Authors: Venu Julapalli, MD; Mehnaz Shafi, MD; Jim Schwartz, MDSeries Editor: Richard Goodgame


April 19, 2004


Although our patient had a rare tumor, the differential diagnosis illustrates the interesting biologic spectrum of neuroendocrine tumors of the pancreas. These tumors can occur sporadically in patients without a family history. However, one tumor can produce multiple hormones. In this situation, the tumors may mimic the MEN syndromes, prompting a search for second or third tumors. The presence of MEN-1 syndrome should be investigated in all patients with concomitant Zollinger-Ellison syndrome and Cushing's syndrome. Only a full hormonal and anatomic evaluation can clarify the clinical alternatives: one tumor producing multiple hormones vs multiple endocrine tumors.

Gastrinomas that produce ACTH are much more biologically aggressive than simple gastrinomas. Therapy is not based on prospective randomized clinical trials but on appreciating the unique combination of hormone production and tumor spread. Treatment decisions first involve symptom control, then a multidisciplinary approach that may include surgical debulking, somatostatin analog therapy, and chemotherapy.


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