Diabetes mellitus, steatorrhea, cholelithiasis and a tumor distorting the duodenum prompted a work-up for somatostatinoma in a 52-year-old man. The responses of pancreatic B-cells but not of A-cells to nutrient stimuli were inhibited, and growth-hormone release was suppressed, suggesting somatostatin resistance in some target tissues. Plasma somatostatin-like immunoreactivity ranged from 9000 to 13,000 pg per milliliter (normal: 88+/-8, mean +/- S.E.M.) and was distributed in four molecular forms, including free somatostatin. The primary tumor contained 5 microgram of somatostatin-like immunoreactivity per milligram of wet tissue, distributed in three of the molecular forms noted in plasma. Plasma calcitonin was also elevated (4650 pg per milliliter; normal: less than 120). Immunocytochemical studies showed that cells of the primary tumor contained somatostatin and calcitonin but no other peptide hormones. Only somatostatin was present in the metastases. Somatostatin was localized electron microscopically in all secretory granules, irrespective of size and shape, whereas calcitonin was present only within a single subpopulation of small granules in the same cells.

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A 30-year-old woman with a 10-year history of recurrent bloody diarrhea and documented colitis of the descending colon, consistent with Crohn's disease, presented with an exacerbation of her gastrointestinal disease and an 18-month history of recurrent facial and genital swelling. Her course evolved to include severe ear pain, dysphagia and colonic dysmotility. She was diagnosed with Melkersson-Rosenthal syndrome and treated with multiple agents. The neurological aspects of her presentation are highlighted, and the Melkersson-Rosenthal syndrome is reviewed.

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