Hippel-Lindau disease: MR imaging

Yutaka Sato; Mary  Waziri; Wilbur  Smith; Edward Frey; William T C Yuh; James  Hanson; Edmund A Franken Jr

doi:10.1148/radiology.166.1.3336687

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Journal article

Peer reviewed

Hippel-Lindau disease: MR imaging

Yutaka Sato, Mary Waziri, Wilbur Smith, Edward Frey, William T C Yuh, James Hanson and Edmund A Franken Jr

Radiology, Vol.166(1), pp.241-246

1988

DOI: 10.1148/radiology.166.1.3336687

PMID: 3336687

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Abstract

Hippel-Lindau disease is an auto-somal-dominant disorder characterized by tumors arising from the central nervous system and abdominal viscera. Because of its progressive nature, frequent multisystem radiologic evaluation of affected persons and family members at risk is desirable for early detection and treatment. During a 2-year study, magnetic resonance (MR) imaging of the head, spine, and abdomen was used for screening and follow-up in 26 members of nine families with the disease. In addition to 13 previously diagnosed cases, five cases were newly diagnosed during the study. Lesions causing significant morbidity and mortality--such as cerebellar and spinal cord hemangioblastoma, renal cell carcinoma, and pheochromocytoma--were correctly depicted with MR imaging, sometimes before symptoms had developed and before the lesions could be seen with other imaging modalities. Central nervous system abnormalities were most clearly shown, but MR imaging also adequately demonstrated abdominal visceral abnormalities. It is therefore highly useful in the diagnosis and follow-up of Hippel-Lindau disease.

Neurology

Biological and medical sciences

Vascular diseases and vascular malformations of the nervous system

Medical sciences

Details

Title: Subtitle: Hippel-Lindau disease: MR imaging
Creators: Yutaka Sato - Univ. Iowa hosp. clin., dep. radiology, Iowa City IA 52242, United States
Mary Waziri - Univ. Iowa hosp. clin., dep. radiology, Iowa City IA 52242, United States
Wilbur Smith - Univ. Iowa hosp. clin., dep. radiology, Iowa City IA 52242, United States
Edward Frey - Univ. Iowa hosp. clin., dep. radiology, Iowa City IA 52242, United States
William T C Yuh - Univ. Iowa hosp. clin., dep. radiology, Iowa City IA 52242, United States
James Hanson - Univ. Iowa hosp. clin., dep. radiology, Iowa City IA 52242, United States
Edmund A Franken Jr - Univ. Iowa hosp. clin., dep. radiology, Iowa City IA 52242, United States
Resource Type: Journal article
Publication Details: Radiology, Vol.166(1), pp.241-246
Publisher: Radiological Society of North America; Oak Brook, IL
DOI: 10.1148/radiology.166.1.3336687
PMID: 3336687
ISSN: 0033-8419
eISSN: 1527-1315
Language: English
Date published: 1988
Academic Unit: Radiology; Stead Family Department of Pediatrics
Record Identifier: 9984051552402771

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