| Multiple focal nodular hyperplasia of the liver associated with vascular malformations of various organs and neoplasia of the brain: a new syndrome. | |
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MedLine Citation:
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PMID: 2813344 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Focal nodular hyperplasia (FNH) is a lesion of the liver in which a large anomalous artery is located within a region of hyperplastic hepatic parenchyma. Patients with FNH commonly have other lesions, often vascular in nature, in the liver or other organs. We have noted that these associated lesions almost always occur in patients with multiple FNH. We therefore studied 27 autopsied patients with FNH. All 13 with multiple FNH had other lesions such as hemangioma of liver, meningioma, astrocytoma, telangiectasis of the brain, berry aneurysm, dysplastic systemic arteries, and portal vein atresia. One patient had several of these lesions including multiple FNH, meningioma, astrocytoma, vascular malformation of the brain stem, and hemangioma of the liver. In contrast, among the 14 patients with solitary FNH there were no associated lesions, except for hepatic hemangioma in one patient. The prevalence of this syndrome was estimated by examination of 2500 serial autopsies and autopsies with various components of the syndrome. On review of 73 consecutive autopsies with meningioma, three had multiple FNH, compared with seven of 2500 consecutive adult autopsies (P less than 0.001). Multiple FNH was found in two of 83 autopsies with astrocytoma (P less than 0.05) and in one of 139 autopsies with berry aneurysm (not significant). We describe a telangiectatic subtype of FNH which occurs in this syndrome as well as in a minority of patients with solitary FNH. The existence and character of this syndrome suggest that there may be an underlying systemic abnormality in some patients having components of the syndrome. Investigation of patients with multiple FNH lesions may reveal significant treatable lesions. |
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Authors:
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I R Wanless; S Albrecht; J Bilbao; J V Frei; E J Heathcote; E A Roberts; D Chiasson |
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Publication Detail:
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Type: Case Reports; Journal Article |
Journal Detail:
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Title: Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc Volume: 2 ISSN: 0893-3952 ISO Abbreviation: Mod. Pathol. Publication Date: 1989 Sep |
Date Detail:
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Created Date: 1989-11-29 Completed Date: 1989-11-29 Revised Date: 2004-11-17 |
Medline Journal Info:
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Nlm Unique ID: 8806605 Medline TA: Mod Pathol Country: UNITED STATES |
Other Details:
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Languages: eng Pagination: 456-62 Citation Subset: IM |
Affiliation:
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Department of Pathology, Toronto Western Hospital, Ontario. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adolescent Astrocytoma / pathology Blood Vessels / abnormalities*, pathology Brain Neoplasms / pathology* Female Hemangioma / pathology* Humans Hyperplasia Intracranial Aneurysm / pathology Liver / pathology* Liver Neoplasms / pathology* Meningioma / pathology Middle Aged Syndrome |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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