Table of Contents
1
UI - 11212886
AU - Granerus M; Johannisson A; Ekblom P; Engstrom W
TI -
Insulin-like growth factors I and II induce cell death in Wilms's tumour
cells.
SO - Mol Pathol 2001 Feb;54(1):30-5
AD - Department of Pathology, Faculty of Veterinary Medicine, Swedish
University of Agricultural Sciences, Uppsala.
AIM: To study the effects of insulin-like growth factors (IGFs) on the
growth phenotype of a Wilms's tumour cell line (WCCS-1). METHODS: WCCS-1
cells were cultured in vitro and exposed to IGF-I and IGF-II, as well as
their antagonists, IGF binding protein 2 and the type I receptor
blocking antibody IGF-IRalpha. The effects on proliferation and cell
cycle parameters were assayed by assessing cell numbers, autoradiography
after labelling with tritiated thymidine, and flow cytometry after
double staining with fluorescein isothiocyanate (FITC) labelled annexin
V and propidium iodide. RESULTS: The addition of IGF-I as well as IGF-II
in physiological doses induced cell death in Wilms's tumour cells. Cell
numbers decreased most dramatically on the fifth to sixth day after
growth factor addition. The occurrence of apoptosis as well as necrosis
was confirmed by annexin-V staining of cell cultures. S-phase indices
were comparable, irrespective of whether the cells were exposed to IGFs
or not, which suggests that WCCS-1 cells undergo cell death at random
during the cell cycle rather that from the prereplicative phase. To
exclude any influences of the IGF binding proteins (IGFBPs), all results
were repeated with Des(1-3)IGF-I, which is unable to bind to any of the
IGFBPs. However, this peptide was equally potent in inducing cell death.
Finally, the addition of IGFBP-2 or the type 1 receptor blocking
antibody IGF-IRalpha partly abrogated the death inducing effects of
IGF-I and IGF-II. CONCLUSIONS: Insulin like growth factors induce cell
death--apoptosis as well as necrosis--in cultured Wilms's tumour cells.
Furthermore, it is proposed that this effect is mediated by the type 1
receptor.
2
UI - 11299842
AU - Granzen B; Efferth T; Keller U; Beniers AJ; Mertens R; Jakse G; Fuzesi L
TI -
Differential expression of the drug resistance markers DNA topoisomerase
II alpha and glutathione S-transferase-pi in the histological
compartments of Wilms' tumors.
SO - Anticancer Res 2001 Jan-Feb;21(1B):771-6
AD - Department of Children and Adolescents, University Hospital Aachen,
Germany.
More than 80% of the patients presenting with Wilms' tumor can be cured
today. Some patients, however, fail to respond to chemotherapy. The
objective of this study was to analyze the immunohistochemical
distribution of two markers of cytostatic drug resistance, e.g. DNA
topoisomerase II alpha (Topo II alpha) and glutathione S-transferase-pi
(GST-pi) in 23 Wilms' tumor patients who had undergone an operation
between 1984 and 1997. Eight patients had stage I disease, seven stage
II, three stage III, four stage IV, and one stage V disease. Five tumors
showed high malignancy histology. Investigations were carried out on
formalin-fixed and paraffin-embedded tissue sections using the indirect
immunoperoxidase method. Topo II alpha was predominantly present in the
epithelial components of the specimens. It was more frequently found in
anaplastic tumors. There was no difference in the presence of Topo II
alpha in the epithelial components between specimens derived from
treated and untreated patients. Topo II alpha was, however, less
expressed in the blastemal and stromal elements of specimens after
preoperative treatment. If GST-pi was present, it was confined to the
epithelial components except for one case. While no expression of GST-pi
was found in preoperatively untreated Wilms' tumors, it was present in
epithelial compartments in 57% of tumors after chemotherapy. In
conclusion, preoperative chemotherapy led to compartment-specific
alterations in the expression levels of both markers indicating a
contribution to treatment response of Wilms' tumors.
3
UI - 11432045
AU - Tejido Sanchez A; de la Morena Gallego JM; Garcia de la Torre JP;
TI -
Villacampa Auba F; Martin Munoz MP; Pamplona Casamayor M; Leiva Galvis O
[Mesoblastic nephroma in the adult: report of a new case]
SO - Arch Esp Urol 2001 Apr;54(3):265-8
AD - Servicio de Urologia, Hospital Hospital Universitario 12 de Octubre,
Madrid, Espana.
