title: Evaluation and management of pancreatic lesions in patients with von Hippel-Lindau disease.
abstract: von Hippel-Lindau (VHL) disease is a heritable cancer-predisposition syndrome with multiorgan involvement. Pancreatic lesions are detected in approximately two-thirds of patients with VHL disease at some point during their lifetime. In these patients, cystic pancreatic lesions are almost exclusively benign and, unless symptomatic, do not require surgical or endoscopic intervention; however, solid pancreatic lesions can often be recognized through imaging screens, and are commonly found to be nonfunctioning pancreatic neuroendocrine tumours (pNETs) with malignant potential. The natural history of these VHL-associated pNETs is variable, and lacks clinical or imaging features that predict disease progression or metastatic potential, and generally needs to be managed more conservatively than their sporadic counterparts. Treatment options for such lesions, which range from active surveillance to surgical intervention, can nevertheless be associated with considerable morbidity and even mortality. Of note, although several guidelines have been established for the management of tumours associated with VHL syndrome, none of these have specifically focused on pancreatic lesions. Thus, we aim to characterize the types of pancreatic lesions associated with VHL disease and their natural history, to identify particular lesions that necessitate treatment, and to define what forms of treatment should be undertaken.
authors.affiliation: Endocrine Oncology Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Building 10, Room 4-5940, Bethesda, Maryland 20891-1201, USA.
authors.forename: Xavier M
authors.initials: XM
authors.lastname: Keutgen
authors.affiliation: Department of Digestive Oncology, Beaujon University Hospital (AP-HP - PRES Paris 7 Diderot), 100 boulevard du Général Leclerc, 92110 Clichy, France.
authors.forename: Pascal
authors.initials: P
authors.lastname: Hammel
authors.affiliation: Molecular Imaging Program, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Building 10, Room B3B69F, Bethesda, Maryland 20892-1088, USA.
authors.forename: Peter L
authors.initials: PL
authors.lastname: Choyke
authors.affiliation: Department of Surgery, Montefiore Medical Center, 111 East 210th Street, Bronx, New York 10467, USA.|Albert Einstein College of Medicine, 1300 Morris Park Avenue, Bronx, New York 10461, USA.
authors.forename: Steven K
authors.initials: SK
authors.lastname: Libutti
authors.affiliation: Department of Genitourinary Medical Oncology, Division of Cancer Medicine, The University of Texas MD Anderson Cancer Center, 1155 Pressler Street, Unit 1374, Houston, Texas 77030-3721, USA.
authors.forename: Eric
authors.initials: E
authors.lastname: Jonasch
authors.affiliation: Endocrine Oncology Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Building 10, Room 4-5940, Bethesda, Maryland 20891-1201, USA.
authors.forename: Electron
authors.initials: E
authors.lastname: Kebebew
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D055088:Early Detection of Cancer; D005838:Genotype; D006801:Humans; D008113:Liver Neoplasms / Q000556:secondary; D008279:Magnetic Resonance Imaging / Q000379:methods; D008875:Middle Aged; D018358:Neuroendocrine Tumors / Q000150:complications / Q000175:diagnosis / Q000628:therapy; D010181:Pancreatic Cyst / Q000150:complications / Q000175:diagnosis / Q000628:therapy; D010190:Pancreatic Neoplasms / Q000150:complications / Q000175:diagnosis / Q000628:therapy*; D010641:Phenotype; D000072078:Positron Emission Tomography Computed Tomography / Q000379:methods; D011230:Precancerous Conditions / Q000175:diagnosis; D014057:Tomography, X-Ray Computed / Q000379:methods; D055815:Young Adult; D006623:von Hippel-Lindau Disease / Q000150:complications / Q000175:diagnosis / Q000628:therapy*