Huszno Joanna, Starzyczny-Słota Danuta, Jaworska Magdalena, Nowara Elżbieta
Maria Skłodowska-Curie Memorial Cancer Center and Institute of Oncology, Gliwice Branch, Poland.
Clinical and Experimental Oncology Department, Maria Skłodowska-Curie Memorial Cancer Center and Institute of Oncology, Gliwice Branch, Poland.
Cent European J Urol. 2013;66(1):39-44. doi: 10.5173/ceju.2013.01.art12. Epub 2013 Apr 26.
Wilms' tumour is one of the commonest malignant tumours of childhood. It appears mainly in the first 5 years of life. Incidental examples of nephroblastoma in adults have been described in literature (about 3% of all described cases). There are diagnostic and therapeutic difficulties in that older age group. The preoperative diagnosis of nephroblastoma in adults is difficult because there are no specific radiographic findings that allow to distinguished it from the more common adult renal tumors. Histopathologically, there is no difference between adult and childhood Wilms' tumor.
The PubMed database and current literature search was conducted for reports on clinical and histopathological features of nephroblastoma in adults. We also reviewed the literature in terms of treatment strategy, toxicity and prognostic factors.
Up till now, several biological factors have been identified that may be in future new prognostic factors. Modern treatment regiments improved OS in this group of patients (OS rates of 90%). The prognosis remain still worse for about 25% of patients with anaplastic, bilateral and recurrent disease.
Due to the fact that nephroblastoma is a very rare type of cancer, adult patients should be treated in an individual way based on the available schemes used in children. Toxicity in adults is higher than in children.
肾母细胞瘤是儿童最常见的恶性肿瘤之一。它主要出现在生命的前5年。文献中已描述了成人肾母细胞瘤的偶发病例(约占所有已描述病例的3%)。在该年龄组中存在诊断和治疗困难。成人肾母细胞瘤的术前诊断困难,因为没有特异性的影像学表现可将其与更常见的成人肾肿瘤区分开来。在组织病理学上,成人和儿童肾母细胞瘤并无差异。
对PubMed数据库及当前文献进行检索,以获取关于成人肾母细胞瘤临床和组织病理学特征的报告。我们还从治疗策略、毒性和预后因素方面对文献进行了综述。
到目前为止,已确定了几种生物学因素,未来它们可能成为新的预后因素。现代治疗方案改善了该组患者的总生存期(总生存率为90%)。对于约25%的间变性、双侧性和复发性疾病患者,预后仍然较差。
由于肾母细胞瘤是一种非常罕见的癌症类型,成人患者应根据儿童可用的方案进行个体化治疗。成人的毒性高于儿童。