vasoactive-intestinal-peptide and Ganglioneuroblastoma

Topics: Colonic Neoplasms; Ganglioneuroblastoma; Humans; Infant; Male; Vasoactive Intestinal Peptide

In this case report we describe a case of mediastinal ganglioneuroblastoma-secreting vasoactive intestinal peptide (VIP), causing secretory diarrhoea in an 18-month-old child. An 18-month-old girl presented with a 2-month history of diarrhoea, abdominal distension and weight loss. Investigations revealed secretory diarrhoea with hypokalaemia, hyponatraemia and hypochloraemia and metabolic acidosis. Her stool output was 2.5-3.lday(-1) with increased stool sodium. VIP levels were strikingly high with normal glucagon and gastrin levels. X-ray of the chest revealed a well-defined mass in the right upper zone with tracheal shift, which was confirmed with computed tomography (CT) of the chest. The mass was resected and the patient became asymptomatic. This case shows that secretory diarrhoea caused by VIP and produced by ganglioneuroblastoma indicates a favourable prognosis, provided it is resectable.

Topics: Diarrhea, Infantile; Female; Ganglioneuroblastoma; Humans; Infant; Mediastinal Neoplasms; Radiography; Vasoactive Intestinal Peptide

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.

Topics: Adrenal Gland Neoplasms; Ataxia; Diarrhea; Female; Ganglioneuroblastoma; Humans; Infant; Lymphatic Metastasis; Paraneoplastic Syndromes, Nervous System; Vasoactive Intestinal Peptide

Topics: Adrenal Gland Neoplasms; Ataxia; Diarrhea; Ganglioneuroblastoma; Humans; Paraneoplastic Syndromes, Nervous System; Vasoactive Intestinal Peptide

A paraneoplastic syndrome is occasionally the first clinical symptom seen with tumours. We report on two children who initially presented with paraneoplastic syndromes due to ganglioneuroblastomas: the first with severe watery diarrhoea caused by a ganglioneuroma producing vasoactive intestinal peptide, the second with non-treatable constipation, caused by ganglioneuroma-produced anti-neuronal nuclear antibodies.. Either severe diarrhoea or chronic constipation may represent rare paraneoplastic syndromes in ganglioneuroblastomas.

Topics: Adolescent; Adrenal Gland Neoplasms; Chronic Disease; Constipation; Fatal Outcome; Female; Ganglioneuroblastoma; Humans; Infant; Paraneoplastic Syndromes; Vasoactive Intestinal Peptide; Vomiting

Topics: Calcitonin; Child, Preschool; Female; Ganglia, Autonomic; Ganglioneuroblastoma; Humans; Microscopy, Immunoelectron; Vasoactive Intestinal Peptide

Topics: Adrenal Gland Neoplasms; Adrenal Glands; Diarrhea, Infantile; Female; Ganglioneuroblastoma; Humans; Infant; Vasoactive Intestinal Peptide

Topics: Adrenal Gland Neoplasms; Child, Preschool; Diarrhea; Female; Ganglioneuroblastoma; Humans; Magnetic Resonance Imaging; Vasoactive Intestinal Peptide

Topics: Cell Differentiation; Cell Division; Child; Child, Preschool; Female; Ganglioneuroblastoma; Humans; Immunoenzyme Techniques; Infant; Male; Neuroblastoma; Radioligand Assay; Receptors, Vasoactive Intestinal Peptide; Survival Analysis; Vasoactive Intestinal Peptide