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

Abstract

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.

Clitoral leiomyoma in a premenopausal woman: a case report

“…Extrauterine leiomyomata localized in the external genitalia is an uncommon lesion arising from smooth muscle cells around vascular epithelium or erectile tissue [1]. The most common extrauterine sites of the disease are the upper digestive tract or the lower respiratory tract, but if the female genital tract is involved, the most frequent localization is the clitoris [2].…”

Endometrial stromal nodule of the vaginal wall with a review of vulvovaginal endometrial stromal neoplasms

“…In contrast, all cases of ESNs reported in the vulvovaginal region ( Table 1 ) have corresponded to endometrial stromal sarcomas of various grades of differentiation. In at least five cases ( Androulaki et al, 2007 , Berkowitz et al, 1978 , Corpa et al, 2004 , Kondi-Paphitis et al, 1998 , Liu et al, 2013 , Masand et al, 2013 ) the tumors appeared to be primary neoplasms, while the remaining ones were likely to be metastatic in nature ( Irvin et al, 1998 , Kondi-Paphitis et al, 1998 , Liu et al, 2013 , Masand et al, 2013 ), since tumor was also found in pelvic locations, colon and lung. Association with endometriosis, locally or elsewhere, was found in 6 cases.…”

“…This is the reason why we made the revision of the cases reported on this localization. We made searches with “vulvovaginal endometrial stromal tumor/sarcoma/nodule”, “vaginal endometrial stromal tumor/sarcoma/nodule” and “vulvar endometrial stromal tumor/sarcoma/nodule” and we only found ten reported cases of sarcomas in the vagina and two in the vulva ( Androulaki et al, 2007 , Berkowitz et al, 1978 , Corpa et al, 2004 , Irvin et al, 1998 , Kondi-Paphitis et al, 1998 , Liu et al, 2013 , Masand et al, 2013 ), half of them being metastases from other sites. Bibliographical analysis demonstrated various types of endometrial stromal sarcoma, often associated to endometriosis, but no cases of stromal nodules like the one we are submitting.…”

Endometrial Stromal Sarcoma Development after Hysterectomy and Tamoxifen Therapy

“…14 Low-grade ESS demonstrates slow growth and an indolent course with a tendency for late recurrence. The 5-year survival rates for low-grade ESS ranges from 67 per cent to nearly 100 per cent, 17 but approximately one-half of patients will develop recurrent disease. [11][12][13] Because there are a limited number of cases of extrauterine ESS, its optimal treatment has not been clearly defined.…”