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Published on: August 14, 2012
Pancreatic neuroendocrine microadenomatosis presenting as a functional VIPoma
Alexei A Krainev1, Viney K Mathavan2, Daniel F Klink3
1St. Vincent Hospital General Surgery Residency Program, St. Vincent Hospital Indianapolis, St. Vincent Ortho Center 2001 West 86th Street, Indianapolis, IN, USA.
Multiple functional pancreatic microadenomas, rare neuroendocrine tumors, caused severe secretory diarrhea and electrolyte imbalances. Surgical removal of these pancreatic head tumors resolved the patient's symptoms.
Area of Science:
- Endocrinology
- Gastroenterology
- Surgical Oncology
Background:
- Pancreatic microadenomas are small, benign neuroendocrine tumors.
- Most are non-functional, but rare cases associated with Multiple Endocrine Neoplasia type 1 (MEN-1) can be functional.
- Functional microadenomas can secrete hormones, leading to complex clinical syndromes.
Observation:
- A unique case of multiple functional microadenomas in the pancreatic head is presented.
- The patient exhibited persistent secretory diarrhea, refractory hypokalemia, and metabolic acidosis.
- Elevated plasma vasoactive intestinal peptide (VIP) levels were a key diagnostic finding.
Findings:
- The microadenomas were identified as the source of excessive VIP secretion.
- Surgical intervention, specifically pancreaticoduodenectomy, was performed.
- Histopathological analysis confirmed the neuroendocrine nature of the microadenomas.
Implications:
- This case highlights the importance of considering functional pancreatic microadenomas in patients with unexplained secretory diarrhea and electrolyte disturbances.
- Early diagnosis and surgical management can lead to complete symptom resolution.
- Further research into the specific mechanisms of hormone secretion by these rare tumors is warranted.
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