PRIMARY EXTRAUTERINE ENDOMETRIAL STROMAL NEOPLASMS - A CLINICOPATHOLOGICAL STUDY OF 20 CASES AND A REVIEW OF THE LITERATURE

We present the results of a clinicopathologic study of 20 patients wit h primary extrauterine endometrial stromal sarcoma (ESS). The sites of the primary neoplasm and the number of patients with sufficient follo w-up for survival analysis are as follows: ovary (three of four), fall opian tube (one of one), pelvic cavity (six of eight), abdominal cavit y (five of six), and retroperitoneum (one of one). Evaluation of all p atients included the mitotic index (MI) and cytologic atypia. Thirteen of the sixteen patients eligible for survival analysis had tumors wit h an MI <10 and would be classified as low-grade stromal sarcomas in t he Norris and Taylor scheme. Eight (62%) of the 13 had one or more rel apses; of these, three died of disease at 35, 108, and 120 months, res pectively, and another patient was alive with disease at 96 months. Th e other four patients who were treated after a relapse showed no evide nce of disease after relapse at 36, 57, 63, and 146 months, respective ly. Two of the 13 patients had tumor considered unresectable at the ti me of diagnosis; both died of disease at 5 and 10 months, respectively . Neither MI nor cytologic atypia were predictive of tumor recurrence or death from tumor. We also extracted clinical and morphologic data f rom all previous reports of primary extrauterine ESS, combined them wi th our 20 patients, and then compared the combined group with 17 cases of primary high-stage uterine ESS we presented in an earlier report. Not surprisingly, the behavior of the primary extrauterine ESS was mor e reminiscent of high-stage primary uterine ESS than low-stage primary uterine ESS.