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Extraskeletal myxoid chondrosarcoma of the heart and review of current literature.
MedLine Citation:
PMID:  20975880     Owner:  NLM     Status:  PubMed-not-MEDLINE    
Abstract/OtherAbstract:
Extraskeletal myxoid chondrosarcoma (EMS) is a rare oncologic phenomenon characterized by chondroid and neurogenic differentiation in extraskeletal locations. These tumours represent fewer than 2.5% of all soft-tissue sarcomas and are most commonly found in the lower extremities, limb girdles, distal extremities, and trunk. Their presence in cardiac tissue is exceedingly unusual; just a single case of ems metastatic to the heart has been reported, and no cases of primary cardiac EMS are known.Here, we report the case of a 26-year-old man who presented to his physician with a chest wall mass. Further evaluation led to the discovery of a large intracardiac mass with multiple end-organ growths. Complete work-up of this patient included cardiac biopsy, echocardiography, magnetic resonance imaging, positron-emission tomography, computed tomography, and fluorescence in situ hybridization studies for the translocation involving the EWSR1 gene locus (22q12). Results of the foregoing studies confirmed the diagnosis of ems, but the origin of this patient's tumours remains elusive and the contention between a primary cardiac source and cardiac metastasis has yet to be resolved.This article describes the histopathology, immunohistochemistry, and chromosomal aberrations common to ems, together with the common presenting features, natural history, and prognosis.
Authors:
H L Geyer; N Karlin
Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Current oncology (Toronto, Ont.)     Volume:  17     ISSN:  1198-0052     ISO Abbreviation:  Curr Oncol     Publication Date:  2010 Oct 
Date Detail:
Created Date:  2010-10-26     Completed Date:  2010-12-10     Revised Date:  2013-05-29    
Medline Journal Info:
Nlm Unique ID:  9502503     Medline TA:  Curr Oncol     Country:  Canada    
Other Details:
Languages:  eng     Pagination:  58-62     Citation Subset:  -    
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