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.)


Benign Extraskeletal Cartilaginous and Osseous Neoplasm

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


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.


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.


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.


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


Laryngeal Soft Tissue Neoplasm

A soft tissue neoplasm that arises from the larynx.


Skin Pleomorphic Lipoma

A pleomorphic lipoma arising from the skin.


Kidney Leiomyoma

A benign smooth muscle neoplasm arising from the kidney.


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.


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  follicular dendritic cell sarcoma that occurs almost exclusively in the liver and spleen and is consistently associated with Epstein-Barr virus.


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.


Extraskeletal osteosarcoma metastatic

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 non-metastasizing soft tissue neoplasm that arises from the oral cavity.


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.


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

A rare variant of kidney angiomyolipoma that consists of at least 80% epithelioid cells. (WHO 2016)


Benign Fibroblastic Neoplasm

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


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.)


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 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.


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.


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.)


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 benign vascular neoplasm that arises in the skin.


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 non-metastasizing soft tissue neoplasm that arises from the larynx.


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


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 soft tissue 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

An inflammatory follicular dendritic cell sarcoma that occurs in the digestive system and is consistently associated with Epstein-Barr virus.


Undifferentiated Epithelioid Sarcoma

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


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 soft tissue neoplasm that arises from the larynx.


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.


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 soft tissue 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 mesenchymal 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 non-metastasizing mesenchymal neoplasm of the stomach.  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.


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 tumor 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 soft tissue 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.


Atypical fibroxanthoma of skin

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


Skin Fibrous Histiocytoma, Fibroblastic Variant

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


Soft Tissue Sarcoma by AJCC v7 Stage

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


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


Intracranial Fibrous Histiocytoma

A benign fibrous histiocytoma involving the dura or cranial bone.


Intracranial lipoma

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


Kaposi Sarcoma Related to Immunosuppressive Treatment

A Kaposi sarcoma that develops after immunosuppressive treatment.


Digestive System Inflammatory Myofibroblastic Tumor

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


Pelvic Fibromatosis

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


Paratesticular Schwannoma

A schwannoma that arises from the paratesticular region.


Stage IIIC Uterine Sarcoma AJCC v7

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


Uterine Angiosarcoma

A malignant vascular neoplasm arising from the uterus.


Stage III Uterine Corpus Endometrial Stromal Sarcoma AJCC v8

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


Stage IVA Uterine Sarcoma AJCC v7

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


Gastric sarcoma

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


Recurrent Skin Angiosarcoma

The reemergence of skin angiosarcoma after a period of remission.


Intermediate Soft Tissue Tumor of Uncertain Differentiation

A soft tissue tumor of uncertain differentiation which is locally aggressive but rarely metastasizes.


Uterine Corpus Sarcoma by AJCC v8 Stage

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


Cervical Low Grade Endometrioid Stromal Sarcoma

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


Kaposi Sarcoma NCI Grade 2

A Kaposi sarcoma with less than 15% necrosis.


Lipofibromatosis

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


Malignant vascular tumor of skin

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


EWSR1-SMAD3-Positive Fibroblastic Tumor

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


Unresectable Undifferentiated Pleomorphic Sarcoma

Undifferentiated pleomorphic sarcoma that is not amenable to surgical resection.


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

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


Prostate Large Cell Neuroendocrine Carcinoma

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


epithelium of external nose

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Epithelial layer that is part of the external part of the nose.


fifth cervical dorsal root ganglion

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


Postpartum Obstetric Thromboembolism

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


Ampulla of Vater Neuroendocrine Tumor G1

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


Tle3-Aes complex

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A transcriptional repressor complex that consists of a heterodimer of the proteins Tle3 (also known as Grg3b) and Aes (Grg5), which are homologs of the Drosophila groucho gene product.


Cyc8(Ssn6)-Tup1 general repressor complex

A corepressor complex containing the WD-repeat protein Tup1p (S. cerevisiae) and Tup11/Tup12 (fission yeast) and the TPR repeat protein Cyc8p (S. cerevisiae) ssn6 (fission yeast) that is recruited to target genes by DNA-bound repressor proteins preferentially at regions where histones are deacetylated by the Clr6 class I HDAC, and recruits the SWI/SNF and SAGA complexes to promoters.


negative cofactor 2 complex

A heterodimeric protein complex that can stably associate with TATA-binding protein on promoters, thereby preventing the assembly of transcription factors TFIIA and TFIIB and leading to repression of RNA polymerase II transcription. The two subunits, NC2alpha (Drap1) and NC2beta (Dr1), dimerize through histone fold domains of the H2A/H2B type present in the amino termini.


