Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Book

Epithelioid Sarcoma

In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025 Jan.
.
Affiliations
Free Books & Documents
Book

Epithelioid Sarcoma

Todd Needs et al.
Free Books & Documents

Excerpt

Epithelioid sarcoma is a rare and aggressive soft tissue sarcoma characterized by both epithelial and mesenchymal differentiation. This condition most commonly affects young adults and typically presents as a painless and slow-growing mass often located in the distal extremities. Epithelioid sarcoma is locally invasive and frequently metastasizes to regional lymph nodes and distant sites. Diagnosis is determined by tissue biopsy, while imaging studies are essential for assessing the extent of the primary tumor and identifying metastatic disease.

Complete surgical resection is curative in early-stage disease. However, local recurrence and late distant metastases may occur, necessitating long-term surveillance. Radiation therapy may be used as an adjunct treatment and as a palliative measure. Recently, treatment options have evolved with the development of targeted therapies, which aim to improve patient outcomes.

PubMed Disclaimer

Conflict of interest statement

Disclosure: Todd Needs declares no relevant financial relationships with ineligible companies.

Disclosure: Eric Fillman declares no relevant financial relationships with ineligible companies.

References

    1. Enzinger FM. Epitheloid sarcoma. A sarcoma simulating a granuloma or a carcinoma. Cancer. 1970 Nov;26(5):1029-41. - PubMed
    1. de Visscher SA, van Ginkel RJ, Wobbes T, Veth RP, Ten Heuvel SE, Suurmeijer AJ, Hoekstra HJ. Epithelioid sarcoma: Still an only surgically curable disease. Cancer. 2006 Aug 01;107(3):606-12. - PubMed
    1. Hollmann TJ, Hornick JL. INI1-deficient tumors: diagnostic features and molecular genetics. Am J Surg Pathol. 2011 Oct;35(10):e47-63. - PubMed
    1. Modena P, Lualdi E, Facchinetti F, Galli L, Teixeira MR, Pilotti S, Sozzi G. SMARCB1/INI1 tumor suppressor gene is frequently inactivated in epithelioid sarcomas. Cancer Res. 2005 May 15;65(10):4012-9. - PubMed
    1. Fisher C. Epithelioid sarcoma of Enzinger. Adv Anat Pathol. 2006 May;13(3):114-21. - PubMed

Publication types

LinkOut - more resources