Unresectable Rhabdomyosarcoma

description: Rhabdomyosarcoma which is not amenable to surgical resection.


name: Stage I Small Intestinal, Esophageal, Colorectal, Mesenteric, and Peritoneal Gastrointestinal Stromal Tumor AJCC v8

category: biolink:Disease biolink:DiseaseOrPhenotypicFeature biolink:BiologicalEntity biolink:ThingWithTaxon biolink:NamedThing

description: Stage I includes: T1 or T2, N0, M0, Low Mitotic Rate. T1: Tumor measuring 2 cm or less. T2: Tumor measuring more than 2 cm but not more than 5 cm. N0: No regional lymph node metastasis or unknown lymph node status. M0: No distant metastasis. Low Mitotic Rate: Five or fewer mitoses per 5 square millimeters, or per 50 HPF. (AJCC 8th ed.)


Childhood Soft Tissue Sarcoma

description: Soft tissue sarcoma occurring in childhood.


name: Atypical Lipomatous Tumor/Well Differentiated Liposarcoma of Deep Soft Tissue

description: An atypical lipomatous tumor/well differentiated liposarcoma that occurs in deep soft tissue.


name: Stage I Gastric and Omental Gastrointestinal Stromal Tumor AJCC v8

description: Stage I includes: IA: (T1 or T2, N0, M0, Low Mitotic Rate); IB: (T3, N0, M0, Low Mitotic Rate). T1: Tumor measuring 2 cm or less. T2: Tumor measuring more than 2 cm but not more than 5 cm. T3: Tumor measuring more than 5 cm but not more than 10 cm. N0: No regional lymph node metastasis or unknown lymph node status. M0: No distant metastasis. Low Mitotic Rate: Five or fewer mitoses per 5 square millimeters, or per 50 HPF. (AJCC 8th ed.)


Sinonasal Sarcoma

description: A sarcoma that arises from the nasal cavity and paranasal sinuses.


Seminal Vesicle Schwannoma

description: A schwannoma that affects the seminal vesicle.


name: Vulvar Smooth Muscle Tumor of Uncertain Malignant Potential

description: A smooth muscle neoplasm that arises from the vulva and cannot be reliably diagnosed as benign or malignant because of the presence of ambiguous morphologic findings.


Transplant-Related Kaposi Sarcoma

description: A Kaposi sarcoma that arises in a patient with a history of organ transplantation.


Esophageal Fibroma

description: A benign neoplasm arising from the fibrous soft tissue of the esophagus. It is characterized by the presence of spindle-shaped fibroblasts.


Conjunctival Stromal Tumor

description: A rare benign stromal tumor that arises from the bulbar conjunctiva. It is characterized by the presence of myxoid/collagenous stroma, spindle-shaped cells, pseudonuclear inclusions, and multinucleated cells.


Head and Neck Lipoma

description: A benign adipose tissue neoplasm that arises from the head and neck. The most frequently affected sites are the lower lip, buccal mucosa, and tongue.


Recurrent Uterine Corpus Sarcoma

description: The reemergence of uterine corpus sarcoma after a period of remission.


Kaposi's sarcoma, metastatic

description: Kaposi sarcoma that has spread from its original site of growth to another anatomic site.


Sinonasal Leiomyoma

description: A leiomyoma that arises from the nasal cavity and paranasal sinuses.


Locally Advanced Rhabdomyosarcoma

description: Rhabdomyosarcoma that has spread from its original site of growth to nearby tissues or lymph nodes.


Advanced Soft Tissue Sarcoma

description: A sarcoma of soft tissue that has spread extensively to other anatomic sites or is no longer responding to treatment.


name: Benign Head and Neck Soft Tissue Neoplasm

description: A non-metastasizing mesenchymal neoplasm that lacks cytological/morphological atypical features and arises from the soft tissue of the head and neck.


Peritoneal Calcifying Fibrous Tumor

description: A benign well-circumscribed lesion arising from the mesentery. It is characterized by the presence of fibroblasts, lymphoplasmacytic infiltrates, collagenous stroma formation, psammoma bodies, and dystrophic calcifications.


Advanced Epithelioid Sarcoma

description: Epithelioid sarcoma that has spread extensively to other anatomic sites or is no longer responding to treatment.


name: Stage IV Gastric Gastrointestinal Stromal Tumor

description: Stage IV includes: (Any T, N1, M0, Mitotic rate: Any rate); (Any T, Any N, M1, Mitotic rate: Any rate). N1: Regional lymph node metastasis. M0: No distant metastasis.  M1: Distant metastasis.  (AJCC 7th ed.)


Skin Syncytial Myoepithelioma

description: A skin myoepithelioma characterized by a solid syncytial growth of ovoid, spindled, or histiocytoid cells. The majority of cases are associated with EWSR1/PBX3 gene fusion.


Undifferentiated spindle cell sarcoma

description: An undifferentiated soft tissue sarcoma characterized by the presence of a malignant spindle cell infiltrate with amphophilic or palely eosinophilic cytoplasm.


Soft Tissue Angiosarcoma

description: An angiosarcoma that arises from the soft tissues, usually in the deep muscles of the lower extremities, retroperitoneum, mediastinum, and mesentery.


Oral Cavity Soft Tissue Sarcoma

description: A soft tissue sarcoma that arises from the oral cavity.


Scrotal Sarcoma

description: A sarcoma involving the scrotum.


name: Childhood Rhabdomyosarcoma by Toronto Guidelines v2 Stage, Tier 2

description: A term that refers to the staging of childhood rhabdomyosarcoma according to the tier 2 Toronto childhood cancer staging guidelines v2.


name: Metastatic Childhood Non-Rhabdomyosarcoma Soft Tissue Sarcoma by Toronto Guidelines v2

description: Distant metastases present. (Childhood cancer staging for population registries according to the Toronto Childhood Cancer Stage Guidelines - Version 2. Cancer Council Queensland and Cancer Australia: Brisbane, Australia; 2021.)


name: Recurrent Extraskeletal Myxoid Chondrosarcoma

description: Reemergence of extraskeletal myxoid chondrosarcoma after a period of remission


Refractory PEComa

description: PEComa that is resistant to treatment.


name: Malignant Epithelioid Cell Type Gastrointestinal Stromal Tumor

description: An epithelioid cell type gastrointestinal stromal tumor with an aggressive clinical course.


Kidney Angiosarcoma

description: A rare angiosarcoma arising from the kidney.


name: Skin Fibrous Histiocytoma, Histiocytic Variant

description: A morphologic variant of fibrous histiocytoma of the skin. It is characterized by the presence of xanthomatous fibrohistiocytic cells, giant cells, and hemosiderin deposition.


Desmoplastic fibroma

description: A rare, benign, locally aggressive osteolytic neoplasm. It is characterized by the presence of a rich collagenous stroma and spindle cells with minimal cellular atypia.


name: Uterine Corpus Smooth Muscle Tumor of Uncertain Malignant Potential

description: A smooth muscle neoplasm that arises from the uterine corpus and cannot be reliably diagnosed as benign or malignant because of the presence of ambiguous morphologic findings.


Esophageal Synovial Sarcoma

description: A synovial sarcoma that affects the esophageal wall.


Salivary Gland Lipoma

description: A lipoma that arises from a salivary gland. It usually occurs in the parotid gland.


Breast Soft Tissue Neoplasm

description: A benign, intermediate, or malignant mesenchymal neoplasm that arises from the breast.


name: Kidney Angiomyolipoma with Epithelial Cysts

description: A rare angiomyolipoma of the kidney characterized by the presence of solid and cystic architectural patterns. The cysts are lined by hobnail epithelial cells.


Broad ligament leiomyoma

description: A leiomyoma that arises from the broad ligament.


name: Stage IIIB Gastric Gastrointestinal Stromal Tumor

description: Stage IIIB includes: T4, N0, M0, Mitotic rate: High. T4: Tumor more than 10 cm in greatest dimension. N0: No regional lymph node metastasis.  M0: No distant metastasis. (AJCC 7th ed.)


GLI1-Altered Soft Tissue Tumor

description: A rare mesenchymal neoplasm of uncertain histogenesis that arises from soft tissue and viscera. It is characterized by the presence of nests and sheets of neoplastic epithelioid cells separated by a rich delicate arborizing vascular network, and GLI1 gene rearrangement. It presents as a slow-growing painless mass. The majority of cases have an indolent clinical course following complete excision. Distant metastases have been reported in a minority of cases.


Benign Skin Fibroblastic Neoplasm

description: A benign neoplasm arising from the dermis. It is characterized by the presence of neoplastic fibroblasts without malignant characteristics.


name: Alveolar soft part sarcoma non-metastatic

description: Alveolar soft part sarcoma that is confined to a specific site without evidence of spread to other anatomic sites.


name: Locally Advanced Undifferentiated Pleomorphic Sarcoma

description: An undifferentiated pleomorphic sarcoma that has spread from its original site of growth to nearby tissues or lymph nodes.


name: Locally Advanced Desmoplastic Small Round Cell Tumor

description: Desmoplastic small round cell tumor that has spread from its original site of growth to nearby tissues or lymph nodes.


Paratesticular Liposarcoma

description: A liposarcoma that arises from the paratesticular region.


Vulvar Lipoblastoma-Like Tumor

description: A benign, well-defined, lobulated, myxoid or gelatinous tumor that arises from the vulva. It shares morphological features with lipoblastoma, myxoid liposarcoma, and spindle cell lipoma. It is characterized by a lobulated growth and is composed of mature adipocytes, lipoblasts, and spindle cells without atypia in a myxoid matrix. DDIT3 and PLAG1 gene rearrangements seen in lipoblastoma and myxoid liposarcoma are not present in this tumor. It can recur infrequently and there are no reports of metastasis.


Recurrent Soft Tissue Sarcoma

description: The reemergence of soft tissue sarcoma after a period of remission.


name: Uterine Corpus Endometrial Stromal and Related Neoplasms

description: A category of benign or malignant mesenchymal neoplasms that arises from the uterine corpus. This category includes the endometrial stromal nodule which is benign and non-invasive, the endometrial stromal sarcoma, and the undifferentiated uterine sarcoma. The endometrial stromal sarcoma is an invasive malignant neoplasm, further subdivided into low grade and high grade endometrial stromal sarcoma. Undifferentiated uterine sarcoma was previously also known as high grade endometrial stromal sarcoma. In 2014, high grade endometrial stromal sarcoma was reclassified and is currently considered a distinct and rare neoplasm. It appears to have a prognosis that falls between low grade endometrial stromal sarcoma and undifferentiated sarcoma.


Childhood Kaposi Sarcoma

description: Kaposi sarcoma that occurs during childhood.


name: Uterine Corpus Tumor Resembling Ovarian Sex Cord Tumor

description: A usually benign neoplasm that arises from the uterine corpus. It resembles ovarian sex cord tumors. It lacks a component of recognizable endometrial stroma and the JAZF1-SUZ12 fusion which is characteristic of the endometrial stromal neoplasms.


Unresectable Alveolar Soft Part Sarcoma

description: Alveolar soft part sarcoma that is not amenable to surgical resection.


name: Stage II Childhood Non-Rhabdomyosarcoma Soft Tissue Sarcoma by Toronto Guidelines v2

description: T1, N0, M0, G2 or G3. (Childhood cancer staging for population registries according to the Toronto Childhood Cancer Stage Guidelines - Version 2. Cancer Council Queensland and Cancer Australia: Brisbane, Australia; 2021.)


name: Central Nervous System Dedifferentiated Solitary Fibrous Tumor

description: A central nervous system solitary fibrous tumor characterized by the presence of areas of abrupt transition to high grade sarcoma.


Vulvar Deep (Aggressive) Angiomyxoma

description: A locally infiltrating, non-metastasizing vulvar angiomyxoma.  It may recur following resection.


name: Peritoneal Low Grade Endometrioid Stromal Sarcoma

description: A very rare sarcoma that arises from the peritoneum and is characterized by the presence of cells that resemble endometrial stromal cells.


name: Stage IIIC Uterine Corpus Endometrial Stromal Sarcoma AJCC v8

description: Stage IIIC includes: T1-3, N1, M0. T1: Uterine corpus endometrial stroma sarcoma with tumor limited to the uterus. T2: Uterine corpus endometrial stroma sarcoma with tumor extending beyond the uterus, within the pelvis. T3: Uterine corpus endometrial stroma sarcoma with tumor infiltrating abdominal tissues. N1: Regional lymph node metastasis. M0: No distant metastasis. (AJCC 8th Ed.)


