Endometrial stromal sarcoma arising from endometriosis


The malignant transformation of endometriosis is a very uncommon event but can occur in 0.7% to 1% of all cases. Any histological type of tumor found in the endometrium might also occur in endometriosis. Most malignant tumors that originate from endometriosis are endometrioid adenocarcinomas and also clear-cell type carcinomas. On the other hand, sarcomas, especially endometrial stromal sarcoma (ESS), are extremely unusual representing 12% of all cases. ESS is an uncommon neoplasm and accounts for 0.2% of the uterine malignances. Malignant tumors arising from endometriosis can derive from the uterine wall as well as from extra-uterine sites. The most frequent extrauterine location is the ovary (78.7%), followed by the pelvic peritoneum (5.7%), the rectovaginal septum (4.3%), the colon (4.3%) and the vagina (2%), representing the majority of extragonadal sites.

ESSs arising from the extrauterine and extraovarian endometriosis sites in the absence of a primary uterine lesion are extremely rare and the treatment options are not clear. Surgical debulking seems to be the best treatment. Adjuvant therapy, such as radiation, hormonal therapy and chemotherapy are not yet proven to be effective. Molecular target therapy could be a future possibility of treatment.

A systematic review of English Medical Literature about incidence, treatment and prognosis of extrauterine ESS arising from endometriosis foci was performed. The selected articles on which this review is based are the following: 9 literature reviews, 8 retrospective studies, 7 case series, 1 prospective trial and 11 case reports.

J Endometr Pelvic Pain Disord 2017; 9(3): 174 - 179

Article Type: REVIEW



Francesco Cosentino, Luigi C. Turco, Gabriella Ferrandina, Anna Fagotti, Salvatore Gueli Alletti, Stefano Cianci, Giuseppe Vizzielli, Virginia Vargiu, Giovanni Scambia

Article History


Financial support: No grants or funding have been received for this study.
Conflict of interest: None of the authors has financial interest related to this study to disclose.

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  •  Foundation of Research and Care John Paul II, Division of Gynecologic Oncology, Catholic University of Sacred Heart, Campobasso - Italy
  •  Foundation Polyclinic A. Gemelli, Division of Gynecologic Oncology, Catholic University of Sacred Heart, Rome - Italy

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