Document Detail

Huge primary pleomorphic leiomyosarcoma in the right ventricle with impending obstruction of both inflow and outflow tracts.
MedLine Citation:
PMID:  19075518     Owner:  NLM     Status:  MEDLINE    
Primary cardiac leiomyosarcoma is an extremely rare disease entity that is associated with very poor prognosis. We describe here a 45-year-old man who had a huge pleomorphic leiomyosarcoma in the right ventricle (RV) that presented with signs of acute pressure and volume overload and impending obstruction of both outflow and inflow tracts of the RV. The tumor was attached to the RV apex and the interventricular septum, and extended into the main pulmonary trunk just above the pulmonary valve as well as into the right atrium through the tricuspid valve. We evaluated the extent of the tumor using various imaging modalities including transthoracic and transesophageal echocardiography, magnetic resonance imaging, computed tomography, and positron emission tomography-computed tomography. Although he underwent urgent debulking surgery to relieve the obstruction, the tumor could not be resected completely because of its extensive local invasion. The tumor has shown aggressive regrowth after surgery despite adjuvant chemotherapy.
Sun Hwa Lee; Won Ho Kim; Jong Bum Choi; Sang Rok Lee; Kyoung Suk Rhee; Jei Keon Chae; Jae Ki Ko
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Publication Detail:
Type:  Case Reports; Journal Article     Date:  2008-12-15
Journal Detail:
Title:  Circulation journal : official journal of the Japanese Circulation Society     Volume:  73     ISSN:  1346-9843     ISO Abbreviation:  Circ. J.     Publication Date:  2009 Apr 
Date Detail:
Created Date:  2009-03-25     Completed Date:  2009-06-30     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101137683     Medline TA:  Circ J     Country:  Japan    
Other Details:
Languages:  eng     Pagination:  779-82     Citation Subset:  IM    
Division of Cardiology, Department of Internal Medicine, Chonbuk National University Medical School, Jeonju, Republic of Korea.
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MeSH Terms
Blood Flow Velocity
Heart Neoplasms / pathology*,  surgery,  therapy
Heart Ventricles / pathology,  radiography
Leiomyosarcoma / pathology*,  surgery,  therapy
Middle Aged
Positron-Emission Tomography*

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