Unresectable Epithelioid Sarcoma

description: Epithelioid sarcoma that is not amenable to surgical resection.


Oral Cavity Soft Tissue Neoplasm

description: A mesenchymal neoplasm that arises from the oral cavity.


name: Adult Desmoplastic Small Round Cell Tumor

description: A desmoplastic small round cell tumor occurring in adults.


Major Salivary Gland Sarcoma

description: A rare malignant soft tissue neoplasm that arises from the parotid gland, submandibular gland, or sublingual gland.  Representative examples include fibrosarcoma, angiosarcoma, leiomyosarcoma, and Kaposi sarcoma.


name: Childhood Non-Rhabdomyosarcoma Soft Tissue Sarcoma by Toronto Guidelines v2 Stage

description: A term that refers to the staging of childhood non-rhabdomyosarcoma soft tissue sarcoma according to the Toronto childhood cancer staging guidelines v2.


name: Childhood Non-Rhabdomyosarcoma Soft Tissue Sarcoma by Toronto Guidelines v2 Stage, Tier 2

description: A term that refers to the staging of childhood non-rhabdomyosarcoma soft tissue sarcoma according to the tier 2 Toronto childhood cancer staging guidelines v2.


Recurrent Kaposi Sarcoma

description: The reemergence of Kaposi sarcoma after a period of remission.


name: adult extraskeletal myxoid chondrosarcoma

description: An extraskeletal myxoid chondrosarcoma occurring in adults.


Head and Neck Angiosarcoma

description: An angiosarcoma that arises from the head and neck. The most frequently affected sites are the oral cavity, nasal cavity, and maxillary sinus.


Peripheral Intraneural Angiosarcoma

description: A malignant vascular neoplasm arising from the peripheral nerves.


Kidney Classic Angiomyolipoma

description: A benign mesenchymal tumor that arises from the kidney and is composed of mature adipose tissue, thick-walled blood vessels, and epithelioid and spindle smooth muscle cells.


Endemic African Kaposi Sarcoma

description: A Kaposi sarcoma that occurs in adults and children in Equatorial Africa.


Stage IIB Uterine Sarcoma AJCC v7

description: Tumor involves other pelvic tissues.  No regional lymph node or distant metastasis.


name: Localized Childhood Rhabdomyosarcoma by Toronto Guidelines v2

description: Tumor confined to the area of origin including the regional lymph nodes. (Childhood cancer staging for population registries according to the Toronto Childhood Cancer Stage Guidelines - Version 2. Cancer Council Queensland and Cancer Australia: Brisbane, Australia; 2021.)


Intermediate Fibrohistiocytic Neoplasm

description: A fibrohistiocytic neoplasm usually affecting younger patients.  It is characterized by an increased risk of local recurrence and a low risk of metastasis.


name: Childhood Rhabdomyosarcoma with Mixed Embryonal and Alveolar Features

description: A mixed rhabdomyosarcoma that occurs in children. The neoplasm is composed of embryonic and alveolar components.  It is characterized by the presence of spindle cells with myoblastic differentiation and a myxoid and a fibrous stroma.


Sinonasal Synovial Sarcoma

description: A synovial sarcoma that arises from the nasal cavity and paranasal sinuses.


Stage IB Soft Tissue Sarcoma AJCC v7

description: Stage IB includes: (T2a, N0, M0, G1, GX); (T2b, N0, M0, G1, GX).  T2a: Superficial tumor more than 5 cm in greatest dimension.  T2b: Deep tumor more than 5 cm in greatest dimension.  N0: No regional lymph node metastasis.  M0: No distant metastasis.  G1: Grade 1.  GX: Grade cannot be assessed. (AJCC 7th ed.)


Sinonasal Hemangioma

description: A hemangioma that arises from the nasal cavity and paranasal sinuses.


name: Benign Epithelioid Cell Type Gastrointestinal Stromal Tumor

description: An epithelioid cell type gastrointestinal stromal tumor with an indolent clinical course.


name: Benign Extraskeletal Cartilaginous and Osseous Neoplasm

description: A benign mesenchymal neoplasm with cartilaginous or osseous differentiation arising from the soft tissues exclusively.  Representative examples include chondroma and osteoma.


Unresectable Uterine Corpus Sarcoma

description: Uterine corpus sarcoma that is not amenable to surgical resection.


Low Grade Endometrial Stromal Sarcoma

description: An infiltrating mesenchymal tumor arising from the uterine corpus, cervix, vagina, and the ovary. It is characterized by the presence of oval to spindle-shape cells that resemble the cells of the endometrial stroma, without evidence of significant atypia and pleomorphism. Numerous small vessels are also present. It is usually characterized by indolent growth and late recurrences.


Vulvar Epithelioid Sarcoma

description: An epithelioid sarcoma that arises from the vulva.


name: Central Nervous System Solitary Fibrous Tumor, Grade 2

description: A solitary fibrous tumor that arises from the central nervous system. It corresponds to the more cellular, less collagenous tumor with plump cells and staghorn vasculature which was diagnosed as central nervous system hemangiopericytoma in the past.


High Grade Paranasal Sinus Sarcoma

description: An aggressive malignant soft tissue neoplasm that arises from the paranasal sinus.  It is characterized by the presence of high-grade histologic features.


Childhood Myoepithelial Tumor

description: A myoepithelial tumor that occurs during childhood.


name: Locally Advanced Unresectable Soft Tissue Sarcoma

description: A soft tissue sarcoma that has spread from its original site of growth to nearby tissues or lymph nodes and is not amenable to surgical resection.


name: Head and Neck Undifferentiated Pleomorphic Sarcoma

description: An undifferentiated pleomorphic sarcoma that arises from the head and neck region.


Vulvar Cellular Angiofibroma

description: A benign neoplasm arising from the fibrous tissue of the vulva. It is characterized by the presence of spindle-shaped fibroblasts, numerous dilated vascular channels, and increased cellularity.


Virus-Related Sarcoma

description: A sarcoma that is caused by a virus.


Visceral Angiosarcoma

description: A soft tissue angiosarcoma arising from the internal organs.


name: Stage IA Soft Tissue Sarcoma of the Trunk and Extremities AJCC v8

description: Stage IA includes: T1, N0, M0, G1, GX. T1: Tumor measuring 5 cm or less in greatest dimension. N0: No regional lymph node metastasis or unknown lymph node status. M0: No distant metastasis. GX: Grade cannot be assessed. G1: Total differentiation, mitotic count and necrosis score of 2 or 3. (AJCC 8th ed.)


Soft Tissue Sarcoma by AJCC v8 Stage

description: A term that refers to the staging of soft tissue sarcoma, following the rules of the TNM AJCC v8 classification system.


Angiofibroma of soft tissue

description: A benign fibroblastic neoplasm composed of uniform spindle cells with abundant fibromyxoid stroma and a prominent network of innumerable branching, thin-walled blood vessels. It typically arises in the extremities, mainly the legs, frequently involving or adjacent to large joints such as the knee. Unusual anatomical locations include the back, abdominal wall, pelvic cavity, and breast. The tumors are often subcutaneous but may be intramuscular and deep. The lesion presents most often as a slow-growing painless mass. (WHO 2020)


name: Thyroid Gland Malignant Peripheral Nerve Sheath Tumor

description: A rare malignant peripheral nerve sheath tumor that arises from the thyroid gland.


Conjunctival Sarcoma

description: A sarcoma that arises from the conjunctiva.


name: Stroma-Derived Neoplasm of Lymphoid Tissues

description: A group of mesenchymal neoplasms that arise from the lymph node and spleen.


description: Osteosarcoma that arises from the soft tissue and is confined to a specific site without evidence of spread to other anatomic sites.


name: Childhood Inflammatory Myofibroblastic Tumor

description: An inflammatory myofibroblastic tumor that occurs during childhood.


Laryngeal Soft Tissue Neoplasm

description: A mesenchymal neoplasm that arises from the larynx.


Skin Pleomorphic Lipoma

description: A pleomorphic lipoma arising from the skin.


Prostate Synovial Sarcoma

description: A rare synovial sarcoma that arises from the prostate gland.


name: Uterine Corpus Low Grade Endometrial Stromal Sarcoma

description: A rare, indolent, invasive mesenchymal tumor that arises from the endometrial stroma. It is characterized by the presence of a plexiform vasculature, infrequent mitoses, and insignificant cytologic atypia. Late recurrences may occur.


name: Malignant fibrous histiocytoma metastatic

description: An undifferentiated pleomorphic sarcoma that has spread to other anatomic sites.


Bone Leiomyoma

description: A rare leiomyoma affecting the bone.


name: Locally Advanced Alveolar Soft Part Sarcoma

description: Alveolar soft part sarcoma that has spread from its original site of growth to nearby tissues or lymph nodes.


Digestive System Rhabdomyosarcoma

description: A rhabdomyosarcoma arising from an anatomic site in the digestive system.


Skin Soft Tissue Neoplasm

description: A mesenchymal neoplasm that arises from the skin.


Mammary-Type Myofibroblastoma

description: A benign mesenchymal neoplasm characterized by the presence of spindle shaped myofibroblasts and mast cells in a collagenous stroma.  It is histologically identical to the myofibroblastoma of breast.  It usually arises from the subcutaneous tissue and the most common sites of involvement are the inguinal/groin, paratesticular, and vulvovaginal areas.


Dermal Fibroma

description: A benign neoplasm arising from the dermis. It is characterized by the presence of spindle-shaped fibroblasts.


Periosteal fibroma

description: A benign fibrous neoplasm arising from the periosteal connective tissue that surrounds a bone.


Metastatic Soft Tissue Sarcoma

description: Soft tissue sarcoma that has spread from the original site of growth to another anatomic site.


Stage I Soft Tissue Sarcoma AJCC v7

description: Stage I includes: IA (T1a, N0, M0, G1, GX); (T1b, N0, M0, G1, GX); IB (T2a, N0, M0, G1, GX); (T2b, N0, M0, G1, GX).  T1a: Superficial tumor 5 cm or less in greatest dimension.  T1b: Deep tumor 5 cm or less in greatest dimension.  T2a: Superficial tumor more than 5 cm in greatest dimension.  T2b: Deep tumor more than 5 cm in greatest dimension.  N0: No regional lymph node metastasis.  M0: No distant metastasis.  G1: Grade 1.  GX: Grade cannot be assessed. (AJCC 7th ed.)


Sinonasal glomangiopericytoma

description: A sinonasal soft tissue neoplasm with hemangiopericytoma-like features.


name: Stage III Adult Soft Tissue Sarcoma AJCC v7


alveolar rhabdomyosarcoma of bladder

description: An alveolar rhabdomyosarcoma arising from the bladder. It is the most common type of rhabdomyosarcoma of the bladder affecting older children and adolescents.


name: Stage IIIA Gastric and Omental Gastrointestinal Stromal Tumor AJCC v8

description: Stage IIIA includes: T3, N0, M0, High Mitotic Rate. T3: Tumor measuring more than 5 cm but not more than 10 cm. N0: No regional lymph node metastasis or unknown lymph node status. M0: No distant metastasis. High Mitotic Rate: Over 5 mitoses per 5 square millimeters, or per 50 HPF. (AJCC 8th ed.)


name: Stage IIIA Uterine Corpus Endometrial Stromal Sarcoma AJCC v8

description: Stage IIIA includes: T3a, N0, M0. T3a: Uterine corpus endometrial stroma sarcoma with tumor infiltrating abdominal tissues at one site. N0: No regional lymph node metastasis. M0: No distant metastasis. (AJCC 8th Ed.)


Recurrent Rhabdomyosarcoma

description: The reemergence of rhabdomyosarcoma after a period of remission.


Low Grade Paranasal Sinus Sarcoma

description: A malignant soft tissue neoplasm that arises from the paranasal sinus.  It is characterized by the presence of low-grade histologic features.


Locally Advanced Epithelioid Sarcoma

description: Epithelioid sarcoma that has spread from its original site of growth to nearby tissues or lymph nodes.


name: Hemangioma, Cavernous, Central Nervous System

description: A cavernous hemangioma arising from the central nervous system.


Vaginal Soft Tissue Neoplasm

description: A benign or malignant mesenchymal neoplasm that arises from the vagina.  Representative examples include leiomyoma, rhabdomyoma, leiomyosarcoma, endometrioid stromal sarcoma, and botryoid-type embryonal rhabdomyosarcoma.


name: Sinonasal Smooth Muscle Tumor of Uncertain Malignant Potential

description: A rare smooth muscle tumor of uncertain malignant potential that arises from the sinonasal tract.


