Table of Contents
1
UI - 11748645
AU - Alami J; Williams BR; Yeger H
TI -
Expression and localization of HGF and met in Wilms' tumours.
SO - J Pathol 2002 Jan;196(1):76-84
AD - Department of Paediatric Laboratory Medicine, Division of Pathology, The
Hospital for Sick Children, 555 University Avenue, Toronto, Ontario, M5G
1X8, Canada.
A number of growth factors and cognate receptors that contribute to
normal kidney development have been shown to play roles in the
pathogenesis of Wilms' tumours. Expression of both hepatocyte growth
factor (HGF) and its tyrosine kinase receptor met has been demonstrated
in normal tissues and their neoplastic counterparts, implicating these
factors in normal development and tumour progression. HGF and met
expression has not been studied in Wilms' tumour. Since HGF and met
function in a paracrine fashion by regulating tubulogenesis in normal
kidney development, they could be involved in the pathogenesis of Wilms'
tumour, in which tubular formation is dysplastic. In the present study,
a series of ten homotypic (consisting of blastemal, epithelial, and
stromal elements) and ten heterotypic (consisting of triphasic histology
and a muscle component) Wilms' tumour cases were examined for expression
of HGF and met, using in situ hybridization, immunohistochemistry, and
western blot analysis. Relatively high met message and protein
expression, compared with normal kidney, were evident in homotypic and
heterotypic tumour blastemal, epithelial, and rhabdomyoblastic cells and
a 145 kD met polypeptide was found in all tumours, with a few cases also
expressing the 170 kD precursor form. No apparent alterations of the met
receptor were observed. Similarly, HGF protein was also abundantly
expressed in blastemal, epithelial, and rhabdomyoblastic cells of the
homotypic and heterotypic Wilms' tumours and a 69 kD HGF polypeptide was
demonstrated by western blot analysis. Immunohistochemistry for the
Ki-67 proliferation marker indicated that the pattern of Ki-67
expression correlated with the HGF and met pattern of expression in both
homotypic and heterotypic tumours. These results reveal, for the first
time, significant co-expression of met/HGF in Wilms' tumours, with a
correspondingly high proliferative index in the same cell groups.
Copyright 2001 John Wiley & Sons, Ltd.
2
UI - 11686507
AU - Arda IS; Tuzun M; Demirhan B; Sevmis S; Hicsonmez A
TI -
Lumbosacral extrarenal Wilms' tumour: a case report and literature
review.
SO - Eur J Pediatr 2001 Oct;160(10):617-9
AD - Baskent University, Faculty of Medicine, Department of Paediatric
Surgery, Ankara, Turkey. serdara@baskent-ank.edu.tr
Occurrence of extrarenal Wilms' tumour (WT) is very exceptional and the
diagnosis is almost always made after surgical intervention. The tumour
can be located in the retroperitoneum, uterus, cervix, testes, skin and
even in the thorax. The exact mechanism whereby a WT occurs in
extrarenal tissues is not known. The presence of ectopic metanephric
blastema cells or the WT gene that cause transformation of extrarenal
primitive mesonephric or pronephric remnants into WT are both considered
in the embryogenesis. Although ultrasonography and CT scan are both
helpful in the definition of retroperitoneal tumours, there is no
characteristic finding to diagnose an extrarenal WT before surgery.
However the histological characteristics are the same as in intrarenal
WT, a retroperitoneal teratoma should be clearly investigated for a
possible admixture of WT cells. Patients with extrarenal WT are given
the same treatment protocol as patients with stage III WT. In this
paper, a 5-year-old female patient with an extrarenal WT located in the
lumbosacral region is presented. Conclusion: as a rule, diagnosis of
extrarenal Wilms' tumour is made after surgery. Surgical excision is the
treatment of choice and the same general therapeutic rules should be
followed as when the kidney were affected. Stage III guidelines for
chemotherapy and radiotherapy are appropriate in these patients.
3
UI - 11687734
AU - Skotnicka-Klonowicz G; Kobos J; Los E; Trejster E; Szymik-Kontorowicz S;
TI -
Daszkiewicz P
Prognostic value of p53 expression in Wilms' tumor in children.
SO - Med Sci Monit 2001 Nov-Dec;7(6):1224-9
AD - Clinic of Surgery and Pediatric Oncology, Institute of Pediatrics,
Medical University of Lodz, Poland.
BACKGROUND: The aim of this report was to evaluate the prognostic value
and clinical correlations of p53 expression in children with Wilms'
tumor. MATERIAL AND METHODS: The study comprised 61 children aged from 2
days to 13 years (median 39 months), diagnosed and treated according to
SIOP and PPGGL criteria in three centers co-operating with the PPGGL.
