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

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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.


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.


Stage I Adult Soft Tissue Sarcoma AJCC v7

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


Oral Cavity Lipoma

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


Paratesticular Deep (Aggressive) Angiomyxoma

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


Nuchal fibroma

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


Meningeal Central Nervous System Solitary Fibrous Tumor, Grade 3

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


Stage IV Adult Soft Tissue Sarcoma AJCC v7

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


Abdominal Inflammatory Myofibroblastic Tumor

Inflammatory myofibroblastic tumor that arises from the abdominal cavity.


Benign Laryngeal Soft Tissue Neoplasm

A 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.


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.


Undifferentiated Epithelioid Sarcoma

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


Stage IV Soft Tissue Sarcoma AJCC v7

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


Gastrointestinal Stromal Tumor by AJCC v8 Stage

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


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

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


Vulvar Angiomyofibroblastoma

An angiomyofibroblastoma arising from the vulva.


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


Vaginal Low Grade Endometrioid Stromal Sarcoma

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


Fallopian Tube Soft Tissue Neoplasm

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


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

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

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

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

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

biolink:PhenotypicFeature biolink:DiseaseOrPhenotypicFeature biolink:BiologicalEntity biolink:NamedThing biolink:Entity biolink:ThingWithTaxon

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 signaling 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 signaling 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 signaling 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 signaling 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.


Malignant Anterior Tongue Neoplasm

A primary or metastatic malignant neoplasm that affects the anterior portion of the tongue.


Benign neoplasm of palate

A non-metastasizing neoplasm that arises from the hard palate, soft palate, or uvula.


Nasopharyngeal hairy polyp

A benign polypoid lesion that arises from the nasopharynx. It usually affects neonates and older infants. It manifests as a pedunculated mass associated with cough, dyspnea, vomiting, or difficulty in swallowing. It is composed of ectoderm and mesoderm.


Recurrent Malignant Head and Neck Neoplasm

The reemergence of a malignant head and neck neoplasm after a period of remission.


Iatrogenic Primary Hypothyroidism

Primary hypothyroidism due to medical or surgical treatment.


Leukoplakia of buccal mucosa

A clinical term that indicates the presence of a white patch on the buccal mucosa 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.


Refractory Malignant Salivary Gland Neoplasm

A malignant salivary gland neoplasm that is resistant to treatment.


Stage IVA Mucosal Melanoma of the Head and Neck AJCC v7

Stage IVA includes: (T4a, N0, M0); (T3-T4a, N1, M0).  T4a: Moderately advanced disease.  Tumor involves deep soft tissue, cartilage, bone, or overlying skin.  T3: Mucosal disease.  N0: No regional lymph node metastases.  N1: Tumor with regional lymph node metastases.  M0: No distant metastasis. (AJCC 7th ed.)


Dentinogenic Ghost Cell Tumor

A benign but locally infiltrating neoplasm arising from tooth-forming tissues. It is more often intraosseous and less frequently extraosseous and occurs in the jaw. It is characterized by the presence of ameloblastoma-like epithelium, connective tissue stroma, ghost cells, and dysplastic dentin. Wide local resection is recommended for intraosseous neoplasms and enucleation for extraosseous neoplasms.


Unresectable Craniopharyngioma

A craniopharyngioma that is not amenable to surgical resection.


Non-Neoplastic Pituitary Gland Disorder

A non-neoplastic disorder that affects the pituitary gland.  Representative examples include pituitary gland hypoplasia and apoplexy.


Sinonasal Inverted Papilloma

A benign neoplasm that arises from the ciliated respiratory mucosa that lines the nasal cavity or paranasal sinuses.  It results from the invagination and proliferation of epithelial cells in the underlying stroma.  Clinical manifestations include nasal obstruction, epistaxis, and anosmia. It has the tendency to recur and extend to adjacent structures.  Inverted papillomas are occasionally associated with the development or presence of carcinomas, usually squamous cell carcinomas.


Diffuse C-Cell Hyperplasia

A non-neoplastic proliferation of morphologically bland C-cells with a diffuse growth pattern in the thyroid gland.


Malignant tumor of palate

A primary or metastatic malignant neoplasm that affects the hard palate, soft palate, or uvula.


Oral Mucosa Langerhans Cell Histiocytosis

Langerhans cell histiocytosis involving the oral mucosa.


