Stage IVA Nasal Cavity and Paranasal Sinus Cancer AJCC v7

Stage IVA includes: (T4a, N0, M0); (T4a, N1, M0); (T1, N2, M0); (T2, N2, M0); (T3, N2, M0); (T4a, N2, M0); T4a: Maxillary sinus: Moderately advanced local disease. Tumor invades anterior orbital contents, skin of cheek, pterygoid plates, infratemporal fossa, cribriform plate, sphenoid or frontal sinuses.  Nasal cavity and ethmoid sinus: Moderately advanced local disease. Tumor invades any of the following: anterior orbital contents, skin of nose or cheek, minimal extension to anterior cranial fossa, pterygoid plates, sphenoid or frontal sinuses.  T1: Maxillary sinus: Tumor limited to the maxillary sinus mucosa with no erosion or destruction of bone.  Nasal cavity and ethmoid sinus: Tumor restricted to any one subsite, with or without bony invasion.  T2: Maxillary sinus: Tumor causing bone erosion or destruction including extension into the hard palate and/or middle nasal meatus, except extension to posterior wall of maxillary sinus and pterygoid plates.  Nasal cavity and ethmoid sinus: Tumor invading two subsites in a single region or extending to involve an adjacent region within the nasoethmoidal complex, with or without bony invasion.  T3: Maxillary sinus: Tumor invading any of the following: bone of the posterior wall of maxillary sinus, subcutaneous tissues, floor or medial wall of orbit, pterygoid fossa, or ethmoid sinuses. Nasal cavity and ethmoid sinus: Tumor invading the medial wall or floor of the orbit, maxillary sinus, palate, or cribriform plate.  N0: No regional lymph node metastasis.  N1: Metastasis in a single ipsilateral lymph node, 3 cm or less in greatest dimension.  N2: Metastasis in a single ipsilateral lymph node, more than 3 cm but not more than 6 cm in greatest dimension, or in multiple ipsilateral lymph nodes, none more than 6 cm in greatest dimension, or in bilateral or contralateral lymph nodes, none more than 6 cm in greatest dimension. M0: No distant metastasis. (AJCC 7th ed.)


Stage III Lip and Oral Cavity Cancer AJCC v8

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Stage III includes: (T3, N0, M0); (T1, N1, M0); (T2, N1, M0); (T3, N1, M0). T3: Tumor size greater than 4 cm or any tumor greater than 10 mm DOI. T1: Tumor size 2 cm or less in greatest dimension and 5 mm or less DOI. T2: Tumor size 2 cm or less in greatest dimension, DOI greater than 5 mm and equal or less than 10 mm or tumor greater than 2 cm but 4 cm or less in greatest dimension and 10 mm or less DOI N1: Metastasis in a single ipsilateral lymph node, 3 cm or smaller in greatest dimension and ENE (extranodal extension)(-). M0: No distant metastasis. (AJCC 8th Ed.)


Recurrent Salivary Gland Squamous Cell Carcinoma

The reemergence of salivary gland squamous cell carcinoma after a period of remission.


Cribriform Adenocarcinoma of Minor Salivary Gland

A minor salivary gland adenocarcinoma characterized by a cribriform pattern. It often metastasizes to the neck lymph nodes.


Refractory Salivary Gland Carcinoma

Salivary gland carcinoma that is resistant to treatment.


Salivary Gland Intraductal Carcinoma

A rare, low-grade adenocarcinoma that arises from the salivary glands.  It is characterized by the presence of cystic structures lined by ductal cells which are arranged in cribriform patterns.  Adjacent ducts show intraductal proliferation.


Stage IVC Paranasal Sinus Cancer AJCC v7

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


Stage I Nasal Cavity and Paranasal Sinus Cancer AJCC v6 and v7

Stage I includes: T1, N0, M0.  T1: Maxillary sinus: Tumor limited to the maxillary sinus mucosa with no erosion or destruction of bone.  Nasal cavity and ethmoid sinus: Tumor restricted to any one subsite, with or without bony invasion.  N0: No regional lymph node metastasis.  M0: No distant metastasis. (AJCC 6th and 7th eds.)


Locally Advanced Head and Neck Carcinoma

A head and neck carcinoma that has spread from its original site of growth to nearby tissues or lymph nodes.


Recurrent Head and Neck Squamous Cell Carcinoma

The reemergence of squamous cell carcinoma in the head and neck region after a period of remission.


basaloid squamous cell carcinoma of floor of mouth

An aggressive variant of squamous cell carcinoma that arises from the floor of the mouth. It is characterized by the presence of small malignant cells with hyperchromatic nuclei and scant amount of cytoplasm forming lobules with peripheral palisading.


Mucoepidermoid carcinoma of the oral cavity stage III

Stage III includes: (T3, N0, M0); (T1, N1, M0); (T2, N1, M0); (T3, N1, M0). T3: Tumor more than 4 cm in greatest dimension. N1: Metastasis in a single ipsilateral lymph node, 3 cm or less in greatest dimension. M0: No distant metastasis. (AJCC 6th and 7th eds.)


Primary Intraosseous Squamous Cell Carcinoma Derived From Odontogenic Cyst

A rare squamous cell carcinoma that arises centrally from the jaw from the lining of an odontogenic cyst.


large cell neuroendocrine carcinoma of salivary gland

A large cell carcinoma that arises from the salivary glands and exhibits neuroendocrine differentiation.


Stage IV Nasal Cavity and Paranasal Sinus Cancer AJCC v7

Stage IV includes: IVA: (T4a, N0, M0); (T4a, N1, M0); (T1, N2, M0); (T2, N2, M0); (T3, N2, M0); (T4a, N2, M0); IVB: (T4b, Any N, M0); (Any T, N3, M0); IVC: (Any T, Any N, M1).  T4a: Maxillary sinus: Moderately advanced local disease. Tumor invades anterior orbital contents, skin of cheek, pterygoid plates, infratemporal fossa, cribriform plate, sphenoid or frontal sinuses.  Nasal cavity and ethmoid sinus: Moderately advanced local disease. Tumor invades any of the following: anterior orbital contents, skin of nose or cheek, minimal extension to anterior cranial fossa, pterygoid plates, sphenoid or frontal sinuses.  T4b: Very advanced local disease.  Tumor invades any of the following: orbital apex, dura, brain, middle cranial fossa, cranial nerves other than maxillary division of trigeminal nerve, nasopharynx, or clivus.  N0: No regional lymph node metastasis.  N1: Metastasis in a single ipsilateral lymph node, 3 cm or less in greatest dimension.  N2: Metastasis in a single ipsilateral lymph node, more than 3 cm but not more than 6 cm in greatest dimension, or in multiple ipsilateral lymph nodes, none more than 6 cm in greatest dimension, or in bilateral or contralateral lymph nodes, none more than 6 cm in greatest dimension. N3: Metastasis in a lymph node, more than 6 cm in greatest dimension.  M0: No distant metastasis.  M1: Distant metastasis.  (AJCC 7th ed.)


