Wilms tumor in Africa: A systematic review of management challenges and outcome in two decades (2000‐2019)

Ekenze, Sebastian O.; Okafor, Okechukwu Charles; Obasi, Akputa Aja; Okafor, David C.; Nnabugwu, Ikenna I

doi:10.1002/pbc.28695

Cited by 17 publications

(29 citation statements)

References 43 publications

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“…A recent systematic review on treatment outcomes in eastern African patients with WT showed that OS adjusted for abandonment in 2010-2019 was 46.1% (range 25%-63.2%), which corresponds to survival rates seen in our study. 30 In the Collaborative Wilms Tumor Project, however, EFS was higher than in our study (respectively, 69% compared to 43.5%), but they excluded patients with bilateral tumors. 9…”

Section: Discussioncontrasting

confidence: 80%

Outcomes of Wilms tumor treatment in western Kenya

Uittenboogaard

Njuguna

Mostert

et al. 2021

Pediatric Blood & Cancer

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Background/objectives Wilms tumor (WT) is a curable type of cancer with 5‐year survival rates of over 90% in high‐income countries, whereas this is less than 50% in low‐ and middle‐income countries. We assessed treatment outcomes of children with WT treated at a large Kenyan teaching and referral hospital. Design/methods We conducted a retrospective record review of children diagnosed with WT between 2013 and 2016. Treatment protocol consisted of 6 weeks of preoperative chemotherapy and surgery, and 4–18 weeks of postoperative chemotherapy depending on disease stage. Probability of event‐free survival (pEFS) and overall survival (pOS) was assessed using Kaplan–Meier method with Cox regression analysis. Competing events were analyzed with cumulative incidences and Fine–Gray regression analysis. Results Of the 92 diagnosed patients, 69% presented with high‐stage disease. Two‐year observed EFS and OS were, respectively, 43.5% and 67%. Twenty‐seven percent of children died, 19% abandoned treatment, and 11% suffered from progressive or relapsed disease. Patients who were diagnosed in 2015–2016 compared to 2013–2014 showed higher pEFS. They less often had progressive or relapsed disease (p = .015) and borderline significant less often abandonment of treatment (p = .09). Twenty‐nine children received radiotherapy, and 2‐year pEFS in this group was 86%. Conclusion Outcome of children with WT improved over the years despite advanced stage at presentation. Survival probabilities of patients receiving comprehensive therapy including radiation are approaching those of patients in high‐income countries. Additional improvement could be achieved by ensuring that patients receive all required treatment and working on earlier diagnosis strategies.

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Section: Discussioncontrasting

confidence: 80%

Outcomes of Wilms tumor treatment in western Kenya

Uittenboogaard

Njuguna

Mostert

et al. 2021

Pediatric Blood & Cancer

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“…Between 2016-2021, two-year OS among patients with Wilms tumor was 48%, consistent with contemporary outcomes in the low-resource settings of sub-Saharan Africa. 23 During this period, regional OS ranged from 0-53% at approximately three years, averaging 44% and 27% in East and West Africa, respectively. Survival reported here is similar to that reported by Queen Elizabeth Central Hospital in Blantyre, Malawi where 2-year OS and EFS was 46%.…”

Section: Discussionmentioning

confidence: 90%

Section: Discussionmentioning

confidence: 98%

“…8 By comparison, OS in relatively high-income South Africa averaged 76% during the same period. 23 In the most recent SIOP and COG reports from high-income countries, OS was >80% across all risk groups, approaching 100% in low-risk disease. 4,16,24 Treatment abandonment, occurring in 27% of patients, was the major determinant of event free survival (EFS) in the cohort.…”

Section: Discussionmentioning

confidence: 98%

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Outcomes of a resource-adapted Wilms tumor treatment protocol in Lilongwe, Malawi, 2016-2021: successes and enduring barriers to cure

