Neuroendocrine tumor in the vaginal reconstruction of a patient with Mayer-Rokitansky-Küster-Hauser syndrome and anorectal malformation
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 arisi...
| Main Authors: | , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Elsevier
2021-03-01
|
| Series: | Journal of Pediatric Surgery Case Reports |
| Subjects: | |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S2213576621000099 |
| _version_ | 1818330015791579136 |
|---|---|
| author | Nicholas Fioravante Tara Tronetti Eric Strauch Roger Voigt Kimberly Lumpkins |
| author_facet | Nicholas Fioravante Tara Tronetti Eric Strauch Roger Voigt Kimberly Lumpkins |
| author_sort | Nicholas Fioravante |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-12-13T12:57:14Z |
| format | Article |
| id | doaj.art-b0c255944f1c4f7faa719c49cd91cfe0 |
| institution | Directory Open Access Journal |
| issn | 2213-5766 |
| language | English |
| last_indexed | 2024-12-13T12:57:14Z |
| publishDate | 2021-03-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Journal of Pediatric Surgery Case Reports |
| spelling | doaj.art-b0c255944f1c4f7faa719c49cd91cfe02022-12-21T23:45:08ZengElsevierJournal of Pediatric Surgery Case Reports2213-57662021-03-0166101787Neuroendocrine tumor in the vaginal reconstruction of a patient with Mayer-Rokitansky-Küster-Hauser syndrome and anorectal malformationNicholas Fioravante0Tara Tronetti1Eric Strauch2Roger Voigt3Kimberly Lumpkins4University of Maryland School of Medicine, Department of Surgery, 29 S Greene St Ste 110, Baltimore, MD, 21201, United StatesUniversity of Maryland School of Medicine, Department of Surgery, 29 S Greene St Ste 110, Baltimore, MD, 21201, United StatesUniversity of Maryland School of Medicine, Department of Surgery, 29 S Greene St Ste 110, Baltimore, MD, 21201, United StatesUniversity of Maryland School of Medicine, Department of Surgery, 29 S Greene St Ste 110, Baltimore, MD, 21201, United StatesCorresponding author. 29 S. Greene St, Ste 110, Baltimore, MD, 21201, United States.; University of Maryland School of Medicine, Department of Surgery, 29 S Greene St Ste 110, Baltimore, MD, 21201, United StatesMayer-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.http://www.sciencedirect.com/science/article/pii/S2213576621000099Mayer-Rokitansky-Küster-HauserAnorectal malformationNeuroendocrine tumorVaginal agenesis |
| spellingShingle | Nicholas Fioravante Tara Tronetti Eric Strauch Roger Voigt Kimberly Lumpkins Neuroendocrine tumor in the vaginal reconstruction of a patient with Mayer-Rokitansky-Küster-Hauser syndrome and anorectal malformation Journal of Pediatric Surgery Case Reports Mayer-Rokitansky-Küster-Hauser Anorectal malformation Neuroendocrine tumor Vaginal agenesis |
| title | Neuroendocrine tumor in the vaginal reconstruction of a patient with Mayer-Rokitansky-Küster-Hauser syndrome and anorectal malformation |
| title_full | Neuroendocrine tumor in the vaginal reconstruction of a patient with Mayer-Rokitansky-Küster-Hauser syndrome and anorectal malformation |
| title_fullStr | Neuroendocrine tumor in the vaginal reconstruction of a patient with Mayer-Rokitansky-Küster-Hauser syndrome and anorectal malformation |
| title_full_unstemmed | Neuroendocrine tumor in the vaginal reconstruction of a patient with Mayer-Rokitansky-Küster-Hauser syndrome and anorectal malformation |
| title_short | Neuroendocrine tumor in the vaginal reconstruction of a patient with Mayer-Rokitansky-Küster-Hauser syndrome and anorectal malformation |
| title_sort | neuroendocrine tumor in the vaginal reconstruction of a patient with mayer rokitansky kuster hauser syndrome and anorectal malformation |
| topic | Mayer-Rokitansky-Küster-Hauser Anorectal malformation Neuroendocrine tumor Vaginal agenesis |
| url | http://www.sciencedirect.com/science/article/pii/S2213576621000099 |
| work_keys_str_mv | AT nicholasfioravante neuroendocrinetumorinthevaginalreconstructionofapatientwithmayerrokitanskykusterhausersyndromeandanorectalmalformation AT taratronetti neuroendocrinetumorinthevaginalreconstructionofapatientwithmayerrokitanskykusterhausersyndromeandanorectalmalformation AT ericstrauch neuroendocrinetumorinthevaginalreconstructionofapatientwithmayerrokitanskykusterhausersyndromeandanorectalmalformation AT rogervoigt neuroendocrinetumorinthevaginalreconstructionofapatientwithmayerrokitanskykusterhausersyndromeandanorectalmalformation AT kimberlylumpkins neuroendocrinetumorinthevaginalreconstructionofapatientwithmayerrokitanskykusterhausersyndromeandanorectalmalformation |