Saga of Wilms′ tumor: Lessons learnt from the past

Abstract

ABSTRACT ABSTRACT ABSTRACTWilms' tumor (WT) represents 6% of childhood cancers. Recent advances in molecular biology have significant implications for the clinical management. A dramatic improvement in overall cure has resulted from the adoption of multimodality treatment during the past few decades. National Wilms' Tumor Study (NWTS), and the Société Internationale d'Oncologie Pédiatrique (SIOP) have laid down the guidelines for standardized treatment of WT, though differing in the diagnostic and therapeutic approach. Both these groups currently aim at intensifying treatment for patients with poor prognosticators while appropriating the therapy to reduce long-term complications for those with favorable prognostic features. Challenges faced in developing nations include poverty, malnutrition, ignorance, and presentation in advanced stages coupled with limited facilities that are necessary for total management of these cases. In this article, we have discussed our approach to deal with patients with nephroblastoma, reviewed the literature on the current management strategies and the long-term outcome. Most countries have adopted the NWTS protocols, while others especially in Europe, South America and some Asian countries follow the SIOP regimen. Both have their advantages and weaknesses and maynot necessarily be suitable to the setup in developing countries. We have discussed the controversial issues in the management of WT including the timing of biopsy, type of biopsy, investigative approach, role of chemotherapy / radiotherapy, management of bilateral Wilms' tumor and parenchymal sparing renal surgery. Despite deviating from NWTS at various points, the overall results have remained satisfactory. Thus, developing countries might adopt their own protocols depending on the prevalent situations and facilities available to them to treat such patients.

Management of Wilms′ tumor: NWTS vs SIOP

“…[ 37 38 ] Doxorubicin was added to the Wilms' tumor treatment protocol in the 1970s. [ 39 ] With the conclusions of the trials conducted by SIOP and NWTS, it is clear that the duration of chemotherapy has been reduced drastically, e.g., 6 months of post-operative chemotherapy instead of 15 months for Stage II to IV is sufficient as per NWTS 4 study,[ 30 ] and 4 instead of 8 weeks for Stage I tumors as per SIOP 93-01 study. [ 33 ] Progressive research and trails have optimized the use of chemotherapeutic agents and in future too, there will be many more modifications in the use of these agents so that acute as well as long-term toxicities are lessened to improve the quality of life of the children affected by Wilms' tumor.…”

“…Very few detailed studies are available from India, and the ones available follow the NWTS protocol. [ 39 40 ] Few centers have tried with upfront chemotherapy in Wilm's tumor patients with FNAC confirmation of the diagnosis as per the UKCCSG protocol. [ 41 ] To date, no published reports have been found regarding the outcome of WT as per the SIOP protocol in our country.…”

Glomerular filtration rate and prevalence of chronic kidney disease in Wilms’ tumour survivors

“…Wilms’ tumour is the most frequently diagnosed tumour in the kidneys of children [1]. It represents approximately 7% of all malignancy in children [2].…”

Imaging spectrum of primary malignant renal neoplasms in children

“…Specific germline mutations on WT1 and WT2 genes located on chromosome 11 may be seen in a minority of the cases. [24]…”