Management recommendations for pancreatic manifestations of von Hippel-Lindau disease.
neuroendocrine tumor
pancreas
recommendations
surveillance
von Hippel-Lindau
Journal
Cancer
ISSN: 1097-0142
Titre abrégé: Cancer
Pays: United States
ID NLM: 0374236
Informations de publication
Date de publication:
01 02 2022
01 02 2022
Historique:
revised:
22
09
2021
received:
26
08
2021
accepted:
23
09
2021
pubmed:
5
11
2021
medline:
11
3
2022
entrez:
4
11
2021
Statut:
ppublish
Résumé
Von Hippel-Lindau disease (VHL) is a multineoplasm inherited disease manifesting with hemangioblastoma of the central nervous system and retina, adrenal pheochromocytoma, renal cell carcinoma, pancreatic neuroendocrine tumors and cysts, and neoplasms/cysts of the ear, broad ligament, and testicles. During 2018-2020, the VHL Alliance gathered several committees of experts in the various clinical manifestations of VHL to review the literature, gather the available evidence on VHL, and develop recommendations for patient management. The current report details the results of the discussion of a group of experts in the pancreatic manifestations of VHL along with their proposed recommendations for the clinical surveillance and management of patients with VHL. The recommendations subcommittee performed a comprehensive systematic review of the literature and conducted panel discussions to reach the current recommendations. The level of evidence was defined according to the Shekelle variation of the Grading of Recommendations, Assessment, Development, and Evaluation grading system. The National Comprehensive Cancer Network Categories of Evidence and Consensus defined the committee members' interpretation of the evidence and degree of consensus. The recommendations encompass the main aspects of VHL-related pancreatic manifestations and their clinical management. They are presented in a clinical orientation, including general planning of screening and surveillance for pancreatic neuroendocrine tumors, utility of biochemical biomarkers, the optimal choice for imaging modality, indirect risk stratification, indications for tissue sampling of VHL-related pancreatic neuroendocrine tumors, and interventions. These recommendations are designed to serve as the reference for all aspects of the screening, surveillance, and management of VHL-related pancreatic manifestations.
Substances chimiques
Von Hippel-Lindau Tumor Suppressor Protein
EC 2.3.2.27
Types de publication
Journal Article
Research Support, N.I.H., Extramural
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
435-446Subventions
Organisme : NEI NIH HHS
ID : 5K08EY027464-02
Pays : United States
Organisme : NEI NIH HHS
ID : 5K08EY027464-02
Pays : United States
Informations de copyright
© 2021 American Cancer Society.
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