Ataxia and secretory diarrhea: two unusual paraneoplastic syndromes occurring concurrently in the same patient with ganglioneuroblastoma.
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.
Gesundheit
Benjamin
B
Unit of Hematology and Oncology, Soroka Hospital, Ben Gurion University, Beer Sheva, Israel.
Smith
Charles R
CR
Gerstle
J Ted
JT
Weitzman
Sheila S
SS
Chan
Helen S L
HS
D000310:Adrenal Gland Neoplasms / Q000175:diagnosis* / Q000378:metabolism / Q000601:surgery; D001259:Ataxia / Q000150:complications* / Q000473:pathology / Q000628:therapy; D003967:Diarrhea / Q000150:complications* / Q000473:pathology / Q000628:therapy; D005260:Female; D018305:Ganglioneuroblastoma / Q000175:diagnosis* / Q000378:metabolism / Q000601:surgery; D006801:Humans; D007223:Infant; D008207:Lymphatic Metastasis; D020361:Paraneoplastic Syndromes, Nervous System / Q000473:pathology* / Q000628:therapy; D014660:Vasoactive Intestinal Peptide / Q000378:metabolism
D014660:Vasoactive Intestinal Peptide