马尾恶性周围神经鞘瘤伴蛛网膜下腔出血:病例报告。

Rafael Aponte-Caballero, Julian Alfonso Sierra-Peña, Juan Felipe Abaunza-Camacho, William Mauricio Riveros-Castillo, Javier M Saavedra
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引用次数: 0

摘要

恶性周围神经鞘瘤(MPNST)是一种不常见的侵袭性肿瘤,常与 I 型神经纤维瘤病有关。这是首例腰椎硬膜内 MPNST,术中发现伴有蛛网膜下腔出血(SAH)。一名72岁的男子因严重急性腰背痛到急诊科就诊。神经系统检查无异常。腰椎钆增强磁共振成像显示,硬膜内病变呈不规则形状,从L3延伸至L5。病灶在T1和T2加权成像中均呈中等信号,周围增强。通过 L3-L5 椎板切除术,发现了弥漫性 SAH 和与马尾神经根紧密粘连的肿瘤。标本检查显示肿瘤呈纺锤形,核多形、高色素,S100 和 SOX10 阳性。随访8个月后,他没有出现神经功能障碍,Karnofsky表现评分为90分。腰椎硬膜内 MPNST 中出现 SAH 的手术证据是一项新发现。
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Cauda equina malignant peripheral nerve sheath tumor presenting with subarachnoid hemorrhage: a case report.

Malignant peripheral nerve sheath tumors (MPNST) are uncommon aggressive neoplasms, frequently associated with type I neurofibromatosis. This is the first case of intradural lumbar spine MPNST with intraoperative findings of associated subarachnoid hemorrhage (SAH). A 72-year-old man presented to the emergency department with severe acute low back pain. Neurological examination was unremarkable. Gadolinium-enhanced MRI of the lumbar spine showed an irregularly shaped intradural lesion extending from L3 to L5. The lesion exhibited a medium signal both on T1 and T2-weighted imaging with peripheral enhancement. Through an L3-L5 laminectomy, a diffuse SAH, and a tumor tightly adherent to cauda equina nerve roots were found. Specimen examination revealed a fusocelular tumor with pleomorphic and hyperchromatic nuclei, positive for S100, and SOX10. On an 8-month follow-up, he had no neurological deficit, with a Karnofsky performance score of 90 points. Surgical evidence of SAH in lumbar spine intradural MPNST is a novel finding.

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