Cardiac Path Online

Pathology of Cardiac Hemangioma

Dr Sampurna Roy MD

Path Quiz Case 91: Case history and images

Diagnosis: 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:

(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 tumours.

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.

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 

                                                                               
Angiosarcoma; Glomus tumour ; Hemangiopericytoma ; Angiolipoma Aggressive angiomyxoma ; Angiomyofibroblastoma ; Angioleiomyoma; Angiomyolipoma ; Dermatofibroma (aneurysmal variant) ; Spindle cell lipoma (Angiomatoid variant);  Kimura's disease  
 

Further reading:

Small cardiac hemangioma: a challenge for diagnosis

Cardiac cavernous hemangioma

Atypically located cardiac haemangioma of the mitral valve

A rare case of a left atrial hemangioma mimicking a  myxoma

Right atrial hemangioma in the newborn

Necrotic cardiac haemangioma masquerading as sepsis with disseminated intravascular coagulation. 

Clinicopathologic studies of 11 cases of primary cardiac valve tumors.

Vascular tumors of the heart in infants and children: case series and review of the literature

Mesothelial/monocytic incidental cardiac excrescences of the heart: case report and literature review.

Haemangioma of the right atrium revealed by cardiogenic shock.

Cardiac hemangioma: a report of two cases and review of the literature.  


 

 

Dr Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)

 

 


 

 

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