[Mediastinal malignant schwannoma disclosed by pericardial tamponade in von Recklinghausen disease]. / Schwannome malin médiastinal révélé par une tamponnade péricardique au cours de la maladie de von Recklinghausen.

The authors report a case of a 47-years-old patient with Von Recklinghausen's disease. He was admitted with pericardial tamponade. 2D echocardiography showed, in addition to the compressive pericardial effusion, an enormous intrapericardial tumor compressing the left cardiac cavities. The patient died soon after pericardial drainage. The post-mortem examination revealed the diagnosis of malignant schwannoma of the left costo-vertebral gutter with an anterior intrapericardial development. A review of the literature showed that malignant schwannomas are seldom localized in the mediastinum, are associated with Von Recklinghausen's disease in 2 to 13% of cases, and have a poor prognosis. Pericardial tamponade is an exceptional presenting sign of mediastinal malignant schwannoma.