Head and Neck Neuroendocrine Carcinoma

A rare neuroendocrine carcinoma that arises from the head and neck.


Mucoepidermoid carcinoma of the oral cavity stage I

Stage I includes: T1, N0, M0.  T1: Tumor 2 cm or less in greatest dimension.  N0: No regional lymph node metastasis.  M0: No distant metastasis. (AJCC 6th and 7th eds.)


Clear cell odontogenic carcinoma

A usually aggressive malignant neoplasm arising from tooth-forming tissues. It more often affects older females and more frequently occurs in the mandible. It is characterized by the presence of malignant epithelial cells with clear cytoplasm and a fibrotic stroma formation. It may recur and metastasize. Metastases may occur in the lymph nodes, lungs, and bones. Treatment of choice is resection with clean margins.


Sinonasal Adenocarcinoma, Intestinal-Type with Mucinous Pattern

An intestinal-type adenocarcinoma that arises from the sinonasal tract. It is characterized by the presence of malignant glandular cells with intracytoplasmic mucin.


Primary ameloblastic carcinoma

A rare, aggressive odontogenic malignant tumor that arises usually from the mandible and less frequently from the maxilla.  It combines the histologic features of an ameloblastoma and carcinoma.


Lip and Oral Cavity Cancer by AJCC v8 Stage

A term that refers to the staging of lip and oral cavity carcinoma according to the American Joint Committee on Cancer, 8th edition.


Stage III Oral Cavity Cancer AJCC v6 and v7


Stage I Cutaneous Squamous Cell Carcinoma of the Head and Neck AJCC v8

Stage I includes: T1, N0, M0. T1: Tumor smaller than 2 cm in greatest dimension. N0: No regional lymph node metastasis. M0: No distant metastasis. (AJCC 8th ed.)


Adenoid cystic carcinoma of the oral cavity

An adenoid cystic carcinoma arising from the minor salivary glands in the oral cavity.


Polymorphous low grade adenocarcinoma of salivary gland

A low grade malignant epithelial neoplasm arising from the salivary gland. It usually arises from the oral cavity, with approximately 60% of the cases involving the palate. The most common presenting sign is a painless mass in the palate. It is characterized by the presence of uniform, small to medium size malignant epithelial cells and an infiltrating pattern. There is a variety of morphologic patterns which may be present within an individual tumor, including lobular, papillary, papillary-cystic, cribriform, and trabecular pattern. It usually has a favorable clinical outcome and a low metastatic potential.


Anterior Tongue Mucoepidermoid Carcinoma

A mucoepidermoid carcinoma arising from the minor salivary glands in the anterior tongue.


Ear carcinoma

A carcinoma that arises from the ear.  Representative examples include ceruminous adenocarcinoma and squamous cell carcinoma of the external ear and adenocarcinoma of the middle ear.


Head and Neck Small Cell Neuroendocrine Carcinoma

A small cell neuroendocrine carcinoma that arises from the head and neck.


Stage IVA Nasal Cavity and Paranasal Sinus Cancer AJCC v8

Stage IVA includes: (T4a, N0, M0); (T4a, N1, M0); (T1, N2, M0); (T2, N2, M0); (T3, N2, M0); (T4a, N2, M0). T4a: Maxillary sinus: Moderately advanced local disease. Tumor invades anterior orbital contents, skin of cheek, pterygoid plates, infratemporal fossa, cribriform plate, sphenoid or frontal sinuses. Nasal cavity and ethmoid sinus: Moderately advanced local disease. Tumor invades any of the following: anterior orbital contents, skin of nose or cheek, minimal extension to anterior cranial fossa, pterygoid plates, sphenoid or frontal sinuses. T1: Maxillary sinus: Tumor limited to the maxillary sinus mucosa with no erosion or destruction of bone. Nasal cavity and ethmoid sinus: Tumor restricted to any one subsite, with or without bony invasion. T2: Maxillary sinus: Tumor causing bone erosion or destruction including extension into the hard palate and/or middle nasal meatus, except extension to posterior wall of maxillary sinus and pterygoid plates. Nasal cavity and ethmoid sinus: Tumor invading two subsites in a single region or extending to involve an adjacent region within the nasoethmoidal complex, with or without bony invasion. T3: Maxillary sinus: Tumor invading any of the following: bone of the posterior wall of maxillary sinus, subcutaneous tissues, floor or medial wall of orbit, pterygoid fossa, or ethmoid sinuses. Nasal cavity and ethmoid sinus: Tumor invading the medial wall or floor of the orbit, maxillary sinus, palate, or cribriform plate. N0: No regional lymph node metastasis. N1: Metastasis in a single ipsilateral lymph node, 3 cm or less in greatest dimension and ENE(-). N2: Metastasis in a single ipsilateral lymph node, 3 cm or smaller in greatest dimension and ENE(+); or metastasis in a single ipsilateral lymph node larger than 3 cm but not larger than 6 cm in greatest dimension and ENE(-); or metastases in multiple ipsilateral lymph nodes , none larger than 6 cm in greatest dimension and ENE(-); or metastases in bilateral or contralateral lymph nodes, none larger than 6 cm in greatest dimension and ENE(-). M0: No distant metastasis. (AJCC 8th ed.)


plasma membrane proton-transporting ATP synthase, stator stalk

biolink:CellularComponent biolink:AnatomicalEntity biolink:OrganismalEntity biolink:BiologicalEntity biolink:NamedThing biolink:PhysicalEssence biolink:SubjectOfInvestigation biolink:ThingWithTaxon biolink:PhysicalEssenceOrOccurrent

