Maruki C, Ito M, Sumie H, Sato K, Ishii S
No Shinkei Geka. 1986 Mar;14(4):579-84.
Intramedullary schwannoma without evidence of von Recklinghausen's disease is extremely rare. Only 23 cases have been reported in the literature. A 42-years-old woman with transverse type myelopathy at the level of Th 6, was referred to our department. Myelography showed diffuse swelling of the thoracic cord. Delayed myelo CT scan revealed the existence of syrinx at C 6 and Th 10 spinal cord level. On NMR spin echo image, high intensity signal with an ovoid shape was visualized in Th 7-8 spinal cord parenchyma and the syrinx was verified below the level of C 5. Based on these findings, she was diagnosed as having Th 7-8 intramedullary spinal cord tumor. Th 6 to Th 9 laminectomy followed by the complete removal of well encapsulated tumor was performed. The rostral and caudal pole of the tumor was found to be located intramedullary. The tumor at the level of Th 7-8, extended not only to the extramedullary space but also to the extradural space. Histopathological examination revealed that the specimen was Antoni A type neurinoma. She showed good recovery. Following hypotheses have been postulated on the cytogenesis of intramedullary schwannoma in the literature: Central displacement of Schwann cells during embryonic development. Schwann cells ensheathing aberrant intramedullary nerve fibers. Schwann cells along the intramedullary perivascular nerve plexus. A critical area where posterior root loose their sheath on entering the pia mater. Transformation of pial cells of neuroectodermal origin into Schwann cells. The cytogenesis of these reported cases may not be identical.(ABSTRACT TRUNCATED AT 250 WORDS)
无冯雷克林霍增氏病证据的髓内神经鞘瘤极为罕见。文献中仅报道过23例。一名42岁女性,胸6水平出现横贯性脊髓病,被转诊至我科。脊髓造影显示胸段脊髓弥漫性肿胀。延迟脊髓CT扫描显示颈6和胸10脊髓节段存在空洞。在核磁共振自旋回波图像上,胸7 - 8脊髓实质内可见椭圆形高强度信号,且在颈5水平以下证实存在空洞。基于这些发现,她被诊断为胸7 - 8髓内脊髓肿瘤。行胸6至胸9椎板切除术,随后完整切除包膜完整的肿瘤。发现肿瘤的头端和尾端均位于髓内。胸7 - 8水平的肿瘤不仅延伸至髓外间隙,还延伸至硬膜外间隙。组织病理学检查显示标本为Antoni A型神经鞘瘤。她恢复良好。文献中对髓内神经鞘瘤的细胞发生提出了以下几种假说:胚胎发育过程中施万细胞的中央移位;包裹异常髓内神经纤维的施万细胞;沿髓内血管周围神经丛的施万细胞;后根进入软脑膜时失去鞘的关键区域;神经外胚层起源的软膜细胞向施万细胞的转化。这些报道病例的细胞发生情况可能并不相同。(摘要截短于250字)