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New Treatment for NF1 Malignant Cancer

Young adults with Neurofibromatosis type I (NF1) are at risk for developing a rare type of sarcoma called a malignant peripheral nerve sheath tumor (MPNST).  These deadly cancers can be exceedingly difficult to treat, and the only known curative therapy requires complete resection of the tumor.  Since many of these cancers cannot be completely removed, radiation therapy is often given before or after surgery. Unfortunately, this treatment approach has not been successful in managing MPNSTs, and chemotherapy is often given after surgery.

To evaluate the benefit of chemotherapy in the management of people with these cancers, Angela C. Hirbe, MD, PhD and her colleagues identified patients at Washington University who had been treated with chemotherapy before surgery. For this study, they treated people with ifosfamide and epirubicin chemotherapy.

In their recent report, they demonstrated that 60% of patients treated before surgery exhibited a response to this combination.  Importantly, one patient had a complete response to chemotherapy, and required no surgery or further treatment.  These exciting data show that MPNSTs can shrink in response to chemotherapy.

Based on these findings, Dr. Hirbe and her team are planning prospective studies to determine whether this treatment approach should become the standard of care for people with MPNST.


Hirbe AC, Cosper PF, Dahiya S, Van Tine BA. Neoadjuvant Ifosfamide and Epirubicin in the Treatment of Malignant Peripheral Nerve Sheath Tumors. Sarcoma. 2017;2017:3761292. doi: 10.1155/2017/3761292. Epub 2017 May 4.