Case Report
Copyright ©The Author(s) 2015. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Jul 7, 2015; 21(25): 7929-7932
Published online Jul 7, 2015. doi: 10.3748/wjg.v21.i25.7929
Refractory diarrhea: A paraneoplastic syndrome of neuroblastoma
Wei Han, Huan-Min Wang
Wei Han, Huan-Min Wang, Oncology Department, Beijing Children’s Hospital, Capital Medical University, Beijing 100045, China
Author contributions: Han W analyzed the data and wrote the paper; Wang HM designed the research and performed the operations.
Ethics approval: The study was reviewed and approved by the Beijing Children’s Hospital Institutional Review Board.
Informed consent statement: All study participants, or their legal guardian, provided informed written consent prior to study enrollment.
Conflict-of-interest statement: All authors have no conflicts of interest to disclose.
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: Huan-Min Wang, MD, Oncology Department, Beijing Children’s Hospital, Capital Medical University, No. 56 Nan Lishi Road, Xicheng District, Beijing 100045, China. hamiwang@aliyun.com
Telephone: +86-10-59616413 Fax: +86-10-59718700
Received: January 8, 2015
Peer-review started: January 12, 2015
First decision: March 10, 2015
Revised: April 9, 2015
Accepted: May 21, 2015
Article in press: May 21, 2015
Published online: July 7, 2015
Processing time: 181 Days and 3.2 Hours
Abstract

Neuroblastoma (NB) is the most common extracranial solid tumor in children. Diarrheal NB is quite rare and is not easy to diagnose in the early stage. Six cases of diarrheal NB in our hospital treated from 1996 to 2006 were retrospectively analyzed, including characteristics such as electrolyte imbalance, pathologic features, vasoactive intestinal peptide (VIP) immunohistochemical staining results, treatment, and prognosis. All patients were boys with 3-8 loose or watery stools each day and routine fecal tests were normal. Abdominal tumors were identified by B-ultrasound. Drugs were ineffective. Three patients underwent surgery, and the remaining three patients received surgery and chemotherapy. Diarrhea stopped after treatment in five patients. Two patients died due to intractable hypokalemia. The tumor was located in the adrenal gland in four patients, in the upper retroperitoneum in one patient, and in the presacral area in one patient. Pathologic findings were NB and ganglioneuroblastoma. Five patients were at clinical stage I-II, and one was at stage III. Four patients survived (followed-up for 6 mo to 4 years). Immunohistochemical staining for VIP was positive. Refractory diarrhea is a paraneoplastic syndrome of NB and is rare. Patients aged 1-3 years who present with chronic intractable diarrhea should be followed closely. Intractable diarrhea, hypokalemia, and dysplasia are the initial clinical manifestations. Increased VIP is characteristic of this disease. Potassium supplementation plays a vital role in the treatment procedure, especially preoperatively. The prognosis of diarrheal NB is good following appropriate treatment.

Keywords: Diarrhea; Hypokalemia; Neuroblastoma; Paraneoplastic syndrome; Vasoactive intestinal peptide

Core tip: Neuroblastoma (NB) is the most common extracranial solid tumor in children. Diarrheal NB is quite rare and is not easy to diagnose in the early stage. Six cases of diarrheal NB were retrospectively analyzed, including characteristics such as electrolyte imbalance, pathologic features, vasoactive intestinal peptide immunohistochemical staining results, treatment, and prognosis.