Cytogenetic abnormalities and clinical outcome in Wilms tumor: A study by the U.K. cancer cytogenetics group and the U.K. Children's Cancer Study Group

2001 ◽  
Vol 38 (1) ◽  
pp. 11-21 ◽  
Author(s):  
Nick Bown ◽  
Simon J. Cotterill ◽  
Paul Roberts ◽  
Mike Griffiths ◽  
Simon Larkins ◽  
...  
Keyword(s):  
Clinical Outcome ◽  
Wilms Tumor ◽  
Study Group ◽  
Cytogenetic Abnormalities ◽  
Cancer Study ◽  
Cancer Study Group

Frequency and Heritability of WT1 Mutations in Nonsyndromic Wilms' Tumor Patients: A UK Children’s Cancer Study Group Study

2004 ◽  
Vol 22 (20) ◽  
pp. 4140-4146 ◽  
Author(s):  
Suzanne E. Little ◽  
Sandra P. Hanks ◽  
Linda King-Underwood ◽  
Chris Jones ◽  
Elizabeth A. Rapley ◽  
...  
Keyword(s):  
De Novo ◽  
Age Of Onset ◽  
Wilms Tumor ◽  
Low Frequency ◽  
Heteroduplex Analysis ◽  
Study Group ◽  
Histologic Subtype ◽  
Cancer Study ◽  
Cancer Study Group ◽  
Wt1 Mutation

Purpose Constitutional WT1 mutations in patients with Wilms' tumor (WT) have specifically been associated with genitourinary abnormalities, such as cryptorchidism and hypospadias. We sought to ascertain the frequency and heritability of constitutional WT1 mutations in nonsyndromic WT patients. Patients and Methods Constitutional DNA from 282 patients treated at seven United Kingdom Children's Cancer Study Group centers was screened for WT1 mutations using heteroduplex analysis. Bidirectional sequencing was used to confirm the mutation and to analyze the corresponding parental DNA samples. Results Five different constitutional WT1 mutations were identified in six children. Mutations in four patients were confirmed to be de novo, and all five mutations are predicted to produce truncated protein. The WT1 mutation group had a young median age at diagnosis of 13.8 months, compared with 34.9 months in the group in whom no WT1 mutations were found; four were female and two were male; and all tumors were of favorable histology. The three tumors with known histologic subtype were stromal-predominant. Contrary to expectation, four of six mutations occurred in children with unilateral tumors without any associated genitourinary abnormality. Conclusion Constitutional WT1 mutations occur with a low frequency (2.1%; 95% CI, 0.8% to 4.6%) in nonsyndromic WT patients. Most mutations occurred in children with unilateral WT without associated genitourinary abnormalities, creating difficulties in identifying individuals with germline mutations on phenotype alone. Two factors that may indicate that an individual is carrying a germline WT1 mutation are an early age of onset and stromal-predominant histology of the WT.

Older Age Is an Adverse Prognostic Factor in Stage I, Favorable Histology Wilms’ Tumor Treated With Vincristine Monochemotherapy: A Study by the United Kingdom Children’s Cancer Study Group, Wilm’s Tumor Working Group

2003 ◽  
Vol 21 (17) ◽  
pp. 3269-3275 ◽  
Author(s):  
K. Pritchard-Jones ◽  
A. Kelsey ◽  
G. Vujanic ◽  
J. Imeson ◽  
C. Hutton ◽  
...  
Keyword(s):  
United Kingdom ◽  
Prognostic Factor ◽  
Wilms Tumor ◽  
Stage I ◽  
Study Group ◽  
Cancer Study ◽  
Adverse Prognostic Factor ◽  
Favorable Histology ◽  
The United Kingdom ◽  
Cancer Study Group