One of two stalks that connect the catalytic core of the hydrogen-transporting ATP synthase to the plasma membrane-associated F0 proteins; is thought to prevent futile rotation of the catalytic core. Examples of this component are found in Bacterial species.


proton-transporting ATP synthase, stator stalk

One of two stalks that connect the catalytic core of the hydrogen-transporting ATP synthase to the membrane-associated Fo proteins; is thought to prevent futile rotation of the catalytic core.


mitochondrial proton-transporting ATP synthase, stator stalk

One of two stalks that connect the catalytic core of the hydrogen-transporting ATP synthase to the mitochondrial membrane-associated F0 proteins; is thought to prevent futile rotation of the catalytic core.


stilbenoid

Any olefinic compound characterised by a 1,2-diphenylethylene backbone.


iminium ion

Cations of structure R2C=N(+)R2.


Cutaneous Indeterminate Dendritic Cell Tumor

A very rare dendritic cell tumor affecting the skin. It is composed of spindle to ovoid cells with a phenotype that is similar to the Langerhans cells. It manifests with cutaneous papules, nodules, and plaques.  The clinical course is variable.


small intestine venule

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Venule of villus of small intestine.


uterine epithelium

An epithelium that is part of a uterus.


epithelium of uterine horn

An epithelium that is part of a uterine horn [Automatically generated definition].


cervical squamo-columnar junction

Region of cervical epithelium where columnar epithelium of endocervic and the stratified non-keratinising squamous epithelium of the ectocervic meet.


ectocervical epithelium

An epithelium that is part of a ectocervix.


endometrium epithelium

An epithelium that is part of an endometrium [Automatically generated definition].


outer layer of endometrium

The layer of endometrial epithelium adjacent to the myometrium.


basal layer of endometrium

The layer of endometrial epithelium adjacent to the uterine cavity.


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

T2 or T3 or T4, N0, M0, G2 or G3 or Any T, N1, M0, Any G (G1, G2, G3 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.)


Seminal Vesicle Angiosarcoma

An extremely rare angiosarcoma that affects the seminal vesicle.


Stage IVA Uterine Corpus Endometrial Stromal Sarcoma AJCC v8

Stage IVA includes: T4, Any N, M0. T4: Uterine corpus endometrial stroma sarcoma with tumor invading bladder or rectum. M0: No distant metastasis. (from AJCC 8th Ed.)


Benign fibrohistiocytic neoplasm of skin

A benign neoplasm arising from the skin. It is composed of fibrohistiocytic cells, spindle fibroblastic cells, and histiocytes arranged in a storiform pattern.


Childhood Tenosynovial Giant Cell Tumor

A tenosynovial giant cell tumor that occurs during childhood.


Central Nervous System Solitary Fibrous Tumor

A mesenchymal, non-meningothelial neoplasm arising from the central nervous system. It is characterized by a collagenous and low cellularity spindle cell and/or hemangiopericytomatous histopathological pattern, recurrent intrachromosomal rearrangement on chromosome 12q that results in the fusion of the NAB2 and STAT6 genes, high recurrence rates, and long-term risk of systemic metastasis.


Extraskeletal osteosarcoma recurrent

The reemergence of extraskeletal osteosarcoma after a period of remission.


Digestive System Kaposi Sarcoma

A Kaposi sarcoma arising from the digestive system.


Dermatomyofibroma

A rare benign cutaneous mesenchymal neoplasm of fibroblastic/myofibroblastic differentiation. (WHO 2018)


Giant Cell Angiofibroma

A morphologic variant of angiofibroma characterized by the presence of multinucleated giant cells, collagenous or myxoid stroma, focal sclerotic areas, and angiectoid spaces.


Parotid Gland Nodular Fasciitis

A self-limiting, rapidly growing, non-encapsulated benign neoplasm that arises from the parotid gland. It is composed of fibroblastic/myofibroblastic cells.


Resectable Undifferentiated Pleomorphic Sarcoma

Undifferentiated pleomorphic sarcoma that is amenable to surgical resection.


Localized Childhood Non-Rhabdomyosarcoma Soft Tissue Sarcoma by Toronto Guidelines v2

Tumor confined to the area of origin including 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.)


Prostate Osteosarcoma

A rare osteosarcoma that arises from the prostate gland.


Oral Cavity Leiomyoma

A benign smooth muscle neoplasm arising from the oral cavity. It is characterized by the presence of spindle cells with cigar-shaped nuclei, interlacing fascicles, and a whorled pattern.


Central Nervous System Myofibroblastoma

A rare myofibroblastoma affecting the central nervous system.


Gastrointestinal Stromal Tumor of the Gastrointestinal Tract

A stromal tumor that originates from the interstitial cells of Cajal. It may involve any part of the gastrointestinal tract. The majority of cases occur in the stomach. Morphologic variants include the epithelioid cell type, spindle cell type, and mixed cell type. Most cases contain KIT- or PDGFRA-activating mutations. Lesions with a diameter greater than 10cm and more than 5 mitotic figures per 50HPF tend to have a more aggressive clinical course. Until recently, surgery has been the only effective therapy for this tumor. However, many patients still experience recurrence. A KIT tyrosine kinase inhibitor, imatinib mesylate (also known as STI-571 or Gleevec), is now effective in the treatment of relapsed and unresectable cases.


Esophageal Soft Tissue Neoplasm

A benign, intermediate, or malignant mesenchymal neoplasm that arises from the esophagus.


Recurrent Adult Soft Tissue Sarcoma

A soft tissue sarcoma occurring in adults that has recurred after a period of remission.


Salivary Gland Nodular Fasciitis

A self-limiting, rapidly growing, non-encapsulated benign neoplasm that arises from the salivary glands. It is composed of fibroblastic/myofibroblastic cells. It usually occurs in the parotid gland.


Stage II Small Intestinal, Esophageal, Colorectal, Mesenteric, and Peritoneal Gastrointestinal Stromal Tumor AJCC v8

Stage II 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.)


Uterine Corpus Inflammatory Myofibroblastic Tumor

A rare inflammatory myofibroblastic tumor that arises from the uterine corpus.


