Tumors of the Heart

Mary N Sheppard; Raad Mohiaddin

Disclosures

Future Cardiol. 2010;6(2):181-193. 

In This Article

Abstract and Introduction

Abstract

Cardiac Tumors are very rare but have devastating consequences given that they involve such an important organ. The majority of tumors are benign myxomas, which can present in very subtle ways causing a subsequent delay in diagnosis. Routine echocardiography is advised for anybody who is feeling generally unwell, since myxomas can cause pyrexia of unknown origin. The use of cardiac imaging has increased the early diagnosis of these tumors. A total of 25% of cardiac tumors are malignant sarcomas and these have a highly aggressive behavior. Early detection of these tumors while they are resectable offers a possibility for cure in the future.

Introduction

Primary tumors of the heart are rare. Metastatic tumors to, or that are directly invasive into, the heart are far more common. Approximately 75% of primary tumors are benign and 50–75% of these are atrial myxomas. The benign tumors include rhabdomyomas, fibromas, papillary fibroelastomas, hemangiomas, pericardial cysts, lipomas, hamartomas, teratomas and paragangliomas/pheochromocytomas. The malignant tumors consist of various sarcomas: myxosarcoma, liposarcoma, angiosarcoma, fibrosarcoma, leiomyosarcoma, osteosarcoma, synovial sarcoma, rhabdomyosarcoma, undifferentiated sarcoma, lymphoma, neurofibrosarcoma and malignant fibrous histiocytoma.

Primary tumors of the heart and pericardium have an incidence of between 0.0017 and 0.028% in collective series.[1] In the USA, based on data from 22 large autopsy series, the prevalence of primary cardiac tumors is approximately 0.02% (200 tumors per million autopsies). Approximately 75% of primary tumors are benign and 50% of benign tumors are myxomas, resulting in 75 cases of myxoma per million autopsies.[2]

In a Chinese study, 39 (95.1%) tumors were benign and included myxoma, fibroma and rhabdomyoma, while two (4.9%) tumors were malignant and included neurofibrosarcoma and malignant mesothelioma.[3] In an Italian series of 125 cases, 113 tumors were benign; myxoma was the most frequent tumor (87 cases) followed by pericardial cyst,[4] endocardial papilloma,[5] fibroma,[3] rhabdomyoma,[3] hematic cyst,[2] teratoma,[2] hemangioma,[1] celothelioma[1] and lipoma.[1] Malignancy was diagnosed in 12 cases, and consisted of pericardial mesothelioma,[3] myxosarcoma,[3] angiosarcoma,[2] fibrosarcoma[2] and leiomyosarcoma.[4]

At the Royal Brompton Hospital, London, UK, over the last 20 years, a total of 94 patients with a histological diagnosis of primary cardiac and pericardial tumors were identified. As observed in other series, the majority (n = 67; 71.3%) of cases were benign, with myxoma being the most common histologic type accounting for 27 cases. Primary malignant tumors (n = 27; 28.7%) included unclassified sarcoma (n = 11), leiomyosarcoma (n = 5) and lymphoma (n = 4) as the most common histologic types.[5]

Thus, there is a large spectrum of cardiac tumors with myxomas predominating among benign tumors while sarcomas predominate among malignant tumors.

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