Benign hemangiomas are
benign vascular
tumours
that
may occur at any site in the heart
and can affect
patients of all age groups.
Site:
These lesions mainly occur in the
visceral layer of the pericardium and may be associated with hemopericardium.
In the
myocardium hemangiomas are
mostly seen in the interventricular septum and anterior wall of the
ventricles.
Subendocardial
hemangiomas are seen in all four cardiac chambers and intracavitary
overgrowth may resemble cardiac myxoma.
Clinical
presentation:
Most
affected patients are asymptomatic and the tumour is often
discovered incidentally.
Symptomatic
patients commonly present with dyspnea on exertion and may
also have chest pain, pericarditis or
pericardial effusion (which may be hemorrhagic), syncope,
and sudden death.
Some
myocardial
and subendocardial hemangiomas cause myocardial dysfunction with
congestive cardiac failure or mimic valvular heart disease.
Intramural hemangioma may cause atrioventricular block.
Kasabach-Merritt
syndrome :
Characterized
by multiple systemic hemangiomas including cardiac hemangioma and
associated with recurrent thrombocytopenia and consumptive
coagulopathy.
Classification :
(i) Localized lesion (ii) Angiomatosis of heart - Diffuse
and extensive involvement of heart.
Gross and
microscopic
appearance :
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It
is identical to hemangioma in other parts of the body and capillary,
cavernous or arteriovenous types have been reported.
Intramural
hemangiomas : Often poorly circumscribed, variably hemorrhagic or
congested, spongy lesions.
Endocardial hemangiomas
: Well-circumscribed, variably myxoid , soft lesions.
Vascular tumours with
epithelioid endothelial cells:
i)
Epithelioid hemangioma (histiocytoid
hemangioma)
:
ii)
Epithelioid Angiosarcoma :
iii)
Epithelioid hemangiendothelioma :
Some of the hemangioendothelioma
patients died of distal metastases, hence they are preferably regarded
as malignant tumors.
Prognosis :
These tumours possess limited growth
potential and will persist if not surgically removed.
Spontaneous involution has
been recorded. Surgical removal gives excellent prognosis.
Differential Diagnosis :
(1) Dilated blood vessels
- Usually in the subendocardium ; Resemble hemorrhoids
(2)
Blood cyst
(crevices in valve
leaflets with blood). These are exclusively seen in the hearts of the
newborns and infants. Often located in the tricuspid and mitral
valves.
(3)
“Mesothelial/monocytic
incidental cardiac excrescence” (cardiac MICE):
Mesothelial/monocytic incidental
cardiac excrescences: cardiac MICE.Mod
Pathol. 1994 Jan;7(1):9-16.
Lesions are attached to: (i ) Mural endocardium : (ii ) Heart valves :
(iii ) Free, floating within pericardial
sac.
These are composed of solid clusters of cells (large polygonal
cells with eosinophilic cytoplasm) within fibrin meshwork.
The cells have a epithelioid or histiocytoid appearance.
Immunohistochemistry : The tumour
cells do not stain with endothelial markers, but are immunopositive
with cytokeratin markers.
Differential diagnosis: Nodular
mesothelial hyperplasia ; Epitheliod hemangioma ; Primary or
metastatic malignant tumour.
Visit:
Cardiac Lipoma
;
Cardiac Fibroma ;
Papillary
Fibroelastoma
REPORTING OF CARDIAC TUMOURS
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