Cellular angiofibroma

A benign mesenchymal neoplasm that usually arises in the superficial soft tissues of the vulva or inguinal and scrotal regions.  It is characterized by the presence of a cellular fibroblastic proliferation in an edematous to fibrous stroma containing numerous vessels.


Localized Soft Tissue Sarcoma

A soft tissue sarcoma that is confined to a specific site without evidence of spread to other anatomic sites.


Prostate Undifferentiated Pleomorphic Sarcoma

A rare undifferentiated pleomorphic sarcoma that arises from the prostate gland.


Advanced Kaposi Sarcoma

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


EBV-Related Sarcoma

Sarcoma that is caused by Epstein-Barr virus infection.


Benign Central Nervous System Mesenchymal, Non-Meningothelial Neoplasm

A mesenchymal, non-meningothelial neoplasm that arises from the central nervous system and is characterized by the absence of atypical or malignant cytological and architectural features, and absence of invasive features or metastatic potential.


Kidney Oncocytoma-Like Angiomyolipoma

A rare angiomyolipoma of the kidney characterized by the presence of polygonal cells with eosinophilic cytoplasm.


Seminal Vesicle Soft Tissue Neoplasm

A mesenchymal neoplasm that arises from the seminal vesicle.


Stage II Uterine Sarcoma AJCC v7

Stage II includes: T2, N0, M0.  T2: Tumor extends beyond the uterus, within the pelvis.  N0: No regional lymph node metastasis.  M0: No distant metastasis.  This staging applies to leiomyosarcoma, endometrial stromal sarcoma, and adenosarcoma. (AJCC 7th ed.)


Stage II Gastric and Omental Gastrointestinal Stromal Tumor AJCC v8

Stage II includes: (T1, N0, M0, High Mitotic Rate); (T2, N0, M0, High Mitotic Rate); (T4, 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. 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.)


Stage IIB Adult Soft Tissue Sarcoma AJCC v7


Plaque-like CD34 positive dermal fibroma

A congenital or acquired benign dermal spindle cell neoplasm with histopathological features mimicking those of superficial dermal dermatofibrosarcoma protuberans. (WHO 2018)


Metastatic Extraskeletal Myxoid Chondrosarcoma

An extraskeletal myxoid chondrosarcoma which has spread to another anatomic site.


Bladder PEComa

A benign or malignant perivascular epithelioid cell tumor arising from the bladder.


Gastrointestinal Clear Cell Sarcoma/Malignant Gastrointestinal Neuroectodermal Tumor

A gastrointestinal tract sarcoma with neuroectodermal differentiation. It is characterized by the presence of neoplastic round cells with pale eosinophilic cytoplasm. Cytoplasmic clearing is seen only in a minority of cases. Half of the cases contain osteoclastic giant cells. It is associated with gene fusion translocations involving the EWSR1 gene, usually EWSR1-ATF1 fusion or EWSR1-CREB1 fusion.


Lacrimal Gland Myoepithelioma

A benign neoplasm with myoepithelial differentiation arising from the lacrimal gland. It lacks an infiltrative growth pattern and does not metastasize.


Uterine Corpus Smooth Muscle Neoplasm

A benign, intermediate, or malignant neoplasm that arises from the uterine corpus and is composed of neoplastic cells with smooth muscle differentiation.


Childhood Desmoplastic Small Round Cell Tumor

A desmoplastic small round cell tumor occurring in children.


Ossifying fibromyxoid tumor, malignant

An ossifying fibromyxoid tumor characterized by the presence of high grade nuclear features or increased cellularity and more than two mitotic figures per 50 HPFs.


Uterine Corpus High Grade Endometrial Stromal Sarcoma

A rare, high grade sarcoma that arises from the endometrial stroma. It is characterized by round cell morphology. It was previously also known as undifferentiated uterine 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.


Salivary Gland Myoepithelial Tumor

A benign or malignant tumor that arises from the salivary glands.  It is characterized by the presence of neoplastic cells with myoepithelial differentiation.  This category includes benign myoepithelioma and myoepithelial carcinoma.


Vulvar Angiosarcoma

A rare angiosarcoma that arises from the vulva.


Childhood Rhabdomyoma

A rhabdomyoma that occurs during childhood.


Liver Soft Tissue Neoplasm

A benign, intermediate, or malignant mesenchymal neoplasm that arises from the liver and intrahepatic bile ducts.


Stage IB Gastric Gastrointestinal Stromal Tumor

Stage IB includes: T3, N0, M0, Mitotic rate: Low.  T3: Tumor more than 5 cm, but not more than 10 cm, in greatest dimension.  N0: No regional lymph node metastasis.  M0: No distant metastasis. (AJCC 7th ed.)


Vulvar Soft Tissue Neoplasm

A benign or malignant mesenchymal neoplasm of the vulva.  Representative examples include leiomyoma, cellular angiofibroma, angiomyxoma, leiomyosarcoma, liposarcoma, and childhood botryoid-type embryonal rhabdomyosarcoma.


Gastric Inflammatory Myofibroblastic Tumor

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


Stage III Small Intestinal, Esophageal, Colorectal, Mesenteric, and Peritoneal Gastrointestinal Stromal Tumor AJCC v8

Stage III includes: IIIA: (T1, N0, M0, High Mitotic Rate); (T4, N0, M0, Low Mitotic Rate); IIIB: (T2, N0, M0, High Mitotic Rate); (T3, N0, M0, High Mitotic Rate); (T4, N0, M0, High 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. 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.)


Sinonasal Desmoid Fibromatosis

Desmoid fibromatosis that arises from the nasal cavity and paranasal sinuses.


Cervical Malignant Peripheral Nerve Sheath Tumor

A rare malignant neoplasm with nerve sheath differentiation arising from the cervix.


Breast Nodular Fasciitis

A self-limiting, rapidly growing, non-encapsulated benign neoplasm that arises from the breast. It has been described in both the subcutis and parenchyma of the breast. It occurs most frequently in the upper-outer quadrant. It is composed of fibroblastic/myofibroblastic cells.


Pilar-Associated Mesenchyme Neoplasm

A mesenchymal neoplasm arising from the pilar sheath of the hair follicle.


