Tumeur maligne des gaines des nerfs périphériques : masse exceptionnelle du médiastin antérieur et moyen

Malignant peripheral nerve sheath tumors (MPNST) are rare nervous tumors usually located in the posterior mediastinum in the paravertebral gutters. We report the case of a non-smoking 62-year-old man who was admitted with a 4 months history of cough, hoarseness and shortness of breath. Physical examination noted a superior vena cava syndrome. CT scan of the chest revealed a right anterior and middle mediastinal mass compressing the superior vena cava, the ascending aorta, the right pulmonary artery, invading the superior root of the pulmonary vein and the right auricle. Flexible bronchoscopy showed extrinsic compression of the right main bronchus, the right upper lobe bronchus and intermedius bronchus. The patient underwent surgical biopsy of the mass by mediastinoscopy. Histological examination revealed a malignant peripheral nerve sheath tumor. The patient received a single cycle of chemotherapy (ifosfamid-adriamycin). Clinical course was marked by the fast worsening of the dyspnea and the general state. Patient died three weeks after the cure of the chemotherapy. This case is original by the exceptional clinical presentation of MPSNT with a superior vena cava syndrome and the very rare location of this tumor in the anterior and middle mediastinum.