The treatment of Wilms' tumour: results of the United Kingdom Children's Cancer Study Group (UKCCSG) second Wilms' tumour study

Mitchell, Christopher; Jones, P.L.; Kelsey, Anna; Vujanić, Gordan; Marsden, Brian D.; Shannon, R S; Gornall, P.; Owens, Cátherine M.; Taylor, Roger; Imeson, John; Middleton, Helen; Pritchard, Jon

doi:10.1054/bjoc.2000.1338

Cited by 114 publications

(72 citation statements)

References 21 publications

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“…Hypercalcemia, which is an uncommon finding in childhood tumors but has been reported in infants with MRT, 8,17 was observed in 3 patients (all aged <12 months). Consistent with past series, 4,5,8 our patients tended to present with advanced disease-all but 3 patients had stage III or IV disease at diagnosis. Like what was reported in the NWTS series, 4 advanced stage was a poor prognostic factor, because all patients who presented with metastases died from progressive disease.…”

Section: Discussionsupporting

confidence: 91%

“…[1][2][3] It arises most commonly in the kidney and comprises from 1.5% to 4% of malignant renal tumors. 4,5 It has been reported that second primary tumors in the central nervous system (CNS), which are classified as atypical teratoid rhabdoid tumors, occur in from 10% to 20% of these individuals. 6,7 A variety of extracranial, extrarenal sites, such as the liver, abdomen, retroperitoneum, or other soft tissues, have been reported.…”

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confidence: 99%

“…4 Regimens containing vincristine, dactinomycin, doxorubicin, and cyclophosphamide produced little improvement in mortality rates of approximately 70% to 80%. 4,5,8 Poorer outcomes have been reported in younger patients, in patients with higher stage or metastases at presentation, and in patients with concurrent CNS disease. 4 Recent attempts to intensify therapy and incorporate alternative agents have resulted in clinical responses and even anecdotal reports of long-term survival in patients with advanced-stage disease.…”

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confidence: 99%

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Extracranial malignant rhabdoid tumors in childhood

Madigan

Armenian

Malogolowkin

et al. 2007

Cancer

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BACKGROUND.Extracranial malignant rhabdoid tumor (MRT) is a rare, aggressive, pediatric malignancy with a historically poor outcome. Recent efforts to intensify treatment for MRT have resulted in isolated reports of long‐term survival.METHODS.The authors conducted a retrospective review of consecutive patients with MRT at Childrens Hospital Los Angeles over the 20 years from 1983 to 2003.RESULTS.Fourteen children were diagnosed with MRT over the 20‐year study period. The median age at presentation was 22.5 months (range, 0.5–108 months). Five patients had renal primary tumors, and 9 patients had extrarenal tumors. Eleven of 14 patients had stage III or IV disease at diagnosis. Five patients (35.7%) were long‐term survivors. The time to disease progression was rapid (mean, 3.6 months). There were no recurrences or deaths beyond 10 months after diagnosis. All survivors received multimodal therapy, including both chemotherapy and surgery with or without radiation. In addition, 2 patients received high‐dose chemotherapy with hematopoietic stem cell rescue (HSCT) after neoadjuvant chemotherapy and local tumor control. Both of those patients were long‐term survivors. There were no survivors after disease recurrence or progression.CONCLUSIONS.Patients with localized disease and complete surgical resection were most likely to survive long‐term. Consolidation with HSCT may benefit selected patients with advanced disease stage. International collaboration and further understanding of the biology of this disease is necessary to improve the survival of children with MRT. Cancer 2007. © 2007 American Cancer Society.

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Section: Discussionsupporting

confidence: 91%

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confidence: 99%

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confidence: 99%

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Extracranial malignant rhabdoid tumors in childhood

Madigan

Armenian

Malogolowkin

et al. 2007

Cancer

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“…Current treatment strategies stratify intensity and scheduling of the different treatment modalities -surgery, radiation and chemotherapy -according to biologic and prognostic features of the initial disease, most importantly, stage and histology. Three large multiinstitutional study groups have contributed to this development: the National Wilms Tumor Study (NWTS), USA, [1][2][3] the United Kingdom Children's Cancer Study Group 4,5 and the International Society of Pediatric Oncology (SIOP). [6][7][8][9] For the 15% of patients with relapse or refractory disease, attempts have been made to use a similar risk-adapted strategy.…”

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confidence: 99%

High-dose chemotherapy with autologous stem cell rescue in children with nephroblastoma

