Department of Human Pathology, Gunma University Graduate School of Medicine, 3-39-22 Showa-machi, Maebashi, Gunma, 371-8511, Japan.
Brain Tumor Pathol. 2014 Jan;31(1):57-61. doi: 10.1007/s10014-012-0131-z. Epub 2013 Jan 11.
Nerve sheath myxoma, a myxoid variant of schwannoma, is a dermal tumor that usually occurs in the upper extremities, head and neck region, or trunk; occasionally, however, it has also been reported to develop in the spinal canal. Here, we describe two cases of intraspinal nerve sheath myxoma. Case 1 was a 74-year-old man with left hypochondrial pain. Gadolinium-enhanced magnetic resonance imaging (MRI) of his spine revealed a well-demarcated intradural extramedullary tumor with peripheral enhancement at the Th8 level. Case 2 was a 58-year-old man with lower back and left buttock pain. Gadolinium-enhanced MRI revealed a well-demarcated intradural extramedullary tumor with peripheral enhancement at the Th12-L1 level. Both cases were clinically diagnosed as schwannoma. Histological studies revealed characteristic myxoid lobules which were separated by fibrous septa or bands of more compact cellular area. The tumor cells were diffusely positive for S-100 and focally positive for Schwann/2E, which reacts with Schwann cells and myelin in the peripheral nervous system. The positive reaction to Schwann/2E confirmed the occurrence of peripheral nerve sheath differentiation. Nerve sheath myxoma should be included in differential diagnosis of spinal canal tumors.
神经鞘黏液瘤，是神经鞘瘤的黏液样变体，是一种真皮肿瘤，通常发生在上肢、头颈部或躯干；然而，偶尔也有报道称其在椎管内发生。在此，我们描述了两例椎管内神经鞘黏液瘤。病例 1 为 74 岁男性，左侧季肋部疼痛。脊柱钆增强磁共振成像（MRI）显示 T8 水平有一个边界清楚的硬脊膜外髓内肿瘤，外周强化。病例 2 为 58 岁男性，有下腰痛和左臀部疼痛。钆增强 MRI 显示 T12-L1 水平有一个边界清楚的硬脊膜外髓内肿瘤，外周强化。两例均临床诊断为神经鞘瘤。组织学研究显示特征性黏液小叶，由纤维间隔或更致密细胞区的带分隔。肿瘤细胞弥漫性 S-100 阳性，局部 Schwann/2E 阳性，Schwann/2E 与周围神经系统中的 Schwann 细胞和髓鞘反应。Schwann/2E 的阳性反应证实了周围神经鞘分化的发生。神经鞘黏液瘤应纳入椎管内肿瘤的鉴别诊断。
