Tumors of the Heart, Primary (Excluding Myxoma)

KEY FEATURES

ESSENTIALS OF DIAGNOSIS

• Positive imaging study for characteristic cardiac masses

• Positive biopsy of cardiac masses

GENERAL CONSIDERATIONS

• Primary tumors of the heart (excluding myxomas) are rare (see Left Atrial Myxoma)

• Metastatic tumors to the heart occur 30 times more frequently than primary tumors

• Most primary cardiac tumors are benign:

  • – Fibromas

  • – Lipomas

  • – Papillary fibroelastomas

  • – Rhabdomyomas

• Malignant tumors include:

  • – Lymphomas

  • – Sarcomas

• Most benign cardiac tumors occur in children:

  • – Fibromas

  • – Hamartomas

  • – Hemangiomas

  • – Rhabdomyomas

• Symptoms and signs are determined by tumor location rather than histology

CLINICAL PRESENTATION

SYMPTOMS AND SIGNS

• Symptoms of heart failure, such as dyspnea

• Palpitation due to arrhythmias

• Constitutional symptoms:

  • – Weight loss

  • – Fatigue

  • – Fever

• Chest pain

• Asymptomatic with incidental finding on an imaging study

PHYSICAL EXAM FINDINGS

• Elevated jugular venous pressure with right heart tumors

• Signs of congestive heart failure

DIFFERENTIAL DIAGNOSIS

• Metastatic tumors

• Thrombus

• Nonbacterial thrombotic endocarditis

• Vegetations from infective endocarditis

• Flail or prolapsing valve leaflets

• Giant aneurysm of the coronary artery

• Pericardial cyst

• Diaphragmatic hernia

• Lipomatous cardiac infiltration

• Normal anatomic variants:

  • – Chiari network

  • – Eustachian valve

  • – Septum spurium

  • – Thebesian valve

  • – Left superior pulmonary vein

  • – Atrial septal aneurysm

DIAGNOSTIC EVALUATION

LABORATORY TESTS

• CBC: anemia, leukocytosis

• Elevated erythrocyte sedimentation rate

ELECTROCARDIOGRAPHY

• Conduction abnormalities

• Arrhythmias

IMAGING STUDIES

• Echocardiography:

  • – Excellent for endocardial tumors

  • – Good for myocardial tumors

  • – Poor for pericardial tumors

• CT and MRI:

  • – Excellent for paracardiac tumors and can often distinguish tissue type, such as lipoma

  • – Positron emission tomography (PET) imaging alone or combined with CT or MRI can help distinguish tumors from other masses and separate benign from malignant tumors

DIAGNOSTIC PROCEDURES

• Coronary angiography may be indicated before surgery

• Cardiac biopsy via catheter or limited surgery may be required

TREATMENT

CARDIOLOGY REFERRAL

• Suspected cardiac tumor

• Heart failure

• Chest pain

• Arrhythmias

HOSPITALIZATION CRITERIA

• Heart failure

• Hemodynamic compromise

• Need for procedure or surgery

MEDICATIONS

• Pharmacologic therapy is adjunctive for specific tumors

THERAPEUTIC PROCEDURES

• Radiation therapy is adjunctive for specific situations

SURGERY

• Surgery can be curative or can be used to reduce symptoms

• Cardiac transplantation is an alternative for unresectable tumors

MONITORING

• ECG monitoring in hospital as appropriate

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