BACKGROUND: Carcinoma arising from an endometriosis-associated abdominal wall scar is a rare entity, with only a few case reports published in the literature. The management is very controversial due to on its own rarity, and there are no specific guidelines. Treatment with a multidisciplinary team is important to achieve the best outcome.

CASE REPORT: We report the case of a 45-year-old woman diagnosed with a growing painless lesion in the right lower quadrant. We decided to perform Tru-Cut biopsy of the abdominal wall lesion, but unfortunately the pathological report was inconclusive at that time. Due to the presence of a highly suspicious lesion, the gynecologic oncologist together with the plastic surgeon and connective tissue surgeon decided to perform a wide resection of the abdominal wall along with hysterectomy and salpingo-oophorectomy. The final pathology report demonstrated endometriosis associated with an endometrioid adenocarcinoma grade II in the abdominal wall tumor. She was restaged with new imaging exams before the definition of the best adjuvant treatment, which showed suspicious bilateral inguinal and right axillary (1.9 cm) lymph nodes, with no other sites of metastatic disease. She was treated with megestrol acetate 160 mg/daily for 8 months, with a partial response.

CONCLUSIONS: Carcinoma arising from an endometriosis-associated abdominal wall scar is a rare entity, and there are no no specific treatment guidelines. Such patients must be assessed by a multidisciplinary team for decision making. Options for adjuvant and palliative treatment for endometrial cancer are generally used for the treatment of this entity. The main purpose of this article is to report this rare presentation and perform a review of the literature about diagnosis, clinical presentation, treatment, and prognosis.

Keywords: Antineoplastic Protocols, Genital Diseases, Female, Genital Neoplasms, Female, Gynecologic Surgical Procedures, Gynecological Examination, Gynecology, Abdominal Wall, Carcinoma, Endometrioid, Cicatrix, Endometrial Neoplasms, Endometriosis, Hysterectomy, Megestrol Acetate, Salpingo-oophorectomy