CLINICAL VIGNETTES
A rare cause of cardiac tumour: an Erdheim–Chester disease with cardiac involvement co-existing with an intracerebral Langerhans cell histiocytosis
Mathieu Granier*, Antoine Micheau, and Isabelle Serre
Department of Cardiology, Arnaud de Villeneuve, Avenue du Doyen G Giraud, Montpellier 34000, France
* Corresponding author. Tel: +33 662 093 886, Fax: +33 467 336 204, Email: mathieugranier@hotmail.com
A 65-year-old man, who was diagnosed few weeks ago an intracerebral Langherans cell histiocytosis (CD68 +, CDA1 +) (Panel A), was admitted for check up of extracerebral localization. CT scan showed lung fibrosis, retroperitoneal fibrosis, and moderate intra-pericardial and pleural effusion. Transthoracic echocardiography revealed a cardiac tumour developed in the interatrial septum and right atrial wall. Transoesophageal echography and MRI confirmed the diagnosis (Panels B and C). An echo-guided biopsy was performed (Panel C). Histology of the cardiac tumour fragments showed histiocytes proliferation, as well as lung and retroperitoneal fragments, but with a different immunohistochemical profile than intracerebral localization (CD68 +, CDA1−), typical of those encountered in Erdheim–Chester disease (Panel D).
Erdheim–Chester disease is a systemic, non-langerhans cell histiocytosis, with various clinical manifestations. The most frequent abnormality is symmetric long tubular bone involvement, but lung or retroperitoneal fibrosis and perirenal or periarotic infiltration can be seen. Infrequently, pericardial or cardiac involvements have been described.
This is the first case of co-existing langerhans and non-langerhans cells histiocytosis with cardiac involvement.
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