【摘要】 目的探讨肾脏横纹肌肉瘤的流行病学、临床表现、诊疗方案及预后。方法回顾收治的1例肾脏横纹肌肉瘤患者的临床资料。男性,43岁,临床表现为右上腹疼痛6 d。B超提示右侧腹膜后肿瘤,CT提示右肾与肝实质之间实质性包块,肿瘤直径约10 cm。结果患者行右腹膜后肿物根治性肾切除术。术中见肿物来源于肾脏中极,与腹膜及肾周广粘连明显,且与下腔静脉紧密粘连,术后病理报告:瘤细胞多形性,胞浆内见嗜酸性包涵体,免疫组化:Vimentin(+),病理诊断右侧肾脏横纹肌肉瘤。术后患者予以舒尼替尼靶向治疗,术后3个月复查肾脏CT,未见肿瘤复发。结论肾横纹肌肉瘤是一种发病年龄低、恶性程度高的少见肾脏恶性肿瘤。光镜下具有嗜酸性包涵体特征性表现,以手术治疗为主,辅以放化疗,术后多靶点酪氨酸激酶抑制剂治疗,效果较好。预后与发病年龄及肿瘤分期有关,预后较差。更多还原

【Abstract】 Objective To Discuss the epidemiology,clinical and diagnostic features and prognosis of malignant rhabdosarcoma of the kidney.Methods One case report of malignant rhabdosarcoma was analyzed restrospectively.The patient was a 43 years old man presenting with 6 days right epigastric pain.B-ultrasound examination showed right retroperitoneal tumor.CT scan revealed a solid mass between right kidney and liver with 10 cm diameter.Results The patient underwent right radical nephrectomy of retroperitoneal mass.The mass originated from the middle of the right kidney.There was obvious adherence with peritoneum,inferior caval vein.The pathology showed tumor cells with various shapes and acidophilic inclusion body.The Vimentin was positive in Vimentin by immunohistochemical stain.The pathological diagnosis was rhabdomsarcoma.The patient received targeted treatment with sunitinib for 3 months.No tumor recurrence was found in CT scan 3 months after operation.Conclusion Rhabdosarcoma is a rare aggressive disease with low age of onset.Acidophilic inclusion body is one of the pathognomonic features and Vimentin is positive in immunohistochemical stain.Surgery is the main therapeutic method and chemoradiotherapy is secondary treatment method.And the patient would get the clinical benefit of postoperative treatment with PTK inhibitor.The prognosis of this disease is related to age of onset and staging.And the prognosis is very poor.更多还原