Glucagonoma is the third most common endocrine-secreting islet cell tumor, and 70% of glucagonomas are malignant. Necrolytic migratory erythema, hyperglycemia, and venous thrombosis comprise a virtually diagnostic triad. The measurement of serum glucagon confirms the diagnosis.
The following classifications are considered under miscellaneous:
- VIPoma (Verner-Morrison Syndrome) - characterized by watery diarrhea, hypokalemia, achlorhydria (WDHA).
- Somatostatinoma - 90% malignant, adult onset diabetes.
- Pancreatic polypeptide.
These are rare but defined clinical syndromes associated with specific polypeptide hormone production by islet cell tumors. Because of their rarity and similar approaches to management, they are grouped in this section.
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