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

doi:10.1097/PAS.0b013e3180377aaf

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

Laboratory Medicine and Pathology

Research output: Contribution to journal › Article › peer-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 language	English (US)
Pages (from-to)	1292-1296
Number of pages	5
Journal	American Journal of Surgical Pathology
Volume	31
Issue number	8
DOIs	https://doi.org/10.1097/PAS.0b013e3180377aaf
State	Published - Aug 2007

Keywords

Cyst
Lung
Von Hippel-Lindau

ASJC Scopus subject areas

Anatomy
Surgery
Pathology and Forensic Medicine

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10.1097/PAS.0b013e3180377aaf

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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.",

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T1 - Multifocal microcysts and papillary cystadenoma of the lung in von Hippel-Lindau disease

AU - Klein, Julianne

AU - Zhuang, Zhengping

AU - Lubensky, Irina

AU - Colby, Thomas V.

AU - Martinez, Felix

AU - Leslie, Kevin O.

PY - 2007/8

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N2 - 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.

AB - 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.

KW - Cyst

KW - Lung

KW - Von Hippel-Lindau

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

Abstract

Keywords

ASJC Scopus subject areas

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