Papillary fibro‐elastic hamartomas of the heart valves

Abstract

TWENTY-EIQHT cases of papillary tumours of the heart valves have been reported hitherto (Prichard, 1951), nine of them on the aortic valve, nine on the tricuspid, five on the mitral and five on the pulmonary. Many of these are very incompletely described and this fact, together with their apparent rarity, is the justification for the publication of two further cases. Case 1A female infant, previously in good health, died from an attack of gastroenteritis at the age of nine months. At the post-mortem examination, idammation of the alimentary tract and fatty change in the liver were present, and in the substance of the aortic cusp of the mitral valve was a firm, apparently fibrous nodule measuring 0.5 by 0.3 cm. The nodule projected mainly on the auricular surface of the valve cusp and the endocardium covering this surface was of velvety texture. There was no other cardiac lesion.Histology. The sections show a papillary nodule, completely covered by endothelium, with villi protruding in the direction of the auricle and composed of a central fibrous core surrounded by delicate collagenous and elastic fibres separated from each other by rnucinous material. The central collagenous zone contains areas of fibrinoid degeneration and of vacuolation due to the mucinous substance.Close beneath the endothelium are coarse elastic fibres, then there is a zone of delicate open collagenous tissue with scattered fibroblasts, and finally a central area of dense collagen fibres. Elastic fibres are present throughout and tend to be arranged in the long axis of the valve cusp ( figs. 1 and 2). The mid-zone has a foamy appearance due to the presence of stromal mucin which stains feebly with Southgate's mucicarmine and by the periodic acid-Schiff technique, and metachromatically with toluidine blue.The villi are avascular but a few large capillaries are present in the solid portion. Iron pigment and glycogen are absent. Case 2A woman aged 80 died from hypertensive cardiac failure and at post-mortem examination a papillary tumour measuring 1.5 by 1.0 cm. waa found on the ventricular surface of the right anterior cusp of the pulmonary valve. It was composed entirely of delicate fronds radiating from a short pedicle arising from an otherwise normal valve cusp (fig. 3). There was no evidence of endocarditis.The basal part of the nodule is solid.It is removed by the action of hyaluronidase.

Primary chondrosarcomatous mesenchymoma of the mitral valve.

“…13), being surrounded by reticulin, seem to represent an area of polymorphous cell sarcoma. There was no evidence of a pre-existing hamartoma (Raeburn, 1953), such as was found by Forker et al (1963), but such a malformation could well have been destroyed by the proliferating tumour. There was no suggestion of the epithelial or cystic structures described by Vacek (1963).…”

Papillary "tumours" of the left ventricle.

“…For a complete bibliography of such case reports the reader is referred to the paper by Pomerance (1961). Usually such lesions occur on the ventricular surface of the semilunar valves or on the atrial surface of the atrio-ventricular valves (Raeburn, 1953). The present case appears to be unique in that the polyps had grown from the endocardial surface of the left ventricle into its cavity.…”

“…To this already imposing list he added the term hemangiofibroma. Raeburn (1953) called these papilliferous masses hamartomas, suggesting that this term should be qualified in a particular case by prefixing a description of the predominant tissue present. He believed them to have a limited capacity for growth, which was not significantly greater than that of the surrounding normal tissues.…”

Video-Assisted Resection of Papillary Fibroelastoma Arising from a Miniature Tendinous Chord in the Apex of the Left Ventricle

“…This finding may provide the basis for the hamartoma hypothesis of PFE. 6) The other challenge in this case was the surgical approach to the tumor. We performed excision via the mitral valve to avoid the potential complications associated with left ventriculotomy.…”