Renal cell carcinoma in children: A clinicopathologic study
Renal cell carcinoma in children: A clinicopathologic study
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DOI：
10.1200/jco.2003.02.072
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发表时间：
2003-02-01
影响因子：
45.3
通讯作者：
Di Tullio, MT
中科院分区：
文献类型：
--
作者：
Indolfi, P;Terenziani, M;Di Tullio, MT
目的：为了确定预后因素，治疗和肾细胞癌（RCC）的儿童影响的结果：患者和方法：该系列包括41例（18名男性和23名女性），中位年龄为124个月，观察11意大利协会儿科血液学和肿瘤中心从1973年1月至2001年1月。临床资料，手术记录，病理结果，和治疗的总结取自charts.Results：7（17%）的41例患者有乳头状组织学，34（82.4%）有非乳头状组织学。18例患者（43.9%）为1期，1例患者（2.4%）为11期，2例患者（4.8%）为IIIA期，10例患者（24.3%）为11113期，9例患者（21.9%）为IV期疾病。1例患者在诊断时双侧受累。7例患者出现疾病复发。肺和肝是最常见的远端病变，通常是致命的。在本研究中，影响预后的主要因素是分期。所有患者的20年无事件生存率为53.5%。20年总生存率为54.9%，所有patients.Conclusion：肾细胞癌是一种罕见的疾病，在儿童和青少年。这种肿瘤在儿童中的临床表现与成人不同，但结局相同。根据我们的经验，局限性疾病的患者可以通过单独的肾切除术治愈。需要在大量患者中进行前瞻性研究，以确认放射治疗和生物反应调节剂作为III期RCC的有效辅助治疗。在晚期疾病患者中，替代疗法似乎是必要的。(C)2003年，美国临床肿瘤学会。
Purpose : To identify the prognostic factors, treatment, and outcome of children affected by renal cell carcinoma (RCC).Patients and Methods: 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.Results: Seven (17%) of the 41 patients had a papillary histology, and 34 (82.4%) had nonpapillary histology. Eighteen patients (43.9%) had stage 1, one patient (2.4%) had stage 11, two patients (4.8%) had stage IIIA, 10 patients (24.3%) had stage 11113, 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.Conclusion: 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 Ill. The alternative therapy seems warranted in patients with advanced disease. (C) 2003 by American Society of Clinical Oncology.