胃肠道恶性神经外胚层肿瘤临床病理分析及文献复习

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Abstract Objective To investigate clinicopathological features，immunophenotype，molecular genetics and differential diagnosis ofmalignant gastrointestinal neuroectodermal tumor. Methods A case of rare malignant gastrointestinal neuroectodermal tumor in December 2015 in our hospitalwas analyzed and reviewed the related literature.The histological and immunohistochemical results were observed，and the fusion status of EWSR1 gene was detected by fluorescence in situ hybridization（FISH）.Results The tumorwas located in the small intestine，the poor-circumscribed lesion located in the entire thickness of the bowelwall extending into themucosal layer with a significant infiltrative growth patterns.The tumors cells composed of round，short spindle or round epithelium-like cells arranged in sheets or nests.A number of multinucleated osteoclast-like giant cells had also been observed.The nuclei thatwas surrounded by variable amounts of eosinophilic cytoplasm showed vesicular chromatin with generally distinct to nucleoli and variable mitotic activity were easy to seen.The tumor cellswere strongly positive for S-100 and Vimentin，and positive for CD99，CD56 and Syn，and weak positive for CR，the Ki-67 proliferating index was about 30%in the aspect of immunohistochemistry.Fluorescence in situ hybridization results showed EWSR1 gene rearrangement.Conclusion Malignant gastrointestinal neuroectodermal tumor is an additionally rare tumorwith high aggressive and poor prognosis.The definite diagnosis for itmainly depends on the histology，immunophenotype，at the same time，need to be differentiated from othermalignant tumors.The rearrangement analysis of EWSR1 gene is helpful for pathologic diagnosis.

Key words： Malignant neuroectodermal tumor Immunophenotype Molecular genetics

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	LIU Hai-ling
	FAN Xin-juan
	LIU Jun
	HUANG Yan

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LIU Hai-ling,　FAN Xin-juan　,LIU Jun　, et al. Clinicopathological analysis and review of literatures of malignant gastrointestinal neuroectodermal tumor[J]. 中国当代医药, 2016, 23(28): 8-11转15.

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http://dangdaiyiyao.com/EN/ OR http://dangdaiyiyao.com/EN/Y2016/V23/I28/8

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