Sinonasal Ameloblastoma

A locally aggressive, primarily gnathic (jaw) tumor with a high propensity for recurrence. It originates wholly within the sinonasal tract, without connection to gnathic sites, arising from sinonasal epithelium and showing histological features identical to those of its counterpart originating in the jaw. (WHO 2017)


Conventional ameloblastoma

An intraosseous ameloblastoma that arises from the jaw, usually the mandible.  It grows slowly, invades locally, and recurs frequently.  It presents with swelling of the jaw.


Head and Neck Melanocytic Neoplasm

A melanocytic neoplasm that arises from the skin or mucosal sites in the head and neck region.


Parotid Gland Sebaceous Lymphadenoma

A rare, benign epithelial neoplasm that arises from the parotid gland.  It is composed of irregular proliferating nests and islands of epithelium, including solid and gland-like sebaceous elements, surrounded by lymphoid stroma. Patients present with a progressively enlarging and painless mass.


Lingual Thyroid Gland

Ectopic thyroid gland located at the base of the tongue.


Hyperthyroidism with Hashimoto disease

Severe, transient hyperthyroidism associated with Hashimoto thyroiditis.


canalicular adenoma of salivary gland

A benign epithelial neoplasm primarily composed of branching and interconnecting cords of single and double-cell thick rows of columnar epithelium in a very loose stroma. It often occurs in the upper lip, where only pleomorphic adenoma is more frequent. Asymptomatic swelling of the upper lip is the most common clinical finding. It may present clinically and histologically as a multifocal lesion, a feature not generally seen with other intraoral salivary gland tumors.


Thyroid Gland Spindle Cell Follicular Adenoma

A rare thyroid gland follicular adenoma composed predominantly of spindle cells.


Middle Ear Paraganglioma

A jugulotympanic paraganglioma arising from paraganglia in the middle ear.


Thyroid Gland Schwannoma

A rare schwannoma that arises from the thyroid gland.


Dual Oxidase 2 Deficiency

Thyroid peroxidase system defect due to presumed mutation(s) in the DUOX2 gene, resulting in decreased activity of dual oxidase 2.


Transient hypothyroxinemia

Reduced concentration of thyroid hormone(s), usually associated with a non-thyroidal illness, that resolves spontaneously.


Refractory Malignant Pharyngeal Neoplasm

Malignant pharyngeal neoplasm that is resistant to treatment.


Non-Neoplastic Head and Neck Disorder

A non-neoplastic disorder that affects the anatomic structures of the head and neck region.


Laryngeal Mucositis

An inflammatory process affecting the mucous membrane of the larynx.


Benign neoplasm of bone of skull

A non-metastasizing neoplasm that arises from the bones and structures of the skull.


External Ear Actinic Keratosis

Actinic keratosis that develops in the skin of the external ear.


Non-Neoplastic Pharyngeal Disorder

A non-neoplastic disorder that affects the pharynx.  Representative examples include pharyngitis and mucositis.


Thyroid Gland Follicular Adenoma with Bizarre Nuclei

A thyroid gland adenoma containing follicular cells with large hyperchromatic nuclei.  The clinical course is benign.


Defective Thyroglobulin Synthesis

Impairment in the intracellular synthesis of thyroglobulin.


Parathyroid gland abscess

An abscess that is located in the parathyroid gland(s).


Hairy leukoplakia of tongue

An epithelial hyperplasia on the surface of the tongue associated with Epstein-Barr virus and found almost exclusively in persons with HIV infection. The lesion consists of a white patch that is often corrugated or hairy.


Pharyngeal Hodgkin Lymphoma

A Hodgkin lymphoma that arises from the pharynx.


Congenital thyroid hypoplasia

Incomplete development of the thyroid gland in a newborn.


Erythroleukoplakia

Leukoplakia of the oral mucosa that is clinically characterized by the presence of erythematous or red and white patches.


Thyroid Gland Paraganglioma

A rare, circumscribed or encapsulated neuroendocrine tumor arising from the thyroid gland. Microscopically, it is characterized by the presence of tumor cells arranged in a nesting (Zellballen) growth pattern. The reported cases have followed a benign clinical course.


Hodgkin's lymphoma of thyroid gland

An exceedingly rare Hodgkin lymphoma that arises from the thyroid gland.


Refractory Malignant Oral Neoplasm

Lip or oral cavity malignant neoplasm that is resistant to treatment.


Sinonasal Respiratory Epithelial Adenomatoid Hamartoma

A benign acquired overgrowth of indigenous glands of the sinonasal tract arising from the surface epithelium. (WHO 2017)


Non-Neoplastic Tongue Disorder

A non-neoplastic disorder that affects the tongue.


