Cardiac lymphangioma: a benign cardiac tumour
Department of Cardiology, Hôpital de la Cavale Blanche, Brest University Hospital, Boulevard Tanguy Prigent, 29609 Brest Cédex, France
* Corresponding author. E-mail address: pierre-yves.pennec{at}chu-brest.fr
A 21-year-old woman without past medical history was referred to our intensive care unit for a first episode of sustained palpitations, but she reported some short lasting episodes during the last 9 months. Electrocardiogram registers a regular rapid (220 bpm) wide QRS complexes tachycardia. The ventricular origin of the tachycardia was confirmed by electrophysiological study.
A large tumoural mass (10x12 cm) included in the postero-lateral wall of the left ventricule was visualized by trans-thoracic echocardiogram (Panel A). The echogenic signal of the tumour was identical to the normal myocardium and there was no limit between tumour and normal myocardium. In order to specify the topography of the tumour and its relationship with adjacent organs, magnetic resonance imaging (Panel B) focused on the thoracic area was performed and the echocardiographic findings confirmed.
Percutaneous myocardial catheter biopsy allows an histological examination of the tumour (Panel C), which demonstrated a cardiac lymphangioma. This benign neoplasm is characterized by endothelial-lined, thin-walled spaces that contain lymph (Panel C-arrow 1) and disjoint native myocites (Panel C-arrow 2). It constitutes an exceptional form of cardiac tumour (only six cases published) mainly discovered during childhood.
Given the extension of the tumour within the myocardium, a surgical resection was impossible. A cardioverter defibrillator was implanted to prevent sudden death. She had some recurrences of ventricular tachycardia converted to sinus rhythm by the device and continue to do well after a follow-up of 18 months. A heart transplant is envisioned.
Panel A. Transthoracic echocardiogram (apical view) showed a large mass in the postero-lateral wall of the left ventricle.
Panel B. Transverse image in T1-weighted spin-echo breath-hold sequence in magnetic resonance imaging: the mass involves the left-ventricular cardiac wall. The signal intensity (SI) is intermediate on T1- and T2-weighted, and identical to the normal myocardium. The tumour is heterogeneous and non-encapsulated with spaces of low SI. There is no contrast enhancement with gadolinium. Note the pericardial effusion.
Panel C. Histological aspect of the tumour. Endothelial-lined, thin-walled spaces that contain lymph (arrow 1) and disjoint native myocites (arrow 2).
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