WDHA syndrome caused by VIP-producing ganglioneuroblastoma

A 3 yr 11 mo-old girl showing classical symptoms of WDHA syndrome was transferred to our department of surgery. In preoperative examination, serum vasoactive intestinal peptide (VIP) was markedly elevated and pancreatic tumor was suspected. However, no tumor was found in the resected pancreas. Unfortunately, an unexpected adrenal tumor (ganglioneuroblastoma) was found in autopsy. The tumor was judged VIPO positive, immunohistochemically. This case was thought to be WDHA syndrome caused by VIPO-producing ganglioneuroblastoma (VIPoma).