von Hippel-Lindau disease

Synopsis

 VHL-associated pulmonary anomalies (#17667557#)

• VHL-associated multifocal microcysts (#17667557#)
• VHL-associated pulmonary papillary cystadenoma of the lung (#17667557#)

 VHL-associated pancreatic anomalies

• VHL-associated pancreatic cysts

 VHL-associated renal anomalies

• VHL-associated renal cell carcinoma
  • VHL-associated cystic renal cell carcinoma

Reviews

 Maher ER. Von Hippel-Lindau disease. Curr Mol Med. 2004 Dec;4(8):833-42. PMID: #15579030

 Barry RE, Krek W. The von Hippel-Lindau tumour suppressor: a multi-faceted inhibitor of tumourigenesis. Trends Mol Med. 2004 Sep;10(9):466-72. PMID: #15350900#

 George DJ, Kaelin WG Jr. The von Hippel-Lindau protein, vascular endothelial growth factor, and kidney cancer. N Engl J Med. 2003 Jul 31;349(5):419-21. PMID: #12890838#

 Kaelin WG Jr. Molecular basis of the VHL hereditary cancer syndrome. Nat Rev Cancer. 2002 Sep;2(9):673-82. PMID: #12209156#

 Kaelin WG Jr, Maher ER. The VHL tumour-suppressor gene paradigm. Trends Genet. 1998 Oct;14(10):423-6. PMID: #9820032#

 Friedrich CA. Genotype-phenotype correlation in von Hippel-Lindau syndrome. Hum Mol Genet. 2001 Apr;10(7):763-7. PMID: #11257110#

References

 Klein J, Zhuang Z, Lubensky I, Colby TV, Martinez F Jr, Leslie KO. Multifocal microcysts and papillary cystadenoma of the lung in von Hippel-Lindau disease. Am J Surg Pathol. 2007 Aug;31(8):1292-6. PMID: #17667557#