Cardiac Path Online

Pathology of Cardiac Paraganglioma

Dr Sampurna Roy MD

 

                                                                                                                      

 

Cardiac Paragangliomas originate from chromaffin paraganglia localized at the base of the heart, close to the aorta and pulmonary trunk, nourished by the branches of the main coronary arteries (coronary glomera).

Their critical location, highly vascularized nature, and 5% to 10% incidence of recurrence or metastatic potential are unfavorable factors.

Clinical presentation:  Paragangliomas occur in patients between the ages of 18 and 85 years (average age, 40 years), affect equally males and females and are occasionally familial.

Intracardiac paraganglioma (pheochromocytoma, chemodectoma) is demonstrated by signs and symptoms of hypertension (due to excessive norepinephrin).

Site: Cardiac paragangliomas are usually seen in the epicardial surface of left atrium, interatrial groove - protruding into the atrial cavities, (resembling atrial myxoma) and root of the great arteries.

The tumour may also occur in the proximal part of right  and left coronary arteries.

Gross features:  These are well-demarcated homogeneous, smooth surfaced, gently lobulated, gray-tan polypoid mass which may project  into the atrial cavity.

Reporting of Cardiac Tumours ; Papillary Fibroelastoma Cardiac Lipoma.

Microscopic features:  Histopathologically, reveal the characteristic patterns of a paraganglioma (chief cells arranged in clusters, "zellballen" surrounded by capillary network).

Immunohistochemical: (Specific neuroendocrine markers - Example- chromogranin) or ultrastructural analysis is often necessary to confirm the endocrine nature of these tumours.

Zellballen pattern (balls of cells) - Images of Paraganglioma

Prognosis:

There is no reliable histopathologic predictor of behavior, and although surgically challenging, most are cured by resection.

The tumours show metastasis and may cause massive hemorrhage and death during surgery.

Reporting of endomyocardial biopsy;  Cardiac rhabdomyoma.

Further reading:

A cardiac paraganglioma presenting with atypical chest pain.

Primary cardiac paraganglioma.

Right Atrial Paraganglioma

A Rare Intracardiac Mass: Cardiac Paraganglioma

Pigmented cardiac paraganglioma.

A left atrial paraganglioma patient presenting with compressive dysphagia.

Cardiac Paraganglioma in an Adolescent

Pigmented cardiac paraganglioma

Pentalogy of Fallot and cardiac paraganglioma: a case report

Tumors of the Adrenal Gland and Extra-adrenal Paraganglia. Washington, DC: Armed Forces Institute of Pathology ; 1997. Atlas of Tumor Pathology ; 3rd series, fascicle 19.

Tumors of the Heart and Great Vessels. Washington, DC: Armed Forces Institute of Pathology ;1996. Atlas of Tumor Pathology ; 3rd series, fascicle 16.

Primary cardiac paraganglioma. Case report and review of surgically treated cases.

 


 

Dr Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)

 

 


 

 

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