Ventricular Outflow Tract Obstruction Secondary to Leiomyosarcoma of the Right Ventricle

Autor: Jan Roelandts, Amir Shoja, Herve Deferm, Philip Claessens, Willem Willaert, Andre Heremans, Marc Vanderheyden
Rok vydání: 2001
Předmět:
Zdroj: Japanese Heart Journal. 42:377-386
ISSN: 1348-673X
0021-4868
DOI: 10.1536/jhj.42.377
Popis: Primary leiomyosarcomas of the heart, particularly those affecting the right ventricle, are uncommon. We report the case of a 70-year-old Belgian woman presenting with the symptoms of progressive exertional dyspnea and left-sided pleuritic pain. A leiomyosarcoma which originated from the right lateral ventricle wall, causing pulmonary outflow obstruction, was diagnosed. Pathology revealed a neoplasm with a myxoid stroma, high mitotic activity and nuclei expressing atypia. Immunohistochemical staining was positive for vimentine and desmin. Seven months after complete surgical resection the tumor relapsed. This case demonstrates the poor outcome, the high relapse rate and inefficiency of treatment associated with primary cardiac leiomyosarcomas. The current literature regarding the incidence, diagnostic techniques, treatment strategies and survival rates of this rare but terminal disease is reviewed.
Databáze: OpenAIRE