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© 2003 European Association of Cardio-Thoracic Surgery
Coexisting chordal papillary fibroelastoma and leaflet Lambl's tumour of the mitral valve
a Service de Chirurgie Cardio-Vasculaire, CHU Gabriel Montpied, B.P. 69, 63003 Clermont-Ferrand Cedex 1, France
* Corresponding author. Tel.: +33-4-7375-1576; fax: +33-4-7375-1579 Received December 20, 2002; received in revised form April 10, 2003; accepted April 11, 2003
Cardiac papillary fibroelastoma (CPF) is a rare and histologically benign tumour. It is the most common valvular tumour and the third cardiac benign tumour, after myxoma and lipoma (less then 16% of all cardiac tumours). CPF is a surgically correctable cause of cerebrovascular and myocardial infarction, making its identification by echocardiography beneficial. We report an unusual case of double localization on both chordae and leaflet of the mitral valve.
Key Words: Cardiac papillary fibroelastoma; Lambl's tumor; Mitral valve tumor
CPF is a rare histologically benign tumour [1] with a malignant propensity for life-threatening complications. It is the most common valvular tumour and the third most common cardiac benign tumour, after myxoma and lipoma (less then 16% of all cardiac tumours) [2]. CPFs are known to be small in size and often isolated in location. They are characterized by multiple papillary fronds formed by a central fibroelastic core surrounded by a myxomatous layer and endothelial cells [3]. The number of CPFs usually detected during life has increased with the advent of echocardiography. Neurological accidents are the most common symptoms [4] but angina, myocardial infarction and atrioventricular block [5] may also reveal left sided CPFs. Pulmonary embolism can also occur in right cavity location [6]. In 90% of cases CPF is located on aortic or mitral leaflets [5,7], but incidental implantation on the mitral chordae and papillary muscle [8], have also been reported. Here we report the case of a patient with a mitral valve and chordal papillary fibroelastoma revealed by cerebrovascular ischemic accident.
A 30-year-old man was admitted for a recent sudden onset of right hemicorporeal deficiency associated to a past transient loss of vision. Cardiac examination, laboratory studies, EKG and chest X-ray were normal. Cerebral computed tomography (CT) scan revealed an ischaemic lesion in the territory of the left profound sylvian artery with a haemorrhagic evolution. Transthoracic (TTE) (Fig. 1d) and transesophageal echocardiography (TEE) (Fig. 1a–c) showed a mobile mass attached to the anterior mitral leaflet chordae originating from the posterior papillary muscle; an additional slight echodensity was detected on the free edge of the A2 leaflet segment (Carpentier classification) (Fig. 1a–d).
Surgical excision of the tumour was performed trough a median sternotomy with normothermic cardiopulmonary bypass (CPB) and intermittent warm blood cardioplegia. A very small mass with a wart aspect of  mm dimension was discovered on the margin of A2 (Fig. 1e). A second mass of  mm was found attached to the primary chordae inserted at the junction of the A2–A3 leaflet segment, originating 1.5 mm from the edge of this leaflet and descending on the chordae just to the top of the posterior papillary muscle (Fig. 1f). The tumour excision required resection of a primary chorda and part of the anterior leaflet tissue (Fig. 1g). For the consequent mitral insufficiency chordal transposition from P2 to A2 and posterior flexible annuloplasty with a 29-mm Duran band (Medtronic Inc., Minneapolis, MN) was performed. At follow-up after 1 year, postoperative outcome was uneventful, neurologically the patient still had a residual right brachio-facial hemiparesis, and he was discharged in sinus rhythm with temporary medication of aspirin. 2.1. Histological and immunohistochemical studies The two excised masses (Fig. 1h) were respectively histologically and immunohistochemically analysed after fixation in AFA (alcohol 75%, formic 20% and acetic acid 5%) and later in paraffin. Consecutive 5-µm sections were stained with haematoxylin and eosin safran (HES), Alcian blue and orcein, that specifically characterized elastic fibres (Fig. 2a–c).
Immunohistochemical studies were performed on each tumour using an Automat (Ventana, Strasbourg, France) after pre-treatment in an autoclave at 120 °C, under pressure, for 5 min at pH 6, with an inhibition of the endogenous peroxidase. Then the primary antibodies vimentin (Fig. 2d,f), actin (Fig. 2e), CD34, CD68, and protein S100 were incubated for 30 min at 35 °C, mixed first with a inhibitor of unspecific antigenic receptor and afterwards with a secondary biotinylated antibody and the streptavidin–biotin–peroxidase complex. The addition of diaminobenzidine in the presence of oxygenated water revealed the presence of the complex, after which counterstaining was carried out with haematoxylin. The two masses had the same histological and immunohistochemical aspect, with specific fronds connected to a common papillary pedicle. This structure was found to contain three different levels: (i) a superficial endothelial layer (vimentin+, CD34+/–, protein S100–); (ii) an intermediate mucopolysaccharidic layer (Alcian blue coloration); and (iii) a central node with a concentric avascular fibrosis and mesenchymal cells, characterized by the presence of elastic and collagen fibres (vimentin+, CD68+, protein S100+/–). A diagnosis of CPF and Lambl's tumour was established considering the histologically and immunohistochemically results.
CPFs have been described in literature with several different names or as separate entity [9]. Sometimes they have been described as Lambl's excrescences [2,7], representing a different state of evolution of the same lesion, differing by size and location, the CPF more frequently presenting on the valve chord or on the valve cusp far away from the edge of the leaflet, while the Lambl tumour remains more usually located on the free edge of the valve leaflets. We have noted that these two masses were histologically the same tumour with two different locations, according to a diagnosis of CPF for the chordal mass and of Lambl's tumour for the small mass on the leaflet. Consequently, preoperative TEE is an important diagnostic step to eliminate multiple locations and to assess valve repair feasibility. Although CPF and the Lambl's tumours are, histologically, benign tumours, they must be considered as a clinically malignant mass in terms of life-threatening complications, but the absence of long-term follow-up series does not permit confirmation of the benefits of anticoagulant medical therapy versus surgical excision in asymptomatic patients [10]. However, a surgical approach seems logical in cases of previous embolisms. doi:10.1016/S1569-9293(03)00081-1
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Abstract
1. Introduction
dimension involving the A2–A3 edge junction. (f) CPF mass of 8x5 mm attached on the primary chordae of A2–A3 anterior mitral leaflet junction. (g) Excision of the two tumours. (h) Dimension of the two masses excised.
. (b) Endothelial layer (arrow) (HES, 
. (c) Lambl vegetation: microscopic aspect similar to papillary frond of fibroelastoma (HES,