DRM complex

A transcriptional repressor complex that contains the lin-9, lin-35, lin-37, lin-52, lin-53, lin-5is involved in 4-, dpl-1 and efl-1 proteins, and is involved in cell fate specification.


beta-catenin-ICAT complex

Transcription factor complex that inhibits binding of Tcf to beta-catenin while preserving interaction of catenin with cadherin thus inhibiting transcription mediated by beta-catenin-Tcf complex.


Mad-Max-mSin3A complex

A transcriptional repressor complex that contains a heterodimer of the bHLH-ZIP proteins Mad and Max, plus mSin3A, a homolog of the yeast Sin3p.


Mad-Max complex

A transcriptional repressor complex that consists of a heterodimer of the bHLH-ZIP proteins Mad and Max.


Osteolytic Lesions of Multiple Myeloma

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


Solitary Lytic Metastatic Bone Lesion

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


Laryngeal Fistula

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


Pharyngocutaneous fistula

An abnormal communication between the skin and the pharynx.


Salivary Gland Fistula

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


External Auditory Canal Fistula

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


Locally Advanced Liposarcoma

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


Liposarcoma metastatic

A liposarcoma that has metastasized to a secondary site.


Metastatic Dedifferentiated Liposarcoma

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


Advanced Myxoid Liposarcoma

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


Myxoid liposarcoma metastatic

Myxoid liposarcoma that has spread to other anatomic sites.


Advanced Dedifferentiated Liposarcoma

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


Locally Advanced Dedifferentiated Liposarcoma

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


Advanced Liposarcoma

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


collagen and cuticulin-based cuticle extracellular matrix

A collagen and cuticulin-based noncellular, multilayered structure that is synthesized by an underlying ectodermal (hypodermal) cell layer. The cuticle serves essential functions in body morphology, locomotion, and environmental protection. An example of this component is found in Caenorhabditis elegans.


chitin-based extracellular matrix

Any constituent part of a chitin-based noncellular, hardened, or membranous extracellular matrix secreted from the apical surface of an epithelial sheet.


An extracellular matrix consisting mainly of proteins (especially collagen) and glycosaminoglycans (mostly as proteoglycans) that provides not only essential physical scaffolding for the cellular constituents but can also initiate crucial biochemical and biomechanical cues required for tissue morphogenesis, differentiation and homeostasis. The components are secreted by cells in the vicinity and form a sheet underlying or overlying cells such as endothelial and epithelial cells.


fertilization envelope

A structure that lies outside the plasma membrane and surrounds the egg. The fertilization envelope forms from the vitelline membrane after fertilization as a result of cortical granule release.


hyaline layer

A multilayered extraembryonic matrix that functions as a substrate for cell adhesion through early development. It is thought to protect and lubricate the embryo, stabilize the blastomeres during morphogenesis, and regulate nutrient intake. The major constituent of the hyaline layer is the protein hyalin. This matrix has been found in echinoderms.


fungal biofilm matrix

An extracellular matrix lying external to fungal cells. The fungal biofilm matrix consists of polysaccharides, proteins, lipids, and nucleic acids. Fungal biofilms mediate adherence to host tissues, and provide protection from host immune defenses.


biofilm matrix

A structure lying external to microbial cells. A biofilm is an aggregate of surface-associated cells, and the biofilm matrix is the envelope of polymeric substances that surrounds the cells.


egg coat

A specialized extracellular matrix that surrounds the plasma membrane of the ovum of animals. The egg coat provides structural support and can play an essential role in oogenesis, fertilization and early development.


bacterial biofilm matrix

A structure lying external to bacterial cells. A biofilm is an aggregate of surface-associated bacteria, and the biofilm matrix is the envelope of polymeric substances that surrounds the bacteria.


organomineral extracellular matrix

An extracellular matrix consisting of a densely packed organomineral assembly in which the mineral phase represents the majority of the material by weight.


perisynaptic extracellular matrix

The portion of the extracellular matrix that lies within the perisynaptic space.


synapse-associated extracellular matrix

The extracellular matrix of the perisynaptic space (the extracellular space adjacent to the synapse) and the synaptic cleft.


interphotoreceptor matrix

A specialized extracellularc matrix that surrounds the photoreceptors of the retina and lies between them and the apical surface of the retinal pigment epithelium. The IPM has been implicated in several important activities required for photoreceptor function and maintenance.


pollen coat

A layer of extracellular matrix deposited onto the surface of the pollen wall upon disintegration of the tapetal layer of the anther wall in the late stages of pollen development. The composition of this material is highly heterogeneous and includes waxes, lipid droplets, small aromatic molecules, and proteins. The pollen coat is proposed to have many functions, such as holding pollen in the anther until dispersal, facilitation of pollen dispersal, protection of pollen from water loss and UV radiation, and facilitation of adhesion of pollen to the stigma.


mineralized extracellular matrix

Extracellular matrix that is mineralized.


vitelline envelope

A glycoprotein-based structure that lies outside the plasma membrane and surrounds the egg before fertilization.


adhesive extracellular matrix

A extracellular matrix which attaches an organism to a substrate.


