Cardiac Path Online
An Approach to reporting of specimens of Cardiac Tumours
Primary heart tumours are rare, but cardiac metastases (usually hematogenously) occur in some patients who die of cancer.
Metastases involve the pericardium or penetrate into the myocardium, or both.
The tumours include:
Myxomas are the most common primary cardiac tumor in adults.
Most tumors (90 %) arise in the region of the fossa ovale, the left to right ratio is 4:1.
It is usually single and 1 to 10 cm in diameter.
They are sessile, pedunculated or papillary type with myxoid appearance.
Microscopically, these are composed of stillate or globular myxoma cells, endothelial cells, smooth muscle cells, all lying in an acid mucopolysccharide matrix.
These are less common than myxoma but are the most common primary heart tumor in children.
Grossly, they may be left-sided or right-sided, gray-white tumor, arising in the ventricular wall.
Microscopically, they are composed of large, rounded, or polygonal cells rich in glycogen and containing myofibrils.
Cytoplasmic strands radiating from the central nucleus to plasma membrane create "spider cells".
Rhabdomyomas are probably hamartomas, some are associated with tuberous sclerosis.
- Papillary Fibroblastoma of the heart valves :
Papillary fibroelastomas may cause emboli but are usually an incidently finding at autopsy.
They are characteristically found on right-sided valves in children and left-sided valves in adults.
Grossly, they appear as 3 to 5 mm hair-like projections.
Microscopically, filaments have a core of myxoid connective tissue, with smooth muscle cells and fibroblasts, covered by endothelium. They probably derive from organized thrombi.
Angiosarcoma is the most common. [These malignant tumors resemble their counterparts in other locations]
Metastatic carcinoma occasionally mimics a primary cardiac neoplasm.
Excision specimens are more usual than biopsy specimens from these tumours.
- Presenting features and their duration:
Cardiac failure , evidence of embolism, arrhythmias, superior vena caval obstruction ;
- Radiological features and diagnosis.
The Pathologist should comment on the following features in the histopathology report:
- Tumour type and grade ;
- Tissues of the heart involved, where applicable ;
- Excision margins if assessable ;
- Presence of overlying thrombus.
- Alcian blue to demonstrate acid mucin in myxomas;
- Immunostain for cytokeratin, Factor VIII-related antigen, other endothelial markers, markers for myocytes.
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