Extradural malignant peripheral nerve sheath tumor of the thoracic spine: A rare case report

Abstract

Background: Malignant peripheral nerve sheath tumors (MPNSTs) typically found in the trunk, limbs, head, and neck represent 3–10% of all soft-tissue sarcomas. Although they typically originating from peripheral nerve Schwann cells, 2–3% arise from the spinal nerves and may be found within the spinal canal. Here, we present a 43-year-old male with an extradural thoracic MPNST contributing to marked cord compression and a progressive paraparesis. Case Description: A 43-year-old male presented with a progressive paraparesis of 16 months’ duration. The MRI showed a posterior T2-T4 extradural tumor in the thoracic spine resulting in significant cord compression. Following a T2-T4 laminectomy and gross total excision of the epidural mass, the patient regained modest neurological function. Immunohistochemistry staining supported the diagnosis of thoracic spinal MPNST. Conclusion: Rarely, spinal MPNST can be considered amongst the differential diagnoses of an extradural spinal tumor. In this case, gross total excision of a posterior T2-T4 epidural MPNST resulted in improvement in the patient’s original paraparesis. Notably, immunohistochemistry staining helped confirm the diagnosis of a MPNST.

Multifocal malignant peripheral nerve sheath tumor in patients with neurofibromatosis type I: Report of two cases and review of literature

“…[ 5 , 10 ] Spinal cases are extremely uncommon with only four cases reported internationally. [ 1 , 7 , 11 ] Our cases are the fifth and sixth spinal MPNST [ Table 1 ].…”

“…Cunha KS, et al [2] 23 F -Sacrum and spine High grade Radiation Survived for 7 months followed by Lung metastasis then deceased. Christi A, et al [1] 43 M -oracic spine High grade…”