Fifty-two cases of the rare entity, uterine endolymphatic stromal myosis, were evaluated in a collaborative study by gynecologic oncologists trained at the M. D. Anderson Tumor Institute. Fifty percent developed recurrent disease subsequent to initial surgical therapy. Of the surgical stage I cases, 47% developed pelvic recurrences, and 9% developed distant recurrences only after initial therapy. The five- and ten-year life-table survival of surgical stages I to IV were 88% for stage I, 66% for stage II, 100% for stage III, and 75% for stage IV. These long-term survival rates were due in part to the hormonal sensitivity of the recurrent tumors. Because of the high recurrence rate after initial therapy, a suggested plan of therapy is presented for patients with uterine endolymphatic stromal myosis.