背景:通常在躯干中发现的恶性周围神经鞘瘤(MPNSTs),四肢,头部,和颈部占所有软组织肉瘤的3-10%。虽然它们通常起源于周围神经雪旺细胞,2-3%来自脊神经,可能在椎管内发现。这里,我们介绍了一名43岁的男性,其硬膜外胸部MPNST导致明显的脊髓压迫和进行性轻瘫。
方法:一名43岁男性出现16个月的进行性轻瘫。MRI显示胸椎后部T2-T4硬膜外肿瘤,导致明显的脊髓压迫。在T2-T4椎板切除术和硬膜外肿块的总切除后,患者恢复了适度的神经功能。免疫组织化学染色支持胸椎MPNST的诊断。
结论:很少,脊髓MPNST可以考虑在硬膜外脊髓肿瘤的鉴别诊断中。在这种情况下,后T2-T4硬膜外MPNST的总切除改善了患者的原始轻瘫。值得注意的是,免疫组织化学染色有助于确认MPNST的诊断。
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.
METHODS: 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.
CONCLUSIONS: 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.