Jain Vishesh, Dhua Anjan, Agarwala Sandeep, Bakhshi Sameer, Srinivas M, Iyer Venkateswaran K, Mathur Sandeep, Jana Manisha, Kandasamy Devasenathipathy, Biswas Ahitagni, Bhatnagar Veereshwar, Bajpai Minu
Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, India.
Department of Medical Oncology, Dr. BRA Institute-Rotary Cancer Hospital, All India Institute of Medical Sciences, New Delhi, India.
J Indian Assoc Pediatr Surg. 2020 Nov-Dec;25(6):372-377. doi: 10.4103/jiaps.JIAPS_168_19. Epub 2020 Oct 27.
Stage IV Wilms tumor is associated with poor prognosis, and recent changes in management have been suggested based on genetic markers and response to chemotherapy in this subgroup of patients.
The objective was to evaluate the outcomes of children with Stage IV Wilms tumor who were managed with the AIIMS-WT-99 protocol.
All the children with Stage IV Wilms tumor who were managed by us from October 2000 to December 2012 were included in the study. All the patients who had received primary treatment elsewhere were excluded from the study. All patients were managed as per the AIIMS-WT-99 protocol. After appropriate investigations, tumors that were deemed resectable underwent an upfront surgery. Unresectable and inoperable tumors received chemotherapy after cytological confirmation of the diagnosis. Chemotherapy was administered as per the NWTS-5 study. Pulmonary and flank radiotherapy was advised to all patients. Patients with poor response to chemotherapy or with recurrence were managed with an alternative chemotherapy regimen. The outcomes that were assessed the 4-year overall survival (OS) and the 4-year event-free survival (EFS).
Kaplan-Meier survival estimates.
During the study period, 219 patients with Wilms tumor were treated. Of these, 36 (16.4%) had Stage IV disease, and they formed the study group. The 4-year OS was 48% with a mean survival time of 59 months limited to 115 months (95% confidence interval: 41.3-75.9 months). The 4-year EFS was 42.4%. Patients with liver metastases had a poor outcome, whereas patients with good response to chemotherapy had a good outcome.
Stage IV Wilms had a poor prognosis, and the survival rates in the index study are lower than those quoted in the literature. Although the exact reason for this poor result eludes us, these patients may benefit from the intensification of chemotherapy.
IV期肾母细胞瘤预后较差，基于该亚组患者的基因标志物和化疗反应，近期有人提出了管理方面的改变。
评估采用全印度医学科学研究所肾母细胞瘤-99方案管理的IV期肾母细胞瘤患儿的治疗结果。
本研究纳入了2000年10月至2012年12月期间由我们管理的所有IV期肾母细胞瘤患儿。所有在其他地方接受过初始治疗的患者均被排除在研究之外。所有患者均按照全印度医学科学研究所肾母细胞瘤-99方案进行管理。经过适当检查后，被认为可切除的肿瘤先进行手术。不可切除和无法手术的肿瘤在细胞学确诊后接受化疗。化疗按照国家肾母细胞瘤研究组-5研究进行。建议所有患者接受肺部和侧腹放疗。化疗反应差或复发的患者采用替代化疗方案进行管理。评估的结果为4年总生存率（OS）和4年无事件生存率（EFS）。
Kaplan-Meier生存估计。
在研究期间，共治疗了219例肾母细胞瘤患者。其中，36例（16.4%）患有IV期疾病，他们组成了研究组。4年总生存率为48%，平均生存时间为59个月，最长为115个月（95%置信区间：41.3 - 75.9个月）。4年无事件生存率为42.4%。有肝转移的患者预后较差，而化疗反应良好的患者预后较好。
IV期肾母细胞瘤预后较差，本研究中的生存率低于文献报道。尽管我们尚不清楚导致这种不良结果的确切原因，但这些患者可能会从强化化疗中获益。
