UI - 11715603
AU - Heij HA
[Childhood neoplasms in the Netherlands (1989-1997)]
SO - Ned Tijdschr Geneeskd 2001 Oct 27;145(43):2104
UI - 11733929
AU - Gow KW; Dome JS; Iglesias J; Novick WM; Hoffer FA; Davidoff AM
Intraatrial and intracaval Wilms' tumor.
SO - J Pediatr Surg 2001 Dec;36(12):1869-71
AD - Department of Surgery, St Jude Children's Research Hospital, Memphis,
UI - 9156283
AU - Fleming S
Genetics of renal tumours.
SO - Cancer Metastasis Rev 1997 Jun;16(1-2):127-40
AD - Department of Pathology, University of Edinburgh, UK.
The two main renal tumours, Wilms' tumour and renal cell carcinoma, are
associated with distinct molecular genetic abnormalities. The genes
involved behave as Knudson oncosuppressor genes. Further dissection of
the molecular biology pathways involving WT1 and VHL genes is providing
fascinating insight into the biology of these genes, the development and
cell biology of the kidney and its tumours.
UI - 11957249
AU - Abdallah FK; Macharia WM
Clinical presentation and treatment outcome in children with
nephroblastoma in Kenya.
SO - East Afr Med J 2001 Jul;78(7 Suppl):S43-7
AD - Department of Haematology and Blood Transfusion, College of Health
Sciences, University of Nairobi, P.O. Box 19676, Nairobi, Kenya.
OBJECTIVE: To review the clinical presentation and management of
children with nephroplastoma and the factors influencing the outcome at
Kenyatta National Referral and Teaching Hospital (KNH). DESIGN: This was
a retrospective case series study based on secondary data accumulated
between 1990 and 1996. SETTING: The relevant data were extracted from
records of all children aged 12 years and below, admitted for cancer at
KNH, Nairobi. RESULTS: Out of 803 children with cancer, 71 (8.8%) had
histologically proven nephroblastoma. At presentation, 1.5% were in
stage I, 13.2% stage II, 36.8% stage III, 41.2% stage IV and 7.4% stage
V. Eighty five per cent presented with stage III-V disease. Ninety five
per cent had nepherectomy and received chemotherapy. Radiotherapy was
given to 50.7% of the patients. Nine patients died before commencement
of chemotherapy, two of whom died in the immediate post-operative
period. The median duration between admission and surgery was 41 days.
Pre-operative chemotherapy was given to 42% of the patients.
Approximately 25.5% of the patients received little or no induction
chemotherapy due to unavailability of drugs while only 2.8% received the
prescribed maintenance treatment with the remainder getting erratic or
no treatment. Overall, only 34.7% remained disease free two years from
time of diagnosis. CONCLUSION: Late presentation, poor availability of
cytotoxic drugs and frequent treatment interruptions for various reasons
have contributed to the poor outcome of nephroblastoma in Kenya.
UI - 11920832
AU - Muto R; Yamamori S; Ohashi H; Osawa M
Prediction by FISH analysis of the occurrence of Wilms tumor in aniridia
SO - Am J Med Genet 2002 Apr 1;108(4):285-9
AD - Department of Pediatrics, Tokyo Women's Medical University, Tokyo,
Aniridia is an autosomal dominant eye anomaly caused by
haploinsufficiency of the PAX6 gene, of which abnormalities include base
alterations, position effects and deletions. When deletion involves its
adjacent genes, i.e., those in the PAX6-WT1 critical region (WTCR),
patients are predisposed to Wilms tumor. We studied 18 patients with
aniridia, five of whom had chromosome deletion involving 11p13, two a
translocation t(10;11)(p13;p13) or a der(14;21)(q10;q10)mat, and 11 had
a normal karyotype. Fluorescence in situ hybridization (FISH) using four
P1-derived artificial chromosome (PAC) clones located at WTCR was
carried out in the 18 patients to identify a deletion extent. Of the 18
patients, eight had a deletion of WTCR: four had microscopic deletion
and four a deletion of WTCR. Deleted region in one patient with a
microscopic deletion was distal to the critical region. Four of the
eight patients with a deletion encompassing WTCR developed Wilms tumor,
and the other four did not (two were too young to be evaluated for the
tumor development). The data in the present study, together with four
similar previous works, indicate that of a total of 102 aniridia
patients, 29 had a deletion spanning WTCR. Wilms tumor developed in 13
(45%) of the 29 patients, whereas patients without deletion in this
region did not develop the tumor. In other words, aniridia patients with
WT1 deletion run a high risk of developing Wilms tumor, and those
without the deletion do not. Copyright 2002 Wiley-Liss, Inc.
UI - 11902736
AU - Anderson J; Slater O; McHugh K; Duffy P; Pritchard J
Response without shrinkage in bilateral Wilms tumor: significance of
SO - J Pediatr Hematol Oncol 2002 Jan;24(1):31-4
AD - Department of Hematology and Oncology, Great Ormond Street Hospital for
Children, NHS Trust and Institute of Child Health, London, United
PURPOSE: To test the hypothesis that poor response to chemotherapy in
patients with bilateral Wilms tumor may be associated with the
appearance of rhabdomyomatous histology, suggesting a differentiation
response. METHODS: Twenty-six patients with bilateral Wilms tumor were
treated at the authors' hospital between 1985 and 1995. Radiologic
response to presurgical chemotherapy was assessed, and postsurgery
histology was reviewed. RESULTS: There was a significant association
between rhabdomyomatous differentiation in postchemotherapy surgical
specimens and poor radiologic response. Poor response did not, however,
necessarily mean poor outcome: of 11 patients with rhabdomyomatous
differentiation, 7 are alive and disease-free, 2 died of complications,
and only 2 died of uncontrolled Wilms tumor. CONCLUSIONS:
Rhabdomyomatous differentiation in postchemotherapy bilateral Wilms
tumor is associated with poor radiologic response. This observation may
indicate a differentiation response rather than an absolute failure of
response to chemotherapy. Clinical measures other than tumor volume are
needed to distinguish between tumors that respond to chemotherapy but do
not shrink, and those that genuinely do not respond.
UI - 11920790
AU - Byrne J; Nicholson HS
Excess risk for Mullerian duct anomalies in girls with Wilms tumor.
SO - Med Pediatr Oncol 2002 Apr;38(4):258-9
AD - Department of Hematology/Oncology, Children's National Medical Center,
111 Michigan Avenue NW, Washington, DC 20010-2970, USA. firstname.lastname@example.org
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