Wilms tumor: “State-of-the-art” update, 2016

Irtan, Sabine; Ehrlich, Peter F.; Pritchard‐Jones, Kathy

doi:10.1053/j.sempedsurg.2016.09.003

Cited by 89 publications

(31 citation statements)

References 54 publications

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“…Wilms tumor is a kidney malignancy that occurs frequently in children and is associated with various genetic alterations . Surgery combined with chemoradiation therapy, based on the stage of disease, has historically been the standard treatment for Wilms tumor . Given the recent advances in the molecular pathogenesis of Wilms tumor and the identification of several novel driver mutations associated with a common set of developmental pathways, therapy targeted at these pathways may be beneficial …”

Section: Introductionmentioning

confidence: 99%

Calcinosis cutis dermatologic toxicity associated with fibroblast growth factor receptor inhibitor for the treatment of Wilms tumor

et al. 2018

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Small-molecule inhibitors (nibs) have revolutionized cancer therapy with the emergence of clinically efficacious treatment for advanced-stage malignancies. Fibroblast growth factor receptor (FGFR) inhibitors have shown therapeutic efficacy in malignancies with molecular-genetic alterations in the FGFR/fibroblast growth factor pathway. In a phase 1 clinical trial, erdafitinib, a pan FGFR inhibitor, was well tolerated with a manageable toxicity profile. Hyperphosphatemia was a frequent adverse event in patients treated with erdafitinib; however, no serious complications were observed with this therapy. Here, we report the development of calcinosis cutis dermatologic toxicity in a patient with hyperphosphatemia while treated with a novel selective FGFR inhibitor, INCB 54828-101. Awareness of this form of dermatologic toxicity from an FGFR inhibitor will be important for close monitoring of serum levels of phosphate, FGF23, vitamin D, and calcitriol, the management of adverse serum chemistry with chelators, and treatment decisions to either reduce dose or withhold FGFR inhibitor.

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Section: Introductionmentioning

confidence: 99%

Calcinosis cutis dermatologic toxicity associated with fibroblast growth factor receptor inhibitor for the treatment of Wilms tumor

et al. 2018

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“…Multidisciplinary treatment methods have led to OS rates of> 90% in patients with early stage disease 11 . However, management of high-risk patients such as anaplastic Wilms tumor has still been a challenge 12 .…”

Section: Discussionmentioning

confidence: 99%

Treatment results of Wilms tumour

Cesur¹,

Küpeli

Özçelik

2019

Cukurova Medical Journal

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The purpose of this study was to evaluate the treatment results of the patients with Wilms tumour (WT). Materials and Methods: The clinical records of 21 children with WT treated between 2012 and 2017 were retrospectively reviewed. Age, nationality, symptoms, diagnostic and surgical methods, surgical complications, the stage of the disease, the status of neoadjuvant chemotherapy, radiotherapy status, presence of recurrent disease and histopathological characteristics of the tumour and outcome of the patients were analyzed. Results: The female/male ratio was 2/1 and the mean age was 4.21±2.26 years. The most common physical examination finding was abdominal mass. The diagnosis was made with clinical examination and radiological investigation in 90.5% of the patients. Total nephroureterectomy was performed in all patients. The most common histopathological subgroup was classical Wilms Tumour that was detected in 81 % of the patients. 6.8 % of the patients had distant metastasis at the time of the diagnosis. The most common distant metastasis site was lungs. Overall survival (OS) of the patients was 85.7% and mean survival time was 36 months (2 months-62 months). Advanced stage at diagnosis, presence of distant metastasis and diffuse anaplastic histology were associated with worse OS rates. Conclusion: Similar to existing knowledge on outcome of patients with Wilms tumor, our results confirmed the negative effect of advanced stage, distant metastasis and diffuse anaplastic histology on survival. Further large-scale studies investigating molecular features of the tumor are needed to decide for proper treatment and to better predict outcome. Amaç: Bu çalişmanın amacı Wilms tümörlü (WT) hastaların tedavi sonuçlarını değerlendirmektir. Gereç ve Yöntem: 2012-2017 yılları arasında WT tanılı 21 hastanın klinik kayıtları geriye dönük olarak incelendi. Yaş, uyruk, semptomlar, teşhis ve cerrahi methotlar, cerrahi komplikasyonlar, hastalığın evresi, neoadjuvan kemoterapi durumu, radyoterapi durumu, rekürren hastalığın varlığı, tümörün histopatolojik özellikleri ve hastaların sonuçları analiz edildi. Bulgular: Kız/erkek oranı 2/1olup, ortalama yaş 4.21±2.26 yıldı. En sık saptanan fizik muayene bulgusu abdominal kitle idi. Hastaların %90,5'ine radyolojik bulgular ve klinik muayene ile tanı konuldu. Hastaların tamamına total nefroüreterektomi uygulandı. Hastaların %81'inde saptanan histopatolojik alt grup klasik Wilms tümörü idi. Hastaların %6,8'inde tanı anında uzak metastaz olup en sık uzak metastaz yeri akciğerdi. Hastaların genel sağkalımı (OS) %85,7 idi ve ortalama sağkalım süresi 36 aydı(2 ay-62 ay). Tanı anında ileri ever hastalık olması, uzak metastaz varlığı ve difüz anaplastik histoloji ile sağkalım oranları belirgin olarak etkilenmekteydi. Sonuç: Literatürdeki verilere benzer şekilde, çalışmamızın bulguları Wilms tümöründe ileri evre, uzak metastaz ve yaygın anaplastik histolojinin sağkalım üzerindeki olumsuz etkilerini doğrulamıştır. Uygun tedaviye karar vermek ve prognozu daha kesin olarak belirleyebilmek için tümörü...

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“…Wilms' tumour (WT) is one of the most common abdominal malignant neoplasms of childhood, with highly successful treatment options developed over the last three decades . Management includes surgical resection, chemotherapy and radiation, administered under different multi‐institutional protocols generated mainly in North America and Europe . The decision to proceed with upfront surgery (radical nephrectomy vs partial/nephron‐sparing surgery [NSS]) vs neoadjuvant chemotherapy (NaC) not only depends on which protocol is being followed, but also on patient variables such as the presence of unilateral vs bilateral disease and diagnosis of a genetic predisposition syndrome .…”

Section: Introductionmentioning

confidence: 99%

Volumetric assessment of unaffected parenchyma and Wilms’ tumours: analysis of response to chemotherapy and surgery using a semi‐automated segmentation algorithm in children with renal neoplasms

et al. 2020

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ConclusionVolumetric analysis is feasible and allows objective assessment of tumour and parenchyma volume in response to chemotherapy and surgery. Our data show changes after therapy that may be otherwise difficult to quantify. Use of such technology may improve surgical planning and quantification of response to treatment, as well as serving as a tool to predict renal reserve and long-term changes in renal function.Additional Supporting Information may be found in the online version of this article: Data S1. Detailed description of segmentation methodology.

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Wilms tumor: “State-of-the-art” update, 2016

Cited by 89 publications

References 54 publications

Calcinosis cutis dermatologic toxicity associated with fibroblast growth factor receptor inhibitor for the treatment of Wilms tumor

Calcinosis cutis dermatologic toxicity associated with fibroblast growth factor receptor inhibitor for the treatment of Wilms tumor

Treatment results of Wilms tumour

Volumetric assessment of unaffected parenchyma and Wilms’ tumours: analysis of response to chemotherapy and surgery using a semi‐automated segmentation algorithm in children with renal neoplasms

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