Speckled oral leukoplakia

Non-homogenous leukoplakia on the oral mucosa.  It is associated with a high risk for malignant transformation.


Leukoplakia of tongue

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


Benign neoplasm of uvula

A non-metastasizing neoplasm that arises from the uvula.


Thyroid Gland Histiocytic and Dendritic Cell Neoplasm

A rare histiocytic and dendritic cell neoplasm that affects the thyroid gland.


Non-Neoplastic Salivary Gland Disorder

A non-neoplastic disorder that affects the major or minor salivary glands.


Hodgkin's lymphoma of salivary gland

A very rare Hodgkin lymphoma that manifests initially in a salivary gland.


Minor Salivary Gland Intraductal Papillary-Mucinous Neoplasm

A rare, low-grade epithelial neoplasm with ductal differentiation that grows within and is limited to the duct system of the minor salivary glands. It is characterized by a proliferation of mucin-producing cells within the minor salivary gland ducts, intraductal accumulation of mucin, and a papillary growth pattern. Cytologic and architectural atypia and mitotic activity are present. AKT1 gene mutations have been identified in the majority of cases. Morphologically it resembles the intraductal papillary-mucinous neoplasms of the pancreas.


Calcifying Odontogenic Cyst

A benign, intraosseous or extraosseous cystic neoplasm arising from tooth-forming tissues. It is characterized by the presence of a cyst lined by an ameloblastoma-like epithelium and ghost cells formation. The ghost cells may undergo calcification. It is treated with enucleation. Few recurrences have been reported for intraosseous neoplasms whereas no recurrences have been reported for extraosseous neoplasms.


Sinonasal Lymphoma

A lymphoma that arises from the sinonasal tract.


Stage IVC Mucosal Melanoma of the Head and Neck AJCC v7

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


Head and Neck Nevus

A benign nevus that arises from the skin in the head and neck region.


Leukoplakia of larynx

A premalignant pathologic process that affects the mucosal epithelium of the larynx.  It appears as a localized or diffuse white patch on the laryngeal mucosa.  Morphologically it is characterized by the pathologic production of keratin in the mucosal epithelial surface with or without epithelial atypia.  It may progress to or co-exist with invasive squamous cell carcinoma.


Maxillofacial Osteoblastoma

A benign bone-forming neoplasm with prominent osteoblastic rimming arising in a maxillofacial bone.


Nasopharyngeal Salivary Gland Anlage Tumor

A rare midline nasopharyngeal lesion that affects infants. It presents with respiratory distress due to nasal airway obstruction. It is a biphasic lesion characterized by the presence of epithelial and myoepithelial components. The histological features suggest that this lesion may be a hamartoma, rather than a neoplasm.


Maxillofacial Neoplasm

A neoplasm that arises in a maxillofacial bone.


Sinonasal Exophytic Papilloma

A papilloma with an exophytic growth arising from the sinonasal track.


Central odontogenic fibroma

A rare, benign, intraosseous neoplasm arising from tooth-forming tissues in the mandible and maxilla. It is characterized by the presence of odontogenic epithelium which is embedded in a fibrous stroma. Local enucleation of the tumor is curative.


Suprasellar Germinoma

A germinoma that arises from the suprasellar region.


Sinonasal Neurofibroma

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


Orbit Paraganglioma

An extra-adrenal paraganglioma arising from the orbit.  Patients may present with visual disturbances or proptosis.


Maxillofacial Sarcoma

A sarcoma that arises in a maxillofacial bone.


Diffuse goiter

Enlargement of the entire thyroid gland without discrete mass(es).


Neonatal hearing impairment

An abnormality that affects hearing within the first month after birth. It may or may not result in hearing loss.


Thyroid Gland Tumor of Uncertain Malignant Potential

An encapsulated or well-circumscribed thyroid gland tumor composed of well-differentiated follicular cells, in which capsular or vascular invasion is indeterminate.


Lymphoepithelial Sialadenitis

A benign salivary gland lesion characterized by acinar atrophy, ductal hyperplasia, and epimyoepithelial islands in lymphoid stroma. (WHO 2017)


Nasopharyngeal Melanoma

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


Oral Cavity Adenoma

A monomorphic or pleomorphic adenoma that arises from the salivary glands in the oral cavity.


