Chemotherapie: Offener Zugang
Offener Zugang

Abstrakt

Adjuvant Treatment For Patients With Malignant Peripheral Nerve Sheath Tumours

Matthias Kolberg, Sigbjørn Smeland and Ragnhild A Lothe

In current clinical practice, there are no curative treatment options for the aggressive neuroectodermal cancer malignant peripheral nerve sheath tumor (MPNST) beyond surgical resection [1]. Nearly one half of the patients have neurofibromatosis type 1 (NF1), a cancer predisposition syndrome that increases the risk of MPNST almost 5000 times as compared to the general population, and MPNST patients with NF1 have been reported to have worse prognosis [2], although the differences in outcome seem to have diminished in recent years. For both NF1 and non-NF1 patients, the 5-year overall survival after MPNST diagnosis is between 30 and 50%.