Cardiac Neoplasms

Basics

Description

Tumors and cysts:
- Cysts and tumors of the pericardium and heart are rare, but are frequently detected when present due to improving imaging modalities of the heart structures. Pseudotumors must be differentiated from the tumors and cysts. It is important to identify the size, location, mobility, and attachment site of these masses.
- 94% of primary tumors are benign.
- Secondary tumors due to metastases are 20-40 times as common as primary tumors.
- Identification and resection of primary tumors is important because the result is likely a cure.
Resection of secondary tumors is palliative.
Metastatic cardiac tumors:
- 10% of malignant neoplasms metastasize to the heart; these metastases are clinically evident 10% of the time.
- Epicardium is the most common location of metastases.
- Carcinomatous invasion of the heart is more common than sarcomatous invasion.
- 90% of clinical presentations are related to pericardial involvement.
- Melanoma, lung, breast, leukemia, and lymphoma are the most common metastatic malignancies.
- The highest percentage of cardiac metastases are melanomas (70%), leukemia, lymphoma, breast, and lung.
- Extension is via direct (lung, breast, esophagus), retrograde lymphatic drainage (most carcinomas), venous extension (renal cell, hepatoma, gonadal), and hematogenous (sarcoma, leukemia, lymphoma, melanoma).
Benign cardiac tumors:
- Intracavitary neoplasms have the potential to cause constitutional symptoms, obstruction, and embolization.
- Myxoma are most common benign cardiac tumor: 75% left atrium, 23% right atrium, and 2% ventricular.
- 30-40% of myxomas demonstrate systemic embolization; embolization related to mobility, not size.
- Rhabdomyoma are most common in infants and typically multiple, pedunculated ventricular masses. Often regresses without surgery; associated with tuberous sclerosis.
- Fibromas most common in children; located in the ventricular free wall. Sudden death occurs in 33% due to conduction abnormalities.
- Lipomas range from small to large, may be located on valves, and can be mistaken for pericardial cysts.
- Hemangiomas may resolve spontaneously.
- Pericardial cyst; AV groove, clear fluid-filled.
Malignant cardiac tumors:
- Primary malignant cardiac neoplasm are mostly sarcomas; invade the right atrium. Frequently find bloody pericardial effusions.
- Local relapse in primary cardiac sarcoma is rare, most patients die from metastatic recurrence.
- Angiosarcoma most frequent, usually originates from the right atrium with large number of vascular channels. Metastases occur in up to 90% of cases, most commonly to the lung.
- Rhabdomyosarcoma is the 2nd most common primary malignancy in adults and has no chamber predilection; most common in children.
- Fibrosarcoma, liposarcoma, and primary malignant lymphoma are infrequent.
Pseudotumors:
- Thrombus, vegetation, coronary artery aneurysm, pheochromocytomas, lipomatous hypertrophy of the septum, Eustachian valve, Chiari network, hiatal hernia
Carcinoid heart disease:
- Carcinoid originates in the GI tract; secretes vasoactive substance serotonin, which is metabolized in the liver and lungs.
- Cardiac involvement after liver involvement; distinctive lesions on the tricuspid and pulmonic valves.
- Fibrous deposits on the tricuspid and pulmonic valves causing retraction of chordae with resultant tricuspid regurgitation and pulmonic stenosis and regurgitation.

Epidemiology

The ratio of metastatic cardiac tumors to primary cardiac tumors is 0.95. Primary neoplasms of the heart and pericardium have a prevalence of 0.01-0.28% in autopsy series. In adults, 1/2 of benign tumors are myxomas. In infants and children, the most common cardiac tumor is rhabdomyosarcoma. Malignant tumors are rare in the pediatric age group.
Incidence
The peak incidence for myxoma is in the 6th decade of life, and 70% of the cases occur in women. 25% of primary tumors are malignant and consist of angiosarcomas (33%), rhabdomyosarcomas (20%), mesotheliomas (15%), and fibrosarcomas(10%).

Risk Factors

10% of cardiac myxomas are familial (autosomal dominant) and are more likely to be multiple and can be located in the ventricular cavity; chromosomes 2 and 17.
Carcinoid heart disease results from hepatic metastases from ileal, bronchial, ovarian, and gonadal carcinoid.

Associated Conditions

Familial myxomas associated with NAME (nevi, atrial myxoma, myxoid neurofibroma, and ephelides), Carney syndrome.
Rhabdomyoma associated with tuberous sclerosis in 33% of patients; associated seizures.
Fibromas may be associated with Gorlin syndrome (basal cell nevus syndrome).
Cardiac hemangiomas associated with skin hemangiomas.