Recurrent Multinodular and Vacuolated Neuronal Tumor

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


brain gray matter

A gray matter that is part of a brain [Automatically generated definition].


ATG2-ATG18 complex

A protein complex essential for autophagy during nutrient deprivation, a catabolic process that sequesters undesired cellular material into autophagosomes for delivery to lysosomes for degradation. Contributes to nutrition homeostasis and damage control in eukaryotic cells. Functions at a late step of autophagosome formation for efficient completion of sequestration, probably through facilitating recruitment of ATG8-phosphatidylethanolamine (PE) to the preautophagosomal structure (PAS) and/or its protection from deconjugation by ATG4. Composed of ATG2 and ATG18 in Saccharomyces cerevisiae.


Stage IV Ureter Cancer AJCC v8

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


Cleft Maxilla

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


zygomaticus muscle

One of: zygomaticus major, zygomaticus minor.


Fallopian Tube Lymphoma

An exceedingly rare lymphoma that arises from the fallopian tube.


intracellular vesicle

Any vesicle that is part of the intracellular region.


post-lysosomal vacuole

A membrane-bounded intracellular vesicle formed late in the endocytic pathway when the pH in the vacuole becomes neutral prior to exocytosis.


piccolo-bassoon transport vesicle

A cytoplasmic dense-core vesicle that transports a range of proteins including piccolo, bassoon, N-cadherin and syntaxin. The transported proteins may be associated with the external side of the vesicle, rather than being contained within the vesicle, therefore forming an aggregate of vesicle and proteins. Piccolo-bassoon transport vesicles (or PTVs) range in size from approximately 80 nm in diameter for dense core vesicles to 130 nm by 220 nm in area for aggregates. They are packaged via the trans-Golgi network before being transported through the axon.


bacteroid-containing symbiosome

A symbiosome containing any of various structurally modified bacteria, such as those occurring on the root nodules of leguminous plants.


food vacuole

Vacuole within a parasite used for digestion of the host cell cytoplasm. An example of this component is found in the Apicomplexa.


synaptic vesicle, readily releasable pool

A synaptic vesicle belonging to the pool of vesicles that are the first to be released as a result of chemical or electrical stimulation e.g. by an action potential, have the highest presynaptic membrane fusion probability and correspond to about 1% of the total number of synaptic vesicles at a resting terminal bouton.


COPI-coated inter-Golgi transport vesicle

A vesicle that mediates transport of cargo within the Golgi complex (for example, between cisternae of the Golgi stack).


neuronal dense core vesicle

A dense core vesicle (granule) that is part of a neuron. These vesicles typically contain neuropeptides. They can be found in all parts of neurons, including the soma, dendrites, axonal swellings (varicosities) and synaptic terminals.


leucosome

A tissue-specific, membrane-bounded cytoplasmic organelle within which uric acid and/or purines crystalize in reflective stacks. Leucosomes are synthesized in leucophore cells and have a whitish cast.


pexophagosome

A membrane-bounded intracellular vesicle involved in the degradation of peroxisome by macropexophagy.


mucocyst

A small subcellular vesicle, surrounded by a membrane, in the pellicle of ciliate protozoans that discharges a mucus-like secretion.


cortical granule

A secretory vesicle that is stored under the cell membrane of an egg. These vesicles fuse with the egg plasma membrane as part of egg activation and are part of the block to polyspermy.


synaptic vesicle, recycling pool

A synaptic vesicle belonging to the pool that repopulate vacancies within the readily releasable pool (RRP) of synaptic vesicles, and require more significant stimuli than the RRP in order to release neurotransmitter; about 10-15% of the total number of synaptic vesicles at a resting terminal bouton are in this state.


neurosecretory vesicle

A large cytoplasmic membrane-bounded vesicle with an electron dense granular core, up to 150-200 nm in diameter, found in neurosecretory cells in the hypothalamus.


clathrin-coated phagocytic vesicle

A clathrin-coated, membrane-bounded intracellular vesicle that arises from the ingestion of particulate material by phagocytosis.


postsynaptic recycling endosome

A recycling endosome of the postsynapse. In postsynaptic terminals with dendritic spines, it is typically located at the base of a dendritic spine. It is involved in recycling of neurotransmitter receptors to the postsynaptic membrane. In some cases at least, this recycling is activated by postsynaptic signalling and so can play a role in long term potentiation.


micropinosome

A membrane-bounded, uncoated intracellular vesicle formed by the process of micropinocytosis.


endosome to plasma membrane transport vesicle

A transport vesicle that mediates transport from the endosome to the plasma membrane, and fuses with the plasma membrane to deliver lipids and membrane proteins to the plasma membrane and to release various cargo molecules, such as proteins or hormones, by exocytosis.


