Multifocal microcysts and papillary cystadenoma of the lung in von Hippel-Lindau disease

Julianne Klein, Zhengping Zhuang, Irina Lubensky, Thomas V. Colby, Felix Martinez, Kevin O. Leslie

Research output: Contribution to journalArticlepeer-review

9 Scopus citations

Abstract

von Hippel-Lindau disease is an autosomal dominant inherited disorder characterized by a predisposition to multiple neoplasms. Renal cell carcinoma and hemangioblastomas of the retina and cerebellum are the most common of these, but other neoplasms and cysts also occur throughout the body. We report a distinctive, yet never described lung lesion in a 43-year-old woman with von Hippel-Lindau disease. Molecular genetic studies confirmed the presence of a VHL gene mutation in the cells of this lesion. We discuss the salient features of this novel lesion, and hypothesize on its origin and nature.

Original languageEnglish (US)
Pages (from-to)1292-1296
Number of pages5
JournalAmerican Journal of Surgical Pathology
Volume31
Issue number8
DOIs
StatePublished - Aug 2007

Keywords

  • Cyst
  • Lung
  • Von Hippel-Lindau

ASJC Scopus subject areas

  • Anatomy
  • Surgery
  • Pathology and Forensic Medicine

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