History and exam

Key diagnostic factors

common

profuse watery diarrhoea

Patients usually present with profuse watery diarrhoea (100% >700 mL/day); stool volumes typically exceed 3 L/day (70% to 80% of patients).[18][19][20]​​

Large tumours can result in 6-8 L stool volumes per day.[21]​

Characteristically, the diarrhoea is odourless and tea-coloured, and persists even after 48-72 hours of fasting.

Profuse watery diarrhoea is part of the clinical syndrome for VIPoma (sometimes referred to as Verner-Morrison syndrome, pancreatic cholera, or WDHA syndrome [watery diarrhoea, hypokalaemia, and achlorhydria]).

Other diagnostic factors

common

age around 50 years

Median age at diagnosis of 51 years (range 11-75 years) has been derived from individual case reports.[9]

headache

Patients may complain of headache and other dehydration-related symptoms.

weight loss

Usually due to chronic volume depletion secondary to diarrhoea, or less commonly metastatic disease. Occurs in 36% to 100% of patients.[9]

poor skin turgor

Due to chronic volume depletion secondary to diarrhoea.

dry mucous membranes

Due to chronic volume depletion secondary to diarrhoea.

muscle weakness

Due to hypokalaemia.

muscle cramps

Due to hypokalaemia.

gastrointestinal symptoms

Excessive secretion of vasoactive intestinal peptide. Frequent bowel movements can cause abdominal discomfort and pain.

hyperglycaemia symptoms

Patients may have symptoms related to hyperglycaemia (e.g., polydipsia, polyuria, fatigue). Due to vasoactive intestinal peptide stimulation of glycogenolysis. May affect 20% to 50% of patients.[30]

uncommon

flushing

Secondary to vasodilation (as a direct effect of vasoactive intestinal peptide). Occurs in 0% to 33% of patients.[9]

hepatomegaly

Due to hepatic metastasis.

history of multiple endocrine neoplasia type 1 (MEN1)

VIPoma is reported in <1% of patients with MEN1.[10]​​[17]

Risk factors

weak

multiple endocrine neoplasia type 1 (MEN1)

VIPoma is reported in <1% of patients with MEN1.​[10]

MEN1 is a rare inherited disorder characterised by multiple tumours arising from the pancreas, parathyroid gland, pituitary gland, or other parts of the digestive tract. See Multiple endocrine neoplasia syndromes.

age around 50 years

Median age at diagnosis has been reported to be 51 years (range: 11-75 years).[9]

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