Neuroendocrine tumor in the vaginal reconstruction of a patient with Mayer-Rokitansky-Küster-Hauser syndrome and anorectal malformation

• Main Authors: Nicholas Fioravante (Author), Tara Tronetti (Author), Eric Strauch (Author), Roger Voigt (Author), Kimberly Lumpkins (Author)
• Format: Book
• Published: Elsevier, 2021-03-01T00:00:00Z.
• Subjects: article
• Online Access: Connect to this object online.
• Notes: 2213-5766; 10.1016/j.epsc.2021.101787
• Keywords: Mayer-Rokitansky-Küster-Hauser; Anorectal malformation; Neuroendocrine tumor; Vaginal agenesis; Pediatrics; RJ1-570; Surgery; RD1-811
• Source: Journal of Pediatric Surgery Case Reports, Vol 66, Iss , Pp 101787- (2021)

Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome occurring in association with an anorectal malformation (ARM) is rare but well described (Wester et al., 2012) [1]. In some patients with MRKH and ARM, a rectovestibular fistula may be left in situ as a neovagina [2,3]. Previously, adenocarcinomas arising in vaginal reconstructions have been reported, however neuroendocrine tumors (NET) have not been previously described in this setting [4,5]. We describe the case of a patient with MRKH associated with ARM that developed a neuroendocrine tumor of the neovagina constructed in infancy from a rectovestibular fistula. She was immunosuppressed following a living related kidney transplant at age 11. At age 20, vaginal bleeding was noted. A 1.5 cm neuroendocrine tumor was found on the anterior vaginal wall and she underwent a complete full-thickness transvaginal resection. The management considerations for neuroendocrine tumors of the neovagina are discussed.