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.
Yu F, Venzon DJ, Serrano J, et al.: Prospective study of the clinical course, prognostic factors, causes of death, and survival in patients with long-standing Zollinger-Ellison syndrome. J Clin Oncol 17 (2): 615-30, 1999.
Danforth DN Jr, Gorden P, Brennan MF: Metastatic insulin-secreting carcinoma of the pancreas: clinical course and the role of surgery. Surgery 96 (6): 1027-37, 1984.
Kahn CR, Rosen SW, Weintraub BD, et al.: Ectopic production of chorionic gonadotropin and its subunits by islet-cell tumors. A specific marker for malignancy. N Engl J Med 297 (11): 565-9, 1977.
Pasieka JL, McLeod MK, Thompson NW, et al.: Surgical approach to insulinomas. Assessing the need for preoperative localization. Arch Surg 127 (4): 442-7, 1992.
van Heerden JA, Grant CS, Czako PF, et al.: Occult functioning insulinomas: which localizing studies are indicated? Surgery 112 (6): 1010-4; discussion 1014-5, 1992.