OBJECTIVE: A case of adult mesoblastic nephroma is presented. The
clinical features, treatment, histological diagnosis and outcome are
discussed. METHODS/RESULTS: A 68-year-old patient in whom a renal mass
had been detected by ultrasound is described. The patient underwent
radical nephrectomy. The anatomopathological analysis demonstrated a
mesoblastic nephroma. No signs of recurrence have been observed at 3
years' follow-up. CONCLUSIONS: Mesoblastic nephroma of adulthood has a
benign behavior and recurrence is rare after surgery.
4
UI - 11455774
AU - Calvo Rodriguez M; Firvida Perez JL; Vega Vazquez F; Salgado Fernandez
TI -
M; Garcia Mata J; Ruben Rodriguez M; Barreiro Mouro A
[Wilms tumor in the adult]
SO - Arch Esp Urol 2001 May;54(4):370-4
AD - Servicio de Oncologia, Complejo Hospitalario de Ourense, C/Ramon Puga,
56 32005 Ourense, Espana.
OBJECTIVE: To report a case of Wilms' tumor in an adult patient.
METHODS: The records of an adult patient with renal tumor is reviewed.
RESULTS: A 23-year-old male consulted for hematuria. Physical
examination and patient assessment by ultrasound and CT showed a solid
tumor in the right kidney. The patient was submitted to radical surgery.
Pathological analysis demonstrated a biphasic nephroblastoma (Wilms'
tumor) with infiltration of renal hilar fat (stage II). After surgery,
adjuvant chemotherapy with vincristine-actinomycin D was administered
for 60 weeks. CONCLUSIONS: Although rare in adults, Wilms' tumor should
be included in the differential diagnosis of all renal tumors. Treatment
is usually by surgery and chemotherapy with or without radiotherapy,
depending on tumor stage.
5
UI - 11465806
AU - Donckerwolcke RM; Coppes MJ
TI -
Adaptation of renal function after unilateral nephrectomy in children
with renal tumors.
SO - Pediatr Nephrol 2001 Jul;16(7):568-74
AD - Department of Pediatrics, University of Calgary, Alberta, Canada.
rdo@skin.azm.nl
Following treatment, survivors of unilateral Wilms tumor (WT) develop
structural and functional changes in the remnant kidney. A
disproportional increase in functional over structural changes results
in hyperfiltration, a condition that may lead to renal damage. We
studied adaptation of renal function after uninephrectomy in ten WT
patients and a child with renal cell carcinoma. Glomerular filtration
rate (GFR) (measured by inulin and creatinine clearances), renal plasma
flow (RPF) by para-aminohippurate (PAH) clearances and segmental tubular
Na+ transport were studied before and following a protein load (renal
functional reserve). Nine patients showed a well-adapted kidney function
with a GFR of 82.27 (+/- 5.6), an RPF of 429.71 (+/- 65.6) ml/min/1.73
m2 and a filtration fracton (FF) of 20%. Absolute proximal Na+
reabsorption was 65.2 (+/- 9.6) ml/min/1.73 m2, distal tubular delivery
was 18.2 (+/- 3.9) ml/min/1.73 m2 and absolute distal Na+ reabsorption
was 2146 (+/- 435) microM/min. A peculiar finding was the high baseline
creatinine clearances (176.17 ml/min/1.73 m2) related to increased
baseline tubular creatinine secretion. Over 120 min following the
protein load, GFR increased by 20%, RPF by 6% and FF remained unchanged.
Absolute proximal reabsorption increased by 20% and distal reabsorption
by 22%. While most changes in renal function induced by a protein load
are similar in healthy individuals and uninephrectomized patients, a
more predominant contribution to Na+ reabsorption by the proximal tubule
was noted. Postload fractional proximal reabsorption remained at 77%
while in healthy persons a decrease from 77% to 62% was reported. Two
patients showed dysfunctional changes following nephrectomy
characterized by an increased GFR (130 ml/min/1.73 m2), increased
filtration fraction (29%) and inability to increase glomerular and
tubular functions following a protein load (loss of functional reserve).
The significance of these abnormalities is not known and requires
long-term follow-up to evaluate whether hyperfiltration will lead to
renal damage.
