8804/3 Epithelioid cell sarcoma


Related terms

Epithelioid sarcoma

Definitions

Connective, subcutaneous and other soft tissues
ICD-O-3 topography code: C49
ICD10: C49
           

An aggressive malignant neoplasm of uncertain lineage, characterized by the presence of epithelioid cells forming nodular patterns. The nodules often undergo central necrosis, resulting in a pseudogranulomatous growth pattern. It usually occurs in young adults. The most common sites of involvement are the extremities (distal-type epithelioid sarcoma), and less frequently the pelvis, perineum, and genital organs (proximal-type epithelioid sarcoma).



Vulva
ICD-O-3 topography code: C51
ICD10: C51
           

Epithelioid sarcoma is an aggressive malignant neoplasm of uncertain lineage, characterized by the presence of epithelioid cells forming nodular patterns. The tumour nodules often undergo central necrosis, resulting in a pseudogranulomatous growth pattern.
Epithelioid sarcoma usually occurs in young adults. The clinical course of epithelioid sarcomas arising in the vulva is marked by frequent recurrences and a high incidence of metastasis1
 
Tavassoli FA, Devilee P (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Breast and Female Genital Organs.
3rd Edition
IARC Press: Lyon 2003



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