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目的探讨消化道原发性未分化多形性肉瘤(UPS)的临床病理学特征。方法回顾性分析2例消化道UPS临床资料,行病理组织学检查及免疫组化观察,结合相关文献进行复习。结果 2例患者均为女性,年龄分别为45和60岁。临床主要表现为消化道肿块并伴有梗阻症状,遂行手术切除。镜下肿瘤细胞异型增生,排列呈旋涡状或席纹状,局灶可见凝固性坏死;细胞形态各异,呈多角形、梭形、类圆形等,并可见多核瘤巨细胞;肿瘤细胞胞质丰富,胞核大、深染,核仁明显,核分裂易见;间质局灶黏液样变性,少许中性粒细胞及淋巴细胞浸润。瘤细胞vimentin、CD68、AACT及lysozyme(+),CD117、Dog-1、CD34、SMA、desmin、S-100、HMB45、CD99、bcl-2、p16、CDK4、MDM2、EMA、广谱CK及ALK均(-),Ki-67阳性指数均>70%。结论消化道UPS是一种罕见的恶性肿瘤,组织起源目前尚不清楚,其诊断依赖于病理组织学检查及免疫组化。
Objective To investigate the clinicopathological features of primary undifferentiated pleomorphic sarcoma (UPS) in the digestive tract. Methods The clinical data of 2 patients with gastrointestinal tract were analyzed retrospectively. Pathological examination and immunohistochemistry were performed to review the data. Results Both patients were female and were 45 and 60 years old, respectively. The main clinical manifestations of digestive tract mass with obstruction symptoms, then surgical resection. Microscopic tumor cells dysplasia, arranged in a spiral or mylar pattern, focal necrosis visible; cell morphology varied, polygonal, fusiform, round and so on, and can be seen in multi-nuclear giant cell tumor cells; tumor cells Rich in quality, large nuclei, deep staining, obvious nucleoli, mitotic easily visible; interstitial focal mucoid degeneration, a small amount of neutrophils and lymphocytes infiltration. Tumor cells vimentin CD68 AACT and lysozyme (+) CD117 Dog-1 CD34 SMA desmin S-100 HMB45 CD99 bcl-2 p16 CDK4 MDM2 EMA broad-spectrum CK and ALK (-), Ki-67 positive index were> 70%. Conclusion The digestive tract UPS is a rare malignant tumor. The origin of the tissue is not clear yet. Its diagnosis depends on histopathological examination and immunohistochemistry.