6
UI - 11527192
AU - Talpallikar MC; Sawant V; Hirugade S; Borwankar SS; Sanghani H
TI -
Wilms' tumor arising in a horseshoe kidney.
SO - Pediatr Surg Int 2001 Jul;17(5-6):465-6
AD - Department of Pediatric Surgery, Seth G.S. Medical College and K.E.M.
Hospital, Acharya Donde Marg, Parel, Mumbai, Maharashtra, India.
The incidence of horseshoe kidney (HK) is estimated at 1 in 400 cases.
The occurrence of Wilms' tumor (WT) in a HK is an uncommon event,
estimated at 0.4%-0.9% of all WTs. We report a case of WT arising from
the isthmus of a HK and review the literature on the subject.
7
UI - 11511981
AU - Ma Y; Singer DB; Gozman A; Ford D; Chai L; Steinhoff MM; Hansen K;
TI -
Maizel AL
Hsal 1 is related to kidney and gonad development and is expressed in
Wilms tumor.
SO - Pediatr Nephrol 2001 Sep;16(9):701-9
AD - Department of Pathology, Rhode Island Hospital, 593 Eddy Street,
Providence, RI 02903, USA.
Townes-Brocks syndrome (TBS) is a human genetic disorder with features
including urogenital, limb, anal and cardiac malformations associated
with mutations of the TBS gene, Hsal 1. To begin to understand the role
of the Hsal 1 protein (p140) in both normal development and disease
pathogenesis, both message and protein expression were evaluated in
specific tissues associated with TBS. DNA sequence information for Hsal
1 predicts that this homeotic, Drosophila homologue (Sal) encodes a
zinc-finger protein consistent with a transcription factor. mRNA for
Hsal 1 was highly expressed in fetal kidney and brain, with detectable
production in thymus and heart. p140 was found in fetal ureteric bud,
fetal and postnatal renal tubular epithelium, and renal blastema. In the
14-week fetal testis, the Hsal 1 protein was specifically expressed in
the testosterone producing Leydig cells while in adult gonads Hsal 1 was
also found in both Leydig and Sertoli cells, spermatogonia of the
testis, and granulosa cells of the ovary. Evaluation of Wilms tumor
revealed consistently high expression of the gene product in the
epithelial and blastemal components. These spatial and temporal patterns
of expression for Hsal 1, and the phenotypic effects associated with
TBS, suggest that Hsal 1 plays an important role in the development and
functional maintenance of the kidney and gonads. Furthermore, the Hsal 1
gene product may play a part in the pathogenesis of specific neoplasms
occurring in these organs in addition to its specific role in
Townes-Brocks syndrome.
8
UI - 11528553
AU - Eggert A; Grotzer MA; Zhao H; Brodeur GM; Evans AE
TI -
[Expression of the neurotrophin-receptor TrkB predicts outcome in
nephroblastomas: results of a pilot-study]
SO - Klin Padiatr 2001 Jul-Aug;213(4):191-6
AD - Universitatskinderklinik Essen, Abteilung fur Hamatologie/Onkologie,
Germany. angelika.eggert@uni-essen.de
BACKGROUND: The neurotrophin-receptor TrkB plays an important role in
pathogenesis, biology and prognosis of neuroblastoma. Expression of TrkB
on aggressive neuroblastomas leads to proliferation and survival of the
tumor cells and is associated with an unfavorable prognosis. It is now
known that Trk receptors are also expressed in extraneural tissues
including the kidney. PATIENTS AND METHODS: To study the role of the
neurotrophin-receptor TrkB in nephroblastoma/Wilms' Tumor (WT), we
determined TrkB mRNA expression by semiquantitative duplex RT-PCR in 39
primary WT. Comparison of mRNA expression levels with clinical variables
was performed using Cox regression analysis. RESULTS: The 5-year overall
survival was significantly worse for patients with tumors expressing
high levels of a functional TrkB-receptor (TrkBfull) in comparison to
patients with low levels of TrkBfull (70 % versus 100 %, p=0.005).
Conversely, children with tumors expressing high mRNA levels of a
functionally inactive truncated TrkB receptor (TrkBtrunc) had a
significantly higher 5-year overall survival rate in comparison to
patients with low levels of TrkBtrunc (100 % versus 68 %, p=0.003). The
hazard ratios for TrkBfull and TrkBtrunc remained significant after
adjusting for tumor stage. All WT with high levels of TrkB also
expressed the ligand brain-derived neurotrophic factor (BDNF).
