MEDIASTINAL MALIGNANT SCHWANNOMA REVEALED BY PERICARDIAL TAMPONADE INVONRECKLINGHAUSEN-DISEASE

The authors report a case of a 47-years-old patient with Von Recklingh ausen's disease. He was admitted with pericardial tamponade. 2D echoca rdiography showed, in addition to the compressive pericardial effusion , an enormous intrapericardial tumor compressing the left cardiac cavi ties. The patient died soon after pericardial drainage. The post-morte m examination revealed the diagnosis of malignant schwannoma of the le ft costo-vertebral gutter with an anterior intrapericardial developmen t. A review of the literature showed that malignant schwannomas are se ldom localized in the mediastinum, are associated with Von Recklinghau sen's disease in 2 to 13 % of cases, and have a poor prognosis, Perica rdial tamponade is an exceptional presenting sign of mediastinal malig nant schwannoma.