Princess Máxima Center for Pediatric Oncology, Utrecht, The Netherlands.
Princess Máxima Center for Pediatric Oncology, Utrecht, The Netherlands.
Crit Rev Oncol Hematol. 2020 Jun;150:102970. doi: 10.1016/j.critrevonc.2020.102970. Epub 2020 Apr 24.
To characterize metanephric tumours in children, we performed a literature review investigating paediatric metanephric adenomas (MA), metanephric stromal tumours (MST) and metanephric adenofibromas (MAF). Including two patients from our own institution (MA, MAF), 110 individual cases (41 MA, 20 MAF, 49 MST) were identified. Additionally, fifteen composite tumours were identified, with areas of MA/MAF and Wilms tumour (WT) or papillary carcinoma. No distinct clinical or radiological features could be defined. In pure metanephric tumours, histologically proven distant metastases were reported once (MA), relapse was reported once (MST) and one tumour-related death occurred (MST). Somatic BRAF-V600E mutations were tested in 15 cases, and identified in 3/6 MA, 3/3 MAF, and 6/6 MST. In our institution the MA harboured a somatic KRAS-G12R mutation. Overall, paediatric metanephric tumours are difficult to discriminate from other renal tumours at presentation, behave relatively benign, and the occurrence of composite tumours warrants analysis of underlying (genetic) pathways.
为了描述儿童的后肾母细胞瘤，我们进行了文献回顾，研究了儿童后肾腺瘤（MA）、后肾间质瘤（MST）和后肾腺纤维瘤（MAF）。包括我们自己机构的两名患者（MA、MAF）在内，共确定了 110 例个体病例（41 例 MA、20 例 MAF、49 例 MST）。此外，还发现了 15 例复合性肿瘤，其中包括 MA/MAF 和 Wilms 瘤（WT）或乳头状癌的区域。没有明确的临床或影像学特征可以定义。在纯后肾母细胞瘤中，有一例组织学证实的远处转移（MA），一例复发（MST），一例肿瘤相关死亡（MST）。在 15 例病例中检测了体细胞 BRAF-V600E 突变，在 6 例 MA、3 例 MAF 和 6 例 MST 中发现了突变。在我们的机构中，MA 携带了体细胞 KRAS-G12R 突变。总体而言，儿童后肾母细胞瘤在发病时很难与其他肾肿瘤区分，其行为相对良性，而复合性肿瘤的发生需要分析潜在的（遗传）途径。
