Clinical, histological and therapeutical aspects in the management of uterine and extrauterine stromal sarcomas: Case reports

Endometrial stromal sarcoma (ESS) is a rare tumor, predominantly occurring as a primary tumor of the uterus. Rare cases of primary extrauterine ESS (EESS) have been reported. Low-grade ESS (LG-ESS) is more common than high-grade ESS (HG-ESS). We present five cases of ESS and one case of EESS. All cases received external radiotherapy (EBRT) at the Radiotherapy Department of the Emergency Clinical Hospital ‘Sfantul Apostol Andrei’ Galati, during 2004-2020. Five cases underwent EBRT in two-dimensional (2D) technique and only one patient received EBRT with three-dimensinal conformational radiotherapy (3DCRT) technique with a linear accelerator, Elekta Synergy. Five patients were referred to postoperative radiotherapy after hysterectomy. The median age of the patients was 57.4 years. One patient was referred to radiotherapy with palliative intent. EESS localized in the retroperitoneum, in the para-aortic region, was identified in one 64-year-old patient with a personal history of hysterectomy and bilateral salpingo-oophorectomy in 1997; EESS was complicated with vertebral extension at the L1-L2 level and spinal cord compression syndrome. ESS represents a rare diagnosis and a high- or low-grade tumor profile is distinguished by immunohistochemistry (IHC) tests. Up to 30% of patients have EESS at presentation. The treatment of ESS is multimodal, its management requiring a multidisciplinary team, and it is different according to the primary tumor location and tumor staging. The role of adjuvant radiotherapy remains controversial in high-grade EESS and due to the rarity of these cases there are limited data concerning the efficacy of adjuvant EBRT available from prospective randomized control clinical trials.