Background: Endometrial stromal sarcoma (ESS) is a malignant tumour with its origin in the endometrial stroma. Little is known about the pathogenesis, risk factors, optimal therapy or outcome of this disease.
Patients and methods: Eleven patients with ESS, treated between 1972 and 1996, are reported on. The hospital records of all the patients, including pathology and operative reports, were reviewed and information on treatment, recurrence and survival was obtained.
Results: The mean age of our patients was 56.6 years. The main symptom was abnormal vaginal bleeding. Most patients were diagnosed at FIGO stage I. Treatment modalities were surgery, radiation and, in one patient, chemotherapy. The median follow-up time was 42.1 months; 27.3% of the patients had local recurrence. The 1-year, 2-year and 5-year survival rates were 36.3%, 18.1% and 9.1%, respectively.
Conclusion: ESS is a uterine sarcoma with a difficult differential diagnosis. Patients are frequently diagnosed in an early tumour stage but still experience local or distant recurrence. The prognosis is poor, with early recurrence and low long-time survival rates. The treatment includes surgery and adjuvant radiation, with endocrine therapy being a promising new approach. In order to obtain more information about the pathogenesis of the tumour and to find the optimal therapy, it is necessary that studies, even with small numbers of patients, are undertaken.