The studies were conducted on tumor tissue removed during surgery, fixed
in formalin and embedded in paraffin blocks. Then 4-micron sections were
evaluated by immunohistochemistry, using the peroxidase method to
determine the expression of p53 in Wilms' tumor cells by means of
primary monoclonal antibody NCL-p53 from Novocastra. RESULTS: The
percentage of immunopositive cells in particular fragments of the tumor
ranged from 0% to 70% (mean 20.4%, median 16.0%). The mean and median
values enabled the children to be divided into two groups: Group A,
where the percentage of cells staining with anti-p53 antibody was >20%
(23 cases), and Group B, where this percentage did not exceed 20%. The
expression of p53 was then evaluated in various stages of advancement
and various histological types, depending on the course of the disease.
In Group A, tumors at higher stages of advancement stages were more
frequent (p<0.05), and showed a higher degree of malignancy (p<0.06;
EFS=56.53%). In Group B, lower stages of advancement were more frequent
(p<0.05), the degree of malignancy was lower, and the EFS was 81.58%. A
discrimination test, however, showed that the determination of p53
expression in Wilms' tumor cells has moderate sensitivity (58.825%),
positive prediction (43.47%), and relatively high specificity (70.45%)
and negative prediction (81.57%), which means that low indexes of p53
expression have higher prognostic value. CONCLUSIONS: The index of p53
expression is not an independent prognostic factor in Wilms' tumor in
children, but this determination may be helpful in identifying high-risk
and low-risk patients.
4
UI - 11720445
AU - Ghanem MA; Van der Kwast TH; Den Hollander JC; Sudaryo MK; Van den
TI -
Heuvel MM; Noordzij MA; Nijman RJ; Soliman EH; van Steenbrugge GJ
The prognostic significance of apoptosis-associated proteins BCL-2, BAX
and BCL-X in clinical nephroblastoma.
SO - Br J Cancer 2001 Nov 16;85(10):1557-63
AD - Department of Paediatric Urology, Josephine Nefkens Institute, The
Netherlands.
Apoptotic cell death represents an important mechanism for the precise
regulation of cell numbers in normal tissues. Various
apoptosis-associated regulatory proteins, such as Bcl-2, Bax and Bcl-X,
may contribute to the rate of apoptosis in neoplasia. The present study
was performed to evaluate the prognostic value of these molecules in a
group of 61 Wilms' tumours of chemotherapeutically pre-treated patients
using an immunohistochemical approach. Generally, Bcl-2, Bax and for
Bcl-X(S/L) were expressed in the blastemal and epithelial components of
Wilms' tumour. Immunoreactive blastema cells were found in 53%, 41% and
38% of tumours for Bcl-2, Bax and for Bcl-X(S/L), respectively. An
increased expression of Bcl-2 was observed in the blastemal component of
increasing pathological stages. In contrast, a gradual decline of Bax
expression was observed in the blastemal component of tumours with
increasing pathological stages. Also blastemal Bcl-X(S/L) expression
decreased with stage. Univariate analysis showed that blastemal Bcl-2
expression and the Bcl-2/Bax ratio were indicative for clinical
progression, whereas epithelial staining was of no prognostic value.
Multivariate analysis showed that blastemal Bcl-2 expression is an
independent prognostic marker for clinical progression besides stage.
These findings demonstrate that alterations of the Bcl-2/Bax balance may
influence the clinical outcome of Wilms' tumour patients by deregulation
of programmed cell death.
5
UI - 11813185
AU - Fong KW; Lee AC; Wong YC; Lee WK; Tsui KY
TI -
Wilms tumor presenting as superior vena cava syndrome.
SO - Med Pediatr Oncol 2002 Feb;38(2):135-6
AD - Department of Paediatrics, Tuen Mun Hospital, New Territories, Hong
Kong, China.
6
UI - 11813169
AU - Beckwith JB
TI -
Revised SIOP working classification of renal tumors of childhood.
SO - Med Pediatr Oncol 2002 Feb;38(2):77-8
7
UI - 11813170
AU - Vujanic GM; Sandstedt B; Harms D; Kelsey A; Leuschner I; de Kraker J;
TI -
SIOP Nephroblastoma Scientific Committee
Revised International Society of Paediatric Oncology (SIOP) working
classification of renal tumors of childhood.
