Somatostatinoma syndrome. Biochemical, morphologic and clinical features

G. J. Krejs, L. Orci, J. M. Conlon, M. Ravazzola, G. R. Davis, Philip Raskin, S. M. Collins, D. M. McCarthy, D. Baetens, A. Rubenstein, T. A. Aldor, 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-yr-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 5μg 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 calcitionin 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.

Original languageEnglish (US)
Pages (from-to)285-292
Number of pages8
JournalNew England Journal of Medicine
Volume301
Issue number6
StatePublished - 1979

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Somatostatinoma
Somatostatin
Calcitonin
Steatorrhea
Neoplasms
Cholelithiasis
Peptide Hormones
Secretory Vesicles
Insulin-Secreting Cells
Duodenum
Growth Hormone
Diabetes Mellitus
Electrons
Neoplasm Metastasis
Food

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Krejs, G. J., Orci, L., Conlon, J. M., Ravazzola, M., Davis, G. R., Raskin, P., ... Unger, R. H. (1979). Somatostatinoma syndrome. Biochemical, morphologic and clinical features. New England Journal of Medicine, 301(6), 285-292.

Somatostatinoma syndrome. Biochemical, morphologic and clinical features. / Krejs, G. J.; Orci, L.; Conlon, J. M.; Ravazzola, M.; Davis, G. R.; Raskin, Philip; Collins, S. M.; McCarthy, D. M.; Baetens, D.; Rubenstein, A.; Aldor, T. A.; Unger, Roger H.

In: New England Journal of Medicine, Vol. 301, No. 6, 1979, p. 285-292.

Research output: Contribution to journalArticle

Krejs, GJ, Orci, L, Conlon, JM, Ravazzola, M, Davis, GR, Raskin, P, Collins, SM, McCarthy, DM, Baetens, D, Rubenstein, A, Aldor, TA & Unger, RH 1979, 'Somatostatinoma syndrome. Biochemical, morphologic and clinical features', New England Journal of Medicine, vol. 301, no. 6, pp. 285-292.
Krejs GJ, Orci L, Conlon JM, Ravazzola M, Davis GR, Raskin P et al. Somatostatinoma syndrome. Biochemical, morphologic and clinical features. New England Journal of Medicine. 1979;301(6):285-292.
Krejs, G. J. ; Orci, L. ; Conlon, J. M. ; Ravazzola, M. ; Davis, G. R. ; Raskin, Philip ; Collins, S. M. ; McCarthy, D. M. ; Baetens, D. ; Rubenstein, A. ; Aldor, T. A. ; Unger, Roger H. / Somatostatinoma syndrome. Biochemical, morphologic and clinical features. In: New England Journal of Medicine. 1979 ; Vol. 301, No. 6. pp. 285-292.
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