Kremens¹,

Gruhn

Klingebiel

et al. 2002

Bone Marrow Transplant

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Summary:Children with Wilms tumor who have a particular risk of failure at relapse or at primary diagnosis were treated with high-dose chemotherapy (HDC) and autologous peripheral blood stem cell rescue in order to improve their probability of survival. Keywords: Wilms tumor; high-dose chemotherapy; autologous bone marrow transplantation A child with newly diagnosed Wilms tumor (WT) has a probability of about 85% of being cured with multimodal treatment nowadays. Current treatment strategies stratify intensity and scheduling of the different treatment modalities -surgery, radiation and chemotherapy -according to biologic and prognostic features of the initial disease, most importantly, stage and histology. Three large multiinstitutional study groups have contributed to this development: the National Wilms Tumor Study (NWTS), USA, 1-3 the United Kingdom Children's Cancer Study Group 4,5 and the International Society of Pediatric Oncology (SIOP). [6][7][8][9] For the 15% of patients with relapse or refractory disease, attempts have been made to use a similar risk-adapted strategy. These efforts were based upon the analysis of Grundy et al 10 demonstrating a 3-year survival of 30% in 367 patients who relapsed following treatment within studies NWTS-2 and -3. The authors identified prognostic factors with respect to time and site of relapse, histology and previous therapy, and used them to identify a good-risk group of relapsed patients who had a survival of >40% with conventional therapy. 10 For the complementary group of very high-risk patients, treatment alternatives were investigated. In phase II studies, single drugs or combinations gave the following results: single drug etoposide: 42% of response; 11 ifosfamide plus etoposide: overall response rate 39.5%; 12 etoposide and ifosfamide: 15% complete and 54% partial remission; 13 etoposide plus carboplatin: 70% response. 14 None of these approaches, however, could improve the long-term survival of these patients above 30%. 14 Consequently, dose-response strategies were investigated. Warkentin et al 15 who administered etoposide and thiotepa, reported eight out of 12 relapse-free survivors after 1 year. The Solid Tumor Registry of the EBMT reviewed 24

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“…It is mainly a childhood cancer, even though it occurs in adults occasionally. In Kenya, Nephroblastoma accounts for 3.3% of childhood cancers [2,3,4,5]. This is a lower incidence than in Western countries where it accounts for up to 8% of childhood cancers [6].…”

Section: Introductionmentioning

confidence: 99%

A Ten Year Retrospective Audit of the Clinic-pathological Management and Treatment Outcomes of Patient’s with Nephroblastoma (Wilms Tumor) at Kenyatta National Hospital

Mwamba¹,

Rogena²,

Kitonyi³

2017

AJCP

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Background and aims: Treatment of Nephroblastoma (Wilms' tumor) in children is one of medicine's success stories. Due to improvements in surgical techniques, drug therapies, and radiation, 85 to 90 percent of children with Wilms tumor who receive state-of-the-art treatment are cured. While this is true for developed countries the outcomes of nephroblastoma (Wilms' tumor, WT) in our hospital and other developing countries are notably below those in these countries. There are continuous reviews and updates of clinical pathologic features, treatment designs, and treatment outcomes every 2 or 3 years, in the developed countries. However, in resource poor countries, such frequent reviews and updates are presently not affordable but, nevertheless, efforts should be made to undertake them every 5 or so years, to improve patient management and treatment outcomes. The objectives of this study were to review the clinical presentation and management of children with Wilms tumor and the factors influencing the outcome at Kenyatta National Referral and Teaching Hospital (KNH). This was a retrospective descriptive cross-sectional study. Analysis of the data was carried out using the statistical package for the social sciences (SPSS) software. The findings of the study will form a basis for revision of treatment design for patients diagnosed with Nephroblastoma at KNH, and by extension, other tertiary public health care facilities in Kenya. Patients and methods: The records of 140 WT patients, aged less than 16 years, who were treated in Kenyatta National Hospital, Kenya, during the period from January 1997 to December 2008 were reviewed. The management protocol followed the scheme of the US National Wilms Tumor Study Group (NSWTG). Results: Thirteen cases (38.2%) were diagnosed as stage I, 4 (11.8%) as stage II, 13 (38.2%) as stage III and 2 (5.9%) as stage IV. Eight cases had bilateral disease (stage V). Four-year overall survival (OS) and event free survival (EFS) rates were xx% and xx%, respectively. Univariate analysis by Log-rank test revealed statistically significant associations between OS and nodal status (p-value < 0.01), manifestation of gross hematuria (p-value 0.02), and tumor size of 10 centimeters or more (p-value 0.02). Multivariate analysis found only the nodal status to be independently associated with OS at a Hazard Ratio of 16.6 (p-value < 0.01). Eight of 13 stage I cases and 6/13 stage III cases had relapsed, with two-year post-relapse survival of 42.8%. Significantly poorer outcome was found in cases with early relapse within 200 days after enrollment (p-value 0.02). Conclusion: Childhood Wilms' tumor presents late in our setting with its consequent management challenges. The need to educate the populace and the primary healthcare providers on the benefits of early diagnosis and treatment of this condition cannot be overemphasized. Large tumor size and gross hematuria were associated with risk of a poorer outcome.

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The treatment of Wilms' tumour: results of the United Kingdom Children's Cancer Study Group (UKCCSG) second Wilms' tumour study

Cited by 114 publications

References 21 publications

Extracranial malignant rhabdoid tumors in childhood

Extracranial malignant rhabdoid tumors in childhood

High-dose chemotherapy with autologous stem cell rescue in children with nephroblastoma

A Ten Year Retrospective Audit of the Clinic-pathological Management and Treatment Outcomes of Patient’s with Nephroblastoma (Wilms Tumor) at Kenyatta National Hospital

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