RAY ALBERT R.

CABIGAN, MD
INTERNIST
INTERVENTIONAL CARDIOLOGY

CARDIAC TUMORS
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CASE #1
▸ 63/F

▸ Cc: easy fatigability and palpitations

▸ No known comorbidities

▸ BP 90/60

▸ HR 122bpm

▸ Irregularly irregular rhythm,

▸ Gr 2/6 diastolic murmur, louder

when lying at the left
MYXOMA
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PRIMARY TUMORS
▸ Approximately three-quarters are histologically benign,
and the majority of these tumors are myxomas

▸ Malignant tumors, almost all of which are sarcomas,

account for 25% of primary cardiac tumors
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MYXOMA
▸ Most common cardiac tumor in adults

▸ Occurs in all ages, most commonly in the third through

sixth decades, with a female predilection

▸ Carney Complex: familial sindome consisting of (1)

myxomas (cardiac, skin, and/or breast), (2) lentigines and/
or pigmented nevi, and (3) endocrine overactivity (primary
nodular adrenal cortical disease with or without Cushing’s
syndrome, testicular tumors, and/or pituitary adenomas
with gigantism or acromegaly)
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MYXOMA
▸ NAME syndrome: nevi, atrial myxoma, myxoid neurofibroma,
and ephelides

▸ LAMB syndrome: lentigines, atrial myxoma, and blue nevi

▸ Pathologically, myxomas are gelatinous structures that consist of

myxoma cells embedded in a stroma rich in
glycosaminoglycans.

▸ Mostly arise from the interatrial septum in the vicinity of the

fossa ovalis (particularly in the left atrium), and are often
pedunculated on a fibrovascular stalk
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MYXOMA
▸ The most common clinical presentation mimics that of
mitral valve disease: either stenosis owing to tumor
prolapse into the mitral orifice or regurgitation resulting
from tumor-induced valvular trauma or distortion.

▸ A characteristic lowpitched sound, a “tumor plop,” may

be appreciated on auscultation during early or mid-
diastole and is thought to result from the impact of the
tumor against the mitral valve or ventricular wall
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TREATMENT
▸ Surgical excision using cardiopulmonary bypass is
indicated regardless of tumor size, and is generally
curative.

▸ Myxomas recur in 12–22% of familial cases but in only 1–

2% of sporadic cases
OTHER BENIGN
TUMORS
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CARDIAC LIPOMA
▸ Usually incidental findings at
postmortem examination;
however, they may grow as large
as 15 cm

▸ Should be resected if they produce

symptoms owing to mechanical
interference with cardiac function,
arrhythmias, or conduction
disturbances
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PAPILLARY FIBROELASTOMA
▸ Friable tumors with frond-like
projections that are usually solitary
and are the most common tumors of
the cardiac valves

▸ Possibly arises from chronic viral

endocarditis

▸ Clinically silent

▸ Can cause valve dysfunction and may

embolize distally, resulting in transient
ischemic attacks, stroke, or myocardial
infarction
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RHABDOMYOMAS AND FIBROMAS

▸ Rhabdomyomas and fibromas are the most common cardiac
tumors in infants and children and usually occur in the ventricles,
where they may produce mechanical obstruction to blood flow,
thereby mimicking valvular stenosis, CHF, restrictive or hypertrophic
cardiomyopathy, or pericardial constriction.

▸ Rhabdomyomas are probably hamartomatous growths, are multiple

in 90% of cases, and are strongly associated with tuberous sclerosis

▸ Fibromas are usually single, universally ventricular in location, often

calcified, tend to grow and cause arrhythmias and obstructive
symptoms
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PARAGANGLIOMAS, HEMANGIOMAS, MESOTHELIOMAS

▸ Paragangliomas are rare chromaffin cell tumors that
represent extra- adrenal pheochromocytomas. Most are
located in the roof of the left atrium

▸ Hemangiomas and mesotheliomas are generally small

tumors, most often intramyocardial in location, and may
cause atrioventricular (AV) conduction disturbances and
even sudden death
SARCOMA
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SARCOMA
▸ Almost all malignant primary cardiac
tumors are sarcomas

▸ Most common in adults: angiosarcoma

▸ Most common in children:

rhabdomyosarcoma

▸ Rapid progression that culminates in the

patient’s death within weeks to months
from the time of presentation as a result of
hemodynamic compromise, local invasion,
or distant metastases.

▸ Commonly involve the right side of the

heart, are rapidly growing
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TREATMENT
▸ The optimal therapy for cardiac sarcoma is complete
resection often with neoadjuvant and postoperative
chemotherapy

▸ Response to radio/chemotherspy is poor EXCEPT

lymphosarcoma
TUMORS METASTATIC
TO THE HEART
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TUMORS METASTATIC TO THE HEART

▸ More common than primary tumors

▸ Incidence is high in malignant melanoma and, to a

somewhat lesser extent, leukemia and lymphoma

▸ Most common primary sites from which cardiac metastases

originate are carcinoma of the breast and lung,
reflecting the high incidence of those cancers.

▸ Cardiac metastases may occur via hematogenous or

lymphangitic spread or by direct tumor invasion
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TUMORS METASTATIC TO THE HEART

▸ The pericardium is most often involved, followed by
myocardial involvement of any chamber and, rarely, by
involvement of the endocardium or cardiac valves.

▸ Electrocardiographic (ECG) findings are nonspecific

▸ On chest x-ray, the cardiac silhouette is most often normal

▸ CT and radionuclide imaging may define the tumor

burden more clearly than 2D echo