SO - Med Pediatr Oncol 2002 Feb;38(2):79-82
AD - Department of Pathology, University of Wales College of Medicine, Heath
Park, Cardiff, United Kingdom. vujanic@cf.ac.uk
The previous International Society of Paediatric Oncology (SIOP) trials
and studies recognized three prognostic groups of renal tumors of
childhood: low risk, intermediate risk, and high risk tumors, which were
further defined in the SIOP (Stockholm) Working Classification of Renal
Tumors of Childhood (1994). The results of the latest SIOP Trials and
Studies showed that certain histological features which remain after
preoperative chemotherapy, such as blastema, are of prognostic
significance while others are not. Therefore, in the next SIOP Trials
and Study a revised classification of renal tumors will be followed. It
still recognizes the three tumor risk groups with different types in
each of them, but for treatment purposes, only three major types of
nephroblastoma need to be recognized: completely necrotic (low risk
tumor), blastemal (high risk tumor), and others (intermediate risk
tumors). Patients will be treated according to tumor histology and
stage. Trials which include preoperative chemotherapy have shown that
the presence of necrotic tumor or chemotherapy induced changes in the
renal sinus or perirenal fat can be ignored for distinguishing between
stage I and II, but if present at resection margins or lymph nodes, it
should be regarded as stage III. Prognostic significance of all
histological component of Wilms tumors will be studied prospectively in
the new trial. Copyright 2002 Wiley-Liss, Inc.
8
UI - 11556536
AU - Hennigar RA; O'Shea PA; Grattan-Smith JD
TI -
Clinicopathologic features of nephrogenic rests and nephroblastomatosis.
SO - Adv Anat Pathol 2001 Sep;8(5):276-89
AD - Department of Pathology and Laboratory Medicine, Emory University School
of Medicine, Atlanta, Georgia 30322, USA. rhennig@emory.edu
Nephrogenic rests are the consequence of residual metanephric tissue in
a fully developed kidney. They usually occur along the perimeter of a
mature renal lobe (i.e., perilobar), within the lobe itself (i.e.,
intralobar), or both (i.e., combined). Nephrogenic rests can be grossly
obvious or microscopically discrete. Nephroblastomatosis designates
nephrogenic rests that are multifocal or diffuse, and implies more
extensive disease. Universal (panlobar) nephroblastomatosis denotes
complete replacement of the renal lobe by nephrogenic tissue. The fate
of nephrogenic rests and nephroblastomatosis varies and includes
obsolescence, sclerosis, dormancy, hyperplasia, or neoplasia. Evidence
strongly suggests that neoplastic transformation of nephrogenic rests
results in Wilms' tumor (nephroblastoma). Nephrogenic rests almost
always occur in the setting of Wilms' tumor; perilobar rests show a
strong association with synchronous bilateral Wilms' tumors, whereas
intralobar rests are more strongly associated with metachronous tumors.
Genetic studies have shown that nephrogenic rests often share many of
the same chromosomal defects as Wilms' tumor, which provides further
evidence that they are precursors to nephroblastoma. Thus, nephrogenic
rests are recognized as clinically significant entities requiring
adequate detection and close surveillance. Heightened awareness
regarding the clinical relevance of nephrogenic rests and
nephroblastomatosis (1) has led to improved detection of these
precancerous lesions, (2) fostered more intensive investigation into
their biologic behavior, and (3) initiated in-depth discussions about
potentially new treatment regimens. The pathologists' ability to
identify and detect nephrogenic rests has benefited from the more
efficient Beckwith classification. Radiologists have deployed
high-resolution radiologic/imaging modalities to improve detection of
nephrogenic rests in situ. Clinicians and surgeons are more aware of the
impact that nephrogenic rests have upon patient management. Despite this
progress, more data is needed to further define these lesions.
9
UI - 11835232
AU - Bown N; Cotterill SJ; Roberts P; Griffiths M; Larkins S; Hibbert S;
TI -
Middleton H; Kelsey A; Tritton D; Mitchell C
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.
SO - Med Pediatr Oncol 2002 Jan;38(1):11-21
AD - School of Biochemistry and Genetics, University of Newcastle upon Tyne,
United Kingdom. Nick.Brown@ncl.ac.uk
BACKGROUND: Tumor genetic features reported to correlate with adverse
outcome in Wilms tumor include karyotype complexity, losses of material
from the short arm of chromosome 1 and from the long arms of chromosomes
11, 16 and 22 and gain of material from the long arm of chromosome 1.
