Histopathology and molecular genetics of multiple cysts and microcystic (serous) adenomas of the pancreas in von Hippel-Lindau patients

Victoria H. Mohr, Alexander O. Vortmeyer, Zhengping Zhuang, Steven K. Libutti, McClellan M. Walther, Peter L. Choyke, Berton Zbar, W. Marston Linehan, Irina A. Lubensky

Research output: Contribution to journalArticle

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Abstract

Microcystic adenoma and cysts of the pancreas occur sporadically or as a part of von Hippel-Lindau (VHL) disease. The pathology of pancreatic cystic disease in VHL patients has not been well characterized. Furthermore, it is presently unknown whether the alteration of the VHL gene is responsible for the development of the entire spectrum of pancreatic serous cystic lesions. We performed a histopathological analysis of 21 cysts and 98 microcystic adenomas in nine VHL patients with a known germline mutation. In addition, PCR-amplified DNA from 27 pancreatic cystic lesions in three informative patients was studied for allelic deletions with polymorphic markers spanning the VHL gene locus. In all patients, pancreatic lesions were multiple: 21 benign serous cysts, 63 microscopic microcystic adenomas (size <0.4 cm), and 35 macroscopic microcystic adenomas (size >0.5 cm). The average number of lesions per patient was 2.1 benign cysts (range, 0-8), 7.7 (1-37) microscopic microcystic adenomas, and 3 (0-21) macroscopic microcystic adenomas. All lesions showed similar histology and contained prominent fibrous stroma, clear and/or amphophilic, glycogen-rich epithelial cells, endothelial and smooth muscle cells. VHL deletions were detected in all types of pancreatic cystic lesions. The presence of VHL gene allelic deletions in the spectrum of multifocal pancreatic cystic lesions provides direct molecular evidence of their neoplastic nature and integral association with VHL disease. The histopathological and molecular data establish a serous cyst-microcystic adenoma continuum in the development of pancreatic cystic neoplasia in VHL disease.

Original languageEnglish (US)
Pages (from-to)1615-1621
Number of pages7
JournalAmerican Journal of Pathology
Volume157
Issue number5
StatePublished - 2000
Externally publishedYes

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Adenoma
Cysts
Molecular Biology
Pancreas
von Hippel-Lindau Disease
Pancreatic Diseases
Germ-Line Mutation
Gene Deletion
Glycogen
Genes
Smooth Muscle Myocytes
Histology
Epithelial Cells
Pathology
Polymerase Chain Reaction
DNA
Neoplasms

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

Cite this

Mohr, V. H., Vortmeyer, A. O., Zhuang, Z., Libutti, S. K., Walther, M. M., Choyke, P. L., ... Lubensky, I. A. (2000). Histopathology and molecular genetics of multiple cysts and microcystic (serous) adenomas of the pancreas in von Hippel-Lindau patients. American Journal of Pathology, 157(5), 1615-1621.

Histopathology and molecular genetics of multiple cysts and microcystic (serous) adenomas of the pancreas in von Hippel-Lindau patients. / Mohr, Victoria H.; Vortmeyer, Alexander O.; Zhuang, Zhengping; Libutti, Steven K.; Walther, McClellan M.; Choyke, Peter L.; Zbar, Berton; Marston Linehan, W.; Lubensky, Irina A.

In: American Journal of Pathology, Vol. 157, No. 5, 2000, p. 1615-1621.

Research output: Contribution to journalArticle

Mohr, VH, Vortmeyer, AO, Zhuang, Z, Libutti, SK, Walther, MM, Choyke, PL, Zbar, B, Marston Linehan, W & Lubensky, IA 2000, 'Histopathology and molecular genetics of multiple cysts and microcystic (serous) adenomas of the pancreas in von Hippel-Lindau patients', American Journal of Pathology, vol. 157, no. 5, pp. 1615-1621.
Mohr, Victoria H. ; Vortmeyer, Alexander O. ; Zhuang, Zhengping ; Libutti, Steven K. ; Walther, McClellan M. ; Choyke, Peter L. ; Zbar, Berton ; Marston Linehan, W. ; Lubensky, Irina A. / Histopathology and molecular genetics of multiple cysts and microcystic (serous) adenomas of the pancreas in von Hippel-Lindau patients. In: American Journal of Pathology. 2000 ; Vol. 157, No. 5. pp. 1615-1621.
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AU - Walther, McClellan M.

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