Intrathoracic paraspinal malignant peripheral nerve sheath tumor.
Intrathoracic paraspinal malignant peripheral nerve sheath tumor.
复制标题
胸腔内椎旁恶性周围神经鞘瘤。
DOI：
10.1016/s1726-4901(09)70109-1
复制
发表时间：
2006
影响因子：
3
通讯作者：
Min
中科院分区：
文献类型：
--
作者：
R. Lai;Shong‐Ling Lin;S. Hsu;Min
神经鞘瘤是后纵隔最常见的神经鞘肿瘤，而胸椎内棘旁恶性周围神经鞘瘤（MPNST）则相当罕见。良性和恶性神经鞘肿瘤都可能有症状，因此临床鉴别的作用有限。在x线影像上，肋骨和椎体的侵蚀、轮廓的不规则和衰减的不均匀性不足以可靠地诊断MPNST。组织学上，mpnst表现为高细胞性、核异型性和有丝分裂活性。手术切除是治疗的主要方式。MPNST术后放射治疗显著降低了局部复发率。预后不佳。在此，我们提出一个不寻常的病例后纵隔肿块在一个50岁的女性延迟诊断2年。手术干预后，组织学发现为MPNST。因切除不完全，术后行放射治疗。5个月后的随访胸部计算机断层扫描显示胸旁棘区有残留的软组织肿块，体积明显减小。术后未发现神经系统后遗症。
Schwannoma is the most common nerve sheath tumor in the posterior mediastinum, whereas intrathoracic paraspinal malignant peripheral nerve sheath tumor (MPNST) is quite rare. Both benign and malignant nerve sheath tumors may be symptomatic, rendering clinical differentiation of limited utility. On radiographic imaging, erosion of the ribs and vertebral bodies, irregularity in contour, and inhomogeneity in attenuation are not sufficiently reliable for diagnosis of MPNST. Histologically, MPNSTs reveal hypercellularity, nuclear atypia, and mitotic activity. Surgical resection is the main modality of treatment. Postoperative radiation therapy for MPNST has led to a significant reduction in local recurrence. The prognosis is unfavorable. Herein, we present an unusual case of a posterior mediastinal mass in a 50-year-old female with delayed diagnosis of 2 years. After surgical intervention, the histologic finding was MPNST. Postoperative radiation therapy was applied because of incomplete resection. The follow-up chest computed tomography 5 months later revealed a residual soft tissue mass with significant reduction in size over the parathoracic spine area. No neurologic sequelae were identified after surgery.