LATE DIAGNOSIS OF GANGLIONEUROBLASTOMA IN AN INFANT

摘要

The neuroendocrine masses that synthesize vasoactive intestinal peptide (VIP) are named VIPomas.Watery diarrhea, hypokalemia, achlorhydria syndrome (WDHA) occurs frequently in adults with pancreatic tumors. WDHA syndrome in children is mainly induces by VIPomas localized in mediastinum or retroperitoneum. Chronic diarrhea in infants is a common condition for addressability to pediatric gastroenterologists. The causes are multiple and the delay in reaching the final diagnosis can lead to fatal complications. The authors present an infant with recurrent watery diarrhea, subocclusion manifestations, hyperchloremic metabolic acidosis and hypokalemia triggered by a retroperitoneal VIPoma that was diagnosed by abdominal ultrasound and tomography. Laboratory investigations indicated an elevated VIP serum level. Tumor excision restored the normal stool consistency, corrected the imbalance of serum electrolytes and normalized VIP level. The diagnosis of N-MYC negative ganglioneuroblastoma was confirmed by immune-histochemical assessment. This paper describes the clinical and histo-genetic aspects of this rare clinical condition.