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Cardiac Tumors

Cardiac Tumors

1. Definition

A. Any benign or malignant neoplasm arising primarily from the myocardium or within a cardiac chamber
B. Approximately 70% are benign and 30% are malignant
C. Metastatic tumors are not classified as cardiac tumors
Papillary fibroelastomaMesothelioma
Granular cell tumorNeurogenic sarcoma
RhabdomyomaSynovial sarcoma

2. Myxoma

A. Morphology
1) Usually pedunculated with a short, broad-based attachment
2) Characteristically polypoid, project into cardiac chamber, and about 5-6 cm in diameter
3) Gelatinous/mucoid texture and covered with endothelium
4) Arises from endocardium as small, uniform cells in myxomatous stroma
5) Rarely metastasizes
B. Location
1) Left atrium: 75%
2) Right atrium: 20%
3) Atrial myxomas usually arise from the atrial septum
4) Ventricles: less than 10%
5) Multicentric: 5%
6) Valve: rare
C. Clinical Presentation
1) Hemodynamic impairment
a) Flow obstruction of venous drainage or across atrioventricular valves
b) Obstruction is usually progressive
c) Intermittent obstruction can cause syncope or sudden death in 1/4 to 1/2 of patients
2) Embolism
a) Occurs in 30 to 45% of left atrial myxomas and over 50% of left ventricular myxomas
b) About 50% of emboli will affect the CNS
c) Right-sided tumors have a lower frequency of embolism
3) Constitutional Symptoms
a) Occur in 30% of patients, most commonly with left atrial tumors
b) Include fever, weight loss, clubbing, Raynaud's, myalgias/arthralgias, hemolytic anemia
c) Laboratory tests may reveal elevated IgM or IgA globulins, ESR, and C-reactive protein
4) Familial Myxoma
a) Familial occurrence in 5% of patients
b) Usually found in young men, more often multiple, and less common in left atrium
c) 20% associated with other conditions: Sertoli cell tumors, Cushing syndrome, centrofacial/labial lentigenosis, cutaneous myxomas
d) Abnormal ploidy pattern in almost all familial cases
e) Strong tendency to recur
D. Diagnostic Tests
1) Echocardiography easily demonstrates the tumor mass and can often demonstrate the site of wall attachment
2) CT and MRI may have a role
3) Cineangiography demonstrates left atrial lesions on the venous phase after right heart injection
E. Indications for Operation
1) At the time of diagnosis
2) Presence of obstructive or embolic symptoms
3) Death occurs within 1 to 2 years after onset of obstructive symptoms
4) Up to 10% of patients will die from embolic complications prior to surgery
5) Natural history with constitutional symptoms only is unknown
F. Surgical Management
1) Complete resection with adequate margin, which usually results in only a small wall defect
2) Left atrial tumors are resected using a trans-septal approach
3) Right atrial tumors are resected through the right atrium
4) Ventricular tumors are resected through the atria
5) Tumor manipulation should be avoided to prevent embolization, and every effort made to remove the tumor intact
G. Results
1) Operative mortality is less than 3%
2) Slightly higher mortality with ventricular tumors
3) 30 to 75% of familial tumors recur

3. Papillary Fibroelastoma

A. Usually small, frond-like tumor that characteristically develops on an aortic or mitral leaflet
B. Core of dense connective tissue that resembles chordae tendinae
C. Produces embolism by fragmentation or thrombus formation
D. Affected valve can often be repaired at surgery
E. Incidental tumors found on the aortic or mitral valves during other surgery should probably be removed

4. Rhabdomyoma

A. Yellow-gray tumor that occur invariably in the ventricles, commonly in multiple locations
B. Altered myocytes, often not discrete from surrounding normal myocardium
C. Associated with tuberous sclerosis
D. Most common primary cardiac tumor in children
E. Cause cardiac failure from obstruction of conduction pathways and ventricular tachycardia
F. Over 90% present before age 15, usually in the first few days of life
G. May require EPS to locate tumors that do not project into ventricular cavity
H. Tumors may be unresectable at surgery
I. About 50% of survivors will eventually develop tuberous sclerosis

5. Fibroma

A. Large, bulky tumors that occur exclusively in the ventricles or ventricular septum
B. Characteristic whorled appearance from fibroblasts, collagen, and elastic fibers
C. Usually present in childhood
D. Free wall tumors can be enucleated without entering the ventricular cavity
E. Results are good, although few patients available for followup

6. Lipoma

A. Well-encapsulated tumors, usually found incidentally
B. Consist of mature fat cells
C. Most commonly occur in atrial septum as part of lipomatous hypertrophy of the interatrial septum
D. Incidental tumors should probably be resected
E. Results are generally good

7. Pheochromocytoma

A. Soft, fleshy tumors occurring in the pericardium and epicardial surface
B. Functionally active chromaffin cells produce large amounts of catecholamines
C. Presents with severe hypertension
D. Diagnosed by urinary catecholamines, CT scanning, and scintography
E. Preoperative alpha- and beta-blockade is mandatory
F. Carefully dissect tumor away from underlying cardiac structures
G. Outcome favorable unless malignant

8. Teratoma

A. Pale, cystic tumor with heterogenous features
B. Up to 20% have malignant features
C. Typically present in very young patients
D. Presence is an indication for operation
E. Results unknown, as very few cases reported

9. Sarcoma

A. Malignant tumor with wide variety of types, originating from mesenchyme
B. Subtypes include angiosarcoma, rhabdomyosarcoma, fibrosarcoma, osteosarcoma, neurogenic sarcoma, leiomyosarcoma, iiposarcoma, and synovial sarcoma
C. Most commonly occur in right atrium and in mid-adulthood
D. Operation indicated to obtain accurate diagnosis
E. Prognosis is poor, as most patients have distant metastases at presentation
F. Adjuvant therapy may have some role

10. Metastatic Tumors

A. Most common neoplastic process involving the heart
B. Common tumors that metastasize to the heart include leukemia, lymphoma, melanoma, lung cancer, and breast cancer
C. Most frequently metastasize to pericardium, then myocardium and endocardium
D. Clinical presentations include pericardial effusion, tamponade, failure, and arrhythmias
E. Prognosis depends on treatment of primary malignancy

11. Right Atrial Extension of Infradiaphragmatic Tumor

A. Most commonly occurs with renal tumors extending up inferior vena cava
B. Usually do not cause cardiac symptoms
C. Combined median sternotomy/midline laparotomy with cardiopulmonary bypass
D. Remove IVC and right atrial portions of tumor prior to primary renal mass