Tumor with watery diarrhoea, hypokalaemia in a 3-year-old girl

Abstract

Watery diarrhoea, hypokalaemia and achlorhydria (WDHA) syndrome was caused by vasoactive intestinal polypeptide (VIP)-producing tumour. A 3-year-old Chinese girl with watery diarrhoea, abdominal distension and hypokalaemia due to a thoracic paraspinal VIP-secreting ganglioneuroma is reported. The girl coughed, fevering up to 39 degrees C after a flu-like episode. She had eight to ten abundant stools daily which is not improved by dietary treatment, resulting in an important weight loss. She weighed 6.8 kg (nl P50 at 6 months of age) and is 76 cm (nl P50 at 9 months of age) in height. Blood electrolytes showed 129 mmol/L sodium, 2.42 mmol/L potassium, 94 mmol/L chloride and 18.6 mmol/L bicarbonate; urinary catecholamines were normal. Computed tomography scan evidenced a left side paravertebral mass of 4 x 6 cm in the lower thoracic region leading to the blood determination of vasoactive intestinal polypeptide which amounted 830 pmol/L(normal < 25 pmol/L). Surgical removal showed a ganglioneuroma of 160 g and was associated with disappearance of the diarrhoea and normalization of VIP level below 20 pmol/L. Review of the 63 reported cases in children with WDHA showed that many of the cases presented with non-treatable watery diarrhoea, hypokalaemia. Achlorhydria is not necessarily part of the WDHA syndrome. The male to female ratio is 1:1.5. Ganglioneuroblastoma and ganglioneuroma are the commonest tumours. Location of the tumour is variable: abdomen, chest or neck. Abdominal distension, flushing, episodic hypertension and colonic dilatation, constipation and ataxia were the other associated features. Surgical resection is the treatment of choice of VIP-producing tumours.

Eosinophilic Esophagitis as Paraneoplastic Syndrome in a Patient with Ganglioneuroblastoma

“…But total restitution of the esophagus and normal weight gain after tumour extirpation suggests a causal relationship between tumour and described symptoms. One other patient with neuroblastoma described in literature presented with 2 concurrent paraneoplasias: an opsoclonusmyoclonus-ataxia-syndrome and a VIP caused diarrhoea [16]. We conclude, that a diagnosis eosinophilic esophagitis in children, especially in combination with diarrhoea, should implicate the exclusion of a neuroblastoma, although it has not been proven that the esophageal disease is of paraneoplastic nature.…”

Case Report: Mediastinal Ganglioneuroma Presented as Chronic Diarrhea in a Young Saudi Girl

“…A syndrome of watery diarrhoea, hypokalaemia and achlorhydria (WDHA) is caused by vasoactive intestinal polypeptide (VIP)-producing tumors. In 2009 Zhang et al reviewed 63 reported cases in children with WDHA syndrome and showed that many of the cases presented with non-treatable watery diarrhea, hypokalemia [14]. Achlorhydria is not necessarily part of the WDHA syndrome.…”

“…Among the 63 tumors, 35 were ganglioneuroblastoma (55.6%), 20 were ganglioneuroma (31.7%), five neuroblastoma (7.9%), one was neurofibroma (1.6%) and one was neurofibromatosis (1.6%). Eleven of the total cases were in the thorax [14]. Harris et al reported one more case of mediastinal GN (4.3 cm by 2.8 cm by 2.8 cm) with WDHA syndrome and high serum level of VIP in an 18.5 months old girl [15].…”

Ganglioneuroma coincidente con una extrusión discal lumbar

“…Si producen síntomas, estos dependerán de la ubicación y del tipo de hormonas que secreten, aunque suelen resultar hormonalmente silentes 5 . En ocasiones pueden secretar polipéptidos intestinalmente vasoactivos, induciendo entonces un síndrome caracterizado por diarrea, hipopotasemia y aclorhidria 28 , o testosterona, lo que conlleva virilizaciones 7 . Se han descrito ubicaciones en cualquier zona del organismo: supratentoriales 3 , cerebelosas 15 , raquídeas 4,8,10,12,13,24 , mediastínicas 27 , retroperitoneales 22 , intestinales 11 o cutáneas 6 .…”