脊髓内梭形细胞横纹肌肉瘤:一例报告并文献复习  

Spindle cell rhabdomyosarcoma in spinal cord:a case report and review of literatures

作　　者：王世凤[1] 刘倩[1] 廖欣[1] 许霞[1] 张尚福[1] 

机构地区：[1]四川大学华西医院病理科

出　　处：《中国现代神经疾病杂志》2009年第2期178-183,共6页Chinese Journal of Contemporary Neurology and Neurosurgery

摘　　要：目的探讨梭形细胞横纹肌肉瘤的临床表现及病理学特征。方法回顾分析1例脊髓内梭形细胞横纹肌肉瘤患者的临床表现、组织形态学及免疫组织化学特征,并复习相关文献。结果男性患者,50岁。临床主要表现为颈肩部疼痛伴左侧上肢麻木、无力,左侧下肢无力,排尿困难。颈椎MRI检查C5～6脊髓内异常信号伴脊髓空洞。手术中可见肿瘤位于颈椎C5～6脊髓,椭圆形,呈灰白、灰褐色,大小约3.00cm×1.50cm×1.50cm,质地坚韧,血液供应丰富,似有包膜,与周围神经组织粘连紧密,蛛网膜与硬脊膜粘连。组织形态学观察肿瘤主要由形态一致的梭形细胞组成,呈＂漩涡状＂紧密排列;肿瘤细胞边界清楚,细胞质少,呈嗜酸性;细胞核深染,呈长卵圆形,偏位,可见小的核仁和核分裂象;肿瘤细胞之间有数量不等的胶原纤维。免疫组织化学染色肿瘤细胞胞质中结蛋白、波形蛋白、肌特异性肌动蛋白表达阳性;胞核中肌浆蛋白表达阳性,Ki-67抗原标记指数约50%;部分肿瘤细胞胞膜和（或）胞质中CD57表达阳性,而平滑肌肌动蛋白、上皮膜抗原、神经元特异性烯醇化酶、胶质纤维酸性蛋白、广谱细胞角蛋白、钙视网膜蛋白和S-100蛋白表达阴性。患者手术后未接受其他辅助治疗,6个月后死亡。结论梭形细胞横纹肌肉瘤为胚胎型横纹肌肉瘤的一种亚型,主要由紧密排列成漩涡状或长束状、形态一致的梭形细胞组成。多见于儿童和青少年,预后良好;发生于成人者,则预后不良。Objective To study the clinicopathological features of spindle cell rhabdomyosarcoma. Methods The clinical presentations, and histopathological and immunohistochemical features were studied in one case with spindle cell rhabdomyosarcoma in spinal cord, and related literatures were reviewed. Results A 50-year-old man mainly presented with neck and shoulder pain, left upperlimb anaesthesia and left lowerlimb adynamia and dysuria. MRI demonstrated abnormal signals and syringomyelia in C5-6 spinal cord. The oval-shape and tenacious tumor measuring 3.00 cm × 1.50 cm × 1.50 cm in C5-6 spinal cord had abundant blood supply, with its capsule adhered to peripheral nerve tissue, and arachnoid co-adhered with spinal dura mater. Histopathological examination revealed the pale and gray color tumor composed of elongated spindle cells arranged in a fascicular or storiform pattern, in which a number of enlarged plump or polygonal shaped rhabdomyoblasts containing some few eosinophilic cytoplasm and eccentrical enlarged hyperchromatic nuclei with small nucleole and karyokinesis. Different amount of collagenous fibers were seen among tumor cells. The immunohistochemistry showed that desmin, vimentin and muscle specific actin （MSA） were positive in cytoplasm, and myogen was positive and Ki-67 labeling index was 50% in nuclei of tumor cells, and CD57 was positive in some parts of tumor, but smooth muscle actin （SMA）, epithelial membrane antigen （EMA）, neuron specific enolase （NSE）, glial fibrillary acidic protein （GFAP）, cytokeratin （AE1 / AE3）, calretinin （CR） and S-100 protein （S-100） were negative in tumor cells. Patient did not receive other treatment and died 6 months after operation. Conclusion Spindle cell rhabdomyosarcoma, composed of spindle cells arranged in a fascicular or storiform pattern, is a variant of embryonal rhabdomyosarcoma. It usually occurs among children and adolescents, with a better prognosis in comparison with other subtypes of rhabdomyosarcoma. The prognosis is poor in ad

关 键 词：横纹肌肉瘤 胚胎型 脊髓 免疫组织化学 

分 类 号：R739.4[医药卫生—肿瘤]