Document Detail


Regional Proteus syndrome: report of an autopsy case.
MedLine Citation:
PMID:  7551015     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
An uncommon autopsy case involving Proteus syndrome with multiple hamartomatous lesions in a 52 year old woman is reported. At birth, hemihypertrophy was noted on the right side of the face. Leiomyoma of the urinary bladder was extirpated at 37 years of age. She died of sepsis due to a brain abscess. At autopsy, diffuse asymmetrical lipomatosis was noted on the right side of the face, scalp, lip, oral mucosa, tongue and on the left side of the cerebellar peduncle. Multiple meningiomas, cavernous hemangiomas and osseous hypertrophy overlapped in the intracranial regions. The present case is considered as a regional type of Proteus syndrome displaying a somatic mosaicism.
Authors:
Y Horie; H Fujita; S Mano; M Kuwajima; K Ogawa
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Pathology international     Volume:  45     ISSN:  1320-5463     ISO Abbreviation:  Pathol. Int.     Publication Date:  1995 Jul 
Date Detail:
Created Date:  1995-11-07     Completed Date:  1995-11-07     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  9431380     Medline TA:  Pathol Int     Country:  AUSTRALIA    
Other Details:
Languages:  eng     Pagination:  530-5     Citation Subset:  IM    
Affiliation:
Department of Clinical Laboratory Medicine, Faculty of Medicine, Tottori University, Yonago, Japan.
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MeSH Terms
Descriptor/Qualifier:
Brain Neoplasms / pathology
Cerebellum / pathology
Diagnosis, Differential
Female
Hemangioma / pathology
Humans
Lipomatosis / pathology
Meningioma / pathology
Middle Aged
Proteus Syndrome / pathology*
Skull / pathology
Tomography, X-Ray Computed
Tongue / pathology

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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