Diagnosis

Signs and symptoms:

Cardiac tumors can simulate common types of cardiac disease.
Signs and symptoms relate to tumor location.
Pericardial involvement is common with metastatic disease, often presenting with signs and symptoms of cardiac tamponade.
Intramyocardial neoplasms can present as conduction system abnormalities.
Left atrial myxomas often present with dyspnea, orthopnea, PND, pulmonary edema, and hemoptysis.
Right atrial or ventricular masses may produce symptoms and physical exam signs of right heart failure.
A diastolic tumor plop may result from right or left atrial myxoma; obstruction of mitral and tricuspid valves.
Myxomatous constitutional symptoms include fever, weight loss, Raynaud syndrome, digital clubbing, and anemia.
Rhabdomyomas may present with ventricular tachycardia, atrioventricular block, ventricular obstruction, pericardial effusion, and sudden death.
Angiosarcoma may produce a continuous precordial murmur secondary to multiple vascular channels.

Tests

Lab
Pericardiocentesis to obtain pericardial fluid for a definitive cytologic diagnosis.
Lab
Elevated ESR, white blood cell count, thrombocytopenia, CRP, and increased Î³-globulins can be seen with myxomas.
Imaging
InitialImagingApproach

Transesophageal echo (TEE) more sensitive and specific for identification of tumors of the heart and pericardium than transthoracic echo (TTE).
TEE better approximates size, shape, location, and stalk attachment for intracardiac masses; helps differentiate true tumors from pseudotumors.
TTE readily available and satisfactory to identify pericardial fluid, tamponade, and aids in pericardiocentesis.
CT is useful for diagnosis of cardiac/pericardial neoplasms but MRI provides improved definition of tumor characteristics.
Selective angiography can be useful in defining the vascular supply of tumors.

Pathological Findings

Carcinoid tumors contain fibroblasts, myofibroblasts, and smooth muscle cells in collagen.
Myxoma cell origin is undefined; microscopically the myxoid matrix is composed of mucopolysaccharide.
Carcinomatous metastases appear as multiple, small, discrete nodules on gross inspection.
Sarcomatous metastases exhibit diffuse infiltration of tissue.
Rhabdomyomas are white to yellow-tan; microscopically; they are circumscribed but not encapsulated and contain abundant glycogen.
Fibromas have calcific deposits.
Intramyocardial lipomas are encapsulated and small in size.

Treatment

Medication

First Line
Adjuvant treatment in resected primary neoplasms is controversial; adjuvant chemotherapy seems advisable in patients with high mitotic rate tumors and adjuvant radiation with high-grade tumors at risk for metastasis.

Additional Treatment

General Measures

Excision of primary tumors to prevent metastases or embolization
Treatment of metastatic tumor is directed at the underlying malignancy but may require surgical intervention for palliation.

Surgery

Pericardial tumor: Biopsy, pericardiocentesis, cardiac window or resection
Intrapericardial tumor resection
Surgical resection can be primary therapy for atrial myxoma, rhabdomyoma, fibroma, and lipoma.
Partial resection can be considered when it eliminates compromise to function of cardiac valves, coronary arteries, or conduction systems.
Hemangioma typically cannot be totally resected.
Surgery for malignant tumors is not recommended because of poor prognosis due to metastases.
Valve surgery can be performed for carcinoid cardiac involvement of the tricuspid and pulmonic valves.

Ongoing Care

Follow-Up Recommendations

Patient Monitoring
Noninvasive imaging with echo, CT, and MRI is used to perform surveillance following surgical excision and to monitor progression/regression of metastatic disease.

Prognosis

Recurrence of atrial myxomas is rare, usually within a 4-yr period.
Angiosarcoma and rhabdomyosarcoma have median survivals of 5-11 mo.
Patients with cardiac carcinoid have a similar survival to patients with metastatic carcinoid without myocardial involvement.

Additional Reading

1Goldman JH, Foster E. Transesophageal echocardiographic (TEE) evaluation of intracardiac and pericardial masses. Cardiol Clin. 2000;18:849-855. [View Abstract]2Meng Q, Lai H, Lima J. Echocardiographic and pathologic characteristics of primary cardiac tumors: A study of 149 cases. Int J Cardiol. 202;84:69-75.3Perchinsky MJ, Lichtenstein SV, Tyers GF. Primary cardiac tumors: Forty years' experience with 71 patients. Cancer. 1997;79:1809-1815. [View Abstract]

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