Ataxia and secretory diarrhea: two unusual paraneoplastic syndromes occurring concurrently in the same patient with ganglioneuroblastoma

J Pediatr Hematol Oncol. 2004 Sep;26(9):549-52. doi: 10.1097/01.mph.0000139414.66455.a4.

Abstract

The presence of rare paraneoplastic syndromes, the opsoclonus-myoclonus-ataxia syndrome (OMA), presumably caused by antineuronal antibody production, and diarrhea, caused by vasoactive intestinal peptide (VIP) secreted by neuroblastoma, may strongly signal the presence of neuroblastoma. The authors describe a child who presented with both syndromes concurrently; this has never been described previously in the same patient. However, diagnosis of neuroblastoma was delayed by a workup focused on the prolonged diarrhea rather than the ataxia. The diarrhea resolved after tumor resection, whereas OMA required further therapy. Increased awareness of VIP-secretory diarrhea, especially in an ataxic child, might contribute to an earlier diagnosis of neuroblastoma.

Publication types

  • Case Reports

MeSH terms

  • Adrenal Gland Neoplasms / diagnosis*
  • Adrenal Gland Neoplasms / metabolism
  • Adrenal Gland Neoplasms / surgery
  • Ataxia / complications*
  • Ataxia / pathology
  • Ataxia / therapy
  • Diarrhea / complications*
  • Diarrhea / pathology
  • Diarrhea / therapy
  • Female
  • Ganglioneuroblastoma / diagnosis*
  • Ganglioneuroblastoma / metabolism
  • Ganglioneuroblastoma / surgery
  • Humans
  • Infant
  • Lymphatic Metastasis
  • Paraneoplastic Syndromes, Nervous System / pathology*
  • Paraneoplastic Syndromes, Nervous System / therapy
  • Vasoactive Intestinal Peptide / metabolism

Substances

  • Vasoactive Intestinal Peptide