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Von recklinghausen's disease associated with duodenal somatostatinoma: Contrast of duodenal versus pancreatic somatostatinomas

✍ Scribed by Chuanyan Mao; Ajay Shah; Daniel J. Hanson; John M. Howard


Publisher
John Wiley and Sons
Year
1995
Tongue
English
Weight
807 KB
Volume
59
Category
Article
ISSN
0022-4790

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✦ Synopsis


A case of Von Recklinghausen's disease with duodenal somatostatinoma is reported. A search of the world's literature revealed 27 patients with Von Recklinghausen' s disease associated with an immunohistologicall y proved duodenal somatostatinoma. Twenty-nine cases of duodenal somatostatinoma not associated with Von Recklinghausen's disease and 32 cases of pancreatic somatostatinomas have been identified for comparison. While their histology may be similar in many respects, the duodenal and pancreatic somatostatinomas show significant differences, especially in hormonal and growth behaviors.

In contrast to its pancreatic counterpart, the duodenal somatostatinoma is frequently associated with Von Recklinghausen's disease, is seldom associated with a recognizable "somatostatin syndrome," often contains psammoma bodies, and is less frequently associated with demonstrable metastases at the time of operation.