Elsevier

The Lancet Oncology

Volume 6, Issue 4, April 2005, Pages 219-228
The Lancet Oncology

Review
Cardiac tumours: diagnosis and management

https://doi.org/10.1016/S1470-2045(05)70093-0Get rights and content

Summary

Primary cardiac tumours are rare, with an autopsy incidence ranging from 0·001% to 0·030%. Three-quarters of these tumours are benign and nearly half of the benign tumours are myxomas. Metastases to the heart are far more common than primary cardiac tumours. Primary cardiac tumours present with one or more of the symptoms of the classic triad of: cardiac symptoms and signs resulting from intracardiac obstruction; signs of systemic embolisation; and systemic or constitutional symptoms. They are diagnosed by use of transthoracic and transoesophageal echocardiograms, MRI, and CT scan. Whereas surgery is indicated in patients with benign tumours, systemic chemotherapy is indicated in those who have widespread or unresectable malignant disease, and chemotherapy and radiotherapy are usually combined in treatment of patients with primary cardiac lymphomas. The prognosis after surgery is usually excellent in the case of benign tumours but is unfortunately still limited in localised malignant diseases. Patients with sarcomas live for a mean of 3 months to 1 year, and those with lymphomas live up to 5 years if treated, but usually die within 1 month if untreated.

Section snippets

Clinical features

Cardiac tumours have many clinical presentations and are known to be great mimickers (panel). Most patients have disseminated metastases, and the diagnosis is made incidentally during assessment of the primary lesion.5 Primary cardiac tumours present with one or more symptoms of the classic triad of: cardiac symptoms and signs resulting from intracardiac obstruction; signs of systemic embolisation; and systemic or constitutional symptoms.5, 6

Dyspnoea and orthopnoea from pulmonary venous

Cardiac myxomas

Cardiac myxomas are the commonest primary cardiac neoplasm in adults, with a mean age at diagnosis of 50 years, and 90% of patients are aged 30–60 years.9 They are found mainly in females,6 and usually arise in the atrial fossa ovalis region; about 75% arise in the left atrium, and 15–20% in the right atrium.5 An atrial myxoma sometimes obstructs the pulmonary or systemic venous drainage and interferes with valve function. Cardiac myxomas can cause recurrent strokes, peripheral or pulmonary

Diagnosis and monitoring

The techniques mainly used in diagnosis of cardiac lesions include transthoracic and transoesophageal echocardiogram, and MRI and CT scans. Echocardiography can quantify the tumour size, shape, attachment, and mobility, and can screen other cardiac chambers accurately for additional tumours.37 Before resection, transoesophageal echocardiography can reconfirm anatomical localisation, quantify baseline valvular stenosis or regurgitation, and guide cannulation.

Preoperative workup should include

Indications for and timing of surgery

Patients can undergo surgery for prognostic or symptomatic considerations. In most patients with benign tumours, or in those with resectable primary malignant disease and no evidence of metastasis, surgery is generally indicated to improve prognosis. By contrast, patients with a small right-sided papillary fibroelastoma,38 no symptoms, and without a patent foramen ovale or atrial septal defect can be managed non-surgically with continued echocardiographic follow-up. Patients with a primary

Search strategy and selection criteria

Data for this review were found by searches of PubMed, and references from relevant articles using the search terms, “cardiac neoplasms”, “myxomas”, “metastatic heart neoplasms”, “angiosarcoma”, and “rhabdomyosarcoma”. Abstracts and reports from meetings were not included. Contemporary textbooks were reviewed. Only papers published in English between 1980 and 2004 were included.

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