Inflammatory myofibroblastic tumour of the heart

Epidemiology

Cardiac inflammatory myofibroblastic tumour is a tumour predominantly of the first two decades of life.

Clinical features

Patients may variously present with fever, myalgia or seizures. Echocardiography shows a luminal lesion, with an equal distribution in the right and left sides of the heart.

Macroscopic appearances

The tumour is endocardial and polypoidal.

Histopathology

There are cellular areas composed of myofibroblastic cells, other areas being loose and myxoid. An inflammatory component comprises lymphocytes and plasma cells. There may be zones of necrosis. Mitotic rates of up to 2 per 10 HPF are seen.

Immunohistochemistry

	SMA	10/101
	ALK	1/71
	S-100	0/71
	Pancytokeratin	0/71
	Desmin	0/71

Differential diagnosis

Cardiac myxoma
Papillary elastofibroma
Low grade sarcoma

References

1 Burke A, Li L, Kling E, et al. Cardiac inflammatory myofibroblastic tumor: a "benign" neoplasm that may result in syncope, myocardial infarction, and sudden death. Am J Surg Pathol 2007; 31:1115-22

This page last revised 16.11.2007.