混合性嗜铬细胞瘤临床病理学特征分析  

Clinicopathological features of composite pheochromocytoma

作　　者：胡琴琴 齐广伟 马杰[1] 杨泽然[1] 何向蕾[1] Hu Qinqin;Qi Guangwei;Ma Jie;Yang Zeran;He Xianglei(Department of Pathology,Zhejiang Provincial People′s Hospital,Hangzhou 310014,China;Department of Pathology,Hangzhou Children′s Hospital,Hangzhou 310014,China)

机构地区：[1]浙江省人民医院病理科,杭州310014 [2]浙江省杭州市儿童医院病理科,310014

出　　处：《中华病理学杂志》2020年第2期145-148,共4页Chinese Journal of Pathology

基　　金：浙江省自然科学基金(LY18H160044);浙江省医药卫生科技计划项目(2019KY024)。

摘　　要：目的探讨混合性嗜铬细胞瘤(composite pheochromocytoma,CP)临床病理特征、免疫表型、诊断及鉴别诊断。方法收集2011年1月至2019年1月浙江省人民医院收治的5例CP,对5例CP的临床、影像学、组织学、免疫组织化学特征及预后进行观察,探讨其临床病理特征及鉴别诊断。结果CP患者中男性4例,女性1例,男女发病比例为4∶1,发病年龄52~68岁(平均年龄59岁,中位年龄54岁),肿瘤最大径3~4 cm(平均最大径3.6 cm,中位最大径3.5 cm);临床表现多为无明显症状的肾上腺肿块;典型的形态为两种成分组成,一种为瘤细胞呈不规则巢状排列,瘤细胞胞质细颗粒状、嗜碱性或双嗜性,核分裂象罕见;另一种为由不规则交叉束状排列的施万细胞及散在分布的神经节细胞组成;免疫组织化学示嗜铬细胞瘤弥漫表达PHOX2B(5/5)、突触素(5/5)、嗜铬粒素A(CgA,5/5),支持细胞S-100蛋白阳性(5/5),节细胞神经瘤弥漫表达S-100蛋白(5/5)、神经丝蛋白(5/5),其内神经节细胞PHOX2B、突触素、CgA弱阳性;5例均接受手术切除,预后良好,随访均无复发。结论CP较少见,临床表现不具有特异性,其具有独特的组织学特征,仔细的观察肿瘤的完整组织结构及免疫组织化学检测PHOX2B、CgA、突触素、S-100蛋白有助于其诊断和鉴别诊断。Objective To detect the clinicopathological features,immunophenotype,diagnosis,and differential diagnosis of composite pheochromocytoma(CP).Methods Five cases of CP were collected at Zhejiang Provincial People′s Hospital from January 2011 to January 2019.The clinical,radiological,histologic,immunohistochemical and outcome data were analyzed;the diagnosis and differential diagnosis were discussed.Results The patients′age range was 52-68 years(mean 59 years,median 54 years),There were 4 males and 1 female,and the male to female ratio was 4∶1.Tumor size was 3-4 cm(mean 3.6 cm,median 3.5 cm).The most common clinical manifestation was adrenal mass.Histologically,the classical feature was two distinct morphologic components,one with tumor cells arranged in irregular nests,and with fine granular and basophilic oramphophilic cytoplasm;the other was composed of scattered ganglion cells in the background of Schwann cells organized in interwoven bundles.The components of pheochromocytoma expressed PHOX2B(5/5),synaptophysin(5/5),CgA(5/5),the sustentacular cells expressed S-100 protein;the components of ganglioneuroma expressed S-100 protein(5/5),NF(5/5),the ganglion cells were weakly positive for PHOX2B,synaptophysin and CgA.All the cases were surgically resected and all patients were free of recurrence at follow-up.Conclusions CP is rare adrenal tumor,and it has typical histologic features but no specific clinical manifestations.Attention should be paid to its characteristic histomorphology with the use of PHOX2B,CgA,synaptophysin and S-100 protein immunohistochemistry that is helpful for its diagnosis.

关 键 词：嗜铬细胞瘤 病理学 外科 免疫组织化学 诊断 鉴别 

分 类 号：R73[医药卫生—肿瘤]