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:
Image Link
Histopathologically,
reveal the characteristic patterns of a paraganglioma (chief cells
arranged in clusters, "zellballen" surrounded by capillary network).
Immunohistochemical (
specific
neuroendocrine markers - eg. chromogranin)
or
ultrastructural analysis is often necessary to confirm the
endocrine nature of these tumours.
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
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