Metastatic low-grade endometrial stromal sarcoma of clitoris: report of a case

• Published in: International journal of gynecological cancer Vol. 17; no. 1; pp. 290 - 293
• Main Authors: Androulaki, A, Papathomas, T G, Alexandrou, P, Lazaris, A C
• Format: Journal Article
• Language: English
• Published: England BMJ Publishing Group LTD 01.01.2007
• Subjects: Clitoris; Clitoris - pathology; Endometrial Neoplasms - pathology; Female; Fibroids; Humans; Middle Aged; Sarcoma; Sarcoma, Endometrial Stromal - pathology; Sarcoma, Endometrial Stromal - secondary; Tumors; Vulvar Neoplasms - secondary
• ISSN: 1048-891X; 1525-1438
• DOI: 10.1111/j.1525-1438.2006.00744.x

Low-grade endometrial stromal sarcoma (ESS) is an uncommon neoplasm, which has a highly recurrent nature. A review of the literature revealed that only one case of low-grade ESS, arising within the vulva from a focus of endometriosis, has been previously published. We describe an additional case of low-grade ESS arising within the vulva and to the best of our knowledge the first report of low-grade ESS metastasized to clitoris. A 46-year-old woman was admitted to our hospital due to a heavy uterine bleeding. A physical examination revealed a lesion in clitoris, which exhibited a densely cellular mesenchymal neoplasm on microscopy. On the basis of the pathologic features alone, a differential diagnosis of a low-grade ESS and cellular leiomyoma was considered. Seven months later, the patient presented again with excessive uterine bleeding and a total hysterectomy was performed. A tumor of white-tan, whorled appearance was found. Its features were suggestive of low-grade ESS. Taking into account the possible extrauterine location of an ESS and reviewing the first case, a diagnosis of rare low-grade ESS metastasized to clitoris was made.