Low-grade stromal sarcoma: a benign appearing malignant uterine tumour; a review of current literature. Differential diagnostic problems illustrated by four cases.

Clinicopathological features of four cases of low-grade stromal sarcoma (LGSS) are presented. Established diagnostic criteria have been followed. Differential diagnoses are discussed with emphasis on the difficulties in separating LGSS from the very common benign uterine leiomyomas. The finding of infiltrating cells, morphologically similar to the cells of the proliferating endometrium, has been decisive for our diagnoses. Prognosis and treatment are discussed on the basis of a compilation of data collected from 31 clinicopathological studies. Of 91 patients with initial disease confined to the uterus, 37 (41%) developed recurrence within 10 years. In contrast to most other uterine sarcomas LGSS is amenable to surgery. We recommend total abdominal hysterectomy with bilateral salpingo-oophorectomy in cases of initial disease confined to the uterus, and radical or palliative surgery in cases of extra-uterine involvement. Long-term follow-up with surgical intervention in cases of recurrence or metastases is advocated.