This is a common congenital tumour affecting infants and children.
Visit:
Fibroblastic/Myofibroblastic tumours
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.
Gross features:
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.
Microscopic features :
Image Link
(AFIP)
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.
Cardiac
Fibromatosis:
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Fibromatosis
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.
Visit:
Cardiac Lipoma
;
Cardiac Angioma ;
Papillary
Fibroelastoma
REPORTING OF CARDIAC TUMOURS
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