Cardiac Tumor

Refers to neoplasm of heart or pericardium.

Types

Primary Secondary
Less commonly occurs Most common
It can be benign or malignant & benign is It can be Benign or malignant.
75% more incident. Includes : breast & lung cancer
Includes : Myxoma Renal carcinoma
Papillary fibroelastoma Melanoma
Fibroma leukaemia
Lipoma
Rhabdomyomas
Angiosarcoma

Myxomas are the most common benign cardiac tumors (50%) and usually affect
middle-aged women. Clinical manifestations of myxomas consist in a triad: constitutional
symptoms, embolization and intracardiac obstruction.

Location – left atrium (75%), right atrium (23%), rarely in the ventricles; usually connected to
the atrial septum (fosa ovalis) .
Diagnosis-- echocardiography – globular, smooth – surfaced or irregular, friable surface with
multilobular appearance, 4 to 8 cm in size and sometimes calcifications.

color Doppler imaging showing signs of mitral/tricuspid stenosis

Transesophageal echocardiography may be needed to better visualize the implantation site

and the potential extension into the pulmonary or caval veins.

Papillary fibroelastomas are the most frequent tumors involving the heart valves. They
account for approximately 8% of primary benign cardiac tumors and are usually encountered
in older ages.

Location – aortic and mitral valves, less often, tricuspid and pulmonary valves or mural
endocardium. Different from vegetations, papillary fibroelastomas are located on the
downstream side of the valve and rarely cause valvular dysfunction .

Echocardiographic appearance – small (< 1 cm), pedunculated by a small stalk, highly

mobile structures.

Lipomas are the second most common benign cardiac tumors (10-20%). They represent
encapsulated fatty accumulations, are variable in size and usually asymptomatic.
Sometimes they may cause symptoms related to compression, arrhythmias and conduction
abnormalities.

Location – anywhere in the heart, most frequently in the left ventricle, right atrium, and atrial
septum.

On echocardiography are homogeneous, broad based masses.

Cardiac CT and MRI are the best imaging tools for diagnosing cardiac lipomas as they
identify fat with high specificity.

Fibromas are the second most common primary cardiac tumors in children. In adults they
account for approximately 3% of all benign cardiac tumors.Fibromas are invariably located
in the ventricular myocardium, usually in the left ventricular free wall or interventricular
septum. They may mimic hypertrophic cardiomyopathy.

Fibromas appear as well circumscribed, solitary tumors often extending into the ventricular
cavity. Typically, their size ranges from 1 to 10 cm and have central calcifications

In 70% of cases, fibromas cause symptoms by impeding intracavitary blood flow or causing
ventricular arrhythmias.
Rhabdomyomas are the most common primary cardiac tumors in infants and young
children, approximately three thirds occurring in patients less than 1 year old. There is a
strong association between cardiac rhabdomyomas and tuberous sclerosis (80%). In more
than half of the patients they regress in size and number after infancy. The presence of
rhabdomyomas in adulthood is uncommon

Rhabdomyomas most often occur in the right and left ventricular myocardium. On
echocardiography they appear as multiple, homogeneous and located intramurally
protruding into the ventricular cavity, and ranging from few mm to few cm in size.

Depending on their location and size they may induce arrhythmias or blood flow obstruction.

Angiosarcomas are the most common histological subtype, typically affecting

middle-aged men. These tumors have a predilection for the right chambers, particularly for
the right atrium.Echocardiography shows, a large, broad based mass near the inferior vena
cava, extending intracavitary and into the pericardium, occasionally invading the caval veins
or tricuspid valve .

Primary cardiac lymphomas involve more often the right side of the heart and usually
associate with pericardial effusion. Prognosis is poor with less than 1 month survival
without treatment and up to 5 years after radio- and chemotherapy.

Treatment

Treatment of benign primary tumors is surgical excision followed by serial

echocardiography over 5 to 6 years to monitor for recurrence. Tumors are excised
unless another disorder (eg, dementia) contraindicates surgery. Surgery is usually
curative (95% survival at 3 years). Exceptions are rhabdomyomas and pericardial
teratoma, Patients with fibroelastoma may also require valvular repair or
replacement.

Treatment of malignant primary tumors is usually palliative (eg, radiation therapy,

chemotherapy, management of complications) because prognosis is poor.

Treatment of metastatic cardiac tumors depends on tumor origin. It may include

systemic chemotherapy or palliation.