Purpose: To identify clinical prognostic factors in children with stage I, favorable histology (FH) Wilms’ tumor treated with vincristine monochemotherapy after immediate nephrectomy to define subgroups for consideration of further reduction in treatment intensity. Patients and Methods: During two consecutive trials of the United Kingdom Children’s Cancer Study Group (UKW2 and UKW3, 1986 to 2001), 242 children with stage I FH Wilms’ tumor were treated with immediate nephrectomy followed by 10 weekly injections of vincristine 1.5 mg/m2. Event-free survival (EFS) and overall survival (OS) were compared by age group. Results: The 4-year EFS rate was 93.2%, 87.2%, and 71.3% for children less than 2 years old, 2 to 4 years old, and 4 years old or older at diagnosis, respectively (log-rank, P = .001); the corresponding 4-year OS rate was 98.1%, 95.0%, and 87.2% (log-rank, P = .01). There were no toxicity- or procedure-related deaths. In multivariate analysis, specimen weight was not of independent prognostic value (P = .66). Among the 186 children younger than 4 years at diagnosis, there were 17 relapses and five deaths, compared with 16 relapses and eight deaths among the 56 children at least 4 years old at diagnosis. OS after relapse was surprisingly poor (61.6% at 4 years). Conclusion: Treatment for stage I FH Wilms’ tumor is generally successful using vincristine monotherapy after immediate nephrectomy, and therefore, the risks of dactinomycin hepatopathy can be avoided. However, age at least 4 years is a significant adverse prognostic factor. This treatment schedule should be considered in any trial of treatment reduction in very young children with stage I FH Wilms’ tumor, regardless of tumor size, and we suggest that the upper age limit for the reduced therapy be set at 4 years.

Wilms' tumor with intracaval thrombus in the UK Children's Cancer Study Group UKW3 trial

2006 ◽  
Vol 41 (2) ◽  
pp. 382-387 ◽  
Author(s):  
Anupam Lall ◽  
Kathy Pritchard-Jones ◽  
Jenny Walker ◽  
Caroline Hutton ◽  
Suzanne Stevens ◽  
...  
Keyword(s):  
Wilms Tumor ◽  
Study Group ◽  
Cancer Study ◽  
Cancer Study Group ◽  
The Uk

Hepatopathy-thrombocytopenia syndrome--a complication of dactinomycin therapy for Wilms' tumor: a report from the United Kingdom Childrens Cancer Study Group.

1991 ◽  
Vol 9 (2) ◽  
pp. 268-273 ◽  
Author(s):  
J Raine ◽  
A Bowman ◽  
K Wallendszus ◽  
J Pritchard
Keyword(s):  
United Kingdom ◽  
Combination Chemotherapy ◽  
Wilms Tumor ◽  
Liver Function Tests ◽  
Study Group ◽  
Severe Thrombocytopenia ◽  
Cancer Study ◽  
The United Kingdom ◽  
Cancer Study Group

We have observed hepatopathy, associated with thrombocytopenia, in children receiving chemotherapy for Wilms' tumor. We have studied this hepatopathy-thrombocytopenia syndrome (HTS) in patients enrolled in the United Kingdom Childrens' Cancer Study Group (UKCCSG) Wilms' tumor trials (UKW1 and UKW2). At the time of this study, 501 patients had completed therapy. Treatment flow sheets were examined for evidence of hepatopathy (hepatomegaly with abnormal liver function tests) and severe thrombocytopenia (platelet count less than 25 x 10(9)/L). No child who developed the syndrome had received irradiation. HTS was seen in five of 355 (1.4%) of patients treated with combination chemotherapy but in none of the 146 patients treated with vincristine alone. In each instance, the onset was less than 10 weeks after diagnosis. In two children, hepatopathy was severe with jaundice, ascites, transaminases greater than 1,000 IU/L, and prolongation of prothrombin time. On average, HTS lasted 12 days, and resolved with supportive treatment. After recovery, the children tolerated chemotherapy, mostly at reduced dosage, without recurrence. There was no evident long-term morbidity. Dactinomycin is the probable cause of this syndrome. We conclude that the HTS is a rare but important complication of dactinomycin-containing combination chemotherapy for Wilms' tumor. Children developing "isolated" thrombocytopenia following dactinomycin are "at risk" of developing the full-blown syndrome and should have their treatment modified accordingly.

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