Unresectable Desmoid Fibromatosis

description: Desmoid fibromatosis that is not amenable to surgical resection.


Parotid Gland Liposarcoma

description: A liposarcoma that arises from the parotid gland.


Laryngeal Soft Tissue Sarcoma

description: A sarcoma that arises from soft tissue of the larynx.


Adult Soft Tissue Sarcoma

description: Soft tissue sarcoma occurring in adults.


Benign Uterine Corpus PEComa

description: A benign neoplasm with perivascular epithelioid cell differentiation arising from the uterine corpus. It is characterized by the absence of pleomorphism and scarcity or absence of mitotic figures. It usually affects perimenopausal women. Patients present with a pelvic mass or abnormal bleeding.


name: Stage IA Adult Soft Tissue Sarcoma AJCC v7


Refractory Malignant Myoepithelioma

description: A malignant myoepithelioma that does not respond to treatment.


Hemosiderotic fibrolipomatous tumor

description: A non-encapsulated, slow-growing, locally aggressive subcutaneous tumor characterized by the presence of adipocytes, hemosiderin-laden spindle cells, hemosiderin-laden macrophages, osteoclast-like giant cells, and scattered chronic inflammatory cells. It usually arises from the dorsum of the foot, ankle, dorsum of the hand, thigh, calf, or cheek.  It may recur if it is not completely excised but does not metastasize.


name: Central Nervous System Undifferentiated Pleomorphic Sarcoma

description: A rare undifferentiated pleomorphic sarcoma (formerly known as malignant fibrous histiocytoma) involving the central nervous system.


name: Uterine Corpus Myxoid Smooth Muscle Tumor of Uncertain Malignant Potential

description: A smooth muscle tumor of uncertain malignant potential that arises from the uterine corpus and displays myxoid changes.


Localized Childhood Rhabdomyosarcoma

description: Rhabdomyosarcoma that occurs during childhood and is confined to a specific site without evidence of spread to other anatomic sites.


Tumor of Specialized Prostatic Stroma

description: A rare neoplasm arising from specialized prostatic stroma.  This category includes prostatic stromal proliferations of uncertain malignant potential and prostatic stromal sarcomas.


Paratesticular Soft Tissue Neoplasm

description: A mesenchymal neoplasm that arises from the paratesticular region.


Vulvar Distal-Type Epithelioid Sarcoma

description: A rare epithelioid sarcoma of the distal type that arises from the vulva.


Adult Synovial Sarcoma

description: A synovial sarcoma occurring in adults.


name: Non-Metastatic Childhood Soft Tissue Sarcoma

description: A soft tissue sarcoma that occurs during childhood and is confined to a specific site without evidence of spread to other anatomic sites.


name: Childhood Rhabdomyosarcoma by Toronto Guidelines v2 Stage

description: A term that refers to the staging of childhood rhabdomyosarcoma according to the Toronto childhood cancer staging guidelines v2.


Fibrous papule of nose

description: An angiofibroma that arises from the nose. It is found in adults as a solitary, dome-shaped papule on the nose.


description: A morphologic variant of dermatofibrosarcoma protuberans characterized by the presence of a fibrosarcomatous component.


Vaginal Solitary Fibrous Tumor

description: A rare solitary fibrous tumor that arises from the vagina.


name: Stage IV Gastric and Omental Gastrointestinal Stromal Tumor AJCC v8

description: Stage IV includes: (Any T, N1, M0, Any Mitotic Rate); (Any T, Any N, M1, Any Mitotic Rate). N1: Regional lymph node metastasis. M0: No distant metastasis. M1: Distant metastasis. (AJCC 8th ed.)


Seminal Vesicle Leiomyoma

description: A leiomyoma that arises from the seminal vesicle.


name: EBV-Positive Inflammatory Follicular Dendritic Cell/Fibroblastic Reticular Cell Tumor

description: A rare, Epstein-Barr virus-associated variant of follicular dendritic cell sarcoma. It usually arises from the liver or spleen and has a favorable prognosis. The name of this entity has been changed from "sarcoma" to "tumor" because of its indolent nature.


name: Childhood Undifferentiated Pleomorphic Sarcoma

description: An undifferentiated pleomorphic sarcoma that occurs during childhood.


name: Stage IVB Uterine Corpus Endometrial Stromal Sarcoma AJCC v8

description: Stage IVB includes: Any T, Any N, M1. M1: M1: Distant metastasis (excluding adnexa, pelvic, and abdominal tissues). (from AJCC 8th Ed.)


name: Superficial Atypical Lipomatous Tumor/Well Differentiated Liposarcoma

description: An atypical lipomatous tumor/well differentiated liposarcoma that occurs in a superficial body structure.


Solitary Adult Fibroma

description: A solitary benign neoplasm arising from the fibrous soft tissues. The tumor is characterized by the presence of spindle-shaped fibroblasts.


name: Vaginal Smooth Muscle Tumor of Uncertain Malignant Potential

description: A smooth muscle neoplasm that arises from the vagina and cannot be reliably diagnosed as benign or malignant because of the presence of ambiguous morphologic findings.


Gastric Gastrointestinal Stromal Tumor

description: A gastrointestinal stromal tumor that arises from the stomach.  It covers a spectrum of benign to malignant soft tissue neoplasms and includes most gastric smooth muscle tumors, leiomyoblastomas, and tumors formerly called gastrointestinal autonomic nerve tumors.


Plexiform fibrohistiocytic tumor

description: An intermediate fibrohistiocytic neoplasm of the skin that usually affects children and young adults. It is a multinodular, poorly circumscribed tumor characterized by the presence of multinucleated giant cells, mononuclear histiocyte-like cells, and spindle fibroblast-like cells arranged in a plexiform pattern.


Subcutaneous Nodular Fasciitis

description: A self-limiting, rapidly growing, non-encapsulated benign neoplasm arising from the subcutaneous tissues. It is characterized by the growth of plump spindle-shaped fibroblasts in a storiform pattern, multinucleated osteoclast-like giant cells, red blood cell extravasation, chronic inflammatory cells, and high mitotic activity.


name: Uterine Corpus Endometrial Stromal Sarcoma by AJCC v8 Stage

description: A term that refers to the staging of uterine corpus endometrial stroma sarcoma according to the American Joint Committee on Cancer, 8th edition.


name: Superficial CD34-positive fibroblastic tumor

description: A low-grade neoplasm arising from the skin and subcutis. It is characterized by the presence of spindled cells with abundant, eosinophilic, granular to glassy cytoplasm, low mitotic activity, marked nuclear pleomorphism, diffuse CD34 expression, and frequent keratin immunoreactivity. The prognosis is excellent.


Skin Spindle Cell Lipoma

description: A spindle cell lipoma arising from the skin.


Cervical Soft Tissue Neoplasm

description: A benign or malignant mesenchymal neoplasm of the cervix.  Representative examples include rhabdomyoma, alveolar soft part sarcoma, and AIDS-related Kaposi sarcoma.


Synovial sarcoma recurrent

description: The reemergence of synovial sarcoma after a period of remission


name: Extragastrointestinal Gastrointestinal Stromal Tumor

description: A rare gastrointestinal stromal tumor that presents as a solitary mass outside the gastrointestinal tract.


Head and Neck Nodular Fasciitis

description: Nodular fasciitis that arises from the head and neck. It usually affects the face, oral cavity, orbit, ear, parotid gland, and neck.


Stage IIB Soft Tissue Sarcoma AJCC v7

description: Stage IIB includes: (T2a, N0, M0, G2); (T2b, N0, M0, G2).  T2a: Superficial tumor more than 5 cm in greatest dimension.  T2b: Deep tumor more than 5 cm in greatest dimension.  N0: No regional lymph node metastasis.  M0: No distant metastasis.  G2: Grade 2.  (AJCC 7th ed.)


Cervical Angiosarcoma

description: A highly aggressive malignant vascular neoplasm that arises from the cervix and shows endothelial cell differentiation.


Lipoma of appendix

description: A benign adipose tissue neoplasm involving the wall of the appendix.


name: Sinonasal Malignant Peripheral Nerve Sheath Tumor

description: A malignant peripheral nerve sheath tumor that arises from the nasal cavity and paranasal sinuses.


Parotid Gland Hemangioma

description: A hemangioma arising from the parotid gland.


Omentum Gastrointestinal Stromal Tumor

description: A gastrointestinal stromal tumor that arises from the omentum. This is an extragastrointestinal tumor, meaning that it does not arise directly from the gastrointestinal tract.


name: Dermatofibrosarcoma Protuberans with Giant Cell Fibroblastoma-Like Differentiation

description: A morphologic variant of dermatofibrosarcoma protuberans characterized by areas of fibroblastoma like-differentiation, and the presence of giant cells.


Vulvar Fibrolipoma

description: A lipoma of the vulva with a predominant fibrous component.


Skin Angiolipoma

description: An angiolipoma arising from the skin.


name: Stage IV Soft Tissue Sarcoma of the Trunk and Extremities AJCC v8

description: Stage IV includes: (Any T, N1, M0, Any G); (Any T, Any N, M1, Any G). N1: Regional lymph node metastasis. M0: No distant metastasis. M1: Distant metastasis. (AJCC 8th ed.)


Uterine Corpus Sarcoma by AJCC v7 Stage

description: A term that refers to the staging of uterine corpus sarcoma, following the rules of the TNM AJCC v7 classification system.


Parotid Gland Sarcoma

description: A sarcoma that arises from the parotid gland.


name: Recurrent Soft Tissue Sarcoma of the Trunk and Extremities

description: The reemergence of a soft tissue sarcoma of the trunk and extremities after a period of remission.


Gastric Plexiform Fibromyxoma

description: A rare soft tissue neoplasm arising from the stomach.  It occurs in the antrum and pyloric region and it may extend into the duodenum.  It occurs in all ages and affects men and women equally.  Morphologically it is characterized by a multinodular plexiform mass that involves the muscular layer.  The neoplastic cells are spindle-shaped and the mitotic activity is low.  It follows a benign clinical course.


Sinonasal Angiosarcoma

description: An angiosarcoma that arises from the nasal cavity and paranasal sinuses.


Breast Vascular Neoplasm

description: A benign or malignant vascular neoplasm that arises from the breast.


name: Extraskeletal Cartilaginous and Osseous Neoplasm

description: A benign or malignant mesenchymal neoplasm with cartilaginous or osseous differentiation arising from the soft tissues exclusively.  Representative examples include chondroma, osteoma, and osteosarcoma.


name: Skin Fibrous Histiocytoma, Cellular Variant

description: A morphologic variant of fibrous histiocytoma of the skin. It is characterized by the presence of spindle-shaped fibrohistiocytic cells and increased cellularity.


name: Resectable Soft Tissue Sarcoma of the Trunk and Extremities

description: Soft tissue sarcoma of the trunk and extremities that is amenable to surgical resection.


Metastatic extraskeletal osteosarcoma

description: A soft tissue osteosarcoma which has spread to another anatomical site.


Paratesticular Hemangioma

description: A capillary or cavernous hemangioma that arises from the paratesticular region.


Paratesticular Leiomyoma

description: A leiomyoma that arises from the paratesticular region.


Uterine Ligament Leiomyoma

description: A leiomyoma that arises from the broad or other uterine ligaments.


name: Perivascular epithelioid tumor, malignant

description: A usually large and aggressive tumor with perivascular epithelioid cell differentiation characterized by the presence of marked nuclear atypia, pleomorphism, increased mitotic activity, necrosis, and infiltrative margins.  The most common metastatic sites are liver, lungs, lymph nodes, and bone.


name: Small Intestinal, Esophageal, Colorectal, Mesenteric, and Peritoneal Gastrointestinal Stromal Tumor by AJCC v8 Stage

description: A term that refers to the staging of small intestinal, esophageal, colorectal, mesenteric, and peritoneal gastrointestinal stromal tumor (GIST), following the rules of the TNM AJCC v8 classification system. (from AJCC 8th Ed.)


Stage IIIB Uterine Sarcoma AJCC v7

description: Stage IIIB includes: T3b, N0, M0. T3b: Tumor infiltrates abdominal tissues, more than one side.  N0: No regional lymph node metastasis. M0: No distant metastasis.  This staging applies to leiomyosarcoma, endometrial stromal sarcoma, and adenosarcoma. (AJCC 7th ed.)


