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

Abstract

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.

Malignant peripheral nerve sheath tumor: Molecular pathogenesis and current management considerations

“…Tumors of the brachial plexus represent a significant surgical challenge. Excision of tumors of the brachial plexus may result in an unpredictable neurologic deficit, and patients should be counseled appropriately [82]. Alternatively, removal of an MPNST involving the brachial plexus may result in no change or an improvement in pain or weakness [83].…”

Medullary metastasis of a malignant peripheral nerve sheath tumor: A case report

“…MPNSTs exhibit a high incidence of local recurrence and distant metastases. Metastases are most frequently identified in the lung, followed by the bone and pleura (2). Currently, there are no recommended adjuvant treatments for MPNST as there is for other soft tissue sarcomas.…”

Long-term survival of a patient with aggressive sporadic malignant peripheral nerve sheath tumour presenting as haemothorax

“…Approximately 25-50% of cases accompany neurofibromatosis type 1 (NF1) -a dominant autosomal defect in which a NF1 gene product -neurofibromin -lacking RAS-GAP function, does not act as a tumour suppressor gene [2,3]. Depending on the type of NF1 mutation, 2-29% of carriers develop MPNST in their lifetime.…”

“…Typical molecular changes include retinoblastoma tumour suppression pathway, aberrant expression of CHFR and aberrant expression of immune system-related genes [4][5][6]. The tumour originates from proliferating Schwann or perineural cells, most frequently surrounding sciatic, brachial and maxillofacial nerves [2]. The most common clinical presentation is a painful, invasively growing mass with accompanying neurological deficit [7,8].…”