CONCLUSIONS: Full-length and truncated TrkB appear to be important
prognostic factors in WT. Their expression should be assessed
prospectively in a larger panel of WT and may have a future role in
patient assignment to risk-based treatment strategies. TrkB signaling
may be reduced in WT with favorable outcome due to low numbers of TrkB
receptors or a competitive effect of functionally inactive TrkBtrunc.
9
UI - 11568898
AU - McNeil DE; Brown M; Ching A; DeBaun MR
TI -
Screening for Wilms tumor and hepatoblastoma in children with
Beckwith-Wiedemann syndromes: a cost-effective model.
SO - Med Pediatr Oncol 2001 Oct;37(4):349-56
AD - Genetic Epidemiology Branch, National Cancer Institute, National
Institutes of Health, 6120 Executive Boulevard, Bethesda, MD 20892-7236,
USA.
BACKGROUND: We undertook a cost-benefit analysis of screening for Wilms
tumor and hepatoblastoma in children with Beckwith-Wiedemann syndrome
(BWS), a known cancer predisposition syndrome. The purpose of this
analysis was twofold: first, to assess whether screening in children
with BWS has the potential to be cost-effective; second, if screening
appears to be cost-effective, to determine which parameters would be
most important to assess if a screening trial were initiated.
PROCEDURES: We used data from the BWS registry at the National Cancer
Institute, the National Wilms Tumor Study (NWTS), and large published
series to model events for two hypothetical cohorts of 1,000 infants
born with BWS. One hypothetical cohort was screened for cancer until a
predetermined age, representing the base case. The other cohort was
unscreened. For our base case, we assumed: (a) sonography examinations
three times yearly (triannually) from birth until 7 years of age; (b)
screening would result in one stage shift downward at diagnosis for
Wilms tumor and hepatoblastoma; (c) 100% sensitivity and 95% specificity
for detecting clinical stage I Wilms tumor and hepatoblastoma; (d) a 3%
discount rate; (e) a false positive result cost of $402. We estimated
mortality rates based on published Wilms tumor and hepatoblastoma stage
specific survival. RESULTS: Using the base case, screening a child with
BWS from birth until 4 years of age results in a cost per life year
saved of $9,642 while continuing until 7 years of age results in a cost
per life-year saved of $14,740. When variables such as cost of screening
examination, discount rate, and effectiveness of screening were varied
based on high and low estimates, the incremental cost per life-year
saved for screening up until age four remained comparable to acceptable
population based cancer screening ranges (< $50,000 per life year
saved). CONCLUSIONS: Under our model's assumptions, abdominal sonography
examinations in children with BWS represent a reasonable strategy for a
cancer screening program. A cancer screening trial is warranted to
determine if, when, and how often children with BWS should be screened
and to determine cost-effectiveness in clinical practice. Published 2001
Wiley-Liss, Inc.
10
UI - 11568899
AU - Rebhandl W; Handisurya A; Memaran N; Felberbauer FX; Aberle J; Paya K;
TI -
Strobl B; Horcher E
Expression of cytokeratin-18-related tissue polypeptide-specific (TPS)
antigen in Wilms tumor.
SO - Med Pediatr Oncol 2001 Oct;37(4):357-64
AD - Department of Paediatric Surgery, University of Vienna Medical School,
Wahringer Gurtel 18-20, A-1097 Vienna, Austria.
