Mucinous Adenocarcinoma of a Sigmoid Vaginoplasty in a Patient with Mayer- Rokitansky-Kuster-Hauser Syndrome: A Second Case Report

Background: Cancers of the neovagina are a rare malignancy. The case of an adenocarcinoma arising in a sigmoid vaginoplasty, in a patient with Mayer-RokitanskyKüster-Hauser syndrom (MRKH), is presented. Case: In 2003 A 41-year-old female patient, suffering from MRKH, underwent a surgical vaginoplasty by laparotomy using a segment of the sigmoid colon. She was then able to have satisfactory sexual intercourse. Seven years after the surgery the patient consulted for vaginal bleeding. A multiple biopsy made it possible to identify a 55 by 40 mm liberkühnien adenocarcinoma on magnetic resonance imaging. An hepatic lesion and a superficial invasion of the first sacral vertebra were also suspected. The patient underwent neoadjuvant chemotherapy, radiochemotherapy and radiofrequency treatment on the hepatic lesion. Then a resection of the transplant, a left hemicolectomy, an ovariectomy, a cholecystectomy and a segment-V liver segmentectomy were performed. The tumor was classified as ypT2N0M0. 3 years later an isolated bone metastasis was diagnosed in the first sacral vertebra. After radiotherapy and chemotherapy the patient showed significant improvement. Conclusion: This is the 2nd case of adenocarcinoma arising from a sigmoid vaginoplasty. This reminds us of how important gynecological follow-up is on patients with MRKH, in particular patients who have a neovaginal construction. Every patient with chronic vaginal bleeding has to undergone an endoscopic exploration with potential biopsy.