Cook Anthony, Lorenzo Armando J, Salle Joao L Pippi, Bakhshi Manijeh, Cartwright Lisa M, Bagi Darius, Farhat Walid, Khoury Antoine
Division of Urology and Department of Pathology, Hospital for Sick Children and University of Toronto, Toronto, Ontario, Canada.
J Urol. 2006 Apr;175(4):1456-60; discussion 1460. doi: 10.1016/S0022-5347(05)00671-3.
RCC represents less than 2% to 6% of pediatric renal tumors. Few reports of long-term outcomes exist. We sought to determine the presentation, treatment and outcome of patients at our institution.
We retrospectively reviewed the age, mode of presentation, mode of treatment, histological subtype, tumor grade, stage and survival of all patients with RCC from 1980 to 2005.
A total of 15 patients were identified. Mean age at presentation was 7.9 years. Symptomatic presentations in nearly 75% of patients included gross hematuria, abdominal pain and polycythemia. The remaining 25% of cases were asymptomatic, and were identified by physical examination or incidentally on imaging. Surgical resection consisted of radical nephrectomy in 10 patients and partial nephrectomy in 5. Pathological analysis revealed papillary RCC in 8 patients and clear cell RCC in 7. Six patients had high stage disease. One patient with stage IV disease died 8 months postoperatively. The remaining 14 patients were alive at a mean followup of 4.9 years. All but 1 patient remain recurrence-free, including all of those who underwent nephron sparing surgery.
To our knowledge this single institution series is the first to include children treated with partial nephrectomy. Pediatric patients with RCC tend to be older and more likely to present symptomatically compared to the typical patient with Wilms tumor. Hematuria and abdominal pain were the most common presentations, and papillary RCC was proportionately more common in this series. Our initial experience suggests that equivalent cure rates can be expected from a nephron sparing approach in appropriately selected cases.
肾细胞癌（RCC）在儿童肾肿瘤中占比不到2%至6%。关于长期预后的报道较少。我们试图确定我院患者的临床表现、治疗方法及预后。
我们回顾性分析了1980年至2005年间所有RCC患者的年龄、临床表现方式、治疗方式、组织学亚型、肿瘤分级、分期及生存情况。
共确定了15例患者。就诊时的平均年龄为7.9岁。近75%的患者有症状表现，包括肉眼血尿、腹痛和红细胞增多症。其余25%的病例无症状，通过体格检查或影像学检查偶然发现。手术切除包括10例行根治性肾切除术和5例行部分肾切除术。病理分析显示8例为乳头状RCC，7例为透明细胞RCC。6例患者疾病分期较高。1例IV期疾病患者术后8个月死亡。其余14例患者存活，平均随访4.9年。除1例患者外，所有患者均无复发，包括所有接受保留肾单位手术的患者。
据我们所知，这个单中心系列研究是首个纳入接受部分肾切除术治疗儿童患者的研究。与典型的威尔姆斯瘤患者相比，儿童RCC患者往往年龄较大，且更有可能出现症状。血尿和腹痛是最常见的表现，在本系列中乳头状RCC相对更常见。我们的初步经验表明，在适当选择的病例中，保留肾单位手术有望获得相当的治愈率。