winfried.rebhandl@akh-wien.ac.at
BACKGROUND: So far, there is no approved tumour marker for diagnosis or
follow-up in Wilms tumour (WT). Tissue polypeptide-specific antigen
(TPS), a cytokeratin 18 proteolytic fragment, has been suggested to be
of value in the clinical management of WT patients. Cytokeratin 18
fragments are an early indicator of apoptosis and cytokeratin 18 might
influence tumour cell behaviour. We investigated TPS expression in
specimens of WT and other paediatric renal malignancies PROCEDURE:
Immunoreactivity of WT sections (n = 9), clear cell sarcomas (CCSK, n =
3), and a renal cell carcinoma (RCC), and two pediatric kidney tumour
cell lines (WT: SK-NEP-1 and rhabdoid tumour of the kidney: G-401) were
investigated using the monoclonal antibody M3. Additionally,
immunoblotting and RT-PCR analysis were performed. Cell culture
supernatants were evaluated for TPS release. Serum TPS was measured in
five patients at diagnosis, during chemotherapy and after surgical
resection. RESULTS: Moderate to strong immunoreactivity for TPS was
found in tubular and blastemal components of nearly all (8/9) WT
specimens. This was confirmed by Western-blotting. Cystic and
epithelial-like portions of CCSKs and RCC showed distinct reactivity
(3/3). The supernatant of G-401 but not of SK-NEP-1 showed a time- and
cell number-dependent increase of TPS release. Interestingly, TPS
synthesis was demonstrated in SK-NEP-1 cells. Median preoperative serum
TPS was elevated (293 U/l) compared to healthy children and lowest after
surgical resection (49.5 U/l). CONCLUSIONS: This is the first study
demonstrating the synthesis and release of TPS by WTs and other
paediatric renal malignancies. Considering the elevated levels of TPS in
serum of these patients, a further investigation of this marker by
larger clinical trials seems to be justified. Copyright 2001 Wiley-Liss,
Inc.
11
UI - 11584401
AU - Jenkner A; Camassei FD; Boldrini R; de Sio L; Rava L; Bosman C; Boglino
TI -
C; Donfrancesco A
111 renal neoplasms of childhood: a clinicopathologic study.
SO - J Pediatr Surg 2001 Oct;36(10):1522-7
AD - Division of Oncology, Department of Pathology and Division of Surgery,
Ospedale Pediatrico Bambino Gesu IRCCS, Rome, Italy.
PURPOSE: The aim of this study was to perform a clinicopathologic
evaluation of a single pediatric institution renal tumor series. Most
patients were treated within the frame of 3 consecutive SIOP trials,
which included preoperative chemotherapy as their main feature. METHODS:
Medical records and diagnoses of 111 patients were reviewed. The
association of pathologic features with outcome was investigated by
means of the Kaplan-Meier method, the Cox model, and a logistic
multivariate analysis. Comparison among different trial results was
carried out. RESULTS: In 98 patients (88%), nephroblastoma was
diagnosed, followed by 6 adult-type renal tumors, 3 cystic nephromas, 2
mesoblastic nephromas, and 2 clear cell sarcomas. For nephroblastoma, a
statistically significant correlation between grade and both
disease-free survival rate and 5-year survival rate, and between stage
and overall survival rate was shown. Lymph node involvement, local
relapse, nephrogenic rests, and older age at presentation appeared to be
less important prognostic factors. Tumor spillage was very sensitive to
chemo or radiotherapy. No significant difference in outcome was observed
among different trials. CONCLUSIONS: Wilms' tumor was the most frequent
neoplasm and resulted in a 5-year cure rate of 90%. Clinical course was
influenced mainly by diffuse anaplasia and, to a minor extent, by lymph
node involvement. Because some tumors followed an unpredictable course,
it is likely that also other biological factors played a significant
role. Copyright 2001 by W.B. Saunders Company.
12
UI - 11584425
AU - Hero B; Kremens B; Sudermann T; Haas RJ
TI -
Collision tumor in children: a review of the literature and presentation
of a rare case of mesoblastic nephroma and neuroblastoma in an infant.
SO - J Pediatr Surg 2001 Oct;36(10):1607-8
13
UI - 11601255
AU - Zhao G; Boysen CD; Brown EF; Hassanein KM; Holmes FF; Holmes GE
TI -
Very long survival in pediatric cancer between 1944 and 1993.
SO - Chin Med J (Engl) 1999 Jul;112(7):615-9
AD - Henan Institute of Medical Sciences, Henan Medical University, Henan
450052, China.
OBJECTIVE: To identify factors associated with very long survival among
all cancer cases diagnosed at age 19 years or younger registered by the
Cancer Data Service at the University of Kansas Medical Center in Kansas
City, Kansas, U.S.A. in the 40-year period between 1944 and 1983, with
follow-up to 1993. METHODS: There were 2720 pediatric patients with 2750
cancers who were studied. Forty-four types of cancer were grouped into
11 diagnostic categories. Diagnosis years spanned four eras: 1944-1953,
1954-1963, 1964-1973, and 1974-1983. Cases were compared using specific
characteristics and were divided into short-term and long-term survivors
with the division generously set at seven years. The proportions of the
long-term survivors were compared by specific characteristics. RESULTS:
Among the diagnostic categories, leukemias were the most common (29.8%),
followed by CNS tumors (15.2%), and Hodgkin's disease (9.0%). Male to
female ratio was 4:3; average age at diagnosis was 8.83 +/- 6.08 years.
