Von Hippel Lindau Disease:
Genetic, Clinical and Imaging Features

Peter L. Choyke, M.D., Gladys M. Glenn, M.D., Ph.D., McClellan M. Walther, M.D., Nicholas J. Patronas, M.D., W. Marston Linehan, M.D., Berton Zbar, M.D.

Radiology (March) 146:629-642,1995

Table of Contents

Abstract
History
Clinical Manifestations
Genetics
Retina Hemangioblastomas
CNS Hemangioblastomas
Endolymphatic Sac Tumors
Renal Cysts and Tumors
Adrenal Tumors
Pancreatic Cystic Disease
Pancreatic Islet Cell Tumors
Papillary Cystadenoma of the Epididymis and Broad Ligament
Other Lesions Associated with VHL Disease
Current Screening Recommendations
The Future
Appendix 1
Appendix 2
References

Note:   Figures 5-20 have been scanned at 50%. The full image can be viewed with an Helper Application that reads JPEG files and by double-clicking the image.

From the:

  1. Diagnostic Radiology Department, Warren Grant Magnuson Clinical Center, Henry M. Jackson Foundation, National Institutes of Health, Bethesda, MD 20892

  2. Cancer Diagnosis Branch, Division of Cancer Biology, Diagnosis and Centers, National Cancer Institute, National Institutes of Health, Bethesda, MD 20892

  3. Surgery Branch, Urologic Oncology Division, National Cancer Institute, National Institutes of Health, Bethesda, MD 20892

  4. Laboratory of Immunobiology, Frederick Cancer Research Facility, National Cancer Institute, National Institutes of Health, Frederick, MD 21701

Corresponding Author

Peter L. Choyke, M.D.
Diagnostic Radiology Department
Warren Grant Magnuson Clinical Center
National Institutes of Health
Bldg. 10, Room 1C660
10 Center Dr 1182
Bethesda, MD 20892-1182

Phone:(301)496-7700
FAX: (301)496-9933
ELECTRONIC MAIL: pchoyke@pop.cc.nih.gov

Acknowledgements

The authors wish to thank Barbara McCoy for her help in manuscript preparation, and Adam Frank MD, Kathleen Hurley RN, Cia Manatos RN, and Kailash Thakore RDMS.