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Malignant duodenal somatostatinoma presenting in association with von Recklinghausen disease
Vijaykumar G Patel1, Vernon J Henderson, David A Fairweather
1Department of Surgery, Morehouse School of Medicine, Atlanta, Georgia 30310, USA.
Abstract:
Somatostatinomas are extremely rare periampullary malignant neuroendocrine tumors that may be associated with von Recklinghausen disease or type-I neurofibromatosis. Duodenal somatostatinomas are distinguished from pancreatic somatostatinomas by their frequent association with type-I neurofibromatosis and typically absence of somatostatinoma syndrome. We report a very rare and atypical case of malignant duodenal somatostatinoma presenting with somatostatinoma syndrome in association with type-I neurofibromatosis.