Golgi to plasma membrane transport vesicle

A transport vesicle that mediates transport from the Golgi to the plasma membrane, and fuses with the plasma membrane to release various cargo molecules, such as proteins or hormones, by exocytosis.


dendritic lamellar body

A specialized secretory organelle found in neurons and associated with the formation of dendrodendritic gap junctions.


symbiosome

A double-enveloped cell compartment, composed of an endosymbiont with its plasmalemma (as inner envelope) and a non-endosymbiotic outer envelope (the perisymbiontic membrane).


contractile vacuole

A specialized vacuole of eukaryotic cells, especially Protozoa, that fills with water from the cytoplasm and then discharges this externally by the opening of contractile vacuole pores. One of its functions is osmoregulatory.


mucin granule

A secretory granule that contains mucin.


apical recycling endosome

Tubulo-vesicular structure located in the apical cytoplasm that participates in apical cargo recycling in polarized epithelial cells.


organellar chromatophore

A bacteroid-containing symbiosome in which the bacterial component is a genetically highly reduced cyanobacterium that is photosynthetically active and incapable of an independent existence outside its host. The chromatophore functions as a photosynthetic organelle, and has been found and characterized in the amoeba Paulinella chromatophora.


cyanosome

A tissue-specific, membrane-bounded cytoplasmic organelle within which an unknown blue pigment is localized. Cyanosomes are synthesized in cyanophores and are blue in appearance.


pterinosome

A tissue-specific, membrane-bounded cytoplasmic organelle within which pteridine pigments are synthesized and stored. Pterinosomes are synthesized in xanthophores and erythrophore cells and are yellow, orange or red in appearance.


presynaptic endosome

An endosome present in the presynapse that fuses with endocytic vesicles arising in the presynaptic endocytic zone. This organelle is believed to be involved in regeneration of synaptic vesicles.


ficolin-1-rich granule

Highly exocytosable gelatinase-poor granules found in neutrophils and rich in ficolin-1. Ficolin-1 is released from neutrophil granules by stimulation with fMLP or PMA, and the majority becomes associated with the surface membrane of the cells and can be detected by flow cytometry.


basolateral recycling endosome

Tubulo-vesicular structure located in the basolateral cytoplasm that participates in basolateral cargo recycling in polarized epithelial cells.


postsynaptic early endosome

An early endosome of the postsynapse. It acts as the major sorting station on the endocytic pathway, targeting neurotransmitter receptors for degregation or recycling.


cytoplasmic vesicle

A vesicle found in the cytoplasm of a cell.


postsynaptic endosome

An endosomal compartment that is part of the post-synapse. Only early and recycling endosomes are typically present in the postsynapse.


CGRP receptor complex

A transmembrane, G protein-coupled signalling receptor complex recognized by calcitonin gene-related peptides (CGRP).


calcitonin family receptor complex

A protein complex which is capable of calcitonin family receptor activity. Calcitonin family receptors may form dimers, trimers or tetramers; adrenomedullin and amylin receptors have only been observed as dimers so far.


adrenomedullin receptor complex

A transmembrane, G protein-coupled signalling receptor complex which is capable of adrenomedullin receptor activity.


amylin receptor complex 3

A G protein-coupled receptor complex that serves as a receptor for amylin polypeptide (AMY) and consists of a calcitonin receptor (CTR/CALCR) and a receptor activity-modifying protein (RAMP) 3. Amylin receptor complex 3 (AMY3) also serves as a receptor for the amyloid-beta complex. Ligand binding to AMY3 results in increased cytosolic calcium ion levels and in activation on multiple intracellular signalling pathways.


amylin receptor complex

A protein complex which is capable of amylin receptor activity.


amylin receptor complex 1

A G protein-coupled receptor complex that serves as a receptor for amylin polypeptide (AMY) and consists of a calcitonin receptor (CTR/CALCR) and a receptor activity-modifying protein (RAMP) 1. Amylin receptor complex 1 (AMY1) also serves as a receptor for the calcitonin related peptide (CGRP) and adrenomedullin (AM/ADM).


amylin receptor complex 2

A G protein-coupled receptor complex that serves as a receptor for amylin polypeptide (AMY) and consists of a calcitonin receptor (CTR/CALCR) and a receptor activity-modifying protein (RAMP) 2. Amylin receptor complex 2 (AMY2) also serves as a receptor for adrenomedullin (AM/ADM).


