Ventricular Outflow Tract Obstruction Secondary to Leiomyosarcoma of the Right Ventricle.

Abstract

SUMMARYPrimary 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. (Jpn Heart J 2001; 42: 377-386)

Pulmonary Artery Obstruction due to Papillary Fibroelastoma on the Pulmonary Valve: A Rare Cardiac Tumor

“…A differential diagnosis of PFE or leiomyosarcomas is helpful to plan the therapy strategies, as it can indicate the probable survival rate before the surgical treatment. MRI is useful in preoperative surgical staging and helps to determine the benign or malignant origin of the neoplasm [9]. In a meta-analysis, Gowda et al documented a 50% cardiac PFErelated mortality rate in patients who did not undergo curative resection for either systemic embolism or outflow obstruction [2].…”