Holmes

Matatiyo

Mpasa

et al. 2022

Preprint

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Purpose Wilms tumor is a common renal cancer of childhood with long-term survival rates exceeding 80% in high-resource countries, yet survival remains below 50% in the low-resource settings of Africa. We assessed outcomes of a resource-adapted treatment protocol at a Malawian hospital to identify actionable factors affecting survival. Methods: We assessed clinical outcomes with a single-center retrospective cohort study of children diagnosed between 2016 and 2021 in Lilongwe, Malawi. Findings: We identified 136 patients with Wilms tumor, most commonly with stage III (25.7%) or IV disease (25.7%). Two-year overall survival (OS) was: Stage I, 78%; Stage II, 27%; Stage III, 62%; Stage IV, 23%, Stage V, 0%. Event-free survival (EFS) was: Stage I, 60%; Stage II, 0%; Stage III, 51%; Stage IV, 13%; Stage V, 0%. After death, treatment abandonment was the most common event comprising EFS, occurring in 26.5% of patients. Among 43% of patients who completed therapy, 2-year OS was 80% and EFS was 69%. Relapse was documented in 9.6% of patients. Radiotherapy was indicated for 40.4% patients, among whom only three received it due to regional unavailability. Factors associated with OS were severe acute malnutrition (Hazard ratio, HR, 1.9), increasing tumor stage (HR, 1.5), and inferior vena cava involvement (HR, 2.7). On multivariable analysis, only tumor stage remained associated with outcome. Interpretation Implementing a curative resource-adapted treatment protocol in an extremely resourced-constrained environment was feasible in Malawi and resulted in relatively favorable outcomes in low-stage disease, particularly among those who completed therapy. However, factors such as late-stage disease, frequent abandonment, and absent radiotherapy represent ongoing implementation barriers that should be the focus of continued research funding and intervention in Africa.

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“…3 The proportion of mortality due to treatment-related neutropenic complications and sinusoidal obstruction syndrome (SOS) is as high as 5%-25% in studies from LMIC. 4,5 Additionally, up to 25% of survivors experience late effects due to the cytotoxic therapy administered for the treatment of WT. 6 Hence, there is a need to identify prognostic factors that can be used to improve the risk-stratification system and stratify candidates for intensive and de-escalated therapy.…”

Section: Introductionmentioning

confidence: 99%

Postchemotherapy tumor volume as a prognostic indicator in Wilms tumor: A single‐center experience from South India

Joseph¹,

Boddu²,

Srinivasan³

et al. 2021

Pediatric Blood & Cancer

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Background: Optimal risk stratification is the key to minimizing relapse and toxicity in children with Wilms tumor (WT). The study evaluated poor tumor volume response to chemotherapy as a risk factor that predicts relapse. Procedure: Children with WT who were treated between 2005 and 2020 at the center were analyzed. Tumor volumes at the time of diagnosis and after preoperative chemotherapy were calculated from cross-sectional imaging. The International Society of Paediatric Oncology (SIOP)-WT-2001 protocol was used for treatment. The area under a receiver operating characteristic curve was estimated to ascertain the ability of tumor volume to predict relapse.Results: Ninety-five patients with a median age of 40 months were included. A postchemotherapy tumor volume cutoff of 270 ml was ascertained to have the best predictive value for relapse. Patients with a tumor volume of <270 ml following preoperative chemotherapy had a better 3-year event-free survival (EFS) than those with a tumor volume of ≥270 ml (89.8% ± 4.0% vs. 57.4% ± 12.5%, p = .001). The data demonstrated that a tumor volume of ≥270 ml after chemotherapy was associated with an increased risk of relapse (hazard ratio [HR]: 5.3, p = .006). The EFS in patients with an epithelial or stromal type of histopathology was not affected by the tumor volume response (p = .437). Conversely, patients with other types of intermediate-risk histopathology who had a poor tumor volume response had an inferior survival (3-year EFS 51.4% ± 18.7%, p = .001). Conclusion:A postchemotherapy tumor volume cutoff of ≥270 ml emerged as a strong predictor of relapse in a low-and middle-income country (LMIC) center study of WT treated with the SIOP protocol.

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Wilms tumor in Africa: A systematic review of management challenges and outcome in two decades (2000‐2019)

Cited by 17 publications

References 43 publications

Outcomes of Wilms tumor treatment in western Kenya

Outcomes of Wilms tumor treatment in western Kenya

Outcomes of a resource-adapted Wilms tumor treatment protocol in Lilongwe, Malawi, 2016-2021: successes and enduring barriers to cure

Postchemotherapy tumor volume as a prognostic indicator in Wilms tumor: A single‐center experience from South India

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