Left Main Coronary Artery Body

The segment of the left main coronary artery that is bounded by its ostium and bifurcation.


Gastric melanoma

A melanoma that arises from mucosa of the stomach.


The (R)-enantiomer of bromazine.


epaxial region somite 12

The epaxial region of somite 12 derived body wall muscle. The epaxial region is dorsal to the horizontal myoseptum. Kimmel et al, 1995.


Deep Palmar Vein

The blood vessel carrying deoxygenated blood and accompanies the deep palmar artery in the hand.


straight-chain fatty acid

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


linolenic acid

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


octadecatrienoic acid

Any trienoic fatty acid having eighteen carbons.


fatty acid 20:3

Any trienoic fatty acid with twenty carbons.


The (5Z,7E,9E,14Z,17Z)-isomer of icosapentaenoic acid.


Head and Neck Disorder

A non-neoplastic or neoplastic disorder that affects the anatomic structures of the head and neck region.  This category includes inflammatory disorders, benign neoplasms, precancerous conditions, and malignant neoplasms.


Thyroid Gland Lipoadenoma

A thyroid gland adenoma composed of follicular structures and mature adipocytes.


Nasopharyngeal Paraganglioma

An extra-adrenal paraganglioma arising from the nasopharynx and nose.  Epistaxis or nasal obstruction are common presenting symptoms.


Follicular ameloblastoma

A histologic variant of solid/multicystic ameloblastoma characterized by the presence of odontogenic epithelial islands in a fibrotic stroma.


Recurrent Malignant Neoplasm of Salivary Gland

The reemergence of a malignant salivary gland neoplasm after a period of remission.


Malignant odontogenic tumor

A sarcoma that arises from tooth-forming tissues.


Salivary Gland Lipoma

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


Sellar Neurocytoma

A rare extraventricular neurocytoma (WHO grade 2) of the hypothalamic-pituitary area. (WHO 2017)


Squamous odontogenic tumor

A rare, locally invasive neoplasm arising from tooth-forming tissues. It usually grows intraosseously in the mandible. The maxilla is less frequently involved. It is characterized by the presence of well differentiated squamous cells which form islands. Recurrences may rarely occur and probably are related to incomplete excision of the tumor.


Parotid Gland Hemangioma

A hemangioma arising from the parotid gland.


Malignant Ameloblastoma

A rare, well differentiated, cytologically benign ameloblastoma which paradoxically metastasizes.


Sellar Choriocarcinoma

A choriocarcinoma that arises from or adjacent to the sellar region.


Oral Intraepithelial Neoplasia

A precancerous neoplastic process affecting the oral mucosa. It is characterized by the presence of dysplasia in the mucosal epithelium.


Thyroid Gland Follicular Tumor of Uncertain Malignant Potential

An encapsulated or well-circumscribed thyroid gland tumor composed of well-differentiated follicular cells with no nuclear features of papillary thyroid carcinoma and with questionable capsular or vascular invasion. This is a tumor indeterminate between follicular adenoma and follicular carcinoma. (WHO)


Non-Neoplastic Laryngeal Disorder

A non-neoplastic pathologic process that affects the larynx.  Representative examples include laryngitis, vocal cord nodule, and laryngeal cleft.


Advanced Malignant Head and Neck Neoplasm

A malignant neoplasm that arises from the head and neck region and has spread extensively to other anatomic sites or is no longer responding to treatment.


Sellar Yolk Sac Tumor

A yolk sac tumor that arises from or adjacent to the sellar region.


Head and Neck Precancerous Condition

A premalignant pathologic process that affects the anatomic structures of the head and neck.  Representative examples include oral leukoplakia and leukoplakia of the vocal cords.


Non-Neoplastic Oral Disorder

A non-neoplastic disorder that affects the oral cavity or the lips.


Polyp of nasal sinus

A polyp that arises from the paranasal sinus mucosa. Causes include allergic rhinitis, chronic sinusitis, and cystic fibrosis. It is characterized by the presence of edematous tissue infiltrated by inflammatory cells, including eosinophils.


Minor Salivary Gland Intraductal Papillary Neoplasm

A rare papillary neoplasm that grows within and is limited to the duct system of the minor salivary glands. This category includes papillary cystadenoma, ductal papilloma (including the intraductal and inverted variants), sialadenoma papilliferum, and intraductal papillary mucinous neoplasm.


Iodine-induced hyperthyroidism

Iodine-induced hyperthyroidism.


