Indolfi Paolo, Spreafico Filippo, Collini Paola, Cecchetto Giovanni, Casale Fiorina, Terenziani Monica, Schiavetti Amalia, Pierani Paolo, Piva Luigi, Cuzzubbo Daniela, De Pasquale Maria D, Pota Elvira, Inserra Alessandro, Bisogno Gianni
Department of Pediatric, Second University of Napoli, Napoli, Italy.
J Pediatr Hematol Oncol. 2012 Oct;34(7):e277-81. doi: 10.1097/MPH.0b013e318267fb12.
Because of the rare occurrence of renal cell carcinoma (RCC) among children very little is known about this malignancy in pediatric age. We aimed adding knowledge on the clinical characteristics and outcome of metastatic (m) RCC in children and adolescents.
The series included 14 stage 4 RCC patients with a median age at diagnosis of 155.5 months, observed at the Italian Pediatric Hematology and Oncology Association (AIEOP) centers from January 1973 to November 2010. We were able to reevaluate histopatology of 11 out of the 14 patients and perform immunostaining for TFE3 in 9 out of the 11 patients.
Of the 14 patients under study, 5 (3 girls) had a translocation morphology TFE+ RCC, 2 were reassigned as papillary type 1 or 2, respectively, 2 tumor specimens with primary clear cell histology had confirmed the initial histologic diagnosis, and 2-whose biopsy specimen was insufficient-had the diagnosis of RCC not further specified with subtyping. In the remaining 3 cases, the initial diagnosis of clear cell carcinoma was left. Overall, 6 patients received chemotherapy, 9 immunotherapy, and 2 adjuvant antiangiogenic therapy. Overall, 11 patients (78.5%) never achieved complete remission and died from progressive disease 1 to 16 months after diagnosis (median overall survival 5.5 mo). Three patients, 2 of whom received adjuvant antiangiogenic therapy, relapsed to lung at 3, 6, and 8 months after diagnosis, and died 18, 32, and 33 months after diagnosis, respectively.
Despite their possibly different biology, childhood and adult mRCC seems to be sharing comparable outcomes. Because of the very low incidence of mRCC (about 20%) in children and adolescents, an international pediatric cooperation to address biological studies and assess the novel targeted approaches is needed.
由于儿童肾细胞癌（RCC）的发病率极低，人们对儿科年龄段的这种恶性肿瘤知之甚少。我们旨在增加对儿童和青少年转移性（m）RCC的临床特征和预后的了解。
该系列包括14例4期RCC患者，诊断时的中位年龄为155.5个月，于1973年1月至2010年11月在意大利儿科血液学和肿瘤学协会（AIEOP）中心接受观察。我们能够重新评估14例患者中11例的组织病理学，并对11例患者中的9例进行TFE3免疫染色。
在研究的14例患者中，5例（3名女孩）具有易位形态的TFE + RCC，2例分别重新分类为1型或2型乳头状癌，2例原发性透明细胞组织学肿瘤标本证实了最初的组织学诊断，2例活检标本不足的患者诊断为RCC但未进一步进行亚型分类。在其余3例中，保留了透明细胞癌的初始诊断。总体而言，6例患者接受了化疗，9例接受了免疫治疗，2例接受了辅助抗血管生成治疗。总体而言，11例患者（78.5%）从未实现完全缓解，在诊断后1至16个月死于疾病进展（中位总生存期5.5个月）。3例患者，其中2例接受了辅助抗血管生成治疗，在诊断后3、6和8个月复发至肺部，分别在诊断后18、32和33个月死亡。
尽管儿童和成人mRCC的生物学特性可能不同，但它们的预后似乎相当。由于儿童和青少年中mRCC的发病率非常低（约20%），需要开展国际儿科合作以进行生物学研究并评估新型靶向治疗方法。
