These tumors are also referred to as Malignant Peripheral Nerve Sheath Tumors (MPNST)
Associated with underlying neurofibromatosis type 1 (NF1)
Malignant tumors associated with nerve sheath. Develop in a pre-existing neurofibroma in about 40% of patients
Therapy
- Surgical resection is very important. Complete resection is the most important factor determining local control
- Radiation therapy may have a role in reducing the risk of local recurrence
- Post-operative for microscopic residual disease which is unresectable increases the chance of local control
- Pre-operative radiation therapy if re-resection is possible
- Chemotherapy
- There are reported responses to chemotherapy in patients who have unresectable tumors at diagnosis
Prognosis
This tumor has a poor prognosis and is often unresectable at presentation.
- 5-year overall survival (OS): just over 50%
- 5 year progression-free survival (PFS): just over 35%
Better prognosis associated with:
- Complete resection (significantly increases the chance of overall survival)
- Tumor size less than 5 cm
- Extremity tumors (early detection and smaller size)
- Good response to chemotherapy
Poor prognosis associated with:
- MPNST in the setting of NF1 with a 10% or so 5 year overall survival.
- Adverse tumor site ( tumors arising in the trunk, intra-abdominal and intra-thoracic tumors)
References and Resources
J Clin Oncol. 2005 Nov 20;23(33):8422-30. Pediatric malignant peripheral nerve sheath tumor: the Italian and German soft tissue sarcoma cooperative group.Carli M, Ferrari A, Mattke A, Zanetti I, Casanova M, Bisogno G, Cecchetto, Alaggio R, De Sio L, Koscielniak E, Sotti G, Treuner J
International Consensus Statement on Malignant Peripheral Nerve Sheath Tumors in Neurofibromatosis 1 Rosalie E. Ferner, and David H. Gutmann
