PDF(Pubmed)
Abstract：
A 13-year-old boy was admitted to the hospital with 1-month history of neck pain and a 2-week history of bilateral hip joint pain accompanied by low fever. Positron emission tomography-computed tomography (PET-CT) revealed the presence of a malignant tumor in the left kidney with metastases to the left renal hilum, retroperitoneum, para-aortic lymph nodes, and multiple bone sites throughout the body. Given that the patient\'s left kidney capsule was intact and the boundary with surrounding tissues was clear, left nephrectomy was performed. Postoperative pathological diagnosis showed desmoplastic small round cell tumor (DSRCT) of the left kidney. CAV-VIP alternating chemotherapy was given 20 days after the first stage surgery. After the end of the 6th cycle, the patient underwent surgery again. The tumor in front of the aorta and postcava, the greater omentum, the retroperitoneal lymph nodes and the hepatic hilum lymph nodes, and the visible tumors in the abdomen were removed. CAV-VIP alternating chemotherapy was continued after the second stage surgery. At the end of the 4th cycle of post operation chemotherapy, radiotherapy was started. An abdominal CT scan conducted 11 months after second-stage surgery did not reveal any recurrence of abdominal tumors; however bone metastases persisted. The patient is currently receiving oral targeted therapy with anlotinib while ongoing follow-up continues.
摘要：
一名13岁男孩入院，有1个月的颈部疼痛史和2周的双侧髋关节疼痛史，伴有低热。正电子发射断层扫描-计算机断层扫描（PET-CT）显示左肾存在恶性肿瘤，并转移到左肾门，腹膜后,主动脉旁淋巴结，以及遍布全身的多个骨骼部位。鉴于患者的左肾囊完整且与周围组织的边界清晰，进行左肾切除术。术后病理诊断为左肾促纤维增生性小圆细胞瘤（DSRCT）。第一阶段手术后20天给予CAV-VIP交替化疗。在第6个周期结束后,病人再次接受了手术。主动脉和后静脉前方的肿瘤，大网膜，腹膜后淋巴结和肝门淋巴结，腹部可见的肿瘤被切除.第二阶段手术后继续进行CAV-VIP交替化疗。在第4周期术后化疗结束时，开始放疗。第二阶段手术后11个月进行的腹部CT扫描未发现腹部肿瘤有任何复发；但是骨转移仍然存在。患者目前正在接受安洛替尼的口服靶向治疗，同时持续随访。
