Cardiac Path Online

Pathology of Papillary Fibroelastoma

Dr Sampurna Roy MD

 

Path Quiz Case 92: Case history and images

Diagnosis: Papillary Fibroelastoma

 

                                                                                                                      

 

 

Syn:   Fibroelastic papilloma ; Papillary tumour of the cardiac valve or giant Lamblís excrescence.

Papillary fibroelastoma is the most common valvular tumour and the second cardiac benign tumour after myxoma.

These are slow growing and asymptomatic lesions seen at any age.

Diagnosis in infants and children should be done carefully  as it may be confused with other lesions at that age.

Despite their benign histology, Papillary fibroelastomas should be excised because of their embolic complications. Emboli may originate either from fragments of the tumor or from a thrombus formed around the tumor.

At present, asymptomatic patients are most frequently diagnosed by two-dimensional echocardiography.

Site: The tumour may occur on all cardiac valves.

Clinical presentation:  

This tumour is an incidental finding at echocardiography, surgery or autopsy.

The tumour may also produce cerebral embolism, or cardiac related symptoms.

Lesion in the aortic valve may cause coronary obstruction, coronary arterial embolism and acute myocardial infarct.

Gross features:

This consists of filiform threads (resembling beautiful sea- anemones with multiple papillary fronds) attached to the myocardium, being either sessile or connected by short pedicle.

Their size varies from mm to cm in diameter.

They occur mainly in the aortic valve but may also arise from mural endocardium or any of the cardiac valves. They may be single or multiple.

 

Microscopic features: 

      

 

Microscopic appearance is highly characteristic. 

 

i) A central core of dense, acellular collagen, may be surrounded by myxomatous matrix.

ii) The peripheral rim contains coarse and fragmented elastin fibres.

iii) The surface lining consists of a layer of endothelium, which may appear hyperplastic.

The central core of collagen is continuous with that of the valve leaflet.

Microscopic appearance is like that of a normal chordae tendineae, hence the lesion should be considered as a hamartoma. Its occurrence in older individuals speaks against a hamartomous nature.

Histologically, it resembles Lamblís excrescence and is considered a similar histogenesis i.e. a proliferative response of the endocardium related to mechanical injury and associated with advancing age (hence the term giant Lamblís excrescence).

Possibility of fibroelastoma should be remembered in all cases of sudden and unexpected cardiac death.

 

Further reading:

Papillary fibroelastoma of the aortic valve: A rare cause of stroke.

Pulmonary valve papillary fibroelastoma. A case report and review of the literature.

Slowly growing cardiac tumor: a case of fibroelastoma.

Papillary fibroelastoma of the aortic valve coincident with a cystic tumor of the atrioventricular node.

Papillary fibroelastoma: increasing recognition of a surgical disease.

Papillary fibroelastoma: echocardiographic characteristics for diagnosis and pathologic correlation.

Cardiac papillary fibroelastoma: a treatable cause of transient ischemic attack and ischemic stroke detected by transesophageal echocardiography.

Lambl's excrescences and papillary fibroelastoma: are they different?

 

 

 

Dr Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)

 

 


 

 

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