Relapsed Wilms’ tumor in pediatric patients: challenges in low- to middle-income countries—a single-center experience (original) (raw)
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How curable is relapsed Wilms' tumour? The United Kingdom Children's Cancer Study Group
Archives of Disease in Childhood, 1990
Three hundred and eighty one children with Wilms' tumour were treated on the United Kingdom Children's Cancer Study Group WT1 Study (1980/6). Seventy one patients relapsed during or after treatment, which included surgery and chemotherapy, with irradiation depending on stage and histology. Despite treatment with various combinations of chemotherapy, surgery, and radiotherapy there were only 17 survivors. For unfavourable histology, any stage, only two of 20 survive. We conclude that, after relapse, even for patients who have had localised disease and favourable histology, the 'salvage' rate is little more than 50% and for all others the likelihood of cure is very small. Three of 41 children who relapsed less than 12 months from diagnosis survive, compared with 14 of 30 who relapsed later. It is essential that even with this 'good prognosis' tumour initial treatment is optimal and given by centres experienced in management of children's cancer. Furthermore, there is a clear need for additional effective chemotherapeutic agents for relapsed patients.
Combined Modality Therapy of Pediatric Wilms' Tumor in Upper Egypt: A Retrospective Study
Middle East Journal of Cancer, 2012
Background: We conducted a retrospective analysis to investigate the clinical outcome of combined modality therapy using multiagent chemotherapy, nephrectomy, and radiotherapy in treatment of children with Wilms' tumor. Methods: This study was conducted on 91 cases of newly diagnosed Wilms' tumor from January 2001 until February 2012. Patients were categorized into two groups according to treatment approach: i) preoperative chemotherapy with delayed surgery (group A; n=66) and ii) immediate surgery (group B; n=25). Results: Preoperative chemotherapy showed a 54.5% partial response rate in group A patients. A final stage distribution indicated that the majority of patients (64%) from both groups were considered to be in the early disease stages (I and II). The median follow up was 49 months (range 3-124). The five-year overall survival rate was 66.5%, whereas the event-free survival rate was 62.5%. In univariate analysis, factors associated with statistically significant reduction in overall (P<0.0001) and event-free survival (P=0.0001) rates included advanced disease stages (P<0.0001 for both) and blastimal subtype (P=0.0067 for overall survival; P=0.012 for event-free survival). Age of >24 months was associated with a significant reduction in the overall survival rate (P=0.038, HR: 0.438, 95% CI: 0.192-0.953), but was not significant in terms of eventfree survival (P=0.104, HR: 0.539, 95% CI: 0.256-1.136). Age >24 months (P=0.0095), disease stage (P=0.0014), and blastimal subtype (P=0.006) were associated with significant increases in relapse rate. Conclusion: Preoperative chemotherapy resulted in a final stage redistribution that placed the majority of patients in the early stages of the disease. Age at diagnosis, disease stage, and histological subtype significantly affected survival and relapse rates.
Survival Analysis of Pediatric Wilms Tumor Based on Risk Identification
Indonesian Journal of Cancer, 2022
Background: Wilms Tumor (WT) or nephroblastoma is the most common primary malignant tumor of the kidney found in children (comprising about > 95% of all kidney tumors). The study of WT prognostic factors has not been elaborated enough in Indonesia. This study aimed to determine the prognostic factors of WT patients in Adam Malik Hospital, Medan. Methods: This study was conducted with a retrospective design due to the rarity of WT cases. A total of 21 WT patients diagnosed from 2003 to 2019 were taken from medical records at Adam Malik Hospital, Medan. Univariate and multivariate Cox regression analyses were performed to determine the independent prognostic factors of WT. The primary endpoint of this study was patients' overall survival (OS) obtained by the Kaplan-Meier analysis on significant variables. Results: From the univariate Cox regression analysis, gender was found to be the sole significant factor (HR = 0.218, p = 0.005) where males have a lower hazard ratio. The multivariate Cox regression analysis yielded an age of diagnosis (HR = 13.860, p = 0.014) and complete tumor removals (HR = 0.056, p = 0.008). The Kaplan-Meier analysis was performed on three significant variables mentioned before. Only gender yielded a significant Mantel-Cox log-rank score (p = 0.002) with male patients found to have better survivability with a median survival of 476 days compared to that of females of 11 days. The three-year survival of males was 45.45% while all females did not survive until the cutoff. Conclusions: Three prognostic factors, including children's gender, age of diagnosis, and tumor removal status, were confirmed to be prognostic factors for the overall survival of children with WT. Further studies covering broader demographic areas were suggested to confirm significant results.
International Journal of Radiation Oncology*Biology*Physics, 2012
Purpose: We analyzed whether the prognosis can differ among Wilms tumors (WT) labeled as Stage III according to currently adopted classification systems. Methods and Materials: Patients with nonanaplastic Stage III WT consecutively registered in two Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP) trials (CNR-92, TW-2003) were the subjects in the present analysis. The steady mainstay of therapy was primary nephrectomy, followed by three-drug chemotherapy with vincristine, dactinomycin, doxorubicin, and abdominal radiotherapy (RT). Results: Ninety-nine WT patients met the criteria for classification as Stage III according to a revised version of the National Wilms Tumor Study-3 staging system (51 patients in CNR-92, 48 patients in TW-2003). Regional lymph nodes (LN) were not biopsied in 16 patients. After a median follow-up of 66 months, the 4-year disease-free survival (DFS) and overall survival (OS) rates were 85% ± 4% and 92% ± 3%, respectively, for the whole group. For 38 children with positive LN, the 4-year DFS rate was 73% ± 7%, as opposed to 98% ± 2% for the 45 children with Stage III WT according to the other criteria but with negative biopsied LN (p = 0.001). The subgroup with the worst prognosis consisted of children more than 2 years old with positive LN (DFS 67% ± 8%). A delay between surgery and RT > 30 days had an adverse impact on the abdominal tumor relapse rate. Conclusions: This study provides further evidence that Stage III tumors with LN metastases might be distinguished from WTs meeting the other criteria for classification as Stage III. The worse outcome of the former may warrant a prospective study on the effects of intensified therapy. A subclassification of Stage III tumors is discussed. Ó 2012 Elsevier Inc.
