Treatment of malignant peripheral nerve sheath tumors in pediatric NF1 disease.

ABSTRACT:

Background

Malignant peripheral nerve sheath tumors (MPNSTs) are rare yet highly aggressive soft tissue sarcomas. Children with neurofibromatosis type 1 (NF1) have a 10% lifetime risk for development of MPNST. Prognosis remains poor and survival seems worse for NF1 patients.

Methods

This narrative review highlights current practices and pitfalls in the management of MPNST in pediatric NF1 patients.

Results

Preoperative diagnostics can be challenging, but PET scans have shown to be useful tools. More recently, functional MRI holds promise as well. Surgery remains the mainstay treatment for these patients, but careful planning is needed to minimize postoperative morbidity. Functional reconstructions can play a role in improving functional status. Radiotherapy can be adm

SUBMITTER: Martin E 

PROVIDER: S-EPMC7575473 | biostudies-literature | 2020 Oct

REPOSITORIES: biostudies-literature