NCI Definition: An aggressive malignant neoplasm of uncertain differentiation, 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). [1]

Epithelioid sarcomas most frequently harbor alterations in SMARCB1 [2].

Most Commonly Altered Genes in Epithelioid Sarcoma

SMARCB1 Loss are the most common alterations in epithelioid sarcoma [2].

Top Alterations in Epithelioid Sarcoma

Significant Genes in Epithelioid Sarcoma

EZH2 +



SS18 +

SSX1 +

Disease Details

ES, Epithelioid Cell Sarcoma
Soft Tissue Sarcoma
Childhood Epithelioid Sarcoma, Adult Epithelioid Sarcoma, Distal-Type Epithelioid Sarcoma, and Proximal-Type Epithelioid Sarcoma
OncoTree Name
Epithelioid Sarcoma
OncoTree Code


1. National Cancer Institute. NCI Thesaurus Version 18.11d. https://ncit.nci.nih.gov/ncitbrowser/ [2018-08-28]. [2018-09-21].

2. The AACR Project GENIE Consortium. AACR Project GENIE: powering precision medicine through an international consortium. Cancer Discovery. 2017;7(8):818-831. Dataset Version 6. This dataset does not represent the totality of the genetic landscape; see paper for more information.

3. All assertions and clinical trial landscape data are curated from primary sources. You can read more about the curation process here.