Gastric Malignant Gastrointestinal Stromal Tumor

A gastrointestinal stromal tumor arising from the stomach.  It is characterized by large size (diameter greater than 10 cm) or more than 5 mitotic figures per 50 high power fields.


penile fibromatosis

Fibromatosis arising from the soft tissues of the penis. It is characterized by the presence of spindle-shaped fibroblasts, and an infiltrative growth pattern. It causes the penis to bend when it becomes erect.


Benign Sinonasal Soft Tissue Neoplasm

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


Stage III Soft Tissue Sarcoma AJCC v7

Stage III includes: (T2a, T2b, N0, M0, G3); (Any T, N1, M0, Any G).  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.  N1: Regional lymph node metastasis.  M0: No distant metastasis.  G3: Grade 3.  There is no T2a for the pathologic group. (AJCC 7th ed.)


Fibrous tissue neoplasm of skin

A neoplasm arising from the dermis. It is characterized by the presence of neoplastic fibroblasts.


Vulvar Angiomyxoma

A deep or superficial angiomyxoma of the vulva.  It may recur following resection.


Stage II Soft Tissue Sarcoma AJCC v7

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.)


Metastatic Uterine Corpus Sarcoma

Uterine corpus sarcoma that has spread from its original site of growth to another anatomic site.


Biphenotypic sinonasal sarcoma

A rare, slow-growing, low-grade soft tissue sarcoma arising from the sinonasal tract. It presents with non-specific obstructive nasal symptoms. Morphologically, it is an infiltrative, cellular spindle cell neoplasm. It is associated with rearrangement of the PAX3 gene.


Breast Inflammatory Myofibroblastic Tumor

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


Bladder Benign PEComa

A benign perivascular epithelioid cell tumor arising from the bladder.


Recurrent Malignant Soft Tissue Neoplasm

Reemergence of a malignant soft tissue neoplasm after a period of remission.


Sinonasal Undifferentiated Pleomorphic Sarcoma

An undifferentiated pleomorphic sarcoma that arises from the nasal cavity and paranasal sinuses.


Locally Advanced Soft Tissue Sarcoma

Soft tissue sarcoma that has spread from its original site of growth to nearby tissues or lymph nodes.


Digestive System Soft Tissue Neoplasm

A benign, intermediate, or malignant mesenchymal neoplasm that arises from the digestive system.


Digestive System Lipoma

A lipoma arising from an anatomic site in the digestive system.


Prostate Solitary Fibrous Tumor

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


Stage I Uterine Corpus Endometrial Stromal Sarcoma AJCC v8

Stage I includes: T1, N0, M0. T1: Uterine corpus endometrial stroma sarcoma with tumor limited to the uterus. N0: No regional lymph node metastasis. M0: No distant metastasis. (AJCC 8th Ed.)


AIDS-Related Gastric Kaposi Sarcoma

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


Vulvar Kaposi Sarcoma

A Kaposi sarcoma arising from the vulva.


Digestive System Leiomyoma

A leiomyoma arising from an anatomic site in the digestive system.


Clear-Cell Myomelanocytic Tumors

A very rare, usually benign neoplasm with perivascular epithelioid cell differentiation characterized by the presence of clear spindle cells arranged in fascicles and nests that usually affects young girls.  Patients usually present with a painful abdominal mass.


Vaginal Superficial Myofibroblastoma

An uncommon, benign, well circumscribed mesenchymal neoplasm that grows beneath the vaginal epithelium. It is characterized by the presence of cells containing bland ovoid, spindle, or stellate-shaped nuclei in a collagenous stroma.


Uterine Corpus Epithelioid Smooth Muscle Tumor of Uncertain Malignant Potential

A smooth muscle tumor of uncertain malignant potential that arises from the uterine corpus and is composed of neoplastic epithelioid cells.


Vaginal Undifferentiated Sarcoma

A high grade infiltrating sarcoma that arises from the vagina. It is characterized by the presence of malignant small spindle cells with scant cytoplasm.


Prostate Soft Tissue Neoplasm

A mesenchymal neoplasm that arises from the prostate gland.


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

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

Soft tissue sarcoma occurring in childhood.


Atypical Lipomatous Tumor/Well Differentiated Liposarcoma of Deep Soft Tissue

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


Stage I Gastric and Omental Gastrointestinal Stromal Tumor AJCC v8

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

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


Seminal Vesicle Schwannoma

A schwannoma that affects the seminal vesicle.


Vulvar Smooth Muscle Tumor of Uncertain Malignant Potential

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

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


Esophageal Fibroma

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

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.


Recurrent Uterine Corpus Sarcoma

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


Kaposi's sarcoma, metastatic

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


Sinonasal Leiomyoma

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


Advanced Soft Tissue Sarcoma

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


Stage IV Gastric Gastrointestinal Stromal Tumor

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.)


Peritoneal Calcifying Fibrous Tumor

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

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


Soft Tissue Angiosarcoma

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

A soft tissue sarcoma that arises from the oral cavity.


Scrotal Sarcoma

A sarcoma involving the scrotum.


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

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.)


Recurrent Extraskeletal Myxoid Chondrosarcoma

Reemergence of extraskeletal myxoid chondrosarcoma after a period of remission


Refractory PEComa

PEComa that is resistant to treatment.


Malignant Epithelioid Cell Type Gastrointestinal Stromal Tumor

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


Kidney Angiosarcoma

A rare angiosarcoma arising from the kidney.


Skin Fibrous Histiocytoma, Histiocytic Variant

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

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.


Uterine Corpus Smooth Muscle Tumor of Uncertain Malignant Potential

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.


Kidney Angiomyolipoma with Epithelial Cysts

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

A leiomyoma that arises from the broad ligament.


Stage IIIB Gastric Gastrointestinal Stromal Tumor

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.)


Benign Skin Fibroblastic Neoplasm

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


Locally Advanced Undifferentiated Pleomorphic Sarcoma

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


Locally Advanced Desmoplastic Small Round Cell Tumor

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


Paratesticular Liposarcoma

A liposarcoma that arises from the paratesticular region.


Vulvar Lipoblastoma-Like Tumor

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

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


Uterine Corpus Endometrial Stromal and Related Neoplasms

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

Kaposi sarcoma that occurs during childhood.


