Intractable watery diarrhea, ganglioneuroblastoma, and vasoactive intestinal peptide

Mitchell, Charles H.; Sinatra, Frank; Crast, Frank W.; Griffin, Robert; Sunshine, Philip

doi:10.1016/s0022-3476(76)80393-9

Cited by 37 publications

(5 citation statements)

References 7 publications

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“…The treatment of choice is surgical excision. Steroids and somatostatin had been used with variable results [9,12,24,27]. However, these should be used only when it is not possible to localise the site of the VIP-secreting tumour.…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2008

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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.

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Section: Discussionmentioning

confidence: 99%

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

et al. 2008

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“…Patients with mediastinal tumors may present with Horner's syndrome, and patients with cervical tumors may present with neck mass. 27,28 Less common presentations, due to paraneoplastic phenomena, include watery diarrhea, caused by secretion of a vasoactive intestinal peptide, 29 and acute cerebellar encephalopathy, marked by opsoclonus, myoclonus, ataxia, and a persistent dementia. Acute cerebellar encephalopathy is seen in three percent of all patients, but is more common in low-stage disease.…”

Section: Signs and Symptomsmentioning

confidence: 99%

Neuroblastoma: a clinical challenge and biologic puzzle

Matthay

1995

CA: A Cancer Journal for Clinicians

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“…Since the diarrhoea ceases after tumour removal it seems likely that a humoral substance secreted by the tumour is responsible. Since then a number of case reports on chronic diarrhoea associated with elevated concentration of VIP in plasma and tumour tissue extract in children with NCT have been published (4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14). Since then a number of case reports on chronic diarrhoea associated with elevated concentration of VIP in plasma and tumour tissue extract in children with NCT have been published (4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14).…”

mentioning

confidence: 99%

“…The exact nature of this substance remained unclear until 1973 when Bloom et al (3) provided evidence that vasoactive intestinal polypeptide (VIP) was the causative agent. Since then a number of case reports on chronic diarrhoea associated with elevated concentration of VIP in plasma and tumour tissue extract in children with NCT have been published (4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14).…”

mentioning

confidence: 99%

Vasoactive Intestinal Polypeptide (Vip) in Children With Neural Crest Tumours

Scheibel¹,

Rechnitzer²,

Fahrenkrug³

et al. 1982

Acta Paediatrica

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The concentration of vasoactive intestinal polypeptide (VIP) in plasma was measured in 22 children with neural crest tumours (NCT) during a 5-year period; the mean concentration of VIP in plasma was 22.5 pmol/l (interval 2.0-95.0 pmol/l). To establish a reference interval the plasma concentration of VIP was measured in 41 children without tumours; the mean concentration of VIP in plasma was 6.2 pmol/l (interval 0.5-19.0 pmol/l). Of the 22 children with NCT 16 had a plasma concentration of VIP within the normal range while 6 children (27%) had elevated plasma concentration of VIP between 28 and 95 pmol/l. Only one child, whose plasma concentration of VIP was 95 pmol/l had diarrhoea. Elevated plasma concentration of VIP in children with NCT but no diarrhoea has not previously been described. The urinary excretion of vanillylmandelic acid (VMA) was increased in 18 of the children with NCT (82%). In 2 of the children with normal excretion of VMA the concentration of VIP in plasma was elevated. Thus, the plasma concentration of VIP may be a supplement to VMA as a tumour marker in some cases of NCT.

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Intractable watery diarrhea, ganglioneuroblastoma, and vasoactive intestinal peptide

Cited by 37 publications

References 7 publications

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

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

Neuroblastoma: a clinical challenge and biologic puzzle

Vasoactive Intestinal Polypeptide (Vip) in Children With Neural Crest Tumours

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