摘要目的 探讨肾脏未分化网状细胞肉瘤(尤因肉瘤)的临床诊治及预后特点. 方法 肾尤因肉瘤患者1例,男,33岁.体检发现左肾肿物3d.B超检查见左肾实质下部占位病变,边界清楚,其内血流信号丰富.CT检查示左肾下极类圆形肿块,5.1 cmu×4.7 cm大小,平扫中等密度,内见多个小斑片状低密度区,增强扫描明显不均匀强化,静脉期强化明显,考虑为肾癌.行后腹腔镜下左肾根治性切除术,手术顺利.检索相关文献复习讨论. 结果 病理报告:左肾下极直径4.0 cm灰白色肿物,部分坏死,瘤细胞小圆形,较均匀一致,成片分布,胞界不清,胞质稀疏,细胞核大小较一致,可见核分裂象.免疫组化染色:Vimentin(+),CD99(+),PAS(+),WT-1(-).病理诊断为肾尤因肉瘤.术后化疗:短周期(环磷酰胺+长春新碱+吡柔比星,每天1次,连续2d),长周期(异环磷酰胺+依托泊苷,每天1次,连续6d),长短周期交替进行,共6个周期,周期间隔21 d.随访14个月未见复发. 结论 肾尤因肉瘤临床罕见,恶性程度高,生物学行为进展快,诊断主要依据病理组织学及免疫组化染色检查,治疗以手术切除加放、化疗为主,但预后不佳.

abstractsObjective To review the clinical features,management and prognosis of renal Ewing's sarcoma (ES) of a single case report. Methods A single case of renal ES was reported.A 33-year-old male presented with a mass in the left kidney found during a three day medical examination.B-ultrasound examination showed a lesion with rich blood flow signals and well defined margins in the inferior portion of the left kidney.The CT scan revealed a solid mass of 5.1 cm × 4.7 cm in the inferior portion of the kidney with un-even enhancement by contrast.A possible diagnosis of renal carcinoma was given prior to surgery.No metastasis was proven.A literature review of ES was then conducted. Results A left retroperitoneoscopic radical nephrectomy was successfully performed.Gross pathologic examination showed a solid tumor with necrosis,localized at the inferior pole of the left kidney.The histopathological examination revealed the tumor consisted of small round tumor cells,which were positive for CD99,vimentin and PAS,but negative for WT-1.A diagnosis of ES of the kidney was then determined.The patient received alternating short cycle ( CTX + VCR + THP) and long cycle ( IFO + VP-16) adjuvant chemotherapy for 6 cycles after the operation.There has been no evidence of recurrence at the 14-month follow up. Conclusions ES of the kidney is a rare disease with no specific clinical feature in most cases.Diagnosis of renal ES must be confirmed with histological features.Surgery combined with radiotherapy and chemotherapy is the main method of therapy for renal ES.The prognosis of renal ES is poor.