Uterine Corpus Tumor Resembling Ovarian Sex Cord Tumor

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.


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

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.)


Central Nervous System Dedifferentiated Solitary Fibrous Tumor

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


Breast Soft Tissue Neoplasm

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


Vulvar Deep (Aggressive) Angiomyxoma

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


Peritoneal Low Grade Endometrioid Stromal Sarcoma

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


Stage IIIC Uterine Corpus Endometrial Stromal Sarcoma AJCC v8

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

Epithelioid sarcoma that is not amenable to surgical resection.


Oral Cavity Soft Tissue Neoplasm

A mesenchymal neoplasm that arises from the oral cavity.


Adult Desmoplastic Small Round Cell Tumor

A desmoplastic small round cell tumor occurring in adults.


Major Salivary Gland Sarcoma

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.


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

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


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

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

The reemergence of Kaposi sarcoma after a period of remission.


adult extraskeletal myxoid chondrosarcoma

An extraskeletal myxoid chondrosarcoma occurring in adults.


Peripheral Intraneural Angiosarcoma

A malignant vascular neoplasm arising from the peripheral nerves.


Kidney Classic Angiomyolipoma

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

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


Stage IIB Uterine Sarcoma AJCC v7

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


Intermediate Fibrohistiocytic Neoplasm

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


Stage IB Soft Tissue Sarcoma AJCC v7

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.)


Vulvar Cellular Angiofibroma

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.


Sinonasal Hemangioma

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


Benign Epithelioid Cell Type Gastrointestinal Stromal Tumor

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


Benign Extraskeletal Cartilaginous and Osseous Neoplasm

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

Uterine corpus sarcoma that is not amenable to surgical resection.


Low Grade Endometrial Stromal Sarcoma

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

An epithelioid sarcoma that arises from the vulva.


Central Nervous System Solitary Fibrous Tumor, Grade 2

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

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

A myoepithelial tumor that occurs during childhood.


Locally Advanced Unresectable Soft Tissue Sarcoma

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.


Virus-Related Sarcoma

A sarcoma that is caused by a virus.


Visceral Angiosarcoma

A soft tissue angiosarcoma arising from the internal organs.


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

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

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

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)


Thyroid Gland Malignant Peripheral Nerve Sheath Tumor

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


Conjunctival Sarcoma

A sarcoma that arises from the conjunctiva.


Stroma-Derived Neoplasm of Lymphoid Tissues

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


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


Childhood Inflammatory Myofibroblastic Tumor

An inflammatory myofibroblastic tumor that occurs during childhood.


Laryngeal Soft Tissue Neoplasm

A mesenchymal neoplasm that arises from the larynx.


Skin Pleomorphic Lipoma

A pleomorphic lipoma arising from the skin.


Uterine Corpus Low Grade Endometrial Stromal Sarcoma

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.


Malignant fibrous histiocytoma metastatic

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


Bone Leiomyoma

A rare leiomyoma affecting the bone.


Skin Soft Tissue Neoplasm

A mesenchymal neoplasm that arises from the skin.


Ectopic hamartomatous thymoma

A benign, well-circumscribed tumor of the neck occurring in adults.  It is characterized by the presence of spindle cells, epithelial islands, and adipose tissue.  There is no evidence of thymic origin or differentiation, despite the use of the term thymoma in the literature.


Mammary-Type Myofibroblastoma

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

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


Periosteal fibroma

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


Metastatic Soft Tissue Sarcoma

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


Stage I Soft Tissue Sarcoma AJCC v7

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

A sinonasal soft tissue neoplasm with hemangiopericytoma-like features.


Stage III Adult Soft Tissue Sarcoma AJCC v7


Stage IIIA Gastric and Omental Gastrointestinal Stromal Tumor AJCC v8

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.)


Stage IIIA Uterine Corpus Endometrial Stromal Sarcoma AJCC v8

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.)


Low Grade Paranasal Sinus Sarcoma

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

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


Hemangioma, Cavernous, Central Nervous System

A cavernous hemangioma arising from the central nervous system.


Vaginal Soft Tissue Neoplasm

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.


Unresectable Desmoid Fibromatosis

Desmoid fibromatosis that is not amenable to surgical resection.


Parotid Gland Liposarcoma

A liposarcoma that arises from the parotid gland.


Laryngeal Soft Tissue Sarcoma

A sarcoma that arises from soft tissue of the larynx.


Adult Soft Tissue Sarcoma

Soft tissue sarcoma occurring in adults.


Benign Uterine Corpus PEComa

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.


Stage IA Adult Soft Tissue Sarcoma AJCC v7


Refractory Malignant Myoepithelioma

A malignant myoepithelioma that does not respond to treatment.


Hemosiderotic Fibrolipomatous Tumor

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.


Central Nervous System Undifferentiated Pleomorphic Sarcoma

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


Uterine Corpus Myxoid Smooth Muscle Tumor of Uncertain Malignant Potential

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


Tumor of Specialized Prostatic Stroma

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

A mesenchymal neoplasm that arises from the paratesticular region.


Vulvar Distal-Type Epithelioid Sarcoma

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


Non-Metastatic Childhood Soft Tissue Sarcoma

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


Angiofibroma of Nose

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


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


Vaginal Solitary Fibrous Tumor

A rare solitary fibrous tumor that arises from the vagina.


Stage IV Gastric and Omental Gastrointestinal Stromal Tumor AJCC v8

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

A leiomyoma that arises from the seminal vesicle.


EBV-Positive Inflammatory Follicular Dendritic Cell Sarcoma

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.


Childhood Undifferentiated Pleomorphic Sarcoma

An undifferentiated pleomorphic sarcoma that occurs during childhood.


Stage IVB Uterine Corpus Endometrial Stromal Sarcoma AJCC v8

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


Superficial Atypical Lipomatous Tumor/Well Differentiated Liposarcoma

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


Solitary Adult Fibroma

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


Vaginal Smooth Muscle Tumor of Uncertain Malignant Potential

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

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

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

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.


Uterine Corpus Endometrial Stromal Sarcoma by AJCC v8 Stage

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


Superficial CD34-Positive Fibroblastic Tumor

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

A spindle cell lipoma arising from the skin.


