Somatostatinoma Syndrome

Authors:
  • GKGuenter J. Krejs
    Guenter J. Krejs
  • LOLelio Orci
    Lelio Orci
  • JCJ. Michael Conlon
    J. Michael Conlon
  • MRMariella Ravazzola
    Mariella Ravazzola
  • GDGlenn R. Davis
    Glenn R. Davis
  • PRPhilip Raskin
    Philip Raskin
  • SCStephen M. Collins
    Stephen M. Collins
  • DMDenis M. McCarthy
    Denis M. McCarthy
  • DBDany Baetens
    Dany Baetens
  • ARArthur Rubenstein
    Arthur Rubenstein
  • TAT. A. M. Aldor
    T. A. M. Aldor
  • RURoger H. Unger
    Roger H. Unger
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Abstract
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 μg 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: <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. (N Engl J Med 301:285–292, 1979)

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