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Maffucci's syndrome: Two case reports with a literature review

Identifieur interne : 005845 ( Istex/Corpus ); précédent : 005844; suivant : 005846

Maffucci's syndrome: Two case reports with a literature review

Auteurs : Richard P. Kaplan ; James T. Wang ; David M. Amron ; Leo Kaplan

Source :

RBID : ISTEX:BC1658E90B35EE2E46A0B8B7F261095A43E4B84F

Abstract

Sixty-three cases of Maffucci's syndrome in the English literature plus two additional cases of our own are reviewed. This syndrome is nonhereditary and is characterized by multiple enchondromas and hemangiomas. It occurs in all races with no sex predominance. The enchondromas and hemangiomas can occur anywhere but are most common in the hands. Long bone involvement is common and leads to progressive skeletal deformity and pathologic fractures. The incidence of malignancies in patients with this syndrome is high. Chondrosarcomas are especially common and occur in 30% oT the patients.

Url:
DOI: 10.1016/0190-9622(93)70265-U

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ISTEX:BC1658E90B35EE2E46A0B8B7F261095A43E4B84F

Le document en format XML

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<sb:title>
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<affiliation>Division of Dermatology, University of California, Los Angeles, Medical CenterDepartment of Pathology, Cedars Sinai Medical Center</affiliation>
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<namePart type="given">James T.</namePart>
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<namePart type="given">David M.</namePart>
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<affiliation>Division of Dermatology, University of California, Los Angeles, Medical CenterDepartment of Pathology, Cedars Sinai Medical Center</affiliation>
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<abstract>Sixty-three cases of Maffucci's syndrome in the English literature plus two additional cases of our own are reviewed. This syndrome is nonhereditary and is characterized by multiple enchondromas and hemangiomas. It occurs in all races with no sex predominance. The enchondromas and hemangiomas can occur anywhere but are most common in the hands. Long bone involvement is common and leads to progressive skeletal deformity and pathologic fractures. The incidence of malignancies in patients with this syndrome is high. Chondrosarcomas are especially common and occur in 30% oT the patients.</abstract>
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<date>199311</date>
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