Cardiac Path Online

Pathology of Cardiac and Pericardial Malignant Tumours

Dr Sampurna Roy MD

 

                                                                                                                      

 

Cardiac sarcoma represents the second most common primary cardiac neoplasm. 

Malignant primary cardiac tumours also include primary pericardial mesotheliomas and primary cardiac lymphomas.

Angiosarcoma

This is probably the most common primary malignant tumour of the  heart.  The tumour usually arises from the right atrium.

Clinical presentation: These tumours occur in all ages and present with a short history.

Patient often presents with features of congestive cardiac failure and dyspnea.

The common variety of tumour presents as a large obstructing mass with associated signs and symptoms.

Less common and less symptomatic variety is the locally infiltrative tumour.

Pericardial lesions present with signs and symptoms of paricarditis.

Prognosis :   Patients die within 10 months of the onset of symptoms. Rarely, patients may survive up to 3 years after treatment with surgery, chemotherapy and/or radiation.

Microscopic features :  It varies from well differentiated neoplastic blood vessels to poorly differentiated solid growth of spindle shaped and anaplastic cells with no vasoformative pattern.

 

Immunohistochemistry:   The tumour  shows positive reaction to endothelial cell markers such as CD31 and CD34.

 

Poorly differentiated cases are to be differentiated  from other poorly differentiated sarcomas, fibrous (sarcomatoid) mesotheliomas and metastatic tumours.

 

Malignant fibrous histiocytoma

Most of the cardiac malignant fibrous histiocytomas are localized in the left atrium (posterior wall or septum) and hence may be mistaken for atrial myxoma, particularly the myxoid variant.

Myxoid variants contain foci of hypercellularity with pleomorphism, mitotic figures, necrosis, and extensive vascularity. All these features are usually lacking in myxoma.

Sections from several sites of the tumour may be helpful.

Prognosis is poor even after surgical resection (varies from 10 months to 5 years.)

Local recurrence is common and show tendency for local invasion.

Rhabdomyosarcoma

Cardian rhabdomyosarcomas are the second common primary malignant cardiac tumour in adults.

A rare case of a botryoid embryonal rhabdomyosarcoma has been recorded in a boy of 8-years-old.

Clinical presentation:  Patients show a general malaise, cardiac murmurs and arrhythmias. Intramyocardial tumours may extend to involve the visceral and parietal pericardium.

Site:  In pediatric age group, tumour usually originates from the atrial or ventricular septum, whereas in adults it commonly arises from left atrium and right ventricle.

Gross and microscopic features: Morphologically the tumour is similar to that in the soft tissues elsewhere.

Most patients die within 1 year of diagnosis.

Fibrosarcoma

Modern techniques reported a series of primary cardiac fibrosarcomas.

Microscopically, the tumours show a storiform or whorled pattern of the spindle cells and the presence of giant cells, suggestive of malignant fibrous histiocytoma.

Diagnosis of cardiac fibrosarcoma is based on the same criteria applied to the tumours of soft tissues elsewhere in the body.

Undifferentiated Sarcoma

These tumours arise from the atria, ventricles and pericardium.

Immunohistochemistry reveals positive staining for smooth muscle actin and vimentin and occasional cells show  positive staining for cytokeratin but more helpful markers are negative.

Prognosis is poor like other sarcomas.

Other rare primary sarcomas of the heart:

Leiomyosarcoma

The histologic diagnosis of cardiac leiomyosarcoma is based on the same criteria as those seen in other sites.

Liposarcoma

This extremely rare tumor arises mainly from left atrium.

Clinical manifestation depends of the position of the tumour mass.

Metastases are common and mostly in lungs and bones. 

Histological diagnosis is based on the same criteria as those applied to liposarcomas in other parts of the body.

Osteosarcoma

Primary cardiac sarcomas with osteosarcomatous differentiation is mainly seen in the left atrium.

Due to this location, they are misdiagnosed as myxoma, despite the atypical echocardiograhic findings.

Prognosis is poor but survival time may be prolonged by surgery.

Histologic diagnosis of cardiac osteosarcoma is no different from that of osteosarcoma elsewhere in the body.

Chondrosarcoma

Primary cardiac chondrosarcoma involves the mitral valve, may be originating from left ventricle.

Histologically, main tumour mass consists of chondrosarcoma , but other areas  show the picture of fibrosarcoma and angiosarcoma.

Clinical course from the onset of symptoms (dyspnea) to death is about 6 months.

Synovial Sarcoma

The rare biphasic malignant tumor composed of epithelial glandular structure and a cellular spindle shaped stroma has been recorded.

Grossly it is a large tumor in the right atrium attached by a stalk to the atriventricular ring.

Immunohistochemical markers confirm the diagnosis by excluding mesothelioma.

Prognosis is poor and patients die within one year.

Malignant Schwannoma

Synonymous: Neurogenic sarcoma ; neurofibrosarcoma ; malignant peripheral nerve sheath tumor.

In the heart the tumours are commonly seen in the right ventricle.

Histological diagnosis of these tumours in the heart is based on the same criteria as applied to neurogenic soft tissue sarcomas elsewhere in the body.

Other primary malignant tumours of the heart and pericardium:

Primary Cardiac Lymphomas

Mesothelioma

Antemortem diagnosis of cardiac mesothelioma is low due to lack of specific clinical findings.

Dyspnea and pericardial effusion are usually the leading clinical findings.

The tumour is more likely to be a metastatic tumour than a primary pericardial mesothelioma.

Occassionally, differentiation from metastatic tumor is difficult due to the diversity of cell character of mesothelioma.

Exfoliative cytology and tissue biopsy helps in establishing the correct diagnosis.

Microscopically, it is similar to the mesotheliomas in other parts of the body.

Myocardial invasion of primary pericardial mesothelioma, without intracavitary extension, is common.

Massive extension into the endocardium differentiates the primary pericardial mesothelioma from other types of sarcoma.

Malignant Teratoma:

Endodermal sinus tumour has been recorded.

The diagnosis of malignant teratoma has been made on the basis of the findings of malignant change in one type of tissue in a benign teratoma.

 

Further reading:

Primary cardiac and pericardial neoplasms: radiologic-pathologic correlation.

Cardiac tumors.

Cardiac Masses on Cardiac CT: A Review.

Primary malignant tumors of the heart: Outcomes of the surgical treatment.

Primary cardiac sarcoma.

 

Dr Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)


 

 

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