摘要
目的探讨儿童原发性恶性非肾母细胞瘤性肾脏肿瘤的临床诊治特点。方法回顾性分析1993年4月至2008年1月间收治的11例儿童原发性恶性非肾母细胞瘤性肾脏肿瘤患儿的临床资料。根据临床表现及术前影像学检查并于术前行穿刺活检。治疗方法主要为术前介入和／或全身化疗、手术切除、术中热灌注化疗和术后化疗。结果肾细胞癌6例，无瘤长期（平均32个月）生存率66．7％。中胚性肾瘤3例，均获3年以上无瘤生存。肾透明细胞肉瘤1例，术后复发死亡。肾横纹肌样瘤1例，术后化疗2个月复发。结论儿童原发性恶性非肾母细胞瘤性肾脏肿瘤发病率低，临床表现与肾母细胞瘤相似，术前诊断较为困难，其中肾细胞癌发病年龄多为年长儿，而肾透明细胞肉瘤、中胚层肾瘤和肾横纹肌样瘤则多见于小婴儿。经过多项系统性治疗，中胚层肾瘤预后较佳，肾细胞癌次之。
Objective To evaluate the diagnostic and therapeutic characteristics of primary malignant non-Wilms＇ renal tumors in children. Methods Between April 1993 and January 2008, the clinical data of 11 cases who suffered from primary malignant non-Wilms＇ renal tumors were retrospectively reviewed. There were renal cell carcinoma in 6 cases , mesoblastic nephroma in 3, clear-cell sarcoma in 1 and renal rhadomyosarcoma 1. Preoperative fine needle biopsy was performed based on clinical manifestation and imaging examinations. The management consisted of preoperative interventional and/or systemic chemotherapy, tumor resection, intraoperative hyperthermic peritoneal perfusion, and postoperative systemic chemotherapy. Results Postoperative follow-up outcome demonstrated that the tumo-free survival rate of renal-cell carcinoma was 66. 7% with mean survival period of 32 months. Long-term survival more than 3 years was in 3 patients of mesoblastic nephroma. One boy of Clear-cell sarcoma died from postoperative tumor recurrence. One case of Rhabdiod tumor recurred after postoperative chemotherapy 2 months later. Conclusions The incidence of primary malignant non- Wilms＇ renal tumors is low in children, and clinical manifestations are similar to those of Wilms＇ tumor. The preoperative differential diagnosis is difficult. Of these group, renal-cell carcinoma is commonly happened in juvenile, whereas mesoblastic nephroma, clear-cell sarcoma and rhabdoid tumor are usually found in infants. The prognosis of mesoblastic nephroma is excellent, and that of renal-cell carcinoma is also good under a multi-systemic therapy.
出处
《中华小儿外科杂志》
CSCD
北大核心
2009年第3期159-162,共4页
Chinese Journal of Pediatric Surgery
关键词
肾肿瘤
诊断
治疗
Kidney neoplasms
Diagnosis
Therapy
