Sarin Yogesh Kumar, Losu Pute U, Nangia Anita
Department of Pediatric Surgery, Lady Hardinge Medical College and Associated Kalawati Children's Hospital, New Delhi, India.
Department of Pathology, Lady Hardinge Medical College and Associated Kalawati Children's Hospital, New Delhi, India.
J Indian Assoc Pediatr Surg. 2024 Nov-Dec;29(6):600-607. doi: 10.4103/jiaps.jiaps_87_24. Epub 2024 Nov 5.
Outcome analysis of patients with Wilms' tumors (WT) is presented.
A retrospective analysis of 23 children having WT managed by a single surgeon over 3 years (2021-2024) using the International Society of Paediatric Oncology Umbrella protocol was done.
The median age at presentation was 36 months; 32 months and 24 months for the unilateral WT (uWT) ( = 19) and bilateral WT (bWT) ( = 4), respectively. M: F ratio was 2.3: 1. WTs were localized in 19 (uWT-16; bWT-3) and metastatic in 4 (uWT-3; bWT-1) patients. Core-needle biopsy was done in 22 patients (26 renal units). Pre-therapy contrast-enhanced computed tomography volumetry ( = 20) showed a median tumor volume of 1023 ml (range: 47-2680 ml). Post-neoadjuvant chemotherapy (NACT) median tumor burden ( = 19) was 612 ml (range 59-3775 ml). Post-NACT, tumor volume decreased in 11/18 patients but increased in seven patients. NACT was avoided in one neonate. Nephroureterectomy (including one with excision of bladder cuff) and nephron-sparing surgery were done in 17 and 10 renal units including 3 with multifocal WT, respectively. Risk stratification was intermediate in 21 and High in 2. Overall staging in 19 uWT included Stage I-7, Stage II-5, Stage III-4, and Stage IV-3 (local staging-stage I in 1 and stage II in 2). Local staging in 8 renal units with bWT was Stage I in 7 and II in 1. One stage IV uWT had bilateral pulmonary metastatectomy. Adjuvant chemotherapy has been completed in 18 patients; two patients are still on adjuvant chemotherapy; flank radiation was administered in six patients. Three patients with synchronous bWT died; two due to acute kidney injury in the immediate postoperative period and one with metastatic disease who had abandoned adjuvant chemotherapy after the 1 cycle. Another patient died of a huge metachronous tumor in the contralateral kidney after a year of completion of therapy. One patient had successful multimodality treatment of local relapse with liver metastasis. 1-year overall and event-free survivals are 84% and 76%, respectively.
Excellent short-term results for localized uWT from a center in a low-middle-income country are reported.
本文介绍了肾母细胞瘤（WT）患者的预后分析。
回顾性分析了在3年（2021 - 2024年）期间由一名外科医生按照国际小儿肿瘤学会综合方案治疗的23例患WT的儿童。
就诊时的中位年龄为36个月；单侧WT（uWT）（n = 19）和双侧WT（bWT）（n = 4）的中位年龄分别为32个月和24个月。男女比例为2.3∶1。WT局限于19例患者（uWT - 16例；bWT - 3例），4例发生转移（uWT - 3例；bWT - 1例）。22例患者（26个肾单位）进行了粗针活检。治疗前对比增强计算机断层扫描容积测量（n = 20）显示肿瘤中位体积为1023 ml（范围：47 - 2680 ml）。新辅助化疗（NACT）后肿瘤中位负荷（n = 19）为612 ml（范围59 - 3775 ml）。NACT后，11/18例患者肿瘤体积减小，但7例患者肿瘤体积增大。1例新生儿未进行NACT。分别在17个和10个肾单位进行了肾输尿管切除术（包括1例切除膀胱袖口）和保留肾单位手术，其中3例为多灶性WT。21例风险分层为中危，2例为高危。19例uWT的总体分期包括Ⅰ期 - 7例，Ⅱ期 - 5例，Ⅲ期 - 4例，Ⅳ期 - 3例（局部分期 - 1例为Ⅰ期，2例为Ⅱ期）。8个bWT肾单位的局部分期为7例Ⅰ期和1例Ⅱ期。1例Ⅳ期uWT患者进行了双侧肺转移瘤切除术。18例患者已完成辅助化疗；2例患者仍在接受辅助化疗；6例患者接受了侧腹放疗。3例同步性bWT患者死亡；2例在术后即刻因急性肾损伤死亡，1例患有转移性疾病，在第1周期后放弃辅助化疗。另1例患者在治疗完成1年后死于对侧肾脏巨大的异时性肿瘤。1例患者对局部复发伴肝转移成功进行了多模式治疗。1年总生存率和无事件生存率分别为84%和76%。
报告了一个中低收入国家中心对局限性uWT取得的优异短期治疗结果。
