Association of BRAF V600E mutations with vasoactive intestinal peptide syndrome in MYCN-amplified neuroblastoma Journal Article

   

Authors:	Shahid, S.; Kushner, B. H.; Modak, S.; Basu, E. M.; Rubin, E. M.; Gundem, G.; Papaemmanuil, E.; Roberts, S. S.
Article Title:	Association of BRAF V600E mutations with vasoactive intestinal peptide syndrome in MYCN-amplified neuroblastoma
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. © 2021 Wiley Periodicals LLC
Keywords:	paraneoplastic syndrome; braf v600e mutation; vasoactive intestinal peptide; mycn-amplified neuroblastoma
Journal Title:	Pediatric Blood and Cancer
Volume:	68
Issue:	10
ISSN:	1545-5009
Publisher:	Wiley Periodicals, Inc
Date Published:	2021-10-01
Start Page:	e29265
Language:	English
DOI:	10.1002/pbc.29265
PUBMED:	34331515
PROVIDER:	scopus
PMCID:	PMC9527949
DOI/URL:	https://www.scopus.com/inward/record.uri?eid=2-s2.0-85111970072&doi=10.1002%2fpbc.29265&partnerID=40&md5=5affa1dd950b24cc9c7ce9a2710c5b4f
Notes:	Article -- Export Date: 1 September 2021 -- Source: Scopus

https://synapse.mskcc.org/synapse/works/has_related_data_chk/186067