Low-grade endometrial stromal sarcoma of the endocervix. Report of a case and review of the literature.

Cervical sarcomas are rare entities comprising 0.5% of all primary cervical malignancies. Endometrial stromal sarcoma (ESS) is the least common (< 10%) type of uterine sarcoma. It has traditionally been divided into two categories: low-grade stromal sarcoma (LGSS), which constitutes 50-60% of all ESS, and high-grade stromal sarcoma (HGSS). Low-grade ESS may arise in extrauterine locations, classically described as arising in foci of endometriosis. In our case, a 44-year-old woman presented with a 4-week history of abnormal vaginal secretions and occasional bleeding. Physical examination revealed a soft, hemorrhagic mass on the posterior cervix, approximately 7 x 4 x 3 cm, looking like a degenerated myoma. The patient underwent a radical hysterectomy with bilateral salpingo-oophorectomy and bilateral pelvic lymphadenectomy. Histopathological findings, including immunohistochemical study, led to the diagnosis of a LGSS of the endocervix, which was in close relation with endometriotic foci. Our case represents an extrauterine low-grade ESS arising in the endocervix, where a problem in the differential diagnosis was encountered and which was finally treated only with surgery. Adjuvant treatment of ESS is controversial. Generally, low-grade ESS is associated with good prognosis and long overall and disease-free survival.