Low Risk Thyroid Gland Neoplasm

A thyroid gland neoplasm in which the incidence of metastatic spread is extremely low. This category includes thyroid gland noninvasive follicular neoplasm with papillary-like nuclear features, thyroid gland hyalinizing trabecular tumor, and thyroid gland tumors of uncertain malignant potential.


Sellar Paraganglioma

An extremely rare paraganglioma arising from chief cells of the dispersed paraganglia of the sellar region. (WHO)


Metastatic malignant neoplasm to orbit

A malignant neoplasm that has spread to the orbit from another anatomic site.


Congenital thyroid disorder

A thyroid gland disorder present at birth. It includes thyroid gland aplasia and thyroid gland hypoplasia, both result in hypothyroidism.


Sellar Immature Teratoma

An immature teratoma that arises from or adjacent to the sellar region.


Congenital hypothyroidism with ectopic thyroid

Thyroid hormone deficiency present at birth that is associated with ectopic thyroid tissue located in the neck region.


Non-Neoplastic Nasal Cavity Disorder

A non-neoplastic disorder that affects the nasal cavity.  Representative examples include rhinitis and Wegener granulomatosis.


Jugular Foramen Neoplasm

A neoplasm that affects the jugular foramen.  Representative examples include paraganglioma, schwannoma, and meningioma.


Salivary Gland Sebaceous Lymphadenoma

A rare, benign epithelial neoplasm composed of irregular proliferating nests and islands of epithelium, including solid and gland-like sebaceous elements, surrounded by lymphoid stroma. Most occur in the parotid gland. Symptoms include a progressively enlarging, painless mass.  Sebaceous lymphadenocarcinoma is the malignant counterpart.


Benign Sinonasal Neoplasm

A non-metastasizing neoplasm that arises from the nasal cavity and paranasal sinuses.


Hard Palate Monomorphic Adenoma

A benign epithelial neoplasm arising from the minor salivary glands in the hard palate. It is characterized by the presence of a monomorphic cellular infiltrate.


Malignant Parapharyngeal Neoplasm

A primary or metastatic malignant neoplasm that affects the parapharyngeal space.


Benign Inner Ear Neoplasm

A non-metastasizing neoplasm that arises from the inner ear. Representative examples include vestibular schwannoma and lipoma.


Non-Neoplastic Tonsillar Disorder

A non-neoplastic disorder that affects the tonsils.


Atypical Parathyroid Gland Tumor

A parathyroid gland tumor that shares certain morphologic characteristics with parathyroid gland carcinomas (e.g. broad fibrous bands formation with or without hemosiderin deposition, presence of mitotic figures) but lacks unequivocal evidence of capsular or vascular invasion. It is considered a tumor of uncertain malignant potential. (WHO)


Disorder of neck

A non-neoplastic or neoplastic disorder that affects the anatomic structures of the neck region.  This category includes disorders of the pharynx, larynx, thyroid gland, and parathyroid gland.


Metastatic Malignant Head and Neck Neoplasm

A malignant neoplasm that arises from the head and neck region and has spread to another anatomic site.


Odontogenic tumor, benign

A benign, slow growing neoplasm arising from tooth-forming tissues. It occurs in the maxillofacial skeleton or the gingiva. Representative examples include adenomatoid odontogenic tumor, calcifying cystic odontogenic tumor, and squamous odontogenic tumor.


Benign neoplasm of orbit

A non-metastasizing neoplasm that arises from the orbit.


Iatrogenic Central Hypothyroidism

Central hypothyroidism due to medical or surgical treatment.


Congenital hyperthyroidism

Hyperthyroidism, the cause of which is present at birth.


Sellar Meningioma

A rare meningioma that arises from the sellar region.


Rhinosinusitis

Inflammation of the mucous membranes lining the nose and paranasal sinuses.


Non-Neoplastic Thyroid Gland Disorder

A non-neoplastic disorder that affects the thyroid gland.  Representative examples include hyperthyroidism, hypothyroidism, thyroiditis, and thyroid gland abscess.


Parapharyngeal Neoplasm

A benign or malignant neoplasm that affects the parapharyngeal space.


Singers' nodes

A non-neoplastic growth in the vocal cord. It is characterized by tissue swelling in both vocal cords in a symmetrical manner. It is caused by vocal abuse behavior. Hoarseness is the presenting symptom.


Mucositis following radiation therapy

Inflammation of the mouth mucosa caused by head and neck radiation therapy.  Signs and symptoms include dry mouth due to lack of saliva, mucosal ulcerations and diffuse erythema of the mouth, taste alteration, and dysphagia.


Nasopharyngeal Non-Neoplastic Disorder

A non-neoplastic disorder that affects the nasopharynx.


Suprasellar germ cell tumor

A germ cell tumor that arises from the suprasellar region.


