Low-grade endometrial stromal sarcoma recurring over three decades

Endometrial stromal sarcoma (ESS) is an uncommon uterine malignancy with a variety of histologic characteristics and clinical courses. We describe a patient who recently underwent her third resection of a locally recurring low-grade ESS 29 years after original diagnosis. Tissue from her recurrent tumor contained high levels of estrogen and progesterone receptors and had a diploid DNA content. In addition to multiple resections, she has previously been treated with vincristine chemotherapy and is now receiving megestrol acetate therapy. Mitotic activity is the most important prognostic feature distinguishing high- and low-grade ESS. The mainstay of therapy for both high- and low-grade tumors is surgical excision. A beneficial adjuvant role for cytotoxic chemotherapy or radiotherapy is yet to be clearly established for low-grade ESS; however, recent reports suggest that progestational agents may produce significant responses in recurrent or persistent disease patients whose tumors produce steroid hormone receptors. Indolent tumors may require years of close observation and multiple treatment approaches to maintain a patient in a functional capacity.