Multiple neuroendocrine tumors of the pancreas in von Hippel-Lindau disease patients: histopathological and molecular genetic analysis.

@article{Lubensky1998MultipleNT,
  title={Multiple neuroendocrine tumors of the pancreas in von Hippel-Lindau disease patients: histopathological and molecular genetic analysis.},
  author={Irina A. Lubensky and Svetlana Pack and D. Ault and Alexander O. Vortmeyer and Steven K. Libutti and Peter L. Choyke and M. M. Walther and W. Marston Linehan and Zhengping Zhuang},
  journal={The American journal of pathology},
  year={1998},
  volume={153 1},
  pages={223-31}
}
Although pancreatic neuroendocrine tumors (NETs) in von Hippel-Lindau (VHL) disease have been reported, their pathological features have not been characterized. In addition, it is unknown whether alterations of the VHL gene are responsible for pancreatic NET development. To evaluate NETs in VHL patients, we performed histopathological analysis of 30 pancreatic tumors in 14 patients. In addition, DNA from NETs and normal pancreatic tissue from 6 patients with documented germ-line VHL gene… CONTINUE READING

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The presence of allelic deletions of the VHL gene in pancreatic NETs provides direct molecular evidence for a role of the gene in their tumorigenesis and establishes NET as an independent tumor type of VHL disease .
Islet Cell TumorMay be associated disease of diseaseVon Hippel-Lindau Syndrome
Although pancreatic neuroendocrine tumors ( NETs ) in von Hippel - Lindau ( VHL ) disease have been reported , their pathological features have not been characterized .
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