椎管内副神经节瘤临床病理学特征

Clinicopathologic features of paraganglioma in vertebral canal

目的探讨原发于椎管内副神经节瘤的组织起源、临床病理特点、鉴别诊断及生物学特征。方法对5例原发于椎管内副神经节瘤进行组织病理学及免疫组化观察,并复习临床资料及相关文献。结果椎管内副神经节瘤好发于中老年男性,临床表现大多无神经内分泌症状,常有局部神经压迫症状。组织学形态具有一般神经内分泌肿瘤的特征。免疫组化标记表达NSE、Syn和CgA,而CK、EMA、GFAP均阴性。随访:1例术后复发。结论副神经节瘤发生在椎管内极少见,诊断主要依靠组织学及免疫组化,其生物学行为应界定为潜在恶性或低度恶性。

Purpose To investigate the pathogenesis, clinicopathologic features, biological behavior of paraganglioma in the vertebral canal and its differential diagnosis. Methods Histopathologic changes and immunohistochemical staining were analyzed in five cases of paraganglioma in vertebral canal. Results The tumor was commonly found in middle-aged men. Patients with paraganglioma in the vertebral canal usually took the form of intradural compression in the vertebral canal but seldom had endocrinological symptoms. The histopathological features were similar to those of the neuroendocrine neoplasm. Immunohistochemical staining showed the tumor cells were positive for NSE, chromogranin A, synaptophysin and negative for cytokeratin, epithelial membrane antigen, and GFAP. Conclusion Paraganglioma of the vertebral canal is a rare and potential malignant tumor. It can be differentiated from other tumors in the region based on histopathology and immunohistochemical staining.