脊髓神经鞘黏液瘤临床病理观察

Intraspinal nerve sheath myxoma: a case report and review of literature

目的探讨脊髓神经鞘黏液瘤的临床病理特征、诊断与鉴别诊断。方法观察1例椎管内神经鞘黏液瘤的组织病理学特点,并复习国内外相关文献。结果患者男性,53岁,以双下肢无力就诊,MRI显示L1水平髓外硬膜下,见一境界清楚的肿物,组织学显示肿物由纤维血管形成的致密区分隔开来,形成特征性的富于黏液的多分叶或多结节结构,免疫组化:肿瘤细胞细胞核及细胞质呈S-100弥漫强(+)。结论脊髓神经鞘黏液瘤极为罕见,国内尚未见报道,肿瘤的组织学形态及免疫组化具有诊断价值,回顾文献并总结其流行病及临床特点,显示脊髓神经鞘黏液瘤多发生于下段脊髓,男性多见,临床症状及影像无明显特殊,目前文献无复发病例报道。

Objective To study the clinicopathological features,diagnosis and differential diagnosis of intraspinal nerve sheath myxoma. Methods We described a case of intradural extramedullary spinal nerve sheath myxoma and reviewed the related literature. Results A 53-year-old male patient presented with bilateral lower limbs weakness and MRI revealed a well-demarcated intradural extramedullary tumor at the L1 lever. Histological study revealed characteristic myxoid lobules that were separated by fibrous septa or bands of more compact cellular area. The tumor cells were diffusely positive for S-100 at cytoplasm and nucleus. Conclusion Intraspinal nerve sheath myxoma is extremely rare. Diagnosis is confirmed by combining pathology with immunohistochemistry. Literatures revealed most intraspinal nerve sheath myxomas are located at lower segment of spinal cord. There is a slight predominance in males. No recurrences are reported.