Cardiac Path Online
Pathology of Cardiac Hemangioma
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 :
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:
Some of the hemangioendothelioma patients died of distal metastases, hence they are preferably regarded as malignant tumours.
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.
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.
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