Abdominal visceral lesions in von Hippel-Lindau disease: incidence and clinical behavior of pancreatic and adrenal lesions at a single center.
BACKGROUND
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.
Delman
Keith A
KA
Department of Surgical Oncology, The University of Texas M.D. Anderson Cancer Center, 1515 Holcombe Boulevard, Unit 444, Houston, Texas 77030, USA.
Shapiro
Suzanne E
SE
Jonasch
Eric W
EW
Lee
Jeffrey E
JE
Curley
Steven A
SA
Evans
Douglas B
DB
Perrier
Nancy D
ND
D000293:Adolescent; D000310:Adrenal Gland Neoplasms / Q000175:diagnosis / Q000453:epidemiology / Q000235:genetics; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002648:Child; D003560:Cysts / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy; D005260:Female; D006801:Humans; D015994:Incidence; D008297:Male; D008875:Middle Aged; D009154:Mutation; D018358:Neuroendocrine Tumors / Q000175:diagnosis / Q000453:epidemiology / Q000235:genetics; D010182:Pancreatic Diseases / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy; D010190:Pancreatic Neoplasms / Q000175:diagnosis / Q000453:epidemiology / Q000235:genetics; D010673:Pheochromocytoma / Q000175:diagnosis / Q000453:epidemiology / Q000235:genetics; D012189:Retrospective Studies; D006623:von Hippel-Lindau Disease / Q000235:genetics*