VIPoma

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A rare neuroendocrine tumor that causes watery diarrhea and electrolyte imbalance


VIPoma
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:

Clinical Presentation[edit | edit source]

Patients with VIPoma typically present with:

Diagnosis[edit | edit source]

Diagnosis involves a combination of clinical suspicion and confirmatory tests:

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]



Classification
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