Ganglioneuroblastoma secretor de péptido intestinal vasoactivo (VIP): a propósito de un caso

The case study involves a 22-month-old child with a 4-month course of chronic diarrhea, stagnant weight, bloated abdomen and hypokalemia. After a wide range of tests, he was diagnosed with a medium-risk right paravertebral ganglioneuroblastoma. Chemotherapy treatment was started, despite frequent hospital admissions because of moderate dehydration due to persistent diarrhea associated with hypokalemia and severe metabolic acidosis. A test for vasoactive intestinal peptide levels in serum was requested, which came out very high, confirming the suspected diagnosis of a ganglioneuroblastoma that was producing VIP. In the end, the patient had surgery, which involved the curative resection of the tumour and led to the resolution of the symptoms. The untreatable chronic diarrhea could be paraneoplastic syndrome from the ganglioneuroblastoma. In situations such as these, the measuring of VIP in serum could be crucial for diagnosis. Tumours that secrete VIP, or vipomas, are very rare in children, as they are most frequently tumours that form on the sympathetic nervous system (ganglioneuroma y ganglioneuroblastoma). They appear principally in children under 2 years old and normally have a favourable prognosis whenever they are removable. It is important to take this diagnostic possibility into account when assessing children with untreatable chronic diarrhea. Se presenta el caso de un niño de 22 meses con diarrea crónica de 4 meses de evolución, estancamiento ponderal, abdomen distendido e hipopotasemia. Tras la realización de pruebas complementarias se le diagnostica de ganglioneuroblastoma paravertebral derecho de riesgo intermedio. Se inicia tratamiento quimioterápico, a pesar del cual persisten los ingresos frecuentes por deshidrataciones moderadas debidas a diarreas persistentes asociadas a hipopotasemias y acidosis metabólicas severas. Se solicitan niveles de péptido intestinal vasoactivo (VIP) en suero, que aparecen muy elevados, lo que confirma el diagnóstico de ganglioneuroblastoma productor de VIP. Por último, se interviene quirúrgicamente al paciente, siendo la resección tumoral curativa y consiguiendo la resolución de los síntomas. La diarrea crónica intratable puede ser un síndrome paraneoplásico del ganglioneuroblastoma. En estas situaciones, las mediciones de VIP en suero pueden ser cruciales para el diagnóstico. Los tumores secretores de VIP o vipomas son muy raros en la infancia, y los tumores del sistema nervioso simpático (ganglioneuroma y gangli