Endometrial stromal sarcoma: treatment and patterns of recurrence

Our objective was to investigate recurrence patterns and conduct an outcome analysis of patients with endometrial stromal sarcoma (ESS).A retrospective review yielded 30 patients with ESS (20 low-grade, 10 high-grade) who underwent primary surgical resection from 1982 to 2005. Median follow-up was 42 months.All patients underwent hysterectomy, whereas pelvic (P) and paraaortic (PA) lymphadenectomy were performed in 12 and 7 patients, respectively. A median of 26 pelvic lymph nodes and 9 PA lymph nodes were removed. Pelvic or PA lymphatic disease was noted in 25% and 29% of patients, respectively. Extrauterine disease was identified in 11 (45%) of 24 patients undergoing exploratory laparotomy; 6 had no residual disease after cytoreductive surgery. None of these 6 patients experienced abdominal failure, but 1 had a hematological recurrence. Thrombotic complications were noted in 13% of patients. The 5-year overall survival was 65%. Overall survival was influenced by grade (79% vs 40%, P = 0.03) and extrauterine disease (77% vs 32%, P = 0.01). No patient who underwent a systematic lymphadenectomy had a lymphatic recurrence, irrespective of nodal status. There were 7 (23%) hematological recurrences; 2 in surgical stage I ESS. Two patients with low-grade ESS remain without evidence of disease 130 and 210 months after secondary cytoreduction. In contrast, no patient with high-grade ESS survived a recurrence.There is high prevalence of extrauterine and nodal disease in ESS. Patients are at high risk for thrombotic complications. If aggressively staged, the predominant failure risk for stage I patients is hematogenous, suggesting the need for improved systemic treatments.