Ameloblastic Fibrosarcoma

A locally aggressive malignant neoplasm arising from odontogenic tissue. It occurs in the mandible and less often in the maxilla.  It is characterized by the presence of a malignant connective tissue component and a benign epithelial component.  The frequency of distal metastases is low.


Sellar Germinoma

A germinoma that arises from or adjacent to the sellar region.


Ceruminous Neoplasm

An adenoma or carcinoma that arises from the ceruminous glands in the external auditory canal.


Laryngeal Precancerous Condition

A premalignant pathologic process that affects the epithelial cells of the laryngeal mucosa.


Non-Neoplastic Neck Disorder

A non-neoplastic disorder that affects the anatomic structures of the neck region.  This category includes non-neoplastic disorders of the pharynx, larynx, thyroid gland, and parathyroid gland.


Ameloblastic fibroma

A rare neoplasm arising from tooth-forming tissues. It usually arises from the posterior mandible. It is characterized by the presence of an epithelial component and fibromyxoid stroma. It may recur and rarely shows malignant transformation to ameloblastic fibrosarcoma. It is treated with enucleation and curettage.


Nasal Cavity Exophytic Papilloma

A benign nasal cavity exophytic papillary neoplasm with fibrovascular cores lined by epithelial cells. It arises from the lower anterior nasal septum. Clinical manifestations include nasal obstruction, epistaxis, and the presence of a mass.


Leukoplakia of lips

A clinical term that indicates the presence of a white patch on the surface of the lip which cannot be characterized as any other disease.  It may be a precancerous condition and in most cases histologic examination reveals keratosis.


Parotid monomorphic adenoma

A benign epithelial neoplasm that arises from the parotid gland. It is characterized by the presence of a monomorphic cellular infiltrate.


Benign Anterior Tongue Neoplasm

A non-metastasizing neoplasm that arises from the anterior portion of the tongue.


Benign Ameloblastoma

An ameloblastoma that does not recur or metastasize.


Oral neoplasm malignant recurrent

Reemergence of a lip or oral cavity malignant neoplasm after a period of remission.


Nasopharyngeal Lymphoma

A lymphoma that arises from the nasopharynx.


Central Hyperthyroidism

Overproduction of thyroid hormones due to a disorder originating within the hypothalamic-pituitary axis.


Thyroglobulin Deficiency

Subnormal production of thyroglobulin, the glycoprotein precursor of thyroid hormones, presumed to result from loss-of-function mutation(s) in the TG gene.


Head and Neck Langerhans Cell Histiocytosis

Langerhans cell histiocytosis involving the head and neck.


sarcoma of oral cavity

A malignant soft tissue neoplasm that arises from the oral cavity.  Representative examples include Kaposi sarcoma and angiosarcoma.


Nasopharyngeal Hodgkin Lymphoma

A Hodgkin lymphoma that arises from the nasopharynx.


Smokers keratosis

A premalignant pathologic process that affects the oral mucosa.  It is associated with the use of smoked tobacco.  It appears as white lesions on the oral mucosa.  Morphologically it is characterized by the pathologic production of keratin in the mucosal epithelial surface with or without epithelial atypia.  It may reverse with the cessation of tobacco use.


Defective Thyroglobulin Transport

Impairment in the intracellular transport of thyroglobulin.


Paranasal sinus and nasal cavity malignant neoplasm

A primary or metastatic malignant neoplasm involving the nasal cavity and paranasal sinuses.


Oropharyngeal Hodgkin Lymphoma

A Hodgkin lymphoma that arises from the oropharynx.


Extraosseous/Peripheral Ameloblastoma

An ameloblastoma that arises from the soft tissues in the gingiva or alveolar mucosa.  It presents as a painless exophytic mass.


Head and Neck Histiocytic and Dendritic Cell Neoplasm

A histiocytic and dendritic cell neoplasm that affects the head and neck.


Sinonasal Seromucinous Hamartoma

A benign overgrowth of indigenous seromucinous glands of the nasal cavity and paranasal sinuses. (WHO 2017)


Locally Advanced Malignant Head and Neck Neoplasm

A malignant neoplasm that arises from the head and neck region and has spread from its original site of growth to nearby tissues or lymph nodes.


Thyroid Gland Clear Cell Follicular Adenoma

A thyroid gland adenoma composed of follicular cells with cytoplasmic clearing.


Parotid Gland Sialolipoma

A benign parotid gland neoplasm composed of mature adipose tissue and epithelial tissue.


Non-Neoplastic Parathyroid Gland Disorder

A non-neoplastic disorder that affects the parathyroid glands.  Representative examples include hyperparathyroidism and hypoparathyroidism.