Kidney Synovial Sarcoma

description: A synovial sarcoma arising from the kidney.


name: Kaposi's sarcoma of extrahepatic bile duct

description: A Kaposi sarcoma that arises from the extrahepatic bile ducts.


Benign Oral Cavity Soft Tissue Neoplasm

description: A mesenchymal neoplasm that arises from the oral cavity and is characterized by the absence of atypical or malignant cytological and architectural features, and absence of invasive features or metastatic potential.


name: Dedifferentiated Gastrointestinal Stromal Tumor

description: A gastrointestinal stromal tumor that through the process of dedifferentiation, results in an uncommon phenotype. Dedifferentiation in the context of GIST, may manifest as loss of CD117 expression and regression from specialized tissue to a more anaplastic/pleomorphic appearance, high nuclear atypia, high mitotic activity, and necrosis.


name: Childhood Angiomatoid Fibrous Histiocytoma

description: An angiomatoid fibrous histiocytoma that occurs during childhood.


sarcoma of tongue

description: A sarcoma that arises from the tongue.


Stage IC Uterine Sarcoma AJCC v7

description: Stage IC includes: T1c, N0, M0.  T1c: For adenosarcoma: tumor invades more than half of the myometrium.  N0: No regional lymph node metastasis.  M0: No distant metastasis.  Stage IC does not apply for leiomyosarcoma and endometrial stromal sarcoma.  (AJCC 7th ed.)


Ectomesenchymal Chondromyxoid Tumor

description: A rare, well circumscribed, non-encapsulated tumor that arises in the oral cavity, most often the anterior tongue.  It is characterized by the presence of round to spindle cells in a chondromyxoid or hyalinized stroma.  Recurrences have been observed in a minority of patients.


name: Metastatic Childhood Rhabdomyosarcoma by Toronto Guidelines v2

description: Distant metastases present. (Childhood cancer staging for population registries according to the Toronto Childhood Cancer Stage Guidelines - Version 2. Cancer Council Queensland and Cancer Australia: Brisbane, Australia; 2021.)


Head and Neck Synovial Sarcoma

description: A synovial sarcoma that arises from the head and neck region.


Tongue Liposarcoma

description: A liposarcoma that arises from the tongue.


Gastric Soft Tissue Neoplasm

description: A benign, intermediate, or malignant mesenchymal neoplasm that arises from the stomach.


Kidney Epithelioid Angiomyolipoma

description: An angiomyolipoma that arises from the kidney and is composed exclusively or predominantly of epithelioid cells. It is often associated with cytologic atypia and may recur or metastasize.


Benign Fibroblastic Neoplasm

description: A benign mesenchymal neoplasm characterized by the presence of neoplastic fibroblasts without malignant characteristics.


name: Childhood Non-Rhabdomyosarcoma Soft Tissue Sarcoma by Toronto Guidelines v2 Stage, Tier 1

description: A term that refers to the staging of childhood non-rhabdomyosarcoma soft tissue sarcoma according to the tier 1 Toronto childhood cancer staging guidelines v2.


name: Abdominal Undifferentiated Pleomorphic Sarcoma

description: Undifferentiated pleomorphic sarcoma involving the organs and structures in the abdomen.


name: Head and Neck EBV-Associated Smooth Muscle Tumor

description: A rare neoplasm of uncertain biological potential that arises from the head and neck. It is associated with EBV infection, exhibits smooth muscle differentiation, and occurs in immunocompromised patients. The most frequent sites of involvement are the larynx, oropharynx, and eye/orbit.


Bladder Angiosarcoma

description: A very rare angiosarcoma that arises from the bladder.


name: Primitive non-neural granular cell tumor

description: A rare, low-grade skin neoplasm of mesenchymal cells of unknown lineage with prominent cytoplasmic granularity. The lesions are most commonly non-ulcerated papules or nodules. Most cases are indolent, despite worrisome histopathologic features. Few cases with regional lymph node metastasis have been reported. (WHO 2018)


Stage IV Uterine Sarcoma AJCC v7

description: Stage IV includes: IVA (T4, Any N, M0); IVB (Any T, Any N, M1).  T4: Tumor involves bladder or rectum.  M0: No distant metastasis.  M1: Distal metastasis (excluding adnexa, pelvic and abdominal tissues).  This staging applies to leiomyosarcoma, endometrial stromal sarcoma, and adenosarcoma. (AJCC 7th ed.)


Prostate Granular Cell Tumor

description: A rare granular cell tumor that arises from the prostate gland.


Stage IV adult soft tissue sarcoma

description: A sarcoma that arises from the soft tissues during adulthood and has spread from the original site of growth to another anatomic site.


Head and Neck Lymphangioma

description: A lymphangioma that arises from the head and neck. It usually affects the skin and soft tissue of the head and neck region.


name: Malignant Extraskeletal Cartilaginous and Osseous Neoplasm

description: A malignant mesenchymal neoplasm with cartilaginous or osseous differentiation arising from the soft tissues exclusively. Extraskeletal osteosarcoma is a representative example.


Stage IVB Uterine Sarcoma AJCC v7

description: Stage IVB includes: Any T, Any N, M1.  M1: Distal metastasis (excluding adnexa, pelvic and abdominal tissues).  This staging applies to leiomyosarcoma, endometrial stromal sarcoma, and adenosarcoma. (AJCC 7th ed.)


name: Spindle Cell Type Gastrointestinal Stromal Tumor

description: A gastrointestinal stromal tumor composed of neoplastic spindle cells.


name: Malignant Head and Neck Soft Tissue Neoplasm

description: A mesenchymal neoplasm with cytological/morphological malignant features and metastatic potential that arises from the soft tissue of the head and neck.


name: Central Nervous System Epithelioid Hemangioendothelioma

description: A low-grade malignant blood vessel neoplasm that arises from the central nervous system.  It is characterized by the presence of epithelioid endothelial cells.


Locally Advanced Synovial Sarcoma

description: Synovial sarcoma that has spread from its original site of growth to nearby tissues or lymph nodes.


rhabdomyosarcoma of kidney

description: An extremely rare rhabdomyosarcoma arising from the kidney. Most cases are of embryonal type.


Stage IIA Uterine Sarcoma AJCC v7

description: Tumor involves adnexa.  No regional lymph node or distant metastasis.


name: Soft Tissue Sarcoma of the Trunk and Extremities by AJCC v8 Stage

description: A term that refers to the staging of soft tissue sarcoma of the trunk and extremities, following the rules of the TNM AJCC v8 classification system.


Extra-abdominal fibromatosis

description: An insidious poorly circumscribed neoplasm arising from the soft tissues outside the abdomen. It is characterized by the presence of elongated spindle-shaped fibroblasts, vascular collagenous stroma formation, and an infiltrative growth pattern.


Epithelioid sarcoma metastatic

description: An epithelioid sarcoma which has spread to another anatomic site.


Uterine Corpus Undifferentiated Sarcoma

description: A rare, high grade sarcoma that arises from the endometrial stroma or myometrium without a specific type of differentiation. It was previously also known as high grade endometrial stromal sarcoma. In 2014, high grade endometrial stromal sarcoma was reclassified and is currently considered a distinct and rare neoplasm. Undifferentiated uterine sarcoma has a worse prognosis.


name: Stage I Childhood Rhabdomyosarcoma by Toronto Guidelines v2

description: Favorable site and any T, any N, M0. (Childhood cancer staging for population registries according to the Toronto Childhood Cancer Stage Guidelines - Version 2. Cancer Council Queensland and Cancer Australia: Brisbane, Australia; 2021.)


name: Stage II Childhood Rhabdomyosarcoma by Toronto Guidelines v2

description: Unfavorable site and T1a, T2a, N0, M0. (Childhood cancer staging for population registries according to the Toronto Childhood Cancer Stage Guidelines - Version 2. Cancer Council Queensland and Cancer Australia: Brisbane, Australia; 2021.)


Scrotal Liposarcoma

description: A liposarcoma involving the scrotum.


Tongue Alveolar Soft Part Sarcoma'

description: An alveolar soft part sarcoma involving the tongue. It usually occurs in children.


Vulvar Lipoma

description: An uncommon benign neoplasm of the vulva, composed of lobules of mature adipocytes.


name: Stage II Uterine Corpus Endometrial Stromal Sarcoma AJCC v8

description: Stage II includes: T2, N0, M0. T2: Uterine corpus endometrial stroma sarcoma with tumor extending beyond the uterus, within the pelvis. N0: No regional lymph node metastasis. M0: No distant metastasis. (AJCC 8th Ed.)


name: Stage IV Childhood Non-Rhabdomyosarcoma Soft Tissue Sarcoma by Toronto Guidelines v2

description: Any T, Any N, M1, Any G (G1, G2, G3, GX). (Childhood cancer staging for population registries according to the Toronto Childhood Cancer Stage Guidelines - Version 2. Cancer Council Queensland and Cancer Australia: Brisbane, Australia; 2021.)


name: Childhood Gastrointestinal Stromal Tumor

description: A gastrointestinal stromal tumor (GIST) occurring in childhood. Pediatric GISTs differ biologically from adult GISTs in that only 11% of pediatric GISTs have activating mutations of KIT and PDGFRA.


Paratesticular Rhabdomyosarcoma

description: A rhabdomyosarcoma that arises from the paratesticular region.


Metastatic rhabdomyosarcoma

description: Rhabdomyosarcoma that has spread from its original site of growth to another anatomic site.


Vaginal Angiomyofibroblastoma

description: An angiomyofibroblastoma arising from the vagina.


Uterine Corpus Metastasizing Leiomyoma

description: A cytologically benign smooth muscle neoplasm that arises from the uterine corpus and has metastasized to the lungs, abdomen, or lymph nodes. It usually presents in women with a history of benign uterine leiomyomas that have been surgically removed years before the extrauterine neoplasm spread.


name: Atypical fibroxanthoma - spindle cell type

description: A morphologic variant of atypical fibroxanthoma characterized by the presence of spindle-shaped fibrohistiocytic cells and a storiform growth pattern.


Sellar Solitary Fibrous Tumor

description: An extremely rare meningeal solitary fibrous tumor that arises from the sellar region.


Recurrent Desmoid Fibromatosis

description: Desmoid fibromatosis that has recurred after a period of remission.


Uterine Corpus Hydropic Leiomyoma

description: A variant of leiomyoma arising from the uterine corpus. It is characterized by conspicuous zonal edema. Hyalinization may also be present.


name: Mesenteric Gastrointestinal Stromal Tumor

description: A gastrointestinal stromal tumor that arises from the mesentery. This is an extragastrointestinal tumor, meaning that it does not arise directly from the gastrointestinal tract.


name: Childhood Plexiform Fibrohistiocytic Tumor

description: A plexiform fibrohistiocytic tumor that occurs during childhood.


undifferentiated sarcoma of soft tissue

description: A term that refers to a heterogeneous group of uncommon soft tissue sarcomas that do not show an identifiable line of differentiation using currently available technologies.  This is a diagnosis of exclusion and includes undifferentiated pleomorphic sarcoma (also known as malignant fibrous histiocytoma), undifferentiated spindle cell sarcoma, undifferentiated round cell sarcoma, and undifferentiated epithelioid sarcoma.


name: Locally Advanced Unresectable Malignant PEComa

description: A malignant PEComa that has spread from its original site of growth to nearby tissues or lymph nodes and is not amenable to surgical resection.


name: Pleomorphic hyalinizing angiectatic tumor of soft tissue

description: A non-metastasizing soft tissue neoplasm characterized by the presence of a neoplastic stroma containing spindle cells, dilated thin-walled vessels lined by fibrin, and a chronic inflammatory infiltrate.  The majority of cases develop in the subcutaneous tissues usually in the lower extremities and present as slow growing masses.  Recurrences occur in approximately half of the cases.


Cellular fibroma

description: A morphologic variant of fibroma characterized by increased cellularity.


name: Locally Advanced Extraskeletal Myxoid Chondrosarcoma

description: Extraskeletal myxoid chondrosarcoma that has spread to nearby tissues or lymph nodes.


name: Central Nervous System Desmoid Fibromatosis

description: Intracranial and/or spinal involvement by desmoid fibromatosis.


