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目的探讨恶性胃肠神经外胚层肿瘤(GNET)的临床病理学特点。方法对1例GNET进行光镜观察、免疫组化标记及荧光原位杂交(FISH)检测,并复习相关文献。结果镜下肿瘤位于胃壁黏膜下层及肌壁间,瘤细胞呈圆形、卵圆形至梭形,片状或巢状分布。局部瘤细胞排列成假腺样和假乳头样,瘤细胞中等大小,胞质丰富、淡嗜酸;核呈空泡状,部分瘤细胞有小核仁及核内包涵体,核分裂象4个/10HPF。间质内可见破骨细胞样巨细胞,散在分布。免疫组化:瘤细胞vimentin和S-100(+),HMB-45、melan-A和CD117等均(-);破骨样多核巨细胞CD68(+)。FISH信号异常(如1F1G1R等)的细胞比例为71%(>阈值15%),EWSR1基因断裂。结论恶性胃肠神经外胚层肿瘤可能为一独特临床病理单元,应与原发于胃肠道的透明细胞肉瘤、恶性黑色素瘤等相鉴别。
Objective To investigate the clinicopathological features of malignant gastrointestinal neuroectodermal tumor (GNET). Methods One case of GNET was examined by light microscopy, immunohistochemistry and fluorescence in situ hybridization (FISH), and reviewed the related literatures. Results Microscopic tumor located in the gastric mucosa and muscular wall, the tumor cells were round, oval to fusiform, flaky or nested distribution. Local tumor cells arranged in a pseudo-gland-like and pseudopapillary, tumor cells of medium size, rich in cytoplasm, pale acid; nucleus was vacuolar, some tumor cells with small nucleoli and inclusion bodies, mitotic figure 4 / 10HPF. Interstitial visible osteoclast-like giant cells, scattered distribution. Immunohistochemistry: The tumor cells vimentin and S-100 (+), HMB-45, melan-A and CD117 (-); osteoclast-like multinucleated giant cells CD68 (+). The proportion of cells with FISH abnormalities (eg, 1F1G1R, etc.) was 71% (> 15% threshold) and the EWSR1 gene was disrupted. Conclusions The malignant gastrointestinal neuroectodermal tumor may be a unique clinical and pathological unit, which should be differentiated from primary clear cell sarcoma and malignant melanoma in the gastrointestinal tract.