Weisbach L, Aziz A, Fisch M, Riechardt S
Klinik und Poliklinik für Urologie, Universitätsklinikum Hamburg-Eppendorf, Martinistraße 52, 20246, Hamburg, Deutschland.
Urologe A. 2017 Jul;56(7):900-904. doi: 10.1007/s00120-017-0420-6.
Renal cell carcinoma is a rare childhood disease. However, it should be considered in the differential diagnosis in the detection of a renal mass.
Incidence and mortality of renal cell carcinomas in childhood. Presentation of clinical symptoms, diagnostics, histology, therapy and course of the disease.
Evaluation of our own prospective data of a small patient population of 8 patients, as well as discussion of the current literature on this tumor entity.
The average follow-up period was 27 months. Preoperative staging studies showed no metastases. The histologically primary predominant subtype was papillary renal cell carcinoma. Tumor stage and lymph node status were the most important prognostic factors in our study.
Renal cell carcinoma in childhood is a rare disease whose treatment is challenging and should be performed in an interdisciplinary team. Typical clinical symptoms, as in adults, are absent. The partial nephrectomy should be, if practicable, the surgical treatment of choice. The most important risk factors for survival are tumor stage and lymph node status. Data on adjuvant target therapy for metastatic disease in childhood are lacking. Further prospective, multicenter studies are necessary to generate more information on the biology and course of this disease and to obtain adjuvant treatment options in locally advanced disease.
肾细胞癌是一种罕见的儿童疾病。然而，在发现肾肿块时的鉴别诊断中应考虑到它。
儿童肾细胞癌的发病率和死亡率。临床症状、诊断、组织学、治疗及疾病进程的呈现。
评估我们自己关于8例小患者群体的前瞻性数据，并讨论有关该肿瘤实体的当前文献。
平均随访期为27个月。术前分期研究显示无转移。组织学上主要的优势亚型是乳头状肾细胞癌。在我们的研究中，肿瘤分期和淋巴结状态是最重要的预后因素。
儿童肾细胞癌是一种罕见疾病，其治疗具有挑战性，应由多学科团队进行。与成人一样，缺乏典型的临床症状。如果可行，部分肾切除术应是首选的手术治疗方法。生存的最重要风险因素是肿瘤分期和淋巴结状态。缺乏关于儿童转移性疾病辅助靶向治疗的数据。需要进一步开展前瞻性、多中心研究，以获取更多关于该疾病生物学和进程的信息，并获得局部晚期疾病的辅助治疗方案。
