VIPoma
(Redirected from Vipoma)
A rare neuroendocrine tumor that causes watery diarrhea and electrolyte imbalance
VIPoma | |
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Synonyms | Verner–Morrison syndrome, Pancreatic cholera, WDHA syndrome (Watery Diarrhea, Hypokalemia, and Achlorhydria) |
Pronounce | |
Specialty | N/A |
Symptoms | Profuse watery diarrhea, dehydration, flushing, cramping, weakness |
Complications | Hypokalemia, achlorhydria, metabolic acidosis, renal failure, malnutrition |
Onset | Typically adults aged 30–50 |
Duration | Chronic unless treated |
Types | Pancreatic or extrapancreatic neuroendocrine tumor |
Causes | Neoplasm of VIP-secreting cells |
Risks | Multiple endocrine neoplasia type 1 |
Diagnosis | Plasma VIP level, imaging (CT scan, MRI, somatostatin receptor scintigraphy) |
Differential diagnosis | Cholera, Carcinoid syndrome, laxative abuse, Zollinger–Ellison syndrome |
Prevention | None known |
Treatment | Surgical resection, somatostatin analogs, supportive care |
Medication | Octreotide, Lanreotide, electrolyte replacement |
Prognosis | Variable; often poor in metastatic cases |
Frequency | ~1 per 10 million annually |
Deaths | Associated with complications of metastatic disease or severe dehydration |
VIPoma is a rare type of neuroendocrine tumor that secretes excessive amounts of vasoactive intestinal peptide (VIP), a hormone involved in regulating intestinal water and electrolyte secretion. The excessive VIP leads to a distinct clinical syndrome characterized by profound watery diarrhea, hypokalemia (low potassium), and achlorhydria (lack of stomach acid), collectively known as WDHA syndrome or Verner–Morrison syndrome. It is also colloquially referred to as pancreatic cholera due to the similarity of symptoms to cholera infection.
Pathophysiology[edit | edit source]
VIPomas most commonly originate from the pancreas, particularly the non-β islet cells, although rare cases have been found in extrapancreatic sites such as the bronchus, adrenal gland, or colon. Approximately 50–75% of VIPomas are malignant at the time of diagnosis, frequently with metastasis to the liver or lymph nodes.
The excessive VIP causes increased intestinal secretion of water and electrolytes, reduced gastric acid secretion, and smooth muscle relaxation, resulting in:
- Chronic watery diarrhea (often exceeding 3 liters/day)
- Electrolyte disturbances including hypokalemia, hypochlorhydria, and metabolic acidosis
- Systemic vasodilation leading to flushing and hypotension
- Possible hypercalcemia and hyperglycemia
Clinical Presentation[edit | edit source]
Patients with VIPoma typically present with:
- Severe, persistent watery diarrhea (even during fasting)
- Dehydration
- Muscle weakness, cramping
- Weight loss
- Facial flushing
- Hypotension
- Nausea or vomiting (less common)
Diagnosis[edit | edit source]
Diagnosis involves a combination of clinical suspicion and confirmatory tests:
- Laboratory tests:
- Elevated serum VIP levels (>75 pg/mL)
- Electrolyte imbalances: low potassium, low bicarbonate
- Metabolic acidosis on arterial blood gas
- Imaging:
- CT scan or MRI to localize tumor
- Somatostatin receptor scintigraphy (Octreoscan) or 68Ga-DOTATATE PET/CT to detect somatostatin receptor-positive lesions
- Endoscopic ultrasound in select cases
Treatment[edit | edit source]
Treatment strategies include:
Medical Management[edit | edit source]
- Octreotide or Lanreotide – long-acting somatostatin analogs that inhibit VIP secretion and reduce diarrhea
- IV fluid and electrolyte replacement – to manage dehydration and correct imbalances
- Antidiarrheal agents – adjunctive, but generally not effective alone
Surgical Management[edit | edit source]
- Surgical resection – curative if the tumor is localized
- Debulking surgery – may help in metastatic cases to control hormone secretion
Advanced/Metastatic Disease[edit | edit source]
- Targeted therapy – e.g., everolimus, sunitinib
- Peptide receptor radionuclide therapy (PRRT) for somatostatin receptor-positive tumors
- Hepatic artery embolization or ablation for liver metastases
Prognosis[edit | edit source]
The prognosis of VIPoma depends on tumor size, location, and metastatic spread. Localized VIPomas have a good prognosis with surgical resection, while metastatic VIPomas have a poorer outcome. However, symptom control with somatostatin analogs can significantly improve quality of life.
Epidemiology[edit | edit source]
VIPomas are extremely rare, with an estimated incidence of approximately 1 per 10 million people per year. They typically occur in adults aged 30–50, with equal incidence in males and females. They may be associated with multiple endocrine neoplasia type 1 (MEN1).
See Also[edit | edit source]
External links[edit | edit source]
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