Tumeur maligne de la gaine d'un nerf périphérique : une lésion rare du médiastin postérieur

We report the case of a 52-year-old male patient who developed a malignant peripheral nerve sheath tumor (MPNST) localized in the posterior mediastinum. The diagnosis of this rare tumor is difficult because the clinical presentation of the benign or malignant types is often similar, i.e. elective pain and bone erosions. Similarly, radiological procedures do not always allow distinction between the two types. MNR and CT-scan are the first line procedures: they localize and characterize the lesions, and CT-scan can also be a guide for biopsies. Histological diagnosis is required, but diagnosis can be compromised by the heterogeneous nature of the tumor. Surgical treatment should be undertaken whenever possible. Survival was unusually long in our patient, more than 5 years after discovery of the MPNST. This type of sarcoma is often very aggressive with frequent development of local recurrences and metastases.