vasoactive-intestinal-peptide and Abdominal-Neoplasms

Vasoactive intestinal polypeptide (VIP) secreting neural crest tumours are an uncommon but important treatable cause of intractable childhood diarrhoea. The radiological appearances of two cases are presented with a review of radiological findings in childhood VIP secreting neural crest tumours. Twenty eight cases of childhood VIP secreting neural crest tumours were reviewed. Nineteen (68%) were ganglioneuroblastomas and nine (32%) were ganglioneuromas. The majority of tumours (66%) were in a paravertebral location in the abdomen indicating that a search for such a tumour should be initiated at this site. Eighteen of the twenty-eight cases reviewed discussed relevant radiological investigations. Calcification was detected in 50% of abdominal radiographs. Gut dilatation was often a prominent feature. A mass was detected in 5 of 5 cases where ultrasound findings were reported, and seven of seven cases with CT findings reported. Prior to the availability of CT and ultrasound the most useful investigation was IVU which demonstrated evidence of a mass in 5 of 9 cases. The presence of paravertebral calcification and gut dilatation on the plain radiograph of a child with intractable diarrhoea suggests the presence of a VIP secreting neural crest tumour. If an abdominal tumour is not found in the appropriate clinical setting and VIP levels are elevated, a widespread search of the paravertebral region is indicated.

Topics: Abdominal Neoplasms; Diagnostic Imaging; Female; Ganglioneuroma; Humans; Infant; Male; Neural Crest; Vasoactive Intestinal Peptide; Vipoma

Topics: Abdominal Neoplasms; Female; Humans; Infant; Neoplasm Proteins; Neuroblastoma; Steroids; Tomography, X-Ray Computed; Vasoactive Intestinal Peptide

A 2-year-old boy with failure to thrive, watery diarrhea, abdominal distention, hypokalemia, metabolic acidosis, and episodes of hypertension and sweating was found to have a calcified right lower quadrant mass. Blood levels of vasoactive intestinal peptide (VIP) and norepinephrine (NE) were elevated. Presurgical management with phenoxybenzamine hydrochloride and metyrosine was associated with an absence of expected postoperative hypotension, and resection of a benign ganglioneuroma resulted in prompt relief of all symptoms and return to normal of VIP and NE levels. Evidence supports the theory that VIP is the substance responsible for the diarrhea that accompanies some neural crest tumors.

Topics: Abdominal Neoplasms; Child; Child, Preschool; Female; Ganglioneuroma; Gastrointestinal Hormones; Humans; Hypertension; Infant; Male; Phenoxybenzamine; Tomography, X-Ray Computed; Tyrosine 3-Monooxygenase; Vasoactive Intestinal Peptide

An 8-month-old boy with persistent watery diarrhoea and failure to thrive developed abdominal distension, hypokalaemia, and flushing of the face and trunk. A high concentration of vasoactive intestinal peptide-like immunoreactivity was found in the serum. Soon after resection of a suprarenal mass, the serum level of vasoactive intestinal peptide became normal and the diarrhoea stopped. Histologically the tumour was a ganglioneuroblastoma: the cells showed fluorescence by the indirect immunofluorescence technique with anti-vasoactive intestinal peptide serum. Electron microscopical examination showed abundant secretory granules in the tumour cells. Reports of chronic watery diarrhoea in children due to neural crest tumours are reviewed, with particular respect to the clinical features of the syndrome.

Topics: Abdominal Neoplasms; Diarrhea, Infantile; Fluorescent Antibody Technique; Ganglioneuroma; Gastrointestinal Hormones; Humans; Infant; Male; Microscopy, Electron; Vasoactive Intestinal Peptide