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

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Abstract
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-ophorectomy (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 <= 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 <= 5/HPF have longer disease-free survival, but P value was not significant.