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Pancreas

Neuroendocrine tumors

Somatostatinoma


Reviewer: Deepali Jain, M.D. (see Reviewers page)
Revised: 29 November 2012, last major update August 2012
Copyright: (c) 2001-2012, PathologyOutlines.com, Inc.

General
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● Functionally active and usually malignant endocrine tumour with evidence of D-cell differentiation and clinical symptoms reflecting the diverse pathophysiologic effects of chronic inappropriate secretion of somatostatin (WHO)
● First reported in 1977 (Lancet 1977;1:666, N Engl J Med 1977;296:963, eMedicine)
● Also called delta cell tumors
● Note: somatostatin expression also seen in occur in gangliocytic paraganglioma, poorly differentiated neuroendocrine carcinoma and hereditary neuroendocrine tumors (MEN1)

Clinical features
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● Only rarely associated with somatostainoma syndrome (diabetes, cholecystolithiasis, steatorrhea, hypochlorhydria, Endocr Relat Cancer 2008;15:229)
● Hard to localize preoperatively
● May be in duodenal wall
● May have psammoma bodies

Clinical images
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Duodenal tumor-endoscopy

Micro images
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Somatostatin+ cells


3 cm, nonangioinvasive, nonfunctioning somatostatin cell tumor shows moderate nuclear crowding with distinct nucleoli; tumor recurred locally 14 years later with gross invasion and somatostatinoma syndrome


This locally invasive tumor shows a medullary pattern and no significant cellular atypia


Paraganglioma-like growth pattern


Various images

     
Duodenal tumors

Electron microscopy images
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D-cell granules in cells of somatostatinoma. Inset: The immunogold reactivity with somatostatin antibodies is seen

End of Pancreas > Neuroendocrine tumors > Somatostatinoma


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