Malignant mediastinal schwannoma in Recklinghausen's disease presenting as cardiac tamponade. Report of two cases

The authors report two cases of Recklinghausen's disease admitted for cardiac tamponade. Two-dimensional echocardiography showed a pericardial tumour in addition to a compressive pericardial effusion. After pericardial drainage, the outcome was rapidly fatal. Histological examination confirmed the diagnosis of a malignant intrapericardial schwannoma. A review of the literature revealed the scarcity of mediastinal malignant schwannoma. Associated with Von Reckinghausen's disease in 2 to 13% of cases, the prognosis is poor. The presentation of malignant mediastinal schwannoma with cardiac tamponade remains very rare.