儿童囊性肾瘤和囊性部分分化型肾母细胞瘤的诊治分析

Cystic nephroma and cystic partially differentiated nephroblastoma in children.

目的总结儿童囊性肾瘤和囊性部分分化型肾母细胞瘤的临床、病理特点，探讨合理的治疗方法。方法回顾性分析本院收治的7例儿童囊性肾瘤、6例囊性部分分化型肾母细胞瘤患儿的临床资料，包括年龄、临床表现、影像学及病理检查结果、治疗和预后。结果13例患儿中，男8例，女5例，年龄4个月至4岁，平均1岁7个月。左侧6例，右侧5例，双侧2例。腹部包块9例，B超偶然发现4例，术前均行B超和增强CT检查。5例7侧行肿瘤剜除术，8例行瘤肾切除术。术后随访6个月至7年，未见肿瘤复发，保留肾脏的5例中，7侧残肾功能良好。结论囊性。肾瘤和囊性部分分化型肾母细胞瘤患儿术前无法鉴别，手术完整切除是主要的治疗方法，肿瘤位于肾脏一极或双侧者可行保留肾脏的肿瘤剜除术。囊性肾瘤为良性病变，术后无需化疗，囊性部分分化型。肾母细胞瘤为低度恶性或潜在恶性，Ⅰ期者可单纯手术治疗，Ⅱ期以上需行手术＋化疗。

Objetive To investigate the characteristic clinical and treatment of cystic nephroma （CN） and cystic partially differentiated nephroblastoma （CPDN）. Methods A retrospective study was conducted on our 7 patients with CN 6 patients with CPDN. The data collected from ~the medical records of these patients were analysed for their clinical presentation, imaging findings and treatment. Results In 13 children （ male 8 and female 5, age rage 4 months to 4 years, mean 19 month） with CN and CPDN, abdominal mass was the main clinical presentation. B-ultrasound and CT were performed in all 13 cases. 5 patients with 7 renals under- went enucleation of the tumors with nephron - sparing surgery and 8 patients underwent nephrectomy. Postoper- ation pathology showed CN in 7 cases and CPDN in 6 patients. During a follow-up 6 months to 7 years, the pa- tients were well with no evidence of recurrence. Conclusion CN is a rare benign disease of the kidney. Ima- ging features are not specific enough to differentiate from CPDN. Nephron sparing resection of tumor is a prima- ry and effective therapeutic method for this disease. CPDN is a rare differentiated variant of Wilmg tumor with low but definite malignant potential. Stage I tumors should be treated with tumor nephreetomy alone, stage II with nephrectomy followed by postoperative vincristine and dactinomycin chemotherapy.