Long-term survivors totaled 1148 (41.7%). Prognosis was better in cases
diagnosed in earlier stages and in later eras. Proportion of long-term
survivors increased from 18.7% in era I to 52.6% in era IV. Improvement
of survival was statistically significant in most diagnostic categories.
CONCLUSIONS: This study shows continuing improvement of survival during
four consecutive eras for childhood and adolescent cancer. Early
diagnosis was associated with better survival. Unstaged cases decreased
over time reflecting progress in diagnostic techniques. Many patients
died before seven years after diagnosis. Those who survived more than
seven years had excellent survival. Pediatricians can expect to
participate in the care of these patients long after the original
dianosis and treatment.
14
UI - 11597793
AU - Blann AD; Li JL; Li C; Kumar S
TI -
Increased serum VEGF in 13 children with Wilms' tumour falls after
surgery but rising levels predict poor prognosis.
SO - Cancer Lett 2001 Nov 28;173(2):183-6
AD - University Department of Surgery,University Hospital of South
Manchester, Nell Lane, Didsbury, M20 8LR, Manchester, UK.
a.blann@bham.ac.uk
Vascular endothelial cell growth factor (VEGF, a potent endothelial cell
mitogen in vitro) may be important in tumour development and its spread
in vivo. In this preliminary study, we tested the hypotheses that (i)
raised serum levels in Wilms' tumour fall after surgery, and (ii) rising
levels predict adverse outcome. Serum VEGF was measured (ELISA) in 13
children about to undergo surgery, and serially on the following day, a
week later, and finally 3-6 months after surgery. A simple follow-up at
6 months was also performed. The control group was 60 healthy adults.
Before surgery, the median (inter-quartile range) VEGF in the children
was 20 ng/ml (10.4-70.5) and was 1 ng/ml (0.5-4.0) in the adults. This
difference is statistically highly significant (P=0.0001). After
surgery, levels in the children fell significantly to 1.3 ng/ml
(0.5-7.95) the following day and to 1.9 ng/ml (0.5-5.0) the following
week (P<0.001, ANOVA). Six months after surgery, three of the children
had died. A level of >10 ng/ml measured 3 months after surgery correctly
identified all three deaths: levels were 1.3 ng/ml (0.5-1.9) in the
survivors and were 20.0 ng/ml (15-104) in those who died. These data
indicate a rapid reduction in raised VEGF following surgery for Wilms'
tumour, but that rising levels predict poor prognosis.
15
UI - 11675960
AU - Tahri A; Benchekroun N; Karkouri M; Dahami Z; Sahraoui S; Acharki A;
TI -
Benider A; Squalli S; Benjelloun S; Kahlain A
[Nephroblastoma in adults. Three case reports]
SO - Ann Urol (Paris) 2001 Sep;35(5):257-61
AD - Centre d'oncologie Ibn Rochd, Casablanca, Maroc.
The authors report three nephroblastoma's cases occurred in adult and
treated at Ibn Rochd Oncology Center at Casablanca. The average of age
was 24 years (19-29 years) and the delay of diagnosis was five months
(3-8 months). The most frequent clinical sign was a lumbar fossa mass.
At diagnosis, radiological exams revealed that patients presented an
advanced stage and metastatic disease in one case. The treatment was a
total nephrectomy with ganglioma dissection when the tumor was
resectable followed by chemotherapy and radiotherapy. Two patients was
in progressive disease despite treatment, and one patient was lost at
follow-up. The prognosis of adult's nephroblastoma remains very severe,
due to the advanced stage at diagnosis and the mediocre reply to the
treatment.
16
UI - 11688464
AU - Muir TE; Cheville JC; Lager DJ
TI -
Metanephric adenoma, nephrogenic rests, and Wilms' tumor: a histologic
and immunophenotypic comparison.
