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©The Author(s) 2017. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastrointest Pathophysiol. Aug 15, 2017; 8(3): 142-149
Published online Aug 15, 2017. doi: 10.4291/wjgp.v8.i3.142
Published online Aug 15, 2017. doi: 10.4291/wjgp.v8.i3.142
Multiple endocrine neoplasia 2B: Differential increase in enteric nerve subgroups in muscle and mucosa
John M Hutson, Pam J Farmer, Cristal J Peck, Bridget R Southwell, Surgical Research/Gastrointestinal Motility Laboratory, Murdoch Children’s Research Institute, Parkville, Victoria 3052, Australia
John M Hutson, Chung W Chow, Bridget R Southwell, Departments of Urology and Anatomical Pathology, The Royal Children’s Hospital, Parkville, Victoria 3052, Australia
John M Hutson, Bridget R Southwell, Department of Paediatrics, University of Melbourne, Melbourne 3010, Australia
Author contributions: Hutson JM surgeon managing patient and wrote manuscript; Farmer PJ and Peck CJ performed histology and immunohistochemistry and generated images; Chow CW provided pathological diagnosis; Southwell BR supervised study and revised manuscript.
Institutional review board statement: The study was performed under ethics approval to do further research on samples collected for diagnositc pathology. Royal Children’s Hospital Ethics Committee approval number 23081B and 24105.
Informed consent statement: The patient and his family gave written consent for the surgery where biopsies were collected for pathological assessment and verbal consent for the samples to be used in this study.
Conflict-of-interest statement: None of the authors has a conflict of interest as the surgery was performed for clinical indications and with informed consent and the histologic analysis was done with commercial reagents.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Correspondence to: Dr. Bridget R Southwell, Surgical Research/Gastrointestinal Motility Laboratory, Murdoch Childrens Research Institute, Royal Children’s Hospital, Flemington Road, Parkville, Victoria 3052, Australia. bridget.southwell@mcri.edu.au
Telephone: +1-61-393455069 Fax: +1-61-393457997
Received: November 17, 2016
Peer-review started: November 22, 2016
First decision: February 30, 2017
Revised: May 24, 2017
Accepted: July 14, 2017
Article in press: July 17, 2017
Published online: August 15, 2017
Processing time: 269 Days and 18.4 Hours
Peer-review started: November 22, 2016
First decision: February 30, 2017
Revised: May 24, 2017
Accepted: July 14, 2017
Article in press: July 17, 2017
Published online: August 15, 2017
Processing time: 269 Days and 18.4 Hours
Core Tip
Core tip: Multiple endocrine neoplasia (MEN) 2B is a rare anomaly caused by an activating mutation of the RET known to produce enteric gangliomatosis. Thyroidectomy is performed to avoid cancer and the colon removed to overcome the constipation. This child presented with constipation very early (1-mo-old) and had subtotal colectomy at 4 years. The classes of nerves affected in MEN2B colon have not been studied before. We used immunohistochemistry with multiple antibodies. There was a massive increase in intrinsic nerve fibres in myenteric and submucosal ganglia, with a differential increase in types of nerve fibres in the muscle and mucosa.