Indolfi Paolo, Terenziani Monica, Casale Fiorina, Carli Modesto, Bisogno Gianni, Schiavetti Amalia, Mancini Antonia, Rondelli Roberto, Pession Andrea, Jenkner Alessandro, Pierani Paolo, Tamaro Paolo, De Bernardi Bruno, Ferrari Andrea, Santoro Nicola, Giuliano Maria, Cecchetto Giovanni, Piva Luigi, Surico Gianmarco, Di Tullio M Teresa
Pediatric Oncology Service-Pediatric Department II, University of Napoli, Napoli, Italy.
J Clin Oncol. 2003 Feb 1;21(3):530-5. doi: 10.1200/JCO.2003.02.072.
To identify the prognostic factors, treatment, and outcome of children affected by renal cell carcinoma (RCC).
The series included 41 patients (18 males and 23 females) with a median age of 124 months observed at the 11 Italian Association for Pediatric Hematology and Oncology centers from January 1973 to January 2001. Clinical data, surgical notes, pathologic findings, and summaries of therapy were taken from the charts.
Seven (17%) of the 41 patients had a papillary histology, and 34 (82.4%) had nonpapillary histology. Eighteen patients (43.9%) had stage I, one patient (2.4%) had stage II, two patients (4.8%) had stage IIIA, 10 patients (24.3%) had stage IIIB, and nine patients (21.9%) had stage IV disease. One patient had a bilateral involvement at diagnosis. Seven patients experienced disease recurrence. Lung and liver were the most common distant lesions and usually were fatal. In this study, the major factor influencing the prognosis was the stage. Event-free survival at 20 years was 53.5% for all patients. Overall survival at 20 years was 54.9% for all patients.
RCC is a rare disease in children and adolescents. This neoplasm has a different clinical presentation in children compared with adults but the same outcome. In our experience, patients with localized disease could be cured by nephrectomy alone. Prospective studies in a larger number of patients are needed to confirm radiation therapy and biologic response modifiers as effective adjunct therapy in RCC stage III. The alternative therapy seems warranted in patients with advanced disease.
确定受肾细胞癌（RCC）影响的儿童的预后因素、治疗方法及结果。
该系列研究纳入了1973年1月至2001年1月期间在意大利11个儿科血液学和肿瘤学协会中心观察的41例患者（18例男性和23例女性），中位年龄为124个月。临床数据、手术记录、病理结果及治疗总结均取自病历。
41例患者中7例（17%）为乳头状组织学类型，34例（82.4%）为非乳头状组织学类型。18例患者（43.9%）为I期，1例患者（2.4%）为II期，2例患者（4.8%）为IIIA期，10例患者（24.3%）为IIIB期，9例患者（21.9%）为IV期疾病。1例患者在诊断时为双侧受累。7例患者出现疾病复发。肺和肝是最常见的远处转移部位，通常是致命的。在本研究中，影响预后的主要因素是分期。所有患者20年无事件生存率为53.5%。所有患者20年总生存率为54.9%。
RCC在儿童和青少年中是一种罕见疾病。与成人相比，这种肿瘤在儿童中有不同的临床表现，但预后相同。根据我们的经验，局限性疾病患者仅通过肾切除术即可治愈。需要对更多患者进行前瞻性研究，以证实放射治疗和生物反应调节剂作为RCC III期有效辅助治疗的作用。对于晚期疾病患者，似乎有必要采用替代疗法。