Skin Radiation-Related Angiosarcoma

description: A malignant vascular neoplasm arising from an area of skin exposed to radiation therapy.


name: Peritoneal Gastrointestinal Stromal Tumor

description: A gastrointestinal stromal tumor that arises from the peritoneum. This is an extragastrointestinal tumor, meaning that it does not arise directly from the gastrointestinal tract.


Intermediate Soft Tissue Neoplasm

description: A soft tissue neoplasm characterized by an increased risk of local recurrence and/or a low risk of metastasis.


Childhood Ectomesenchymoma

description: An ectomesenchymoma that occurs during childhood.


Gardner Fibroma

description: An uncommon, poorly circumscribed, benign neoplasm arising in the soft tissues of infants, children and adolescents. It is characterized by the presence of haphazardly arranged spindle-shaped fibroblasts, collagenous stroma formation, and plaque-like growth pattern. There is a strong genetic component associating the neoplasm with Gardners syndrome and deep fibromatosis/desmoid tumor.


Endocervical Undifferentiated Sarcoma

description: A high grade undifferentiated sarcoma that arises from the endocervix.


Recurrent Childhood Rhabdomyosarcoma

description: The reemergence of childhood rhabdomyosarcoma after a period of remission.


name: Stage I Soft Tissue Sarcoma of the Trunk and Extremities AJCC v8

description: Stage I includes: IA: (T1, N0, M0, G1, GX); IB: (T2, T3, T4, N0, M0, G1, GX). T1: Tumor measuring 5 cm or less in greatest dimension. T2: Tumor measuring more than 5 cm and less than or equal to 10 cm in greatest dimension. T3: Tumor measuring more than 10 cm and less than or equal to 15 cm in greatest dimension. T4: Tumor measuring more than 15 cm in greatest dimension. N0: No regional lymph node metastasis or unknown lymph node status. M0: No distant metastasis. GX: Grade cannot be assessed. G1: Total differentiation, mitotic count and necrosis score of 2 or 3. (AJCC 8th ed.)


name: Stage IB Gastric and Omental Gastrointestinal Stromal Tumor AJCC v8

description: Stage IB includes: T3, N0, M0, Low Mitotic Rate. T3: Tumor measuring more than 5 cm but not more than 10 cm. N0: No regional lymph node metastasis or unknown lymph node status. M0: No distant metastasis. Low Mitotic Rate: Five or fewer mitoses per 5 square millimeters, or per 50 HPF. (AJCC 8th ed.)


name: Stage IIIA Small Intestinal, Esophageal, Colorectal, Mesenteric, and Peritoneal Gastrointestinal Stromal Tumor AJCC v8

description: Stage IIIA includes: (T1, N0, M0, High Mitotic Rate); (T4, N0, M0, Low Mitotic Rate). T1: Tumor measuring 2 cm or less. T4: Tumor measuring more than 10 cm in greatest dimension. N0: No regional lymph node metastasis or unknown lymph node status. M0: No distant metastasis. High Mitotic Rate: Over 5 mitoses per 5 square millimeters, or per 50 HPF. Low Mitotic Rate: Five or fewer mitoses per 5 square millimeters, or per 50 HPF. (AJCC 8th ed.)


Unresectable Soft Tissue Sarcoma

description: Soft tissue sarcoma that is not amenable to surgical resection.


name: Unresectable Desmoplastic Small Round Cell Tumor

description: A desmoplastic small round cell tumor that is not amenable to surgical resection.


Childhood PEComa

description: A PEComa that occurs during childhood.


Refractory Desmoid Fibromatosis

description: Desmoid fibromatosis that is resistant to treatment.


Maxillofacial Desmoplastic Fibroma

description: A rare, benign, locally aggressive osteolytic neoplasm that arises in a maxillofacial bone. It is characterized by the presence of a rich collagenous stroma and spindle cells with minimal cellular atypia.


Lymphangioleiomyomatosis of skin

description: Lymphangiomyomatosis involving the dermis.


Benign Skin Vascular Neoplasm

description: A vascular neoplasm that arises from the skin and is characterized by the absence of atypical or malignant cytological features, and absence of invasive features or metastatic potential.


name: Stage I Adult Soft Tissue Sarcoma AJCC v7

description: Stage I includes: IA (T1a, N0, M0, G1, GX); (T1b, N0, M0, G1, GX); IB (T2a, N0, M0, G1, GX); (T2b, N0, M0, G1, GX).  T1a: Superficial tumor 5 cm or less in greatest dimension.  T1b: Deep tumor 5 cm or less in greatest dimension.  T2a: Superficial tumor more than 5 cm in greatest dimension.  T2b: Deep tumor more than 5 cm in greatest dimension.  N0: No regional lymph node metastasis.  M0: No distant metastasis.  G1: Grade 1.  GX: Grade cannot be assessed. (AJCC 7th ed.)


Oral Cavity Lipoma

description: A benign adipose tissue neoplasm of the oral cavity. It usually occurs within the lingual and buccal mucosa.


name: Paratesticular Deep (Aggressive) Angiomyxoma

description: A locally infiltrating, non-metastasizing angiomyxoma that arises from the paratesticular region.


Nuchal fibroma

description: A rare non-encapsulated and poorly circumscribed tumor that usually arises from the posterior neck. It is seen more frequently in men and is characterized by the presence of thick collagen fibers. It may recur but does not metastasize.


name: Meningeal Central Nervous System Solitary Fibrous Tumor, Grade 3

description: A solitary fibrous tumor that arises from the meninges. It most often corresponds to the tumor previously diagnosed as anaplastic hemangiopericytoma.


name: Stage IV Adult Soft Tissue Sarcoma AJCC v7

description: Stage IV includes: Any T, Any N, M1, Any G.  M1: Distant metastasis. (AJCC 7th ed.)


name: Abdominal Inflammatory Myofibroblastic Tumor

description: Inflammatory myofibroblastic tumor that arises from the abdominal cavity.


Benign Laryngeal Soft Tissue Neoplasm

description: A mesenchymal neoplasm that arises from the larynx and is characterized by the absence of atypical or malignant cytological and architectural features, and absence of invasive features or metastatic potential.


Intermediate Lipomatous Neoplasm

description: A lipomatous neoplasm characterized by a high risk of local recurrence and no risk of metastasis.


Vascular neoplasm of skin

description: A benign, intermediate, or malignant vascular neoplasm that arises from the skin.


Angiomyofibroblastoma

description: A benign myofibroblastic neoplasm, usually arising in the pelviperineal region. It is characterized by the presence of neoplastic spindle to round cells, dilated thin walled vessels, and stromal edema.  Most patients present with painless circumscribed masses.


Recurrent Alveolar Rhabdomyosarcoma

description: The reemergence of alveolar rhabdomyosarcoma after a period of remission.


name: Metastatic Desmoplastic Small Round Cell Tumor

description: A desmoplastic small round cell tumor that has spread from the original site of growth to another anatomic site.


name: Metastatic Childhood Soft Tissue Sarcoma

description: A sarcoma that arises from the soft tissues during childhood and has spread from the original site of growth to another anatomic site.


name: Stage II Soft Tissue Sarcoma of the Trunk and Extremities AJCC v8

description: Stage II includes: T1, N0, M0, G2, G3. T1: Tumor measuring 5 cm or less in greatest dimension. N0: No regional lymph node metastasis or unknown lymph node status. M0: No distant metastasis. G2: Total differentiation, mitotic count and necrosis score of 4 or 5. G3: Total differentiation, mitotic count and necrosis score of 6, 7, or 8. (AJCC 8th ed.)


Vulvar Solitary Fibrous Tumor

description: A rare solitary fibrous tumor that arises from the vulva.


name: Stage IIIB Gastric and Omental Gastrointestinal Stromal Tumor AJCC v8

description: Stage IIIB includes: T4, N0, M0, High Mitotic Rate. T4: Tumor measuring more than 10 cm in greatest dimension. N0: No regional lymph node metastasis or unknown lymph node status. M0: No distant metastasis. High Mitotic Rate: Over 5 mitoses per 5 square millimeters, or per 50 HPF. (AJCC 8th ed.)


Epithelioid fibrous histiocytoma

description: A morphologic variant of fibrous histiocytoma of the skin. It is characterized by the presence of epithelioid fibrohistiocytic cells.


Parotid Gland Kaposi Sarcoma

description: A Kaposi sarcoma arising from the parotid gland.


Recurrent Malignant Myoepithelioma

description: The reemergence of a malignant myoepithelioma after a period of remission.


Stage IA Soft Tissue Sarcoma AJCC v7

description: Stage IA includes: (T1a, N0, M0, G1, GX); (T1b, N0, M0, G1, GX).  T1a: Superficial tumor 5 cm or less in greatest dimension.  T1b: Deep tumor 5 cm or less in greatest dimension.  N0: No regional lymph node metastasis.  M0: No distant metastasis.  G1: Grade 1.  GX: Grade cannot be assessed. (AJCC 7th ed.)


name: Esophageal Malignant Peripheral Nerve Sheath Tumor

description: A malignant peripheral nerve sheath tumor that affects the esophageal wall.


Fascial fibroma

description: A benign fibrous tumor arising from the fascial connective tissue.


name: Stage IA Gastric Gastrointestinal Stromal Tumor

description: Stage IA includes: T1 or T2, N0, M0, Mitotic rate: Low.  T1: Tumor 2 cm or less in greatest dimension.  T2: Tumor more than 2 cm, but not more than 5 cm, in greatest dimension. N0: No regional lymph node metastasis.  M0: No distant metastasis. (AJCC 7th ed.)


Uterine Corpus Malignant PEComa

description: A malignant neoplasm with perivascular epithelioid cell differentiation arising from the uterine corpus. The neoplasm is usually a large size, and charcaterized by the presence of marked nuclear atypia, pleomorphism, increased mitotic activity, necrosis, and infiltrative margins.  The most common metastatic sites are lungs, lymph nodes, and bone. Patients present with a pelvic mass or abnormal bleeding.


name: Stage IV Uterine Corpus Endometrial Stromal Sarcoma AJCC v8

description: Stage IV includes: IVA: T4, Any N, M0; IVB: Any T, Any N, M1. T4: Uterine corpus endometrial stroma sarcoma with tumor invading bladder or rectum. M0: No distant metastasis. M1: Distant metastasis (excluding adnexa, pelvic, and abdominal tissues). (from AJCC 8th Ed.)


name: Stage IV Childhood Rhabdomyosarcoma by Toronto Guidelines v2

description: Any site and any T, any N, M1. (Childhood cancer staging for population registries according to the Toronto Childhood Cancer Stage Guidelines - Version 2. Cancer Council Queensland and Cancer Australia: Brisbane, Australia; 2021.)


Advanced Synovial Sarcoma

description: A synovial sarcoma that has spread extensively to other anatomic sites or is no longer responding to treatment.


name: Benign Pilar-Associated Mesenchyme Neoplasm

description: A mesenchymal neoplasm arising from the pilar sheath of the hair follicle. It is characterized by the absence of atypical or malignant cytological and architectural features, and absence of invasive features or metastatic potential. Representative examples include fibrofolliculoma, perifollicular fibroma, pilar leiomyoma, and trichogenic myxoma.


name: Seminal Vesicle Mammary-Type Myofibroblastoma

description: An extramammary myofibroblastoma that affects the seminal vesicle.


Stage I Uterine Sarcoma AJCC v7

description: Stage I includes: T1, N0, M0.  T1: Tumor limited to the uterus.  N0: No regional lymph node metastasis.  M0: No distant metastasis.  This staging applies to leiomyosarcoma, endometrial stromal sarcoma, and adenosarcoma. (AJCC 7th ed.)


Malignant Cervical Soft Tissue Neoplasm

description: A rare malignant mesenchymal neoplasm that arises from the cervix.


name: Skin Intermediate Fibrohistiocytic Neoplasm

description: A borderline fibrohistiocytic neoplasm arising from the dermis and extending to the subcutaneous tissues.


Head and Neck Epithelioid Hemangioma

description: An epithelioid hemangioma that arises from the head and neck. The most frequently affected sites are the forehead, preauricular region, and scalp.


Digestive System Sarcoma

description: A sarcoma that arises from the digestive system.


