Department of Urology, Chongqing Key Laboratory of Children Urogenital Development and Tissue Engineering, Chongqing Key Laboratory of Pediatrics, Ministry of Education Key Laboratory of Child Development and Disorders, National Clinical Research Center for Child Health and Disorders, China International Science and Technology Cooperation base of Child development and Critical Disorders, Children's Hospital of Chongqing Medical University, 2 ZhongShan Rd, 400013, Chongqing, PR China.
Department of Urology, Yunnan Provincial Key Research Laboratory of Pediatric Major Diseases, Yunnan Province Clinical Research Center for Children's Health and Disease, Kunming Children's Hospital, Children's Hospital Affiliated to Kunming Medical University, Kunming, PR China.
BMC Pediatr. 2022 Oct 13;22(1):591. doi: 10.1186/s12887-022-03643-1.
To discuss the clinical and prognostic indicators of pediatric malignant rhabdoid tumor of the kidney (MRTK), and to increase the understanding of the occurrence and development of MRTK.
From July 2014 to September 2021, all cases were confirmed by postoperative pathological examination. Among the 42 patients, there were 25 males and 17 females, with a median age of 10 (1-84) months. Abdominal mass or hematuria were the main clinical manifestations. Preoperative chemotherapy was performed in 9 cases (VC). The tumor stages were stage I-IV. Preoperative metastasis was found in 9 cases; the most common site was the lung. Postoperative patients received conventional chemotherapy, including VDACE regimen and UH-1 regimen. Among the 42 children in this group, survival at follow-up in this study was 26.2%(11/42).
Preoperative anemia was found by univariate analysis, hypertension and hypercalcemia had shorter survival time. In addition, tumor-related factors had a significant impact on survival, with incomplete tumor resection, lymph node metastasis, stage III-IV had a lower survival rate. The impact of postoperative factors on survival included postoperative complications had a lower survival rate. The children were younger than 12 months, preoperative metastasis, no chemotherapy was performed after surgery was an independent risk factor for the prognosis of MRTK.
The main clinical manifestations about MRTK were abdominal mass and hematuria. Preoperative chemotherapy did not significantly improve the prognosis. Postoperative chemotherapy can significantly improve the survival rate. Diagnosis depends on clinical manifestations, imaging, histopathology, immunohistochemistry and other comprehensive judgment. Age less than 12 months, preoperative metastasis, and no postoperative chemotherapy were independent risk factors for prognosis.
探讨小儿肾横纹肌样瘤（MRTK）的临床及预后指标，提高对 MRTK 发生发展的认识。
回顾性分析 2014 年 7 月至 2021 年 9 月收治的 42 例经术后病理检查证实的 MRTK 患儿的临床资料，男 25 例，女 17 例；中位年龄 10（1～84）个月。临床表现主要为腹部包块或血尿。9 例行术前化疗（VC），肿瘤分期为Ⅰ～Ⅳ期，术前转移 9 例，最常见转移部位为肺部。术后均行常规化疗，方案包括 VDACE 方案和 UH-1 方案。本组 42 例患儿随访生存 26.2%（11/42）。
单因素分析发现术前贫血、高血压、高钙血症患者的生存时间较短，此外肿瘤相关因素对生存有显著影响，肿瘤不完全切除、淋巴结转移、Ⅲ～Ⅳ期患者的生存率较低。术后因素对生存的影响包括术后并发症患者的生存率较低。患儿年龄<12 个月、术前转移、术后未行化疗是影响 MRTK 预后的独立危险因素。
MRTK 的主要临床表现为腹部包块和血尿，术前化疗并不能显著改善预后，术后化疗可显著提高生存率。诊断依赖于临床表现、影像学、组织病理学、免疫组织化学等综合判断。年龄<12 个月、术前转移、术后未行化疗是独立的预后危险因素。
