混合性嗜铬细胞瘤-节细胞神经瘤临床病理特征并文献复习

Composite pheochromocytoma-ganglioneuroma: a clinicopathologic and literature review

目的探讨混合性嗜铬细胞瘤(composite pheochromocytoma,CP)-节细胞神经瘤的临床病理学特征、免疫表型、鉴别诊断及预后。方法对3例CP-节细胞神经瘤行免疫组化SP法染色,并复习相关文献。结果男性1例,女性2例,年龄37~64岁;其中例3为纵隔原发。镜下见瘤组织均由两种成分构成:一种为瘤细胞,核圆形或卵圆形,胞质细颗粒状,排列成器官样,核分裂象罕见;一种为束状排列的神经纤维瘤样背景下,散在及聚集分布的神经节细胞;结节边缘尚可见残留的肾上腺组织。免疫表型:嗜铬细胞瘤成分中CD56、CgA、Syn、vimentin均阳性,SMA、Melan-A、α-inhibin、NF均阴性,S-100支持细胞阳性,Ki-67增殖指数低;节细胞神经瘤成分中NF、S-100均阳性,Ki-67增殖指数低,其内神经节细胞中CgA、Syn弱阳性或阴性。结论CP是相对较罕见的肿瘤,而节细胞神经瘤为相对常见的一种混合成分,其在临床及放射学上无法与嗜铬细胞瘤区分,目前仅基于肿瘤的组织病理学特征进行诊断,需根据其内混合的不同成分(良性或恶性)来采取相应的临床治疗与随访。

Purpose To explore the clinicopathological features, immunophenotype, differential diagnosis and prognosis of the composite pheochromocytoma （CP） -ganglioneuroma. Methods 3 cases of CP-ganglioneuroma were stained by immunohistochemical SP method, and the related literatures were reviewed. Results 3 cases of CP-ganglioneuroma were one male and 2 females, the age were 37 - 64. Case 3 were of primary mediastinal. Microscopically, the tumor tissues were composed of two components： one type of tumor cells were arranged in nests with a predominant Zellballen pattern, round or oval nuclei, fine granular cytoplasm and rare mitotic, another part of the neoplasm showed scattered and aggregated distributed ganglion cells in the background of neurofibromatosis which aligned bundles and interwoven, the edge of the tumor was still residual adrenal tissue. Immunohistochemically, components of phcochromocytoma were positive for CD56, CgA, Syn, vimentin and negative for SMA, Melan-A,α-inhibin NF with low Ki-67 proliferation index. S-100 was positive in supporting cells, ganglioneuroma components were positive for NF, S-100 with low Ki-67 proliferation index. CgA and Syn were weakly positive or negative in the ganglion cells. Conclusion CP is a relatively rare tumor, which can not be distinguished from pheochromocytoma in clinical and radiological diagnosis. The corresponding clinical treatment and follow-up management should be taken according to the different ingredients （benign or malignant）.