Stage III Uterine Sarcoma AJCC v7

description: Stage III includes: IIIA (T3a, N0, M0); IIIB (T3b, N0, M0); IIIC (T1-T3, N1, M0).  T1: Tumor limited to the uterus. T2: Tumor extends beyond the uterus, within the pelvis.  T3: Tumor infiltrates abdominal tissues.  T3a: Tumor infiltrates abdominal tissues, one side. T3b: Tumor infiltrates abdominal tissues, more than one side.  N0: No regional lymph node metastasis. N1: Regional lymph node metastasis.  M0: No distant metastasis.  This staging applies to leiomyosarcoma, endometrial stromal sarcoma, and adenosarcoma. (AJCC 7th ed.)


name: Peritoneal Desmoplastic Small Round Cell Tumor

description: A desmoplastic small round cell tumor that occurs in the abdominal and/or pelvic peritoneum.


name: Stage II Adult Soft Tissue Sarcoma AJCC v7

description: Stage II includes: IIA (T1a, N0, M0, G2, G3); (T1b, N0, M0, G2, G3); IIB (T2a, N0, M0, G2); (T2b, N0, M0, G2).  T1a: Superficial tumor 5 cm or less in greatest dimension.  T1b: Deep tumor 5 cm or less in greatest dimension.  T2a: Superficial tumor more than 5 cm in greatest dimension.  T2b: Deep tumor more than 5 cm in greatest dimension.  N0: No regional lymph node metastasis.  M0: No distant metastasis.  G2: Grade 2.  G3: Grade 3. (AJCC 7th ed.)


Intermediate Fibroblastic Neoplasm

description: A locally aggressive or rarely metastasizing neoplasm that arises from the soft tissues.  It is characterized by the presence of neoplastic spindle-shaped fibroblasts.


Gastric Lymphangioma

description: A lymphangioma arising from the stomach.


Bladder Malignant PEComa

description: A malignant perivascular epithelioid cell tumor arising from the bladder.


Leiomyoma, Epithelioid

description: A benign or malignant gastrointestinal stromal tumor with epithelioid morphology.


Giant Cell Angioblastoma

description: A rare, slow-growing, locally aggressive soft tissue tumor characterized by the presence of oval to spindle cells forming concentric aggregates around small vessels.  Giant multinucleated cells are also present.


name: Undifferentiated Sarcoma, Not Otherwise Specified

description: An undifferentiated soft tissue sarcoma which cannot be further characterized.


Stage IB Uterine Sarcoma AJCC v7

description: Stage IB includes: T1b, N0, M0.  T1b: For leiomyosarcoma and endometrial stromal sarcoma: tumor more than 5 cm in greatest dimension.  For adenosarcoma: tumor invades to less than half of the myometrium.  N0: No regional lymph node metastasis.  M0: No distant metastasis.  (AJCC 7th ed.)


Vulvar Superficial Myofibroblastoma

description: An uncommon benign polypoid or nodular mesenchymal neoplasm that arises in the vulvar region. It is characterized by the presence of oval or spindle-shaped cells in a collagenous stroma and is separated from the epidermis by a variably thick Grenz zone.


AIDS-Related Cervical Kaposi Sarcoma

description: A Kaposi sarcoma arising from the cervix in patients who are infected with the human immunodeficiency virus.


Laryngeal Granular Cell Tumor

description: A granular cell tumor that arises from the larynx.


name: Childhood EWSR1-SMAD3-Positive Fibroblastic Tumor

description: An EWSR1-SMAD3-positive fibroblastic tumor that occurs during childhood.


Breast Angiolipoma

description: An angiolipoma that arises from the breast. The majority are located in the subcutaneous tissue overlying the breast parenchyma, rather than in breast parenchyma.


Stage IIIA Uterine Sarcoma AJCC v7

description: Stage IIIA includes: T3a, N0, M0.  T3a: Tumor infiltrates abdominal tissues, one side. N0: No regional lymph node metastasis. M0: No distant metastasis.  This staging applies to leiomyosarcoma, endometrial stromal sarcoma, and adenosarcoma. (AJCC 7th ed.)


Skin Spindle Cell/Pleomorphic Lipoma

description: A spindle cell/pleomorphic lipoma arising from the skin.


Central Nervous System Angiolipoma

description: An angiolipoma that arises from the central nervous system.


name: Malignant Sinonasal Soft Tissue Neoplasm

description: A malignant mesenchymal neoplasm that arises from the nasal cavity and paranasal sinuses.


Sclerosing PEComa

description: A tumor with perivascular epithelioid cell differentiation characterized by the presence of cords of neoplastic cells in a densely collagenous stroma.


Bladder Solitary Fibrous Tumor

description: A solitary fibrous tumor that arises from the bladder. Most of these tumors are benign.


name: Uterine Corpus Central Primitive Neuroectodermal Tumor

description: A rare uterine corpus malignant small round cell tumor with neuroglial differentiation. The prognosis is poor.


alveolar rhabdomyosarcoma of prostate

description: A rare alveolar rhabdomyosarcoma arising from the prostate gland.


name: EBV-Positive Inflammatory Follicular Dendritic Cell/Fibroblastic Reticular Cell Tumor of the Digestive System

description: EBV-positive inflammatory follicular dendritic cell/fibroblastic reticular cell tumor that arises from the digestive system.


Orbital Alveolar Soft Part Sarcoma

description: An alveolar soft part sarcoma involving the orbit. It usually occurs in children.


name: Gastric and Omental Gastrointestinal Stromal Tumor by AJCC v8 Stage

description: A term that refers to the staging of gastric and omental gastrointestinal stromal tumor (GIST), following the rules of the TNM AJCC v8 classification system. (from AJCC 8th Ed.)


name: Mixed Cell Type Gastrointestinal Stromal Tumor

description: A gastrointestinal stromal tumor composed of a mixture of neoplastic epithelioid and spindle cells.


name: Conventional Extraskeletal Myxoid Chondrosarcoma

description: A well differentiated extraskeletal myxoid chondrosarcoma.


Undifferentiated Stromal Sarcoma

description: An infiltrating high-grade malignant mesenchymal tumor arising from the uterine corpus, cervix, vagina, and ovary.


name: Orbit Extraskeletal Myxoid Chondrosarcoma

description: An extraskeletal myxoid chondrosarcoma that arises from the orbit. The orbit is the most frequently affected site for extraskeletal myxoid chondrosarcomas in the head and neck region.


name: Skin Atypical Lipomatous Tumor/Well Differentiated Liposarcoma

description: A rare atypical lipomatous tumor/well differentiated liposarcoma arising from the skin.


Myxoid leiomyoma

description: A leiomyoma characterized by the presence of abundant myxoid matrix.


Hemangioendothelioma of skin

description: A vascular proliferation arising from the skin.  It is characterized by the presence of prominent endothelial cells and formation of vascular channels.


name: Malignant Laryngeal Soft Tissue Neoplasm

description: A malignant mesenchymal neoplasm that arises from the larynx.


Degeneration of uterine fibroid

description: A morphologic variant of uterine corpus leiomyoma characterized by the presence of hyalinized or myxoid stroma, edema, cystic changes, hemorrhage, and calcifications.


Poorly Differentiated Synovial Sarcoma

description: A synovial sarcoma characterized by the presence of poorly differentiated morphological features and absence of spindle cells.


name: Stage III Childhood Rhabdomyosarcoma by Toronto Guidelines v2

description: Unfavorable site and (T1a, T2a, N1, M0); (T1b, T2b, Any N, M0). (Childhood cancer staging for population registries according to the Toronto Childhood Cancer Stage Guidelines - Version 2. Cancer Council Queensland and Cancer Australia: Brisbane, Australia; 2021.)


Undifferentiated Epithelioid Sarcoma

description: An undifferentiated soft tissue sarcoma characterized by the presence of a malignant cellular infiltrate with epithelioid morphology.


Stage IV Soft Tissue Sarcoma AJCC v7

description: Stage IV includes: Any T, Any N, M1, Any G.  M1: Distant metastasis. (AJCC 7th ed.)


name: Gastrointestinal Stromal Tumor by AJCC v8 Stage

description: A term that refers to the staging of gastrointestinal stromal tumor (GIST), following the rules of the TNM AJCC v8 classification system. This staging system does not apply to pediatric GIST, familial GIST (germline mutant KIT or PDGFRA), or syndromic GIST (no AJCC staging system available). (from AJCC 8th Ed.)


name: Stage IIIB Small Intestinal, Esophageal, Colorectal, Mesenteric, and Peritoneal Gastrointestinal Stromal Tumor AJCC v8

description: Stage IIIB includes: (T2, N0, M0, High Mitotic Rate); (T3, N0, M0, High Mitotic Rate); (T4, N0, M0, High Mitotic Rate). T2: Tumor measuring more than 2 cm but not more than 5 cm. T3: Tumor measuring more than 5 cm but not more than 10 cm. T4: Tumor measuring more than 10 cm in greatest dimension. N0: No regional lymph node metastasis or unknown lymph node status. M0: No distant metastasis. High Mitotic Rate: Over 5 mitoses per 5 square millimeters, or per 50 HPF. (AJCC 8th ed.)


description: A morphologic variant of dermatofibrosarcoma protuberans characterized by the presence of poorly differentiated sarcomatous components.


name: Vaginal Low Grade Endometrioid Stromal Sarcoma

description: An infiltrating sarcoma that arises from the vagina.  It is characterized by the presence of cells that resemble endometrial stromal cells.


Fallopian Tube Soft Tissue Neoplasm

description: A benign or malignant mesenchymal neoplasm of the fallopian tube.  Representative examples include leiomyoma and leiomyosarcoma.


name: Childhood NTRK-Rearranged Spindle Cell Neoplasm

description: An NTRK-rearranged spindle cell neoplasm that occurs during childhood.


name: Stage IIIA Soft Tissue Sarcoma of the Trunk and Extremities AJCC v8

description: Stage IIIA includes: T2, N0, M0, G2, G3. T2: Tumor measuring more than 5 cm and less than or equal to 10 cm in greatest dimension. N0: No regional lymph node metastasis or unknown lymph node status. M0: No distant metastasis. G2: Total differentiation, mitotic count and necrosis score of 4 or 5. G3: Total differentiation, mitotic count and necrosis score of 6, 7, or 8. (AJCC 8th ed.)


Bladder Soft Tissue Neoplasm

description: A mesenchymal neoplasm that arises from the bladder.


Vulvar Angiomyofibroblastoma

description: An angiomyofibroblastoma arising from the vulva.


name: Tongue Ectomesenchymal Chondromyxoid Tumor

description: Ectomesenchymal chondromyxoid tumor that arises from the tongue, usually the anterior dorsal aspect.


Synovial sarcoma non-metastatic

description: Synovial sarcoma that is confined to a specific site without evidence of spread to other anatomic sites.


Superficial angiomyxoma

description: An angiomyxoma arising from the dermis or subcutaneous tissues.  It may recur following resection.


name: Stage III Gastric and Omental Gastrointestinal Stromal Tumor AJCC v8

description: Stage III includes: IIIA: (T3, N0, M0, High Mitotic Rate); IIIB: (T4, N0, M0, High Mitotic Rate). T3: Tumor measuring more than 5 cm but not more than 10 cm. T4: Tumor measuring more than 10 cm in greatest dimension. N0: No regional lymph node metastasis or unknown lymph node status. M0: No distant metastasis. High Mitotic Rate: Over 5 mitoses per 5 square millimeters, or per 50 HPF. (AJCC 8th ed.)


Resectable Soft Tissue Sarcoma

description: Soft tissue sarcoma that is amenable to surgical resection.


Epithelioid sarcoma non-metastatic

description: Epithelioid sarcoma that is confined to a specific site without evidence of spread to other anatomic sites.


Desmoplastic fibroblastoma

description: A rare benign soft tissue neoplasm characterized by the presence of an abundant collagenous or myxocollagenous matrix, spindle-shaped, and stellate-shaped fibroblasts.  It usually presents as an asymptomatic, slowly growing subcutaneous mass. The most common sites of involvement are the upper arm, shoulder, and lower limb.


name: Uterine Corpus Leiomyoma, Mitotically Active Variant

description: A morphologic variant of uterine corpus leiomyoma characterized by an increased number of mitoses (up to 19 mitoses per 10 high-powered fields).


Sinonasal Soft Tissue Neoplasm

description: A mesenchymal neoplasm that arises from the nasal cavity and paranasal sinuses.


Recurrent Childhood Soft Tissue Sarcoma

description: The reemergence of soft tissue sarcoma in childhood after a period of remission.


Lacrimal Gland Myoepithelial Carcinoma

description: A carcinoma that arises from the lacrimal gland. It is characterized by the presence of a malignant cellular infiltrate exhibiting myoepithelial differentiation.


name: Optic Nerve Sheath Solitary Fibrous Tumor

description: A rare solitary fibrous tumor that arises from the optic nerve sheath.


