Endometrial Stromal Sarcoma Arising in Colorectal Endometriosis: A Case Report and Review of the Literature

Abstract

Extrauterine endometrial stromal sarcoma (ESS) arising in endometriosis is extremely rare, particularly in the colorectum. It should always be included in the differential diagnosis of primary tumors originating from gastrointestinal tract in females, given that preoperative endoscopical biopsy may reveal no specific changes. We reported a case of ESS arising in colorectal endometriosis and reviewed the previous 7 cases reported in the English literature. Our patient, who was unavailable for tumor resection and refused further adjuvant therapy, played a role in representing the natural history of low-grade extragenital ESS. This case was the only death from ESS arising in colorectal endometriosis.

An unexpected diagnosis of primary omental endometrial stromal sarcoma in a patient with acute right abdominal pain: A case report and review of literature

“…Most cases reviewed had multifocal disease, with either local or distant dissemination, while only one death was reported for a patient with unresectable low grade EESS [6]. The clinical behaviour of EESS is similar to low grade uterine ESS.…”

“…Lesions in the gastrointestinal tract may involve the serosa (9 cases) or invade bowel wall to create polypoid lesions [14] , with resulting carcinoma-like obstructive symptoms (8 cases). Only four patients presented with disseminated disease [6] , [10] , [15] , [16] , while localised lesion or local spread comprised the majority of cases (9 and 7 cases respectively). Eight women had previous hysterectomy for benign pathologies, and four received unopposed hormonal therapy at the time of diagnosis, including the present case [7] , [12] , [17] .…”

“…The commonest complaint was abdominal pain (9 cases). Other complaints included hematochezia (4 cases) [5] , [6] , [7] , [8] , change in bowel habits (4 cases) [5] , [6] , [8] , [9] , and tenesmus (1 case) [9] . Only 2 cases, including the current one, presented acutely and were diagnosed following an emergent surgical intervention [10] .…”

“…Although EESS was previously believed to have worse prognosis than its uterine counterpart [21] , accumulating evidence, does not support this. Most cases reviewed had multifocal disease, with either local or distant dissemination, while only one death was reported for a patient with unresectable low grade EESS [6] . The clinical behaviour of EESS is similar to low grade uterine ESS.…”

Endometrial Stromal Sarcoma Arising from Endometriosis

“…In 1925, Sampson (11) first described and suggested three criteria for diagnosis of malignancy arising from endometriosis: (i) demonstration of a clear example of benign endometriosis lesions in close proximity to the tumor, (ii) no other primary site identified for the tumor, and (iii) histologic significant cellular atypia and prominent nucleoli in a histology compatible with an endometrial primary (11, 12).…”