Soft Palate Benign Granular Cell Tumor

A rare benign neoplasm that arises from the soft palate.  It is characterized by the presence of plump eosinophilic cells with abundant granular cytoplasm.  The neoplastic cells extend into the surrounding tissues.  Recurrences are rare after removal of the tumor.


Thyroid Gland Mucinous Follicular Adenoma

A thyroid gland adenoma composed of follicles and characterized by the presence of abundant extracellular mucin.


Adenoma, Basal Cell

A benign epithelial neoplasm with a uniform, monomorphic appearance that is dominated by basal cells forming trabecular structures. It is rare, and occurs mostly on the parotid gland. The average age of patients has been reported to be 58 years. Swelling is the most constant clinical finding.


Iodine Deficiency Hypothyroidism

Hypothyroidism due to insufficient intake of iodine.


Thyroid Gland Signet Ring Cell Follicular Adenoma

A thyroid gland adenoma characterized by the presence of signet ring cells that stain positive for thyroglobulin.


Head and Neck Neuroendocrine Neoplasm

A neoplasm with neuroendocrine differentiation that arises from the head and neck. This category includes neuroendocrine tumors, neuroendocrine carcinomas, and paragangliomas.


Thyroid Gland Langerhans Cell Histiocytosis

Langerhans cell histiocytosis involving the thyroid gland focally or diffusely. It is exceedingly rare and usually occurs in patients with multifocal disease.


Pharyngeal Lymphoma

A lymphoma that arises from the pharynx.


Stage III Mucosal Melanoma of the Head and Neck AJCC v7

Stage III includes: T3, N0, M0.  T3: Mucosal disease N0: No regional lymph node metastases.  M0: No distant metastasis. (AJCC 7th ed.)


Thyroid Gland Follicular Nodular Disease

A benign, non-inflammatory, multifocal proliferation of thyroid follicular cells that results in the formation of multiple thyroid nodules. The clinical term multinodular goiter refers to the changes caused in the thyroid gland by follicular nodular disease.


neoplasm of anterior dorsal surface of tongue

A benign or malignant neoplasm that affects the anterior portion of the tongue.


Salivary Gland Infection

A viral (such as mumps) or bacterial infectious process affecting the salivary gland. The salivary gland becomes enlarged and is often painful.


Refractory Malignant Head and Neck Neoplasm

A malignant head and neck neoplasm that is resistant to treatment.


Disorder of tonsil (palatine tonsil)

A non-neoplastic or neoplastic disorder that affects the tonsils.


inverted ductal papilloma of salivary gland

A very rare benign tumor of the minor salivary glands, characterized by a luminal papillary proliferation that arises at the junction of the salivary gland duct and the oral mucosal surface epithelium and expands as a nodular mass into the lamina propria.  It presents as an asymptomatic mucosal swelling.


Unicystic ameloblastoma

An ameloblastoma that presents as a unilocular cyst.  It usually arises from the mandible.


Sellar ependymoma

A very rare, low-grade neoplasm that arises from the posterior pituitary. It is composed of epithelioid and oncocytic cells forming sheets and fascicles. It shows histopathological features reminiscent of ependymomas, including perivascular pseudorosettes and true rosettes. There is no evidence of necrosis or increased mitotic activity. Despite the presence of ependymal histopathological features, these neoplasms probably are not related to ependymomas. Their prognosis is unknown.


malignant neoplasm of skull

A primary or metastatic malignant neoplasm that affects the bones and structures of the skull.


Benign neoplasm of accessory sinus

A non-metastasizing neoplasm that arises from the paranasal sinuses.  Representative examples include Schneiderian papilloma and salivary gland-type adenoma.


Oral Cavity Myeloid Sarcoma

An extramedullary myeloid tumor that arises from the oral cavity. It occurs de novo or can precede, coincide with, or follow the presentation of acute myeloid leukemia, or can constitute blastic transformation of a myelodysplastic syndrome or myeloproliferative neoplasm. (WHO 2017)


Malignant Inner Ear Neoplasm

A malignant neoplasm that affects the inner ear.


Benign Skull Base Neoplasm

A non-metastasizing neoplasm that arises from the skull base.


Stage IVB Mucosal Melanoma of the Head and Neck AJCC v7

Stage IVB includes: T4b, Any N, M0.  T4b: Very advanced disease.  Tumor involves brain, dura, skull base, lower cranial nerves (IX, X, XI, XII), masticator space, carotid artery, prevertebral space, or mediastinal structures.  M0: No distant metastasis. (AJCC 7th ed.)


