Atypical and malignant peripheral nerve-sheath tumors of the brachial plexus: report of three cases and review of the literature.

Tumor involvement of the brachial plexus is uncommon. The most common intrinsic neoplasms involving the brachial plexus are benign neurilemmomas and neurofibromas that are usually associated with neurofibromatosis-1 (NF-1). Solitary neurofibromas unassociated with NF-1 are very uncommon. Malignant peripheral nerve-sheath tumors (MPNST) are rare at this site, arising spontaneously or in the context of NF-1. This presentation discusses the clinical presentation, pathology, and management of these tumors, which usually occur in young adults. MPNST are intermediate or high-grade sarcomas with a high risk of local and distant spread. Approximately 50% of MPNST arise in patients with NF-1, and therefore these patients should be thoroughly investigated for any new symptoms or masses. MPNST of the brachial plexus should be treated with an adequate wide local excision, with adjuvant high-dose radiotherapy pre- or postoperatively. The role of chemotherapy in the treatment of MPNST is not clearly defined, but it may have some benefit in salvaging treatment failures.