Abstract
Von Hippel-Lindau disease (VHL) is a dominantly inherited multi-system syndrome. Although pheochromocytoma is the hallmark endocrine neoplasm, pancreatic lesions occur frequently, and their management can be complex. This report describes 26 patients from a single institution with pancreatic or adrenal lesions (or both) in the background of VHL. We reviewed records for all patients treated for adrenal (type 2 VHL) or pancreatic manifestations of VHL at our institution from 1990 to 2004. Forty patients with VHL were identified from 24 pedigrees; 26 (65%) had adrenal or pancreatic involvement (or both). Seven patients had isolated pheochromocytomas, 16 had isolated pancreatic lesions, and 3 had both. Of the 10 patients with pheochromocytomas, 4 had bilateral adrenal involvement. All but three pheochromocytomas were diagnosed in patients aged 40 years or younger. Of the 19 patients with pancreatic lesions, 8 had cystic lesions, 8 had neuroendocrine tumors, and 3 had both. Of 11 patients with neuroendocrine tumors, 4 had metastases (3 hepatic, 1 nodal). No patient with cystic pancreatic lesions developed carcinoma. VHL should be familiar to the endocrine surgeon because of the high incidence (65% in our series) of visceral endocrin...Continue Reading
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