Abstract

Cardiac tumors are rare, and of these, primary cardiac tumors are even rarer. Metastatic cardiac tumors are about 100 times more common than the primary tumors. About 90% of primary cardiac tumors are benign, and of these the most common are cardiac myxomas. Approximately 12% of primary cardiac tumors are completely asymptomatic while others present with one or more signs and symptoms of the classical triad of hemodynamic changes due to intracardiac obstruction, embolism and nonspecific constitutional symptoms. Echocardiography is highly sensitive and specific in detecting cardiac tumors. Other helpful investigations are chest X-rays, magnetic resonance imaging and computerized tomography scan. Surgical excision is the treatment of choice for primary cardiac tumors and is usually associated with a good prognosis. This review article will focus on the general features of benign cardiac tumors with an emphasis on cardiac myxomas and their molecular basis.

Highlights

  • Cardiac tumors are rare and are divided into primary and metastatic tumors with an autopsy series showing metastatic cardiac tumors to be about 100 times more common than primary cardiac tumors [1]

  • Rare case reports of myxoid leiomyosarcoma and angiosarcoma mimicking clinically, radiologically and grossly as myxoma are present in the literature [69,70,71]

  • Myxoid Liposarcoma is a malignant tumor composed of a mixture of uniform round to oval shaped primitive non-lipogenic mesenchymal cells and small signet ring lipoblasts in a prominent myxoid stroma, rich in a delicate arborizing, “chicken wire” capillary vasculature

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Summary

Introduction

Cardiac tumors are rare and are divided into primary and metastatic tumors with an autopsy series showing metastatic cardiac tumors to be about 100 times more common than primary cardiac tumors [1] In another autopsy series, the prevalence of primary cardiac tumors was found to be 0.001%–0.03% [2]. Recent studies show that papillary fibroelastomas are the most common primary benign cardiac tumors [5,6]. Amongst the primary malignant cardiac tumors, the most common ones are sarcomas (90%) followed by lymphomas [4]. The clinical presentation of primary cardiac tumors depends on their site, size, mobility and infiltration of adjacent structures. Malignant primary cardiac tumors may cause symptoms relatable to metastases to different organs such as the lungs, brain and bones

Cardiac Myxomas
Histogenesis
Clinical Presentation
Macroscopy
Histopathology
Immunohistochemistry
Electron Microscopy
Differential Diagnosis
Inflammatory Myofibroblastic Tumor
Myxofibrosarcoma
Others
Diagnosis of Primary Benign Cardiac Tumors
Treatment
Prognosis
Molecular Genetics of Cardiac Myxomas
Markers of Poor Prognosis
Drug Targets in CM
Cytogenetic
Findings
Conclusions
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