Objective Endometriosis-associated abdominal wall cancer (EAAWC) is rare, and few reports are available. This article provides a review of reports in the literature on the pathology, diagnosis, management, and outcome of patients with EAAWC.

Method We performed a review of existing reports in the English language literature on cancer arising from abdominal wall endometriosis. MEDLINE and EMBASE searches were conducted for articles published from September 1986 to August 2014 using combinations of medical subject heading terms.

Results We identified 26 articles reporting on EAAWC and added 1 patient who was treated at our institution. In all of these patients, EAAWC was described after uterine surgery (mostly cesarean section). The delay between the first surgery and the diagnosis of malignant disease was more than 20 years. Clear cell carcinoma was the most common histology, followed by endometrioid carcinoma. Death was described in 44% of women within a few months of diagnosis.

Conclusions Endometriosis-associated abdominal wall cancer is rare and aggressive. It seems to be associated with cesarean section, and it shows poor prognosis. The mainstay of treatment remains extensive surgery and chemotherapy.