Carcinoid syndrome due to a malignant somatostatinoma.
Somatostatinoma is one of the rarest tumours of the endocrine pancreas. Cardinal manifestations of a somatostatinoma include gallstones, mild diabetes mellitus, steatorrhoea, diarrhoea and dyspepsia. Like any other pancreatic islet cell carcinoma, a somatostatinoma may also produce several different hormones such as adrenocorticotropic hormone, calcitonin, vasoactive intestinal polypeptide, pancreatic polypeptide, gastrin, insulin, and glucagon. In many cases, the clinical picture is dominated by the effect of these other hormones. We present a patient with somatostatinoma in which an immunocytochemical study of the specimens from pancreas and liver showed a weak positive reaction for gastrin besides a strong positive reaction for somatostatin. Interestingly, this patient also showed the signs of carcinoid syndrome which was successfully treated with octreotide.
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- Krejs GJ, Collins SM, McCarthy D, Unger RH, Gardner JD. Follow-up of a patient with somatostatinoma. N Engl J Med. 1986 Nov 13;315(20):1295–1295. [PubMed] [Google Scholar]
- Pipeleers D, Couturier E, Gepts W, Reynders J, Somers G. Five cases of somatostatinoma: clinical heterogeneity and diagnostic usefulness of basal and tolbutamide-induced hypersomatostatinemia. J Clin Endocrinol Metab. 1983 Jun;56(6):1236–1242. [PubMed] [Google Scholar]
- Gerlock AJ, Jr, Muhletaler CA, Halter S, Goncharenko V. Pancreatic somatostatinoma: histologic, clinical, and angiographic features. AJR Am J Roentgenol. 1979 Nov;133(5):939–943. [PubMed] [Google Scholar]
- Larsson LI, Hirsch MA, Holst JJ, Ingemansson S, Kühl C, Jensen SL, Lundqvist G, Rehfeld JF, Schwartz TW. Pancreatic somatostatinoma. Clinical features and physiological implications. Lancet. 1977 Mar 26;1(8013):666–668. [PubMed] [Google Scholar]
- Ganda OP, Weir GC, Soeldner JS, Legg MA, Chick WL, Patel YC, Ebeid AM, Gabbay KH, Reichlin S. "Somatostatinoma": a somatostatin-containing tumor of the endocrine pancreas. N Engl J Med. 1977 Apr 28;296(17):963–967. [PubMed] [Google Scholar]
- Unger RH. Somatostatinoma. N Engl J Med. 1977 Apr 28;296(17):998–1000. [PubMed] [Google Scholar]
- Asa SL, Kovacs K, Killinger DW, Marcon N, Platts M. Pancreatic islet cell carcinoma producing gastrin, ACTH, alpha-endorphin, somatostatin and calcitonin. Am J Gastroenterol. 1980 Jul;74(1):30–35. [PubMed] [Google Scholar]
- Kvols LK, Moertel CG, O'Connell MJ, Schutt AJ, Rubin J, Hahn RG. Treatment of the malignant carcinoid syndrome. Evaluation of a long-acting somatostatin analogue. N Engl J Med. 1986 Sep 11;315(11):663–666. [PubMed] [Google Scholar]