Estrada Carlos R, Suthar Anjali M, Eaton Samuel H, Cilento Bartley G
Department of Urology, Children's Hospital Boston, Harvard Medical School, Boston, Massachusetts, USA.
Urology. 2005 Dec;66(6):1296-300. doi: 10.1016/j.urology.2005.06.104.
To review our experience to better define pediatric renal cell carcinoma (RCC). Pediatric RCC is rare, and recent data suggest it may be a unique disease.
A retrospective review was conducted of hospital and pathology records from 1965 to 2003. Patients with RCC were identified, and the clinical and pathologic data were extracted.
Since 1965, 11 patients with RCC were treated, accounting for 3% of all renal tumors. In the first 20 years, 191 patients with renal tumor were treated, of whom 3 had RCC. In the most recent 15 years, 172 patients with renal tumor were treated, of whom 8 had RCC. The mean age at presentation was 14.7 years (range 9 to 17 years), with a female predominance (2.7:1). The clinical signs and symptoms included hematuria in 36%, flank pain in 27%, and an abdominal mass in 9%; 36% were discovered incidentally. Of the 11 RCC tumors, 45% were papillary and 55% were clear cell carcinoma. Papillary tumors presented at a worse stage and displayed more aggressive clinical behavior. Of 10 patients with available follow-up data, 6 had no evidence of RCC, 1 had died of other causes, 2 had died of metastatic RCC, and 1 was alive with recurrent RCC at a mean follow-up of 4.9 years.
The clinical presentation, pathologic characteristics, and clinical behavior of pediatric RCC are different than those for adult RCC. A possible increasing incidence of RCC in children would parallel an increased incidence in adults. Our findings warrant additional and coordinated efforts to better characterize RCC in children.
回顾我们的经验以更好地界定小儿肾细胞癌（RCC）。小儿RCC较为罕见，近期数据表明它可能是一种独特的疾病。
对1965年至2003年的医院及病理记录进行回顾性研究。确定患有RCC的患者，并提取临床和病理数据。
自1965年以来，共治疗了11例RCC患者，占所有肾肿瘤的3%。在最初的20年里，共治疗了191例肾肿瘤患者，其中3例为RCC。在最近的15年里，共治疗了172例肾肿瘤患者，其中8例为RCC。就诊时的平均年龄为14.7岁（范围9至17岁），女性占优势（2.7:1）。临床体征和症状包括血尿占36%、胁腹痛占27%、腹部肿块占9%；36%为偶然发现。在11例RCC肿瘤中，45%为乳头状，55%为透明细胞癌。乳头状肿瘤分期较差，表现出更具侵袭性的临床行为。在10例有随访数据的患者中，6例无RCC证据，1例死于其他原因，2例死于转移性RCC，1例在平均随访4.9年时存活但有复发性RCC。
小儿RCC的临床表现、病理特征和临床行为与成人RCC不同。儿童RCC发病率可能增加，这与成人发病率增加相平行。我们的研究结果需要进一步协同努力，以更好地描述儿童RCC的特征。
