Malignant Peripheral Nerve Sheath Tumor is a rare type of soft tissue malignant tumor that occurs in only 5% of cases.
Among mediastinal tumors, only 18.8% occur primarily in the nerves, and of these, only 10.3% are malignant.
We studied six cases of mediastinal primary malignant peripheral nerve sheath tumors in which surgery was carried out.
Nerve sheath tumors localized within the membrane offer good long-term prognosis even if malignant.
Furthermore, long-term survival is possible even if the tumor has invaded neighboring organs, provided it can be completely excised.