摘要目的 探讨松果体区乳头状肿瘤的临床表现、病理诊断、鉴别诊断、组织发生、术后治疗及与生存相关因素.方法 收集松果体区及第三脑室后部肿瘤386例,新生儿松果体组织2例,成人松果体组织2例.HE染色、过碘酸雪夫(PAS)组织化学染色、16种抗体免疫组织化学染色(EnVision法).结果 确诊松果体区乳头状肿瘤5例,患者4例男性,1例女性.1例为复发病例,组织病理学表现为乳头状结构及实性细胞区,乳头状结构特征为单层、假复层柱状或立方上皮细胞围绕血管排列,朝向血管的细胞表面和血管形成明显的界限,乳头中心血管玻璃样变,实性细胞区细胞排列呈巢片状,可见核分裂象及坏死.5例PAS染色胞质内均偶见粉色黏蛋白样物质.免疫组织化学染色细胞角蛋白(CK)、CK8/18、突触素、巢蛋白、微管相关蛋白、S-100蛋白、波形蛋白5例均阳性,嗜铬粒素A、神经元特异性烯醇化酶4例阳性,神经丝蛋白2例阳性,Ki-67阳性指数1％ ～6％,上皮细胞膜抗原、CK5/6、癌胚抗原、NeuN 5例均阴性.除病例5随访失败外,其余4例患者中,3例健在,1例死亡,死亡病例为复发病例.结论 松果体区乳头状肿瘤从儿童到成人均可发病,男性居多；组织病理学表现为乳头状结构及实性细胞区,肿瘤细胞具有上皮细胞学特征；应注意与乳头状结构肿瘤鉴别,特别是室管膜瘤；可能来源于联合下器官室管膜细胞；早诊断、肿瘤小、及早手术、争取全切、术后放疗有助于延长患者生存期.

abstractsObjective To study the clinicopathologic features of papillary tumor of the pineal region (PTPR).Method Three hundred and eighty six cases of pineal region and posterior third ventricle tumors,two newborn and two adult pineal glands were analyzed by HE,PAS and immunohistochemistry of 16 antibodies (EnVision method).Results Five cases of PTPR were diagnosed with mixed papillary features and densely cellular areas,and included one recurrent case.In the papillary areas,the vessels were lined by one or several layers of cuboidal/columnar cells; the vessel wall was hyalinized.In the densely cellular areas,sheets or nests of tumor cells were seen.The tumor cells of these five cases were immunoreactive to CK,CK8/18,synaptophysin,MAP2,nestin,S-100,and vimentin.Four cases were immunoreactive to NSE and CgA; and 2 cases were immunoreactive to NF.All five cases were negative for EMA,CK5/6,CEA,and NeuN.Ki-67 labeling index ranged from 1％ to 6％.Three patients were alive,and the recurrent one died.Conclusions PTPR occurs in patients with over a wide age range,from children to adults,and is more commonly found in male than female.PTPR is composed of both papillary and solid areas,characterized by epithelial cytology,and needs to be differentiated from ependymoma.PTPR may originate from the specialized ependymocytes of the subcommissural organ.The prognostic factors are early diagnosis,complete surgical resection and radiotherapy.