Simple jQuery Dropdowns
Please use this identifier to cite or link to this item: http://repository.li.mahidol.ac.th/dspace/handle/123456789/45073
Title: Preventive medicine of von Hippel-Lindau disease-associated pancreatic neuroendocrine tumors
Authors: Tobias Krauss
Alfonso Massimiliano Ferrara
Thera P. Links
Ulrich Wellner
Irina Bancos
Andrey Kvachenyuk
Karina Villar Gómez De Las Heras
Marina Y. Yukina
Roman Petrov
Garrett Bullivant
Laura Von Duecker
Swati Jadhav
Ursula Ploeckinger
Staffan Welin
Camilla Schalin-Jäntti
Oliver Gimm
Marija Pfeifer
Joanne Ngeow
Kornelia Hasse-Lazar
Gabriela Sansó
Xiaoping Qi
M. Umit Ugurlu
Rene E. Diaz
Nelson Wohllk
Mariola Peczkowska
Jens Aberle
Delmar M. Lourenço
Maria A.A. Pereira
Maria C.B.V. Fragoso
Ana O. Hoff
Madson Q. Almeida
Alice H.D. Violante
Ana R.P. Quidute
Zhewei Zhang
Mònica Recasens
Luis Robles Díaz
Tada Kunavisarut
Taweesak Wannachalee
Sirinart Sirinvaravong
Eric Jonasch
Simona Grozinsky-Glasberg
Merav Fraenkel
Dmitry Beltsevich
Viacheslav I. Egorov
Dirk Bausch
Matthias Schott
Nikolaus Tiling
Gianmaria Pennelli
Stefan Zschiedrich
Roland Därr
Juri Ruf
Timm Denecke
Karl Heinrich Link
Stefania Zovato
Ernst Von Dobschuetz
Svetlana Yaremchuk
Holger Amthauer
Ozer Makay
Attila Patocs
Martin K. Walz
Tobias B. Huber
Jochen Seufert
Per Hellman
Raymond H. Kim
Ekaterina Kuchinskaya
Francesca Schiavi
Angelica Malinoc
Nicole Reisch
Barbara Jarzab
Marta Barontini
Andrzej Januszewicz
Nalini Shah
William F. Young
Giuseppe Opocher
Charis Eng
Hartmut P.H. Neumann
Birke Bausch
The Second Affiliated Hospital Zhejiang University School of Medicine
Endocrinology Research Centre
Istituto Oncologico Veneto IOV - IRCCS
Instytut Kardiologii im. Prymasa Tysiaclecia Stefana Kardynała Wyszynskiego
V. P. Komisarenko Institute of Endocrinology and Metabolism of the Ukraine Academy of Medical Sciences
Maria Sklodowska-Curie Institute – Oncology Center
Taussig Cancer Center
Ludwig-Maximilians-Universität München
Universität Hamburg
Marmara Üniversitesi
Charité – Universitätsmedizin Berlin
Universität Freiburg im Breisgau
Heinrich Heine Universität
Semmelweis Egyetem
Univerzitetni Klinični Center Ljubljana
Universidade Federal do Ceara
Hospital de Ninos Ricardo Gutierrez
University of Toronto
Akademiska Sjukhuset
University of Texas MD Anderson Cancer Center
Hospital Universitari de Girona Dr. Josep Trueta
Universidad de Chile
Universität zu Lübeck
Faculty of Medicine, Siriraj Hospital, Mahidol University
Linköpings universitet
Ontario Cancer Institute University of Toronto
Universidade de Sao Paulo - USP
Hospital del Salvador
University of Groningen, University Medical Center Groningen
Mayo Clinic
Nanyang Technological University
Universitätsklinikum Hamburg-Eppendorf und Medizinische Fakultät
Hospital Universitario 12 de Octubre
Helsingin Yliopisto
King Edward Memorial Hospital India
Università degli Studi di Padova
Hadassah University Medical Centre
Universidade Federal do Rio de Janeiro
Division of Endocrine Surgery
Central Services
Bakhrushin Brothers Moscow City Hospital
Asklepios Paulinen Klinik
Huyssens Foundation Clinics
Wenzhou Medical University
Keywords: Biochemistry, Genetics and Molecular Biology;Medicine
Issue Date: 1-Sep-2018
Citation: Endocrine-Related Cancer. Vol.25, No.9 (2018), 783-793
Abstract: © 2018 Society for Endocrinology Published by Bioscientifica Ltd. Printed in Great Britain. Pancreatic neuroendocrine tumors (PanNETs) are rare in von Hippel-Lindau disease (VHL) but cause serious morbidity and mortality. Management guidelines for VHL-PanNETs continue to be based on limited evidence, and survival data to guide surgical management are lacking. We established the European-American-Asian-VHL-PanNET-Registry to assess data for risks for metastases, survival and long-term outcomes to provide best management recommendations. Of 2330 VHL patients, 273 had a total of 484 PanNETs. Median age at diagnosis of PanNET was 35 years (range 10-75). Fifty-five (20%) patients had metastatic PanNETs. Metastatic PanNETs were significantly larger (median size 5 vs 2cm; P<0.001) and tumor volume doubling time (TVDT) was faster (22 vs 126 months; P=0.001). All metastatic tumors were ≥2.8cm. Codons 161 and 167 were hotspots for VHL germline mutations with enhanced risk for metastatic PanNETs. Multivariate prediction modeling disclosed maximum tumor diameter and TVDT as significant predictors for metastatic disease (positive and negative predictive values of 51% and 100% for diameter cut-off ≥2.8cm, 44% and 91% for TVDT cut-off of ≤24 months). In 117 of 273 patients, PanNETs >1.5cm in diameter were operated. Ten-year survival was significantly longer in operated vs non-operated patients, in particular for PanNETs <2.8cm vs ≥2.8cm (94% vs 85% by 10 years; P=0.020; 80% vs 50% at 10 years; P=0.030). This study demonstrates that patients with PanNET approaching the cut-off diameter of 2.8cm should be operated. Mutations in exon 3, especially of codons 161/167 are at enhanced risk for metastatic PanNETs. Survival is significantly longer in operated non-metastatic VHL-PanNETs.
URI: https://www.scopus.com/inward/record.uri?partnerID=HzOxMe3b&scp=85050883238&origin=inward
http://repository.li.mahidol.ac.th/dspace/handle/123456789/45073
ISSN: 14796821
13510088
Appears in Collections:Scopus 2018

Files in This Item:
There are no files associated with this item.


Items in DSpace are protected by copyright, with all rights reserved, unless otherwise indicated.