Metastatic Malignant PEComa

description: A malignant PEComa that has spread from its original site of growth to another anatomic site.


name: Stage IA Uterine Corpus Endometrial Stromal Sarcoma AJCC v8

description: Stage IA includes: T1a, N0, M0. T1a: Uterine corpus endometrial stroma sarcoma with tumor limited to the uterus, measuring 5 cm or less in greatest dimension. N0: No regional lymph node metastasis. M0: No distant metastasis. (AJCC 8th Ed.)


name: Central Nervous System Inflammatory Myofibroblastic Tumor

description: A multinodular intermediate fibroblastic neoplasm affecting the central nervous system.  It is characterized by the presence of spindle-shaped fibroblasts and myofibroblasts, and a chronic inflammatory infiltrate composed of eosinophils, lymphocytes and plasma cells.


name: Soft Tissue Sarcoma of the Trunk and Extremities

description: A soft tissue sarcoma that arises from the trunk or the extremities.


name: Stage II Gastric Gastrointestinal Stromal Tumor

description: Stage II includes: (T1, N0, M0, Mitotic rate: High); (T2, N0, M0, Mitotic rate: High); (T4, N0, M0, Mitotic rate: Low). T1: Tumor 2 cm or less in greatest dimension.  T2: Tumor more than 2 cm, but not more than 5 cm, in greatest dimension.  T4: Tumor more than 10 cm in greatest dimension. N0: No regional lymph node metastasis.  M0: No distant metastasis. (AJCC 7th ed.)


name: Recurrent Desmoplastic Small Round Cell Tumor

description: The reemergence of desmoplastic small round cell tumor after a period of remission.


Stromal Neoplasm

description: A benign or malignant mesenchymal neoplasm composed of stromal cells.  Representative examples include gastrointestinal stromal tumor, endometrial stromal sarcoma, and prostate stromal sarcoma.


NTRK-rearranged spindle cell neoplasm

description: An emerging rare group of soft tissue tumors, usually characterized by a monomorphic spindle cell infiltrate, stromal hyalinization, and frequent coexpression of S100 and CD34 by immunohistochemistry. Most tumors harbor NTRK1 fusions with a variety of partners. Rare cases involving NTRK2 and NTRK3 fusions have also been reported.


description: An extramammary myofibroblastoma that arises from the paratesticular region.


Classic Type Atypical Fibroxanthoma

description: A morphologic variant of atypical fibroxanthoma characterized by the presence of atypical mitotic figures, high mitotic activity, and pleomorphic cells.


Conjunctival Angiosarcoma

description: An angiosarcoma that arises from the conjunctiva.


Buccal Mucosa Lipoma

description: A benign adipose tissue neoplasm that arises from the buccal mucosa.


Fibromatosis, Abdominal

description: An insidious poorly circumscribed neoplasm arising from the deep soft tissues of the abdomen. It is characterized by the presence of elongated spindle-shaped fibroblasts, collagenous stroma formation, and an infiltrative growth pattern.


name: Soft Tissue Tumor of Uncertain Differentiation

description: A benign,intermediate, or malignant soft tissue neoplasm in which the line of differentiation is uncertain. Representative examples include neoplasm with perivascular epithelioid cell differentiation, alveolar soft part sarcoma, desmoplastic small round cell tumor, epithelioid sarcoma, extraskeletal myxoid chondrosarcoma, and synovial sarcoma.


Benign Gastric Soft Tissue Neoplasm

description: A mesenchymal neoplasm that arises from the stomach and is characterized by the absence of atypical or malignant cytological and architectural features, and absence of invasive features or metastatic potential. Representative examples include leiomyoma, lipoma, and hemangioma.


name: Stage IV Small Intestinal, Esophageal, Colorectal, Mesenteric, and Peritoneal Gastrointestinal Stromal Tumor AJCC v8

description: Stage IV includes: (Any T, N1, M0, Any Mitotic Rate); (Any T, Any N, M1, Any Mitotic Rate). N1: Regional lymph node metastasis. M0: No distant metastasis. M1: Distant metastasis. (AJCC 8th ed.)


Vulvar Prepubertal Fibroma

description: A rare, benign mesenchymal neoplasm that arises from the vulvar region, usually the labia majora of prepubertal girls. It presents as a slow-growing, unilateral painless mass. It is characterized by an infiltrative growth of bland spindle cells in a collagenous, edematous, or myxoid stroma. It may recur following incomplete excision.


name: Prostate Inflammatory Myofibroblastic Tumor

description: A rare inflammatory myofibroblastic tumor that arises from the prostate gland.


name: Locally Invasive Desmoid-Type Fibromatosis

description: Desmoid-type fibromatosis that has invaded the surrounding tissues.


Superficial Acral Fibromyxoma

description: A benign fibroblastic neoplasm that arises from acral sites, usually the periungual area of the digits.  It is characterized by the proliferation of spindled and stellate-shaped fibroblasts in the dermis in a myxoid or collagenous stroma.  Nuclear atypia is usually minimal.  It usually presents as a solitary and slow-growing dome-shaped mass on a digit. The recurrence rate is low.


name: Stage IIIB Soft Tissue Sarcoma of the Trunk and Extremities AJCC v8

description: Stage IIIB includes: T3, T4, N0, M0, G2, G3. T3: Tumor measuring more than 10 cm and less than or equal to 15 cm in greatest dimension. T4: Tumor measuring more than 15 cm in greatest dimension. N0: No regional lymph node metastasis or unknown lymph node status. M0: No distant metastasis. G2: Total differentiation, mitotic count and necrosis score of 4 or 5. G3: Total differentiation, mitotic count and necrosis score of 6, 7, or 8. (AJCC 8th ed.)


name: Dermatofibrosarcoma protuberans with myoid differentiation

description: A morphologic variant of dermatofibrosarcoma protuberans characterized by the presence of areas of myofibroblastic differentiation.


Advanced Malignant PEComa

description: A malignant PEComa that has spread extensively to other anatomic sites or is no longer responding to treatment.


name: Head and Neck Epithelioid Hemangioendothelioma

description: A rare epithelioid hemangioendothelioma that arises from the head and neck. The most frequently affected sites are the oral cavity, soft tissue of the neck, and salivary glands.


name: Unresectable Malignant Gastrointestinal Stromal Tumor

description: Malignant gastrointestinal stromal tumor that is not amenable to surgical resection.


name: Stage IB Soft Tissue Sarcoma of the Trunk and Extremities AJCC v8

description: Stage IB includes: T2, T3, T4, N0, M0, G1, GX. T2: Tumor measuring more than 5 cm and less than or equal to 10 cm in greatest dimension. T3: Tumor measuring more than 10 cm and less than or equal to 15 cm in greatest dimension. T4: Tumor measuring more than 15 cm in greatest dimension. N0: No regional lymph node metastasis or unknown lymph node status. M0: No distant metastasis. GX: Grade cannot be assessed. G1: Total differentiation, mitotic count and necrosis score of 2 or 3. (AJCC 8th ed.)


Childhood Rhabdomyosarcoma

description: An aggressive malignant mesenchymal neoplasm arising from skeletal muscle in children.  Only a small percentage of tumors arise in the skeletal muscle of the extremities. The majority arise in other anatomic sites.


Pulmonary Artery Angiosarcoma

description: A malignant vascular neoplasm arising from the pulmonary artery.


Appendix Kaposi Sarcoma

description: A rare Kaposi sarcoma that arises from the appendiceal wall.


pleomorphic liposarcoma of skin

description: A rare pleomorphic liposarcoma arising from the skin.


name: Recurrent Gastrointestinal Stromal Tumor

description: The reemergence of a gastrointestinal stromal tumor after a period of remission.


Breast Atypical Myoepitheliosis

description: A neoplastic process characterized by the proliferation of spindle to cuboidal myoepithelial cells in and around small breast ducts exhibiting cytologic atypia and mitotic activity.


name: Angiosarcoma of the Skin of the Arm after Radical Mastectomy followed by Lymphedema

description: An angiosarcoma arising from the skin of the arm following radical mastectomy and resulting lymphedema.


name: Stage I Childhood Non-Rhabdomyosarcoma Soft Tissue Sarcoma by Toronto Guidelines v2

description: Any T, N0, M0, G1 or GX. (Childhood cancer staging for population registries according to the Toronto Childhood Cancer Stage Guidelines - Version 2. Cancer Council Queensland and Cancer Australia: Brisbane, Australia; 2021.)


Vaginal Deep (Aggressive) Angiomyxoma

description: A locally infiltrating, non-metastasizing vaginal angiomyxoma.  It may recur following resection.


name: Malignant Soft Tissue Tumor of Uncertain Differentiation

description: A malignant soft tissue neoplasm in which the line of differentiation is uncertain.


Paratesticular Cellular Angiofibroma

description: A cellular angiofibroma that arises from the paratesticular region.


Jaw Desmoplastic Fibroma

description: A desmoplastic fibroma that arises from the jaw. It affects the mandible more often than the maxilla.


Skin Myoepithelioma

description: A benign skin neoplasm composed exclusively of myoepithelial cells.


name: Intermediate Sinonasal Soft Tissue Neoplasm

description: A mesenchymal neoplasm that arises from the sinonasal tract and is characterized by an increased risk of local recurrence and/or a low risk of metastasis.


name: Gastric Gastrointestinal Stromal Tumor of Uncertain Malignant Potential

description: A gastrointestinal stromal tumor arising from the stomach.  It is characterized by a maximum diameter greater than 5 cm and equal or less than 10 cm and no more than 5 mitotic figures per 50 high power fields.


Head and Neck Hemangioma

description: A hemangioma that arises from the head and neck. The most frequently affected sites are the oral cavity and sinonasal tract mucosa.


Vulvar Superficial Angiomyxoma

description: A vulvar angiomyxoma arising from the dermis or subcutaneous tissues. It may recur following resection.


description: A rare variant of dermatofibrosarcoma protuberans characterized by plaque-like growth, resembling plaque-like CD34-positive dermal fibroma.


name: Prostate Malignant Solitary Fibrous Tumor

description: A rare malignant solitary fibrous tumor that arises from the prostate gland.


Hepatobiliary Soft Tissue Neoplasm

description: A benign, intermediate, or malignant mesenchymal neoplasm that arises from the hepatobiliary system.


Thyroid Gland Leiomyoma

description: A leiomyoma that arises from the thyroid gland.


Atypical fibroxanthoma of skin

description: An intermediate cutaneous mesenchymal neoplasm of uncertain differentiation, usually affecting the actinic-damaged skin of the elderly.  The tumor presents as a single cutaneous nodule which is often ulcerated.  Microscopically, it is characterized by the presence of highly atypical cytologic features, pleomorphism and abundant mitotic figures. The vast majority of patients have an excellent prognosis following conservative therapy. Recurrences are infrequent, and metastasis is rare.


name: Skin Fibrous Histiocytoma, Fibroblastic Variant

description: A morphologic variant of fibrous histiocytoma of the skin. It is characterized by the presence of spindle-shaped fibrohistiocytic cells and associated acanthosis.


Soft Tissue Sarcoma by AJCC v7 Stage

description: A term that refers to the staging of soft tissue sarcoma, following the rules of the TNM AJCC v7 classification system.


description: An epithelioid hemangioendothelioma that arises from the nasal cavity and paranasal sinuses.


Intracranial Fibrous Histiocytoma

description: A benign fibrous histiocytoma involving the dura or cranial bone.


Intracranial lipoma

description: A rare benign adipose tissue neoplasm located in the intracranial region. It is a congenital hamartomatous malformation derived from the embryologic meninx primitiva.


name: Kaposi Sarcoma Related to Immunosuppressive Treatment

description: A Kaposi sarcoma that develops after immunosuppressive treatment.


Head and Neck Soft Tissue Neoplasm

description: A mesenchymal neoplasm that arises from the soft tissue of the head and neck.


name: Digestive System Inflammatory Myofibroblastic Tumor

description: A multinodular intermediate fibroblastic neoplasm arising from the digestive system. It is characterized by the presence of spindle-shaped fibroblasts and myofibroblasts, and a chronic inflammatory infiltrate composed of eosinophils, lymphocytes and plasma cells.


