Low grade Endometrial Stromal Sarcoma of uterine corpus, a clinico-pathological and survey study in 14 cases

BACKGROUND Endometrial stromal sarcoma (ESS) is a rare disease with probably less than 700 new cases in the USA or Europe per year. The aim of this study was to evaluate the behavior of low-grade endometrial stromal sarcoma (LGESS) in relation to their clinical and pathological features and to identify possible prognostic factors. PATIENTS AND METHODS Fourteen patients with histologically proven ESS were included in the analysis. Endometrial stromal sarcoma is characterized by proliferations composed of cells with Endometrial stromal cell differentiation. Low-grade endometrial stromal sarcoma has an infiltrating margin and typically show extensive worm-like vessel invasion. RESULTS The median age was 44.35 +/- 6 years. The most common presenting symptom was vaginal bleeding, occurring in twelve patients (86%). Diagnosis was made through Fractional dilatation and curettage in four patients (28.5%). Eight patients had a total abdominal hysterectomy and salpingo-oophorectomy (57%). Radiotherapy as adjuvant therapy was administered to four patients (28.5%). The median follow-up time was 45.6 months (range 24-84). The median overall survival of the 14 patients was 45.35 +/- 21 months (range 20-83). Three of 14 patients demonstrated a recurrence of disease at 9, 72, and 96 months respectively. The recurrent diseases were treated with surgery, chemotherapy, and radiotherapy. No patient died of the disease. Clinico-pathological parameters did not significantly differ between patients with and without recurrence, but patients with no myometrial invasion and low mitotic count < or = 5/HPF showed longer disease-free survival. CONCLUSION Five-year survival rate was 93%. Survival probabilities were calculated by the product limit method of Kaplan and Meier that showed, patients with no myometrial invasion and low mitotic count < or = 5/HPF have longer disease-free survival, but P value was not significant.