Improved Survival of Children With Wilms Tumor
Journal of Pediatric Hematology/Oncology, 2006
To analyze changes in the overall survival (OS) rate of children with Wilms tumor treated in a single institute over nearly 30 years. This study included 327 children with a newly diagnosed Wilms tumor. Their median age was 3 years, and the male:female ratio was 1.1. Survival rates were analyzed according to the stage of disease, histopathology, and different treatment regimens used between 1972 and 1999. At diagnosis, 51.1% of patients had advanced stage disease. Ten patients had anaplasia, and; 97% (317 patients) of the tumors had favorable histopathology. The 10-year OS rate was 60.6% for the entire group, but varied according to the years in which the patients were treated, the chemotherapy regimen, and stage of disease.
Advances in Clinical and Experimental Medicine, 2014
Background. The risk factors responsible for recurrences of Wilms' tumor (nephroblastoma) are still under discussion. The aim of the study was to analyze the relationship between relapses of Wilms' tumor and the patients' clinical history. Material and Methods. Clinical data from children registered in the Polish Pediatric Solid Tumors Study Group were analyzed. The clinical stages (CS), pathology variants (high risk: HR, intermediate risk: INT, and low risk: LOW) and chemotherapy regimens were correlated with the outcomes. Results. Recurrences developed in 34 out of 288 (11.8%) patients with Wilms' tumor treated in accordance with International Society for Pediatric Oncology 2001 (SIOP 2001) protocols. Of these 34 patients, 11 initially had CS I, seven were at CS II, four were at CS III, 11 were at CS IV and one had CS V. There were eight patients with second recurrences; of these, seven were in the INT risk group and one in the high histological risk group. There was no correlation between age (p = 0.256) or gender (p = 0.538) and the risk of tumor recurrence. In the study group, seven out of 10 patients with local recurrences are alive; as are 13 out of 22 patients with distant recurrences
Annals of Oncology, 2002
Background: The outcome of children with relapsed Wilms' tumor is poor, especially with poor-risk factors such as unfavorable histology, early recurrence, previous three-drug therapy, relapse not confined to lungs and abdominal relapse following abdominal radiotherapy. We report the overall response rate, progression-free survival and overall survival of 11 children with relapsed and poor-risk Wilms' tumor following ifosfamide/carboplatin/etoposide (ICE) chemotherapy. Patients and methods: ICE therapy consisted of ifosfamide 1800 mg/m 2 /day (on day 0-4), carboplatin 400 mg/m 2 /day (on day 0-1) and etoposide 100 mg/m 2 /day (on day 0-4). The median age at diagnosis was 39 months (range from 13 months to 16 years) and the median time to relapse after initial diagnosis was 9 months (range 4-72 months). All but one patient had at least one poor prognostic feature, with eight patients showing three or four. Results: After ICE chemotherapy the number of patients showing a complete response (CR) was three (27%) and a partial response (PR) was six (55%). The overall response rate (CR+PR) was 82%. Five of the six patients with a PR subsequently achieved a CR with further therapy. The 3-year event-free survival and overall survival were 63.6 ± 14.5%. Conclusions: The response rate in children with relapsed and poor-risk Wilms' tumor is >80% with ICE re-induction chemotherapy followed by post-ICE therapy. The optimal approach for post-ICE consolidation therapy has yet to be determined.
Pediatric Hematology Oncology Journal, 2016
Background: Wilms tumor is a very common renal malignancy in children. Prognosis has been improved dramatically during the last few decades because of multimodal treatment and successful sequential studies. Through a retrospective study conducted in the National Institute of Oncology of Rabat, concerning children with Wilms tumor treated following the International Society of Pediatric Oncology protocol (93-01) between 2005 and 2010, we report the experience of our institute in treatment of this malignancy. We analyze also the clinicopathologic and therapeutic aspects impacting the outcome results and compared to literature data. Results: Fifty-two patients with Wilms tumor treated in the department of radiotherapy after receiving chemotherapy and surgery at the department of hemato-oncology in children hospital of Rabat were enrolled. The main characteristic was the high prevalence of locally advanced and metastatic stages (32.6% of stage IV). With a median follow up of 54.8 months [20e79], we observed a complete response in 32 cases (61.5%), local recurrence in only one case (1.9%), metastatic relapse in 3 cases (5.8%), both local and metastatic recurrence in 3 cases (5.8%) and disease progression in 8 cases (15.4%). The mean duration of overall survival was 91.2 months. The estimated 2-year and 5-year overall survival were 78.7% and 70.1% and for metastatic patients 68.8% and 62.5% respectively. At univariate analysis several parameters were tested for survival, but only age, anaplasia, lymph node involvement, type of metastasis and response to treatment were found to significantly impact the overall survival. Outcome was better for localized tumors (stage I, II and III) compared with disseminated tumors (stage IV and V) combined. Also a better survival rate was found in the low and intermediate risk group compared to high risk, but not statistically significant. Conclusion: The relatively low outcome found in this series compared to literature can be mainly explained by the higher prevalence of metastatic disease compared to other series, but also by diagnosis and therapeutic delay, more likely because of bad socioeconomic conditions and lack of coordination between different operators. However, our results are nevertheless comparable to maghrebian series. Our department has established many procedures for improving the outcome and further studies are necessary to evaluate their efficiency.