Introduction: Malignant Peripheral Nerve Sheath Tumor is a rare type of soft tissue malignant tumor that occurs in only 5% of cases. It tends to occur in the arms and legs and trunk of the body, with mediastinal occurrence in only a few cases. Among mediastinal tumors, only 18.8% occur primarily in the nerves, and of these, only 10.3% are malignant, with very few reports indicating the long-term progress of patients with MPNST of the mediastinum.
Case presentation: We studied six cases of mediastinal primary malignant peripheral nerve sheath tumors in which surgery was carried out at this Center between 1977 and 2000. Four of the cases were men and two were women. The period of observation was between 14 and 277 months and the median follow-up time was 119.5 months. The tumors were contained in a membrane and completely excisable in three cases, with the patient demonstrating long-term survival with no recurrence in each case. The remaining three cases were not completely excisable due to invasion into the aorta. In two cases, the patients were treated post-surgically with chemotherapy; however, in all three cases the patient died as a result of the original condition.
Discussion and conclusions: 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. In cases where complete excision is difficult, a multidisciplinary approach including radiotherapy and anti-cancer drug treatment may contribute to improved prognosis but this is a subject that requires further study.
Keywords: MPNST; Malignant peripheral nerve sheath tumor; Malignant schwannoma; Mediastinal tumor; Surgery.
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