Metastatic Malignant Neoplasm in the Head and Neck

The spread of a malignant neoplasm to the head and neck.  This may be from a primary head and neck malignant neoplasm, or from a malignant neoplasm at a distant site.


Recurrent Sinonasal Inverted Papilloma

The reemergence of inverted Schneiderian papilloma after a period of remission.


Thyroid Gland Hyperfunctioning Adenoma

A thyroid gland adenoma producing thyroxin.  It is associated with hyperthyroidism.  Radioactive iodine scan reveals a hot nodule.


Adult Craniopharyngioma

A craniopharyngioma that occurs during adulthood.  It can be either adamantinomatous or papillary.  The papillary histologic variant occurs virtually exclusively in adults.  Clinical signs and symptoms include visual disturbances and endocrine deficiencies.


Acquired Hyperthyroidism

Hyperthyroidism, the cause of which is not present from birth.


Thyroid Gland Follicular Adenoma with Papillary Architecture

A benign, encapsulated thyroid gland neoplasm characterized by the presence of large follicles with intrafollicular papillary architecture. The cells lining the papillae are usually columnar. It is usually a cystic neoplasm and lacks nuclear atypia and capsular invasion. Psammoma bodies are not present. Activating TSHR mutations have been described in the majority of cases. In a small number of cases GNAS mutations have been identified.


Non-Neoplastic Inner Ear Disorder

A non-neoplastic disorder that affects the inner ear.  Representative examples include infection and sensory hearing loss.


Thyroid Gland Solitary Fibrous Tumor

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


Oral Squamous Cell Papilloma

A benign, exophytic and polypoid growth that arises from the lips or oral cavity. It is covered by hyperplastic epithelium. Some cases are caused by human papillomavirus infection.


Odontogenic myxoma

An intraosseous odontogenic neoplasm with good prognosis, arising from the mandible and less frequently from the maxilla. It is characterized by the presence of stellate cells, myxoid stroma formation, and prominent collagen. Small tumors may be cured with enucleation. Complete excision may be required for larger tumors.


Interarytenoid leukoplakia

A clinical term that indicates the presence of a white mucosal patch in the interarytenoid area.  In most cases histologic examination reveals keratosis.


Aneuploid Dysplastic Oral Leukoplakia

Oral leukoplakia associated with advanced dysplasia in which genetic studies show aneuploidy.  The vast majority of patients develop aggressive invasive squamous cell carcinomas.  Complete resection of the lesion does not reduce the risk of developing invasive squamous cell carcinoma.


Non-Neoplastic Oral Cavity Disorder

A non-neoplastic disorder that affects the oral cavity.  Representative examples include inflammation, fibroepithelial polyp, and cleft palate.


Acquired central hypothyroidism

Central hypothyroidism, the cause of which is not present at birth.


Recurrent Malignant Pharyngeal Neoplasm

Reemergence of a malignant pharyngeal neoplasm after a period of remission.


Sclerosing Polycystic Adenosis

A rare, benign, well-circumscribed salivary gland neoplasm that predominantly affects females.  It is characterized by the presence of microcysts, ducts, and acini in a sclerotic stroma.  Focal lymphocytic infiltrates may also be present.


Osteoma of skull

A benign bone-forming neoplasm arising from the skull.


Mucosal Melanoma of the Head and Neck

A melanoma that arises in the mucosa of the nasal cavity, paranasal sinuses, oral cavity, oropharynx, nasopharynx, larynx, and hypopharynx.


Fibro-odontoma, Ameloblastic

A rare benign neoplasm arising from tooth-forming tissues. It is characterized by the presence of a fibromyxoid stroma, epithelial component, dentin, and enamel. Recurrences are rare.


Non-toxic nodular goiter

Sporadic nodular enlargement of the thyroid gland that is not associated with changes in thyroid function or malignancy.


Non-Neoplastic Gingival Disorder

A non-neoplastic disorder that affects the gums.


Maxillofacial Osteoma

A benign, well-circumscribed bone-forming neoplasm arising in a maxillofacial bone.


Reactive C-Cell Hyperplasia

A non-neoplastic proliferation of C-cells in the thyroid gland caused by external stimuli such as hypercalcemia and chronic lymphocytic thyroiditis.


Transient Primary Hypothyroidism

Primary hypothyroidism that resolves spontaneously.


Sellar Teratoma

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


Acanthomatous ameloblastoma

A solid/multicystic ameloblastoma with follicular pattern in which the central cells in the odontogenic epithelial islands show squamous differentiation.