Cervical Soft Tissue Neoplasm

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


Extragastrointestinal Gastrointestinal Stromal Tumor

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


Stage IIB Soft Tissue Sarcoma AJCC v7

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

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


Lipoma of appendix

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


Sinonasal Malignant Peripheral Nerve Sheath Tumor

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


Omentum Gastrointestinal Stromal Tumor

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.


Dermatofibrosarcoma Protuberans with Giant Cell Fibroblastoma-Like Differentiation

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


Vulvar Fibrolipoma

A lipoma of the vulva with a predominant fibrous component.


Skin Angiolipoma

An angiolipoma arising from the skin.


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

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

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


Parotid Gland Sarcoma

A sarcoma that arises from the parotid gland.


Recurrent Soft Tissue Sarcoma of the Trunk and Extremities

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


Gastric Plexiform Fibromyxoma

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

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


Breast Vascular Neoplasm

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


Extraskeletal Cartilaginous and Osseous Neoplasm

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


Skin Fibrous Histiocytoma, Cellular Variant

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


Metastatic extraskeletal osteosarcoma

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


Paratesticular Hemangioma

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


Paratesticular Leiomyoma

A leiomyoma that arises from the paratesticular region.


Uterine Ligament Leiomyoma

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


Perivascular epithelioid tumor, malignant

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.


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

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

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.)


Kaposi's sarcoma of extrahepatic bile duct

A Kaposi sarcoma that arises from the extrahepatic bile ducts.


Benign Oral Cavity Soft Tissue Neoplasm

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.


Dedifferentiated Gastrointestinal Stromal Tumor

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.


Childhood Angiomatoid Fibrous Histiocytoma

An angiomatoid fibrous histiocytoma that occurs during childhood.


sarcoma of tongue

A sarcoma that arises from the tongue.


Stage IC Uterine Sarcoma AJCC v7

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.)


Abdominal Undifferentiated Pleomorphic Sarcoma

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


Ectomesenchymal Chondromyxoid Tumor

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.


Tongue Liposarcoma

A liposarcoma that arises from the tongue.


Gastric Soft Tissue Neoplasm

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


Kidney Epithelioid Angiomyolipoma

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

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


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

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.


Bladder Angiosarcoma

A very rare angiosarcoma that arises from the bladder.


Primitive non-neural granular cell tumor

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

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

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


Stage IV adult soft tissue sarcoma

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


Malignant Extraskeletal Cartilaginous and Osseous Neoplasm

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

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.)


Spindle Cell Type Gastrointestinal Stromal Tumor

A gastrointestinal stromal tumor composed of neoplastic spindle cells.


Central Nervous System Epithelioid Hemangioendothelioma

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


Stage IIA Uterine Sarcoma AJCC v7

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


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

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

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

An epithelioid sarcoma which has spread to another anatomic site.


Scrotal Liposarcoma

A liposarcoma involving the scrotum.


Vulvar Lipoma

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


Stage II Uterine Corpus Endometrial Stromal Sarcoma AJCC v8

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.)


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

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.)


Childhood Gastrointestinal Stromal Tumor

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.


Vaginal Angiomyofibroblastoma

An angiomyofibroblastoma arising from the vagina.


Uterine Corpus Metastasizing Leiomyoma

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.


Atypical fibroxanthoma - spindle cell type

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


Sellar Solitary Fibrous Tumor

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


Recurrent Desmoid Fibromatosis

Desmoid fibromatosis that has recurred after a period of remission.


Uterine Corpus Hydropic Leiomyoma

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


Mesenteric Gastrointestinal Stromal Tumor

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.


Childhood Plexiform Fibrohistiocytic Tumor

A plexiform fibrohistiocytic tumor that occurs during childhood.


undifferentiated sarcoma of soft tissue

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.


Locally Advanced Unresectable Malignant PEComa

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.


Pleomorphic hyalinizing angiectatic tumor of soft tissue

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

A morphologic variant of fibroma characterized by increased cellularity.


Central Nervous System Desmoid Fibromatosis

Intracranial and/or spinal involvement by desmoid fibromatosis.


Skin Radiation-Related Angiosarcoma

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


Peritoneal Gastrointestinal Stromal Tumor

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

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


Gardner Fibroma

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

A high grade undifferentiated sarcoma that arises from the endocervix.


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

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.)


Stage IB Gastric and Omental Gastrointestinal Stromal Tumor AJCC v8

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.)


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

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

Soft tissue sarcoma that is not amenable to surgical resection.


Unresectable Desmoplastic Small Round Cell Tumor

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


Childhood PEComa

A PEComa that occurs during childhood.


Refractory Desmoid Fibromatosis

Desmoid fibromatosis that is resistant to treatment.


Maxillofacial Desmoplastic Fibroma

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

Lymphangiomyomatosis involving the dermis.


Benign Skin Vascular Neoplasm

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.


Stage I Adult Soft Tissue Sarcoma AJCC v7

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

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


Paratesticular Deep (Aggressive) Angiomyxoma

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


Nuchal fibroma

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.


Meningeal Central Nervous System Solitary Fibrous Tumor, Grade 3

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


Stage IV Adult Soft Tissue Sarcoma AJCC v7

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


Abdominal Inflammatory Myofibroblastic Tumor

Inflammatory myofibroblastic tumor that arises from the abdominal cavity.


Benign Laryngeal Soft Tissue Neoplasm

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

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


Vascular neoplasm of skin

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


Angiomyofibroblastoma

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.


Metastatic Desmoplastic Small Round Cell Tumor

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


Metastatic Childhood Soft Tissue Sarcoma

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


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

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

A rare solitary fibrous tumor that arises from the vulva.


Stage IIIB Gastric and Omental Gastrointestinal Stromal Tumor AJCC v8

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

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


Parotid Gland Kaposi Sarcoma

A Kaposi sarcoma arising from the parotid gland.


Recurrent Malignant Myoepithelioma

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


Stage IA Soft Tissue Sarcoma AJCC v7

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.)


Esophageal Malignant Peripheral Nerve Sheath Tumor

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


Fascial fibroma

A benign fibrous tumor arising from the fascial connective tissue.


Stage IA Gastric Gastrointestinal Stromal Tumor

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

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.