Benign Parapharyngeal Neoplasm

A benign neoplasm that arises from the parapharyngeal space.


Sellar Mature Teratoma

A mature teratoma that arises from or adjacent to the sellar region.


Oropharyngeal Lymphoma

A lymphoma that arises from the oropharynx.


Hemangioma of salivary gland

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


Thyroid Gland Rosai-Dorfman Disease

Sinus histiocytosis with massive lymphadenopathy affecting the thyroid gland. It is rare and is usually accompanied by involvement of cervical lymph nodes.


Adenoma, Monomorphic

A benign epithelial neoplasm arising from the salivary glands. It is characterized by the presence of a monomorphic cellular infiltrate.


Non-Neoplastic Middle Ear Disorder

A non-neoplastic disorder that affects the middle ear.


Oral Cavity Monomorphic Adenoma

A benign epithelial neoplasm arising from the salivary glands in the oral cavity. It is characterized by the presence of a monomorphic cellular infiltrate.


Thyroid Gland Follicular Adenoma with Papillary Hyperplasia

A thyroid gland adenoma characterized by the presence of papillae lined by cells that range from cuboidal to columnar. The papillae are usually cystic and non-branching. Aggregates of follicles may be found within the papillary structures.


Oropharyngeal Polyp

A benign exophytic growth that arises from the oropharynx.


Dental surface disorders

A disorder that affects the surface of the tooth.


Abscess of thyroid

A rare disorder characterized by the development of an abscess in the thyroid gland.  It usually follows an upper respiratory tract, pharyngeal, or middle ear infection.  It is usually caused by Staphylococcus or Streptococcus infection.  It presents with fever, pain, and neck mass.


Salivary gland adenoma

A benign adenoma that arises from the salivary gland. This group includes pleomorphic adenoma, canalicular adenoma, basal cell adenoma, and sebaceous adenoma.


Orbital Melanoma

A melanoma that arises from the structures of the orbit.


Primordial Odontogenic Tumor

A benign odontogenic tumor composed of variably cellular loose fibrous tissue with areas similar to the dental papilla, entirely surrounded by cuboidal to columnar epithelium resembling the internal epithelium of the enamel organ. It occurs intraosseously, with a marked preference for the mandible. (WHO 2017)


Mouth Mucous Membrane Leukoplakia

A clinical term that indicates the presence of a white patch on the mucous membrane in the mouth which cannot be characterized as any other disease.  It may be a precancerous condition and in most cases histologic examination reveals keratosis.


Pharyngeal Melanoma

A melanoma that arises from the mucosal surface of the pharynx.


Benign Head and Neck Neoplasm

A non-metastasizing neoplasm that arises from the head and neck.


Salivary Gland Lymphadenomas

A rare benign salivary gland tumor that consists of a well-circumscribed biphasic proliferation of epithelial cells and reactive lymphoid tissue. Sebaceous and non-sebaceous forms can be distinguished. (WHO 2017)


Agenesis of larynx

A congenital deformity in which there is no laryngeal structure.


Wolff-Chaikoff Phenomenon

Hypothyroidism induced by excessive levels of iodine in the blood.


ductal papilloma of salivary gland

A benign papillary neoplasm that arises from the salivary glands.  This category includes intraductal papilloma, inverted ductal papilloma, and sialadenoma papilliferum.


Metastatic malignant neoplasm to lip

A malignant neoplasm that has spread to the lip from another anatomic site.


Posterior Tongue Neoplasm

A benign or malignant neoplasm that affects the base of the tongue.


Benign neoplasm of base of tongue

A non-metastasizing neoplasm that arises from the base of the tongue.


Ameloblastic fibrodentinoma

A rare neoplasm arising from tooth-forming tissues. It usually arises from the posterior mandible. It is characterized by the presence of an epithelial component, fibromyxoid stroma, and dentin formation. It is treated with enucleation and curettage. Recurrences may occur.


Basal Cell Ameloblastoma

A solid/multicystic ameloblastoma with follicular pattern in which the odontogenic epithelial islands are composed of uniform basaloid cells.


Non-Neoplastic Paranasal Sinus Disorder

A non-neoplastic disorder that affects the paranasal sinuses.  Representative examples include sinusitis and mucocele.


Suppurative parotitis

Parotitis that is caused by a bacterial agent.


Sinonasal Disorder

A non-neoplastic or neoplastic disorder that affects the nasal cavity or paranasal sinuses.  Representative examples include inflammatory disorders, papillomas, and carcinomas.


Benign Maxillofacial Neoplasm

A non-metastasizing neoplasm that arises in a maxillofacial bone.


Non-Neoplastic External Ear Disorder

A non-neoplastic disorder that affects the pinna and/or the ear canal.