When to Look for Von Hippel-Lindau Disease in Gastroenteropancreatic Neuroendocrine Tumors?Ling H.a · Cybulla M.a · Schaefer O.b · Arnold C.c · Schories M.c · Neumann H.P.H.a
Departments of aNephrology and Hypertension, bRadiology, and cGastroenterology and Endocrinology, Albert-Ludwigs-Universität, Freiburg, Germany
Do you have an account?
- Rent for 48h to view
- Buy Cloud Access for unlimited viewing via different devices
- Synchronizing in the ReadCube Cloud
- Printing and saving restrictions apply
Rental: USD 8.50
Cloud: USD 20.00
Von Hippel-Lindau (VHL) disease is a progressive autosomal dominant multisystem disorder that is associated with a germ line mutation of the VHL gene on the short arm of chromosome 3. A variety of benign and malignant diseases, including eye and CNS hemangioblastomas, renal cell carcinoma and pheochromocytoma are the major components. Gastroenteropancreatic neuroendocrine tumors are also listed among the typical complications, although these occur seldom. Virtually all such tumors are pancreatic islet cell tumors. VHL-associated islet cell tumors are mostly hormone-inactive. They can be detected during screening investigations according to the multidisciplinary disorder or by workup of space-occupying lesions. There are no specific predictors for malignancy in VHL-associated islet cell neoplasias, but tumors smaller than 3 cm in diameter are believed to be always benign. Gadolinium-enhanced MRI is currently the imaging method of choice, but contrast-enhanced CT is also a diagnostic option. The spectrum of manifestations is illustrated by selected cases.
© 2004 S. Karger AG, Basel
Article / Publication Details
Copyright / Drug Dosage / DisclaimerCopyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.