Stage IV Uterine Corpus Endometrial Stromal Sarcoma AJCC v8

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.)


Benign Pilar-Associated Mesenchyme Neoplasm

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.


Seminal Vesicle Mammary-Type Myofibroblastoma

An extramammary myofibroblastoma that affects the seminal vesicle.


Stage I Uterine Sarcoma AJCC v7

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

A rare malignant mesenchymal neoplasm that arises from the cervix.


Skin Intermediate Fibrohistiocytic Neoplasm

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


Digestive System Sarcoma

A sarcoma that arises from the digestive system.


Stage III Uterine Sarcoma AJCC v7

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.)


Peritoneal Desmoplastic Small Round Cell Tumor

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


Stage II Adult Soft Tissue Sarcoma AJCC v7

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

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

A lymphangioma arising from the stomach.


Bladder Malignant PEComa

A malignant perivascular epithelioid cell tumor arising from the bladder.


Leiomyoma, Epithelioid

A benign or malignant gastrointestinal stromal tumor with epithelioid morphology.


Giant Cell Angioblastoma

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.


Undifferentiated Sarcoma, Not Otherwise Specified

An undifferentiated soft tissue sarcoma which cannot be further characterized.


Stage IB Uterine Sarcoma AJCC v7

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

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

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


Laryngeal Granular Cell Tumor

A granular cell tumor that arises from the larynx.


Childhood EWSR1-SMAD3-Positive Fibroblastic Tumor

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


Breast Angiolipoma

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

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

A spindle cell/pleomorphic lipoma arising from the skin.


Central Nervous System Angiolipoma

An angiolipoma that arises from the central nervous system.


Malignant Sinonasal Soft Tissue Neoplasm

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


Sclerosing PEComa

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


Epithelioid Sarcoma NCI Grade 2

An epithelioid sarcoma with less than 15% necrosis.


Bladder Solitary Fibrous Tumor

A solitary fibrous tumor that arises from the bladder. Most tumors are benign.


Uterine Corpus Central Primitive Neuroectodermal Tumor

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


Undifferentiated Stromal Sarcoma

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


EBV-Positive Inflammatory Follicular Dendritic Cell Sarcoma of the Digestive System

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


Gastric and Omental Gastrointestinal Stromal Tumor by AJCC v8 Stage

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.)


Mixed Cell Type Gastrointestinal Stromal Tumor

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


Conventional Extraskeletal Myxoid Chondrosarcoma

A well differentiated extraskeletal myxoid chondrosarcoma.


Skin Atypical Lipomatous Tumor/Well Differentiated Liposarcoma

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


Myxoid leiomyoma

A leiomyoma characterized by the presence of abundant myxoid matrix.


Hemangioendothelioma of skin

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


Malignant Laryngeal Soft Tissue Neoplasm

A malignant mesenchymal neoplasm that arises from the larynx.


Undifferentiated Epithelioid Sarcoma

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


Stage IV Soft Tissue Sarcoma AJCC v7

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


Gastrointestinal Stromal Tumor by AJCC v8 Stage

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.)


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

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.)


Vulvar Angiomyofibroblastoma

An angiomyofibroblastoma arising from the vulva.


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


Vaginal Low Grade Endometrioid Stromal Sarcoma

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

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


Childhood NTRK-Rearranged Spindle Cell Neoplasm

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


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

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

A mesenchymal neoplasm that arises from the bladder.


Superficial angiomyxoma

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


Stage III Gastric and Omental Gastrointestinal Stromal Tumor AJCC v8

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

Soft tissue sarcoma that is amenable to surgical resection.


Epithelioid sarcoma non-metastatic

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


Desmoplastic fibroblastoma

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.


Uterine Corpus Leiomyoma, Mitotically Active Variant

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

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


Recurrent Childhood Soft Tissue Sarcoma

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


Lacrimal Gland Myoepithelial Carcinoma

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


Optic Nerve Sheath Solitary Fibrous Tumor

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


Metastatic Malignant PEComa

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


Stage IA Uterine Corpus Endometrial Stromal Sarcoma AJCC v8

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.)


Central Nervous System Inflammatory Myofibroblastic Tumor

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.


An extramammary myofibroblastoma that arises from the paratesticular region.


Soft Tissue Sarcoma of the Trunk and Extremities

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


Stage II Gastric Gastrointestinal Stromal Tumor

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.)


Recurrent Desmoplastic Small Round Cell Tumor

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


Stromal Neoplasm

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

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.


Classic Type Atypical Fibroxanthoma

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


Conjunctival Angiosarcoma

An angiosarcoma that arises from the conjunctiva.


Fibromatosis, Abdominal

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.


Soft Tissue Tumor of Uncertain Differentiation

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

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.


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

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

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.


Prostate Inflammatory Myofibroblastic Tumor

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


Locally Invasive Desmoid-Type Fibromatosis

Desmoid-type fibromatosis that has invaded the surrounding tissues.


Superficial Acral Fibromyxoma

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.


Paratesticular Cellular Angiofibroma

A cellular angiofibroma that arises from the paratesticular region.


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

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.)


Dermatofibrosarcoma protuberans with myoid differentiation

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


Advanced Malignant PEComa

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


Unresectable Malignant Gastrointestinal Stromal Tumor

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


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

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.)


Pulmonary Artery Angiosarcoma

A malignant vascular neoplasm arising from the pulmonary artery.


Appendix Kaposi Sarcoma

A rare Kaposi sarcoma that arises from the appendiceal wall.


Recurrent Gastrointestinal Stromal Tumor

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


Breast Atypical Myoepitheliosis

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.


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

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


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

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

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


Malignant Soft Tissue Tumor of Uncertain Differentiation

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


Skin Myoepithelioma

A benign skin neoplasm composed exclusively of myoepithelial cells.


Intermediate Sinonasal Soft Tissue Neoplasm

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.


Gastric Gastrointestinal Stromal Tumor of Uncertain Malignant Potential

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.


Vulvar Superficial Angiomyxoma

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


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


Prostate Malignant Solitary Fibrous Tumor

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


Hepatobiliary Soft Tissue Neoplasm

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


Thyroid Gland Leiomyoma

A leiomyoma that arises from the thyroid gland.