Pelvic Fibromatosis

description: Fibromatosis that occurs in the pelvis. It affects almost always females. It is characterized by the presence of elongated spindle-shaped fibroblasts, collagenous stroma formation, and an infiltrative growth pattern. It recurs if incompletely resected but lacks metastatic potential.


Paratesticular Schwannoma

description: A schwannoma that arises from the paratesticular region.


Stage IIIC Uterine Sarcoma AJCC v7

description: Stage IIIC includes: T1-T3, N1, M0.  T1: Tumor limited to the uterus. T2: Tumor extends beyond the uterus, within the pelvis.  T3: Tumor infiltrates abdominal tissues.  N1: Regional lymph node metastasis.  M0: No distant metastasis.  This staging applies to leiomyosarcoma, endometrial stromal sarcoma, and adenosarcoma. (AJCC 7th ed.)


Uterine Angiosarcoma

description: A malignant vascular neoplasm arising from the uterus.


Unresectable Synovial Sarcoma

description: Synovial sarcoma that is not amenable to surgical resection.


name: Stage III Uterine Corpus Endometrial Stromal Sarcoma AJCC v8

description: Stage III includes: IIIA: T3a, N0, M0; IIIB: T3b, N0, M0; IIIC: T1-3, N1, M0. T1: Uterine corpus endometrial stroma sarcoma with tumor limited to the uterus. T2: Uterine corpus endometrial stroma sarcoma with tumor extending beyond the uterus, within the pelvis. T3: Uterine corpus endometrial stroma sarcoma with tumor infiltrating abdominal tissues. T3a: Uterine corpus endometrial stroma sarcoma with tumor infiltrating abdominal tissues at one site. T3b: Uterine corpus endometrial stroma sarcoma with tumor infiltrating abdominal tissues at more than one site. N0: No regional lymph node metastasis. N1: Regional lymph node metastasis. M0: No distant metastasis. (AJCC 8th Ed.)


Stage IVA Uterine Sarcoma AJCC v7

description: Stage IVA includes: T4, Any N, M0. T4: Tumor involves bladder or rectum.  M0: No distant metastasis.  This staging applies to leiomyosarcoma, endometrial stromal sarcoma, and adenosarcoma. (AJCC 7th ed.)


Gastric sarcoma

description: A sarcoma that arises from the stomach. Representative examples include Kaposi sarcoma, leiomyosarcoma, and liposarcoma.


Recurrent Skin Angiosarcoma

description: The reemergence of skin angiosarcoma after a period of remission.


name: Intermediate Soft Tissue Tumor of Uncertain Differentiation

description: A soft tissue neoplasm of uncertain differentiation which is locally aggressive but rarely metastasizes.


Uterine Corpus Sarcoma by AJCC v8 Stage

description: A term that refers to the staging of uterine corpus sarcoma according to the American Joint Committee on Cancer, 8th edition. This staging system applies to leiomyosarcomas, endometrial stromal sarcomas, and adenosarcomas of the uterine corpus. It does not apply to carcinosarcomas which are staged according to the carcinomas and carcinosarcomas of the uterine corpus. (from AJCC 8th Ed.)


name: Cervical Low Grade Endometrioid Stromal Sarcoma

description: A very rare sarcoma that arises from the cervix and is characterized by the presence of cells that resemble endometrial stromal cells.


Alveolar soft part sarcoma recurrent

description: The reemergence of alveolar soft part sarcoma after a period of remission.


Hemangioma of salivary gland

description: A hemangioma that arises from a salivary gland. It occurs almost exclusively in the parotid gland.


Lipofibromatosis

description: A rare, slow-growing, painless tumor of infancy and early childhood that usually affects the distal extremities. It is characterized by the presence of alternating bands of mature adipose tissue and spindle-cell fibrous tissue, without destruction of the adipose tissue architecture. It does not metastasize, however the rate of local recurrences is high.


Malignant vascular tumor of skin

description: A malignant vascular neoplasm arising from blood vessels and lymphatics of the skin.


EWSR1-SMAD3-positive fibroblastic tumor

description: A benign fibroblastic neoplasm characterized by a fusion of exon 7 of EWSR1 gene with exon 5 of SMAD3 gene. Patients usually present with a small painless superficial tumor in the hands and feet. Morphologically, there is zonation with acellular hyalinized center and peripheral proliferation of fibroblastic spindle cells without atypia. It may recur following incomplete excision.


name: Unresectable Undifferentiated Pleomorphic Sarcoma

description: Undifferentiated pleomorphic sarcoma that is not amenable to surgical resection.


name: Stage III Soft Tissue Sarcoma of the Trunk and Extremities AJCC v8

description: Stage III includes: IIIA: (T2, N0, M0, G2, G3); IIIB: (T3, T4, N0, M0, G2, G3). T2: Tumor measuring more than 5 cm and less than or equal to 10 cm in greatest dimension. T3: Tumor measuring more than 10 cm and less than or equal to 15 cm in greatest dimension. T4: Tumor measuring more than 15 cm in greatest dimension. N0: No regional lymph node metastasis or unknown lymph node status. M0: No distant metastasis. G2: Total differentiation, mitotic count and necrosis score of 4 or 5. G3: Total differentiation, mitotic count and necrosis score of 6, 7, or 8. (AJCC 8th ed.)


Atypical Ductal Breast Hyperplasia

description: A neoplastic process that arises from the terminal ductal lobular unit of the breast parenchyma.  It is usually solitary and confined to a single lobular unit.  It is characterized by the presence of a monomorphic epithelial proliferation in a cribriform, micropapillary, or solid growth pattern.  Focal architectural features of low grade ductal carcinoma in situ are present.  There is an increased risk for development of invasive breast carcinoma.


name: Grade 3 Pleura Infection with Unknown Absolute Neutrophil Count, CTCAE

category: biolink:PhenotypicFeature biolink:DiseaseOrPhenotypicFeature biolink:BiologicalEntity biolink:ThingWithTaxon biolink:NamedThing


name: Skin Breakdown or Decubitus Ulcer, CTCAE


name: Grade 2 Skin Breakdown or Decubitus Ulcer, CTCAE


name: Grade 4 Skin Breakdown or Decubitus Ulcer, CTCAE


name: Grade 5 Skin Breakdown or Decubitus Ulcer, CTCAE


name: Prostate Large Cell Neuroendocrine Carcinoma

description: An exceptionally rare neuroendocrine carcinoma that arises from the prostate gland.  It is characterized by the presence of malignant large cells.


Postpartum Obstetric Thromboembolism

description: Presence of a blood clot in the maternal vascular system which originated from a distant site during the postpartum period.


name: Clear Cell Calcifying Epithelial Odontogenic Tumor

description: A calcifying epithelial odontogenic tumor that contains a variable proportion of clear cells containing diastase-labile PAS-positive material that is consistent with glycogen.


name: Early T Precursor Acute Lymphoblastic Leukemia, Not Otherwise Specified

description: Early T precursor acute lymphoblastic leukemia in which further genetic/molecular characterization is not available.


name: Ampulla of Vater Neuroendocrine Tumor G1

description: A well differentiated, low grade neoplasm with neuroendocrine differentiation that arises from the ampulla of Vater and the periampullary region. The mitotic count is less than 2 per 10 HPF.


name: Grade 1 Intraoperative Renal Injury, CTCAE

description: Primary repair of injured organ/structure indicated


Osteolytic Lesions of Multiple Myeloma

description: A radiologic finding indicating the presence of multiple radiolucent osteolytic bone lesions observed in a patient with a diagnosis of multiple myeloma.


Solitary Lytic Metastatic Bone Lesion

description: A radiologic finding indicating the presence of solitary area of bone destruction that results from the spread of cancer to the bone(s).


glucose

An aldohexose used as a source of energy and metabolic intermediate.


Laryngeal Fistula

description: An abnormal communication between the larynx and another organ or cavity.


Pharyngocutaneous fistula

description: An abnormal communication between the skin and the pharynx.


Salivary Gland Fistula

description: An abnormal communication between a salivary gland and another organ or anatomic site.


External Auditory Canal Fistula

description: An abnormal communication between the external auditory canal and the adjacent tissues.


name: Grade 5 Oral Cavity or Gum Infection Documented Clinically or Microbiologically with Grade 3 or 4 Neutrophils, CTCAE


Locally Advanced Liposarcoma

description: A liposarcoma that has spread from its original site of growth to nearby tissues or lymph nodes.


Liposarcoma metastatic

description: A liposarcoma that has metastasized to a secondary site.


Metastatic Dedifferentiated Liposarcoma

description: Dedifferentiated liposarcoma that has spread from its original site of growth to another anatomic site.


Advanced Myxoid Liposarcoma

description: A myxoid liposarcoma that has spread extensively to other anatomic sites or is no longer responding to treatment.


Myxoid liposarcoma metastatic

description: Myxoid liposarcoma that has spread to other anatomic sites.


Advanced Dedifferentiated Liposarcoma

description: A dedifferentiated liposarcoma that has spread extensively to other anatomic sites or is no longer responding to treatment.


Metastatic Pleomorphic Liposarcoma

description: Pleomorphic liposarcoma that has spread from its original site of growth to another anatomic site.


Advanced Pleomorphic Liposarcoma

description: Pleomorphic liposarcoma that has spread extensively to other anatomic sites or is no longer responding to treatment.


name: Locally Advanced Dedifferentiated Liposarcoma

description: Dedifferentiated liposarcoma that has spread from its original site of growth to nearby tissues or lymph nodes.


Advanced Liposarcoma

description: A liposarcoma that has spread extensively to other anatomic sites or is no longer responding to treatment.


name: Recurrent Multinodular and Vacuolated Neuronal Tumor

description: The reemergence of multinodular and vacuolated neuronal tumor following a period of remission.


name: Adult Multinodular and Vacuolated Neuronal Tumor

description: A multinodular and vacuolated neuronal tumor that occurs in adults.


Grade 5 Ovarian Infection, CTCAE

description: Death


Stage IV Ureter Cancer AJCC v8

description: Stage IV includes: (T4, N0, M0); (Any T, N1, M0); (Any T, N2, M0); (Any T, Any N, M1). T4: Tumor invades adjacent organs, or through the kidney into the perinephric fat. N0: No regional lymph node metastasis. N1: Metastasis in a single lymph node, 2 cm or less in greatest dimension. N2: Metastasis in a single lymph node, more than 2 cm in greatest dimension; or multiple lymph nodes. M0: No distant metastasis. M1: Distant metastasis. (AJCC 8th ed.)


Grade 5 Mucosal Infection, CTCAE

description: Death


Cleft Maxilla

description: A congenital abnormality consisting of a fissure in the midline of the upper jawbone.


Fallopian Tube Lymphoma

description: An exceedingly rare lymphoma that arises from the fallopian tube.


Bacteriological Finding

description: Objective evidence of bacterial infection perceptible to the examiner by laboratory procedure.


name: Grade 3 Lower Extremity Vein Injury, CTCAE


name: Grade 4 Lower Extremity Vein Injury, CTCAE


name: Grade 2 Lower Extremity Vein Injury, CTCAE


name: Grade 1 Lower Extremity Vein Injury, CTCAE


name: Grade 5 Lower Extremity Vein Injury, CTCAE


Gastric melanoma

description: A melanoma that arises from mucosa of the stomach.


(R)-bromazine

The (R)-enantiomer of bromazine.


name: Grade 2 Oral Cavity or Gum Infection with Normal Absolute Neutrophil Count or Grade 1 or 2 Neutrophils, CTCAE


name: Oral Cavity or Gum Infection with Normal Absolute Neutrophil Count or Grade 1 or 2 Neutrophils, CTCAE


name: Grade 5 Oral Cavity or Gum Infection with Normal Absolute Neutrophil Count or Grade 1 or 2 Neutrophils, CTCAE


name: Grade 4 Oral Cavity or Gum Infection with Normal Absolute Neutrophil Count or Grade 1 or 2 Neutrophils, CTCAE


name: Grade 3 Oral Cavity or Gum Infection with Normal Absolute Neutrophil Count or Grade 1 or 2 Neutrophils, CTCAE


straight-chain fatty acid

Any fatty acid whose skeletal carbon atoms form an unbranched open chain.


linolenic acid

A two-membered subclass of octadecatrienoic acid comprising the (9Z,12Z,15Z)- and (6Z,9Z,12Z)-isomers. Linolenic acids are nutrients essential to the formation of prostaglandins and are also used in making paints and synthetic resins.


octadecadienoic acid

Any straight-chain, C18 polyunsaturated fatty acid having two C=C double bonds.