Cardiac Path Online
Pathology of Cardiac Fibroma
This is a common congenital tumour affecting infants and children.
Site: The site of cardiac fibroma is usually the ventricle or interventricular septum and the right atrium.
The left ventricle is usually more frequently involved than the right.
In patients with ventricular septal involvement the symptoms may include heart failure, arrhythmias, sudden death, cyanosis and chest pain.
Rarely, the tumour involving ventricular wall or the atrium, tend to be asymptomatic for a long time and eventually valvular or intracardiac obstruction appears.
Patients with heart failure had large, bulky tumours in either ventricle that were difficult to excise.
Tumours located within the ventricular myocardium, often extend into or even obliterating the chamber lumen.
Tumours are always single and range in size from 2 to 10 cm
The gross appearance of the tumours is typically described as fibrous, white, and whorled.
It is a well circumscribed tumour which is clearly demarcated from the surrounding myocardium.
Cut surface reveals fibromatous nature and occasional calcification.
Fibromas are composed of fibrous tissue with peripheral parts intermingled with the surrounding myocardium.
Thus it is less demarcated than the gross appearance suggests.
Occasional presence of elastic fibres leads to the term "fibroelastic hamartoma".
There may be hyalinization or cystic degeneration in the central part of a fibroma.
Foci of calcification, and rarely ossification, are seen in a number of cases.
Small groups of lymphocytes and mononuclear inflammatory cells may be present, especially surrounding vessels and at the junction with the normal myocardium.
Mitotic figures are rare even in cellular areas.
There is no evidence of malignancy.
Complete resection may not be possible in cases of large lesions or involvement of the almost whole interventricular septum.
Association with Gorlin Syndrome:
There is an increased risk of cardiac fibroma in patients with Gorlin syndrome.
The abnormalities noted in this syndrome include - Multiple naevoid basal cell carcinoma ; Rib and vertebral anomalies ; Large head with a characteristic appearance ; Calcification of falx cerebri ; Odontogenic keratocysts of the mandible.
Due to its origin from fibroblasts and the similarity between fibromatosis and cardiac fibroma, the term cardiac fibromatosis is suggested.
Cardiac fibroma is part of the spectrum of soft tissue lesions that embraces soft tissue fibromas and fibromatoses.
Many of the latter tumours are considered aggressive lesions with the capacity for local invasion and growth.
In contrast to fibromatosis, cardiac fibromas do not appear to continue to grow and proliferate after 1 or 2 years of age. The degree of fibrosis increases with age, and the degree of cellularity decreases with age.
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