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目的 探讨促结缔组织增生性小圆细胞瘤的临床病理特征。 方法 报告 1例 11岁促结缔组织增生性小圆细胞瘤患儿的诊治情况 ,复习有关文献。 结果 患儿以腹痛为主诉 ,左腹膜后 9.0cm× 6.0cm× 3 .0cm囊性肿瘤 ,细胞巢周围结缔组织显著增生 ,免疫组化染色CD99( + )、EWS/WT1( + )。腹膜后肿瘤切除术后联合化疗 ,随访 16个月无肿瘤复发。 结论 促结缔组织增生性小圆细胞瘤有特异的临床病理特征 ,好发于男性青少年。细胞巢周围结缔组织显著增生 ,表达MIC2基因产物CD99及特异的EWS/WT1蛋白 ,手术切除肿瘤联合化疗是治疗的主要方法。
Objective To investigate the clinicopathological features of connective tissue proliferative small round cell tumor. Methods A report of 11 cases of children with connective tissue proliferative small round cell tumor diagnosis and treatment, review of the literature. Results Abdominal pain was the main complaint in children. The left retroperitoneal tumor was 9.0 cm × 6.0 cm × 3.0 cm in size. The connective tissue around the nests became hyperplastic. The expressions of CD99 (+) and EWS / WT1 (+) were also detected by immunohistochemistry. Retroperitoneal tumor resection combined with chemotherapy, followed up for 16 months without tumor recurrence. Conclusions Prominent connective tissue proliferative small round cell tumor has specific clinicopathological features, occurs in male adolescents. Connective tissue around the cell nest significantly proliferated, the expression of the MIC2 gene product CD99 and specific EWS / WT1 protein, surgical resection of the tumor combined with chemotherapy is the main method of treatment.