Composite pheochromocytoma-ganglioneuroma: a clinicopathologic and literature review / 临床与实验病理学杂志

ABSTRACT

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 componentsone 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 pheochromocytoma 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).