混合性嗜铬细胞瘤1例报道并文献复习

Report of a case of composite pheochromocytoma and review of literature

目的 :分析1例肾上腺混合性嗜铬细胞瘤(composite pheochromocytoma,CP)患者的临床病理特征及预后。方法:对CP患者的病理组织进行HE染色及免疫组织化学(免疫组化)染色,观察肿瘤组织形态和免疫表型,分析CP的临床病理特征。结果:光学显微镜镜下可见CP组织由2种成分组成,一种为嗜铬细胞瘤成分,其肿瘤细胞排列成器官样结构,细胞质嗜碱性或嗜双色性,核分裂相罕见;另一种为节细胞神经瘤(ganglioneuroma,GN)成分,可见施万细胞呈束状及编织状排列,其间散在分布神经节细胞。免疫组化检查示,肿瘤组织中嗜铬粒蛋白A(chromogranin A,CgA)、突触素(synaptophysin,Syn)呈强阳性表达,施万细胞S100弥漫阳性表达,肿瘤细胞Ki-67增殖指数<1%。患者随访5年间肿瘤无复发。结论:CP是一种罕见肿瘤,由嗜铬细胞瘤与其他成分混杂。本研究的1例患者为CP-GN,预后良好。

Objective: To study the clinicopathological characteristics and immunotype of composite pheochromocytoma-ganglioneuroma(CP-GN). Methods: The clinicopathological features of one CP-GN patient were analyzed by HE and immunochemistry staining,and related literatures were reviewed. Result: The patient was a male with adrenal mass.Pathological examination revealed that the tumor consisted of two components: the first component was pheochromocytoma,tumor cells were arranged in nests with basophilic or amphiphilic cytoplasm;the other component was ganglioneuroma, the ganglion cells were scattered in the background of the bundle and braided schwann cells. Both CgA and Syn were strongly and diffusely positive in the pheochromocytoma, while staining for S100 protein and NF identified Schwann cells and ganglion cells were observed in the ganglioneuroma. The Ki-67 index was low in CP-GN. Conclusions: CP-GN is rare, and its histological structure is mixed. Patient has better prognosis after operation.