Colonic Adenocarcinoma of a Neovagina in a Patient with Mullerian AgenesisCourtney N Ruegner1*, Melanie Benton2, Nathan Dunsmore2, George Perry3 and Rudy J Castellani1
- *Corresponding Author:
- Courtney N. Ruegner
Department of Pathology, University of Maryland
Baltimore, 620 W. Lexington Street, Baltimore, MD 21201, USA
E-mail: [email protected]
Received date: June 25, 2014; Accepted date: July 29, 2014; Published date: July 31, 2014
Citation: Ruegner CN, Benton M, Dunsmore N, Perry G, Castellani RJ (2014) Colonic Adenocarcinoma of a Neovagina in a Patient with Mullerian Agenesis. Reprod Syst Sex Disord 3:138. doi:10.4172/2161-038X.1000138
Copyright: © 2014 Ruegner CN, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome, also known as Mullerian agenesis, is a largely sporadic disorder resulting in absence or hypoplasia of the vagina, uterus, and fallopian tubes. In order to foster adequate psychosocial development and sexual intercourse, a number of surgical procedures are available, one of which is the creation of a neovagina from a segment of sigmoid colon. In this report, we describe a patient who presented at age 17 amenorrhea and was found to have MRKH. She underwent vaginal reconstruction with a neovagina from a segment of her sigmoid colon at age 19. Subsequent to this procedure, the patient developed carcinoma of the breast and renal cell carcinoma, and in addition developed an invasive mucinous adenocarcinoma of her neovagina. This is only the third case of an adenocarcinoma arising in a neovagina in the setting of MRKH, and the first case described with an accompanying adenomatous component. All three cases to date demonstrated a mucinous phenotype. Overall, this case emphasizes that neovaginal mucosa may undergo neoplastic transformation, and that recognition of this possibility is important in long-term follow up care for patients affected MRKH treated by surgical reconstruction.