Tonsillopharyngitis

Inflammation of the palatine tonsils and the posterior pharynx (throat).


Leukoplakia of vocal cords

A clinical term that indicates the presence of a white patch on the mucosal surface of the vocal cords.  In most cases histologic examination reveals keratosis.


Intrasellar Meningioma

A rare meningioma arising from the intrasellar region.


Sinonasal Schwannoma

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


Ameloblastic Fibroodontosarcoma

An odontogenic sarcoma producing enamel/enameloid and dentin.


Oral Cavity and Lip Precancerous Condition

A premalignant pathologic process that affects the mucosa surface of the oral cavity or lip.  It includes leukoplakia, erythroplakia, and smoker's keratosis.


Oral Cavity Granular Cell Tumor

A rare neoplasm that arises from the oral cavity, usually the tongue.  It is characterized by the presence of plump eosinophilic cells with abundant granular cytoplasm.  The neoplastic cells extend into the surrounding tissues, usually skeletal muscle.  The vast majority of cases follow a benign clinical course.  Recurrences are rare after removal of the tumor.


Recurrent Malignant Thyroid Gland Neoplasm

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


Refractory Malignant Thyroid Gland Neoplasm

Malignant thyroid gland neoplasm that is resistant to treatment.


Thyroid Gland Black Follicular Adenoma

A thyroid gland follicular adenoma seen in patients treated with minocycline. The tumors have black discoloration visible on macroscopic examination and cytoplasmic accumulation of black pigment. (WHO 2017)


Head and Neck Follicular Dendritic Cell Sarcoma

A follicular dendritic cell sarcoma that arises from the head and neck. The most frequently affected sites are the cervical lymph nodes, the Waldeyer ring, and the soft tissue of the neck.


Sellar Schwannoma

An extremely rare schwannoma that arises from the sellar region.


Recurrent Craniopharyngioma

The reemergence of a craniopharyngioma after a period of remission.


Granular cell ameloblastoma

A solid/multicystic ameloblastoma with follicular pattern in which the odontogenic epithelial islands contain granular cells.


Mucosal Melanoma of the Head and Neck by AJCC v7 Stage

A term that refers to the staging of mucosal melanoma of the head and neck according to the American Joint Committee on Cancer, 7th edition. No prognostic stage grouping is proposed in the 8th edition.


Non-Neoplastic Sinonasal Disorder

A non-neoplastic disorder that affects the nasal cavity or paranasal sinuses.  Representative examples include rhinitis, sinusitis, and mucocele.


Hypothyroid Goiter

Goiter associated with reduced thyroid hormone secretion.


Lymphedema of the Head and Neck

Lymphedema that manifests in the head and neck.


Choanal polyp

A benign exophytic growth that arises from the nasopharynx.


Thyroid Gland Teratoma

A teratoma that arises from the thyroid gland. Thyroid gland teratomas with high grade/malignant histological features carry DICER1 gene mutations and have been reclassified as thyroblastomas. They are no longer considered true teratomas.


Nasal Cavity Papilloma

A papilloma that arises from the ciliated respiratory mucosa that lines the nasal cavity.


Oral Cavity Benign Granular Cell Tumor

A rare benign neoplasm that arises from the oral cavity, usually the tongue.  It is characterized by the presence of plump eosinophilic cells with abundant granular cytoplasm.  The neoplastic cells extend into the surrounding tissues, usually skeletal muscle.  Recurrences are rare after removal of the tumor.


Sellar Embryonal Carcinoma

An embryonal carcinoma that arises from or adjacent to the sellar region.


Maxillofacial Osteosarcoma

An osteosarcoma that arises in a maxillofacial bone.


Malignant Skull Base Neoplasm

A primary or metastatic malignant neoplasm that affects the skull base.


Oral melanocytic nevus

A melanocytic nevus that arises from the lip and/or oral cavity. The majority are benign. Dysplastic nevi that can lead to the development of melanoma can rarely arise from the lip and/or oral cavity.


Non-invasive follicular thyroid neoplasm with papillary like nuclear features

A non-invasive neoplasm of thyroid follicular cells with a follicular growth pattern and nuclear features of papillary thyroid carcinoma that has an extremely low malignant potential. These tumors were formerly classified as non-invasive encapsulated follicular variant of papillary thyroid carcinoma or well-differentiated tumor of uncertain malignant potential. (WHO)


Ameloblastic Fibrodentinosarcoma

An odontogenic sarcoma producing dentin/dentinoid.