This study sought to test these associations in a large series of tumors
studied by cytogenetic analysis. Identification of markers associated
with elevated risk of relapse and fatal outcome could allow more
effective treatment stratification at presentation. PROCEDURE: Thirteen
member laboratories of the U.K. Cancer Cytogenetics Group provided
results from a 12-year period. Karyotype abnormalities were correlated
with clinical data (age, tumor stage, and histology) and outcome data
provided by the central register of the U.K. Children's Cancer Study
Group. RESULTS: Of 127 abnormal karyotypes, 78 included a reputedly
"poor prognosis" feature. Univariate survival analysis showed no
significant adverse effect for karyotype complexity, 1p loss or 11q
loss. The poor outcome of cases with 16q loss was of borderline
significance, but this effect was restricted to those tumors with
unbalanced translocation der(16)t(1q;16q). The association between
relapse risk and gain of 1q material was not significant. Only monosomy
22 was a significant marker of poor outcome in univariate analysis (13
cases showing 50% relapse free survival at 5 years compared to 79%
survival for the remaining 114 cases, P = 0.02). In multivariate
analysis, significant independent predictors of poor outcome were 1q
gain (Hazard Ratio 3.4), stage IV disease (HR 5.0), and monosomy 22 (HR
5.9). CONCLUSIONS: Loss of chromosome 22 identifies high risk Wilms
tumors. The prognostic significance of 1q gain, 16q loss and unbalanced
translocation der(16)t(1q;16q) is unresolved and warrants further
investigation. Copyright 2002 Wileyb Liss, Inc.
10
UI - 10811666
AU - Porteus MH; Narkool P; Neuberg D; Guthrie K; Breslow N; Green DM; Diller
TI -
L
Characteristics and outcome of children with Beckwith-Wiedemann syndrome
and Wilms' tumor: a report from the National Wilms Tumor Study Group.
SO - J Clin Oncol 2000 May;18(10):2026-31
AD - Dana-Farber Cancer Institute, Department of Pediatrics and Department of
Biostatistics, Harvard Medical School, and Children's Hospital, Boston,
MA 02115, USA.
PURPOSE: Children with Beckwith-Wiedemann syndrome (BWS) are at
increased risk for developing Wilms' tumor (WT). We reviewed the
National Wilms Tumor Study Group (NWTSG) records to assess clinical
characteristics and outcome of patients with WT and BWS. METHODS: In the
NWTSG, treating clinicians were asked to report, for each enrolled
patient, whether the patient had BWS. Between 1980 and 1995, 4,669
patients were treated on two consecutive NWTSG protocols (NWTS 3 and
NWTS 4). We retrospectively reviewed the clinical characteristics and
treatment outcomes of BWS patients compared with patients with WT
without BWS. RESULTS: Fifty-three children enrolled onto NWTS 3 and 4
were reported to have BWS. BWS patients were more likely to present with
lower-stage tumors (P =.0001), with more than half (27 of 53) presenting
with stage I disease. The overall treatment outcomes for the BWS
patients were nearly identical to those without BWS, with overall
survival at 4 years from diagnosis at 89% and 90%, respectively.
Overall, 21% of the patients with BWS had bilateral disease, either at
diagnosis (nine of 53) or as metachronous contralateral recurrence (two
of 53). BWS patients enrolled onto NWTS 4 had smaller tumors than those
enrolled onto NWTS 3 (P =.02), a trend not seen in the non-BWS patients.
CONCLUSION: Like children without BWS, children with BWS and WT have an
excellent prognosis with modern treatment regimens. There is a high risk
of bilateral disease, and increasingly smaller tumors are being
detected. This suggests that a national trial assessing the role of
ultrasound screening followed by nephron-sparing surgery for some
patients may be appropriate.
11
UI - 11426492
AU - Korones DN; Brown MR; Palis J
TI -
"Liver function tests" are not always tests of liver function.
SO - Am J Hematol 2001 Jan;66(1):46-8
AD - Department of Pediatrics, University of Rochester, School of Medicine
and Dentistry, Children's Hospital at Strong, New York 14642, USA.
david-korones@urmc.rochester.edu
A child with Wilm's tumor and a child with immune thrombocytopenic
purpura (ITP) were each noted to have persistent elevations of aspartate
aminotransferase (AST), alanine aminotransferase (ALT), and lactate
dehydrogenase (LDH). Both children underwent thorough evaluation for
liver disease and, as a result, experienced delays in treatment of the
Wilm's tumor and ITP. Eventually both children were found to have
extremely elevated serum creatine kinase (CK). Muscle biopsy confirmed
diagnoses of Duchenne's muscular dystrophy in one child, and Becker's
muscular dystrophy in the second. Hematologists/oncologists should
consider obtaining a serum CK to rule out muscle disease in patients
with unexplained elevations of AST, ALT, and LDH.
12
UI - 11208855
AU - Grundy R; Pritchard J
TI -
Ascertainment of the incidence of Beckwith-Wiedemann syndrome in the
National Wilms Tumor Study Group.
SO - J Clin Oncol 2001 Jan 15;19(2):593-4
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