Faculty Collaboration Database

Abdominal visceral lesions in von Hippel-Lindau disease: incidence and clinical behavior of pancreatic and adrenal lesions at a single center. World J Surg 2006 May;30(5):665-9

Date

04/18/2006

Pubmed ID

16617420

DOI

10.1007/s00268-005-0359-4

Scopus ID

2-s2.0-33646545660 (requires institutional sign-in at Scopus site)   35 Citations

Abstract

INTRODUCTION: 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.

METHODS: We reviewed records for all patients treated for adrenal (type 2 VHL) or pancreatic manifestations of VHL at our institution from 1990 to 2004.

RESULTS: 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.

CONCLUSIONS: VHL should be familiar to the endocrine surgeon because of the high incidence (65% in our series) of visceral endocrine lesions (pancreas 40%, adrenal gland 18%, both 7%). As seen in other hereditary endocrinopathy syndromes, pheochromocytomas are frequently bilateral; therefore,close follow-up of the contralateral gland in young patients with a unilateral lesion is critical. Cystic lesions of the pancreas may be closely monitored, whereas neuroendocrine tumors should be removed because of the risk of metastasis. Pancreatic pathology is not uncommon in VHL patients with pheochromocytoma and should be sought during the preoperative assessment.

Author List

Delman KA, Shapiro SE, Jonasch EW, Lee JE, Curley SA, Evans DB, Perrier ND

Author

Douglas B. Evans MD Chair, Professor in the Surgery department at Medical College of Wisconsin

MESH terms used to index this publication - Major topics in bold

Adolescent
Adrenal Gland Neoplasms
Adult
Aged
Aged, 80 and over
Child
Cysts
Female
Humans
Incidence
Male
Middle Aged
Mutation
Neuroendocrine Tumors
Pancreatic Diseases
Pancreatic Neoplasms
Pheochromocytoma
Retrospective Studies
von Hippel-Lindau Disease