Association of BRAF V600E mutations with vasoactive intestinal peptide syndrome in MYCN-amplified neuroblastoma. Academic Article uri icon

Overview

abstract

  • Very rarely, vasoactive intestinal peptide-related diarrhea (VIP-D) is observed in patients with high-risk neuroblastoma (HR-NB) where the associated fluid and electrolyte abnormalities can pose a major clinical challenge for administering the required aggressive multimodality treatment. Two patients with HR-NB developed VIP-D during induction and were found to have a somatic BRAF V600E mutation. Serum VIP levels and diarrhea promptly resolved in both patients after initiating treatment with BRAF and MEK inhibitors. This illustrates an association of VIP-D with BRAF V600E mutations and demonstrates a therapeutic strategy in the specific context of VIP-D and BRAF V600E mutations in HR-NB patients. The addition of BRAF and MEK inhibitors allows continued conventional tumor-directed treatment by decreasing the severity of symptoms caused by this life-threatening complication.

authors

  • Shahid, Sanam
  • Kushner, Brian H
  • Modak, Shakeel
  • Basu, Ellen M
  • Rubin, Elyssa M
  • Gundem, Gunes
  • Papaemmanuil, Elli
  • Roberts, Stephen S

publication date

  • July 31, 2021

Research

keywords

  • Diarrhea
  • N-Myc Proto-Oncogene Protein
  • Neuroblastoma
  • Proto-Oncogene Proteins B-raf
  • Vasoactive Intestinal Peptide

Identity

PubMed Central ID

  • PMC9527949

Scopus Document Identifier

  • 85111970072

Digital Object Identifier (DOI)

  • 10.1002/pbc.29265

PubMed ID

  • 34331515

Additional Document Info

volume

  • 68

issue

  • 10