Neurofibrosarcoma

Hui-Kuo G. Shu, MD, PhD
Last Modified: March 24, 2002

Dear OncoLink "Ask The Experts,"

My friend's daughter is 2 and has been diagnosed with neurofibrosarcoma. The cancer is in the upper thigh, her sciatic nerve and thigh muscle have been removed before Xmas but they had to go back in 2 weeks ago (end of January) and take out more. It is still there (very small) and apparently in the spine. What are the prognosis and/or options for this type of cancer? What would be the best course for treatment? Is chemo the only recourse? Thank you for reading this.

Hui-Kuo G. Shu, MD, PhD, Assistant Professor in the Department of Radiation Oncology at the University of Pennsylvania, responds:

Neurofibrosarcoma, or a malignant peripheral nerve sheath tumor is a very difficult problem. The prognosis will depend on a number of factors including tumor grade, whether the patient has neurofibromatosis, the extent of surgery, presence of distant metastasis, etc. The general approach to treatment is to obtain a maximal resection, which could include amputation in some cases. In the setting of wide local excision, radiation therapy is often added to improve local control. However, since relatively high doses of radiation are required for the treatment of this sarcoma, it may be difficult to treat a patient this young. The role of adjuvant chemotherapy has not been clearly established although responses can be obtained. This modality can certainly be considered especially given the difficulties with giving adjuvant radiation in this patient. This patient's physician would have the most details about this particular case and should be able to provide you with the best information about prognosis and potential treatment approaches in this situation.