目的 探讨马尾副神经节瘤的临床表现及病理学特征.方法 报告1例临床罕见伴节细胞神经瘤分化的马尾副神经节瘤病例,分析其临床表现、组织病理学特点,并复习相关文献.结果 患者男性,47岁,临床表现为右足底麻木并渐进性双下肢麻木.MRI检查显示L4～S1椎管内占位性病变.术中可见S1节段硬脊膜部分缺损未闭合,硬脊膜受压,肿瘤约5 cm×3 cm×3cm大小,位于神经根,形状不规则,血运丰富,压迫马尾囊及神经根.组织形态学观察肿瘤由副神经节瘤和节细胞神经瘤组成,副神经节瘤为实性细胞巢结构,细胞呈器官样排列,部分为实性片状、癌巢样或条索样、假“菊形”团样、血管外皮瘤样及乳头状排列；节细胞神经瘤可见典型节细胞,背景为神经纤维、许旺细胞等,形成节细胞神经瘤图像,两种成分单独或混杂存在.免疫组织化学染色巢状成分的肿瘤细胞胞质表达广谱细胞角蛋白,以及α-突触核蛋白和嗜铬素A,肿瘤细胞巢周围胞质可见表达S-100蛋白的支持细胞,Ki-67抗原标记指数＜5％；节细胞成分表达α-突触核蛋白、神经元特异性烯醇化酶,并不同程度表达细胞角蛋白,但不表达神经元核抗原.结论 该例患者肿瘤组织中伴有节细胞神经瘤成分,含有多种组织学和细胞学形态,伴骶椎隐裂,临床罕见.细胞角蛋白表达阳性可能是马尾副神经节瘤不同于其他部位副神经节瘤的重要病理学特点之一.%Objective To investigate the clinical manifestations and pathological features of paraganglioma of cauda equine. Methods The clinical manifestations, histopathology and immunophenotype features were studied in one rare case of cauda equine paraganglioma with ganglioneuroma differentiation, positive cytokeratin (CK) and bifid occult spine. Related literatures were reviewed. Results The patient was a 47-year-old male. Numbness of the right sole lasted for 2 months, then developed to anaesthesia of two legs. The MR image demonstrated a mass in L4-S1. In operation, it was found that the irregular tumor was 5 cm × 3 cm × 3 cm in size, yellow-white in color, with abundant blood supply, attached to the caudal nerve root and varied in consistency from soft to hard. Cauda equine and nerve root were compressed. The histopathological examination revealed the tumor presented a biphasic tissue pattern with paraganglioma and ganglioneuroma - components. The cells of paraganglioma were arranged in solid sheets, or organoid growth pattern, or carcinoid patterns, or pseudorosette pattern, or hemangio-perithelioma patterns or papillary patterns. The other component presented ganglioneuroma in a solid pattern. Two components existed separately or mixed together. Immunohistochemical examination found cytokeratin, a-synuclein and chromogranin A (CgA) were positive in the paraganglioma area. Some paraganglioma cells expressed S-100 protein-like sustentacular cells. Ki-67 antigen labeling index was less than 5%. A-synuclein, neuronspecific enolase and CK were positive, and neuronal nuclei (NeuN) antigen was negative in the ganglioneuroma cells. Conclusion Paraganglioma is an uncommon benign primarytumor of cauda equine characterized by a slow growth like ependymomacase is very rare because of thecoexistance of ganglioneuroma, multi - histological and cytological morphology, strong positive CK expression and occult cleft spine. The expression of CK may be an important feature of paraganglioma of cauda equine.
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