American Journal of Roentgenology, Vol 162, 1091-1094, Copyright © 1994 by American Roentgen Ray Society

ARTICLES

Pancreatic lesions in von Hippel-Lindau disease: prevalence, clinical significance, and CT findings

DM Hough, DH Stephens, CD Johnson and LA Binkovitz
Department of Diagnostic Radiology, Mayo Clinic, Rochester, MN 55905.

OBJECTIVE. The purposes of this study were to determine the nature, prevalence, and CT findings of pancreatic lesions in patients with von Hippel-Lindau disease and to determine whether identification of pancreatic cysts and neoplasms is important in establishing the diagnosis of von Hippel-Lindau disease. SUBJECTS AND METHODS. The medical records and radiologic images of 52 patients with von Hippel- Lindau disease who were evaluated at our institution between 1976 and 1992, and who at some stage underwent abdominal CT, sonography, or MR imaging, were reviewed. The nature, prevalence, and CT findings of the pancreatic lesions were determined, and the role of the pancreatic abnormalities in establishing the diagnosis of von Hippel-Lindau disease was studied. RESULTS. Twenty-nine (56%) of the 52 patients had pancreatic lesions. Nineteen patients had pancreatic cysts and no other pancreatic lesion. Four patients had islet cell tumors only, one had a microcystic adenoma only, and three had indeterminate pancreatic masses. One patient had cysts and an islet cell tumor, and another patient had cysts, an islet cell tumor, and a microcystic adenoma. In six patients (12%), pancreatic lesions were the only abdominal manifestation of von Hippel-Lindau disease. In three patients screened because of a family history of von Hippel-Lindau disease, no CNS abnormalities were present, and the only abdominal lesions were in the pancreas (cysts in two cases, islet cell carcinoma in the other). Thus, the pancreatic lesion was an important factor in establishing a diagnosis of von Hippel-Lindau disease in these patients. CONCLUSION. Pancreatic lesions may be the only abdominal manifestation of von Hippel-Lindau disease. CT findings include cysts, islet cell tumors, and microcystic adenomas. Pancreatic lesions, including cysts, may precede any other manifestation of von Hippel-Lindau disease by several years, and recognition permits earlier diagnosis in patients being screened for von Hippel-Lindau disease.
CiteULike    Complore    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this?

This article has been cited by other articles:

				H. J. Kim, D. H. Lee, Y. T. Ko, J. W. Lim, H. C. Kim, and K. W. Kim CT of Serous Cystadenoma of the Pancreas and Mimicking Masses Am. J. Roentgenol., February 1, 2008; 190(2): 406 - 412. [Abstract] [Full Text] [PDF]

				R. S. Leung, S. V. Biswas, M. Duncan, and S. Rankin Imaging Features of von Hippel-Lindau Disease RadioGraphics, January 1, 2008; 28(1): 65 - 79. [Abstract] [Full Text] [PDF]

				E. R Woodward and E. R Maher Von Hippel-Lindau disease and endocrine tumour susceptibility. Endocr. Relat. Cancer, June 1, 2006; 13(2): 415 - 425. [Abstract] [Full Text] [PDF]

				K. J. Mortele, T. C. Rocha, J. L. Streeter, and A. J. Taylor Multimodality Imaging of Pancreatic and Biliary Congenital Anomalies RadioGraphics, May 1, 2006; 26(3): 715 - 731. [Abstract] [Full Text] [PDF]

				S. J. Handrich, D. M. Hough, J. G. Fletcher, and M. G. Sarr The Natural History of the Incidentally Discovered Small Simple Pancreatic Cyst: Long-Term Follow-Up and Clinical Implications Am. J. Roentgenol., January 1, 2005; 184(1): 20 - 23. [Abstract] [Full Text] [PDF]

				T. C. Demos, H. V. Posniak, C. Harmath, M. C. Olson, and G. Aranha Cystic Lesions of the Pancreas Am. J. Roentgenol., December 1, 2002; 179(6): 1375 - 1388. [Full Text] [PDF]

				H. B. Marcos, S. K. Libutti, H. R. Alexander, I. A. Lubensky, D. L. Bartlett, M. M. Walther, W. M. Linehan, G. M. Glenn, and P. L. Choyke Neuroendocrine Tumors of the Pancreas in von Hippel-Lindau Disease: Spectrum of Appearances at CT and MR Imaging with Histopathologic Comparison Radiology, December 1, 2002; 225(3): 751 - 758. [Abstract] [Full Text] [PDF]

				S. T. Lott, D. S. Chandler, S. A. Curley, C. J. Foster, A. El-Naggar, M. Frazier, L. C. Strong, M. Lovell, and A. M. Killary High Frequency Loss of Heterozygosity in von Hippel-Lindau (VHL)-associated and Sporadic Pancreatic Islet Cell Tumors: Evidence for a Stepwise Mechanism for Malignant Conversion in VHL Tumorigenesis Cancer Res., April 1, 2002; 62(7): 1952 - 1955. [Abstract] [Full Text] [PDF]

				V. H. Mohr, A. O. Vortmeyer, Z. Zhuang, S. K. Libutti, M. M. Walther, P. L. Choyke, B. Zbar, W. M. Linehan, and I. A. Lubensky Histopathology and Molecular Genetics of Multiple Cysts and Microcystic (Serous) Adenomas of the Pancreas in von Hippel-Lindau Patients Am. J. Pathol., November 1, 2000; 157(5): 1615 - 1621. [Abstract] [Full Text] [PDF]

				I. A. Lubensky, S. Pack, D. Ault, A. O. Vortmeyer, S. K. Libutti, P. L. Choyke, M. M. Walther, W. M. Linehan, and Z. Zhuang Multiple Neuroendocrine Tumors of the Pancreas in von Hippel-Lindau Disease Patients : Histopathological and Molecular Genetic Analysis Am. J. Pathol., July 1, 1998; 153(1): 223 - 231. [Abstract] [Full Text] [PDF]