Endometrial stromal sarcoma arising from endometriosis of the terminal ileum: The role of immunohistochemistry in the differential diagnosis

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AI-generated summary by claude@2026-06, 2026-06-07

This case report details a rare instance of low-grade endometrial stromal sarcoma arising from ectopic endometriosis in the terminal ileum, highlighting the crucial role of immunohistochemistry in its diagnosis.

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Abstract

Low-grade endometrial stromal sarcoma (LESS) is an uncommon uterine malignancy. Occasionally, it may develop in extrauterine endometriotic lesions and present morphological characteristics mimicking various neoplasms, making its diagnosis very challenging. We report a rare case of a 56-year-old woman presenting with a pelvic mass, initially presumed to be of ovarian origin. After surgical excision the diagnosis of a LESS arising from foci of endometriosis of the terminal ileum was established. Pelvic lymph nodes and omentum were also infiltrated. The patient received adjuvant chemotherapy and medroxyprogesterone; she is alive with no evidence of disease after a follow-up of 38 months. Immunohistochemical characteristics of the tumor are very important for the differential diagnosis of this rare neoplasm and include diffuse strong positivity for CD 10, estrogen receptor expression and CD 34 negativity.

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Condition tags

mesh:D004715endometriosis

MeSH descriptors

Endometrial Neoplasms Endometriosis Ileal Diseases Sarcoma, Endometrial Stromal Antigens, CD34 Antigens, CD34 Diagnosis, Differential Endometrial Neoplasms Endometrial Neoplasms Endometrial Neoplasms Endometriosis Endometriosis Endometriosis Female Humans Ileal Diseases Ileal Diseases Ileal Diseases Immunohistochemistry Middle Aged

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europepmc
last seen: 2026-06-04T01:30:01.192114+00:00
openalex
last seen: 2026-06-04T00:00:01.174412+00:00
pubmed
last seen: 2026-05-13T22:16:17.081435+00:00
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