SO - Am J Surg Pathol 2001 Oct;25(10):1290-6
AD - Department of Laboratory Medicine and Pathology, Mayo Foundation,
Rochester, Minnesota 55905, USA.
Metanephric adenoma (MA) is a renal tumor that is generally detected in
adults and occasionally in children. These tumors usually behave in a
benign fashion. Although the histogenesis of MA is unclear, a
morphologic similarity to Wilms' tumor (WT) complex exists. Six cases of
MA, five cases of childhood WT (CWT), two cases of adult WT (AWT), and
four cases of treated MWT and/or nephrogenic rests (MWT/NR), with
paraffin blocks available for use, were retrieved from the surgical
pathology files of the Mayo Clinic. Clinical information was extracted
from the medical record. Immunoperoxidase stains for WT1, AE1, CK7,
CD57, CD56, and desmin were performed on paraffin sections from all
cases. All six cases of MA were strongly and diffusely positive with
antibodies to WT1 and CD57 and focally positive with antibodies to CK7.
Three cases showed focal faint staining in <5% of the cells with keratin
AE1. Stains for CD56 and desmin were negative. All seven cases of WT,
including five CWT and two AWT, were strongly and diffusely positive
with WT1 in the blastema and epithelium but showed only weak focal
positivity in stromal cells. Six cases were diffusely positive for CD56
and one case showed focal positivity. Keratin AE1 was positive in one
case of AWT and focally positive in the other AWT. The blastema of all
cases of WT were negative for desmin, CK7, and CD57, although staining
for keratin AE1, CD56, and CD57 was seen in maturing tubules of CWT
cases. Of the five CWT cases, two had associated NR and two showed
maturing WT after treatment. The areas of NR and maturing WT were
histologically similar to MA and were composed of small tubules with
uniform nuclei with no mitotic activity, scant cytoplasm, and focal
calcifications. All four cases of maturing WT/NR were positive for WT1
and focally positive for CD57, CK7, and AE1. Stains for CD56 and desmin
were negative except in foci of residual blastema, which stained for
CD56 but lacked CD57 and CK7 staining. Five cases each of renal cell
carcinoma, papillary renal cell carcinoma, and oncocytoma were negative
for WT1. Two of five cases of chromophobe carcinoma showed very weak
staining present in <10% of tumor nuclei. Metanephric adenoma is
histogenetically related to WT and is morphologically and
immunophenotypically identical to maturing WT and nephrogenic rests.
17
UI - 11692602
AU - Hosokawa Y; Saiki S; Hanafusa T; Meguro N; Maeda O; Kinouchi T; Kuroda
TI -
M; Usami M; Kotake T
[A case of adult Wilms' tumor]
SO - Hinyokika Kiyo 2001 Sep;47(9):641-3
AD - Department of Urology, Osaka Medical Center for Cancer and
Cardiovascular Diseases.
Wilms' tumor is very rarely found in adults and there are no established
treatment guidelines for such tumors in adults. A 56-year-old woman was
referred to our hospital for further examination of macroscopic
hematuria. Computed tomography scan revealed a large right renal mass
with enlarged lymph nodes. Angiography showed a hypovascular tumor. She
underwent right nephrectomy and resection of lymph node metastasis with
a diagnosis of malignant renal tumor. Histopathological examination
revealed nephroblastoma with lymph node metastasis. The disease was
classified as stage III according to the National Wilms' Tumor Study
classification. The patient received adjuvant chemotherapy consisting of
ifosfamide, cisplatin, and etoposide. This protocol was selected because
of the published poor results with the standard Wilms' tumor
chemotherapeutic agents when used in adults. She remained without tumor
recurrence as of six months after surgery. Development of better
therapeutic approaches to adult Wilms' tumor is awaited.
18
UI - 11715603
AU - Heij HA
TI -
[Childhood neoplasms in the Netherlands (1989-1997)]
SO - Ned Tijdschr Geneeskd 2001 Oct 27;145(43):2104
19
UI - 11712786
AU - Efferth T; Schulten HG; Thelen P; Bode ME; Beniers AJ; Granzen B;
TI -
Ringert RH; Mertens R; Gefeller O; Jakse G; Fuzesi L
Differential expression of the heat shock protein 70 in the histological
compartments of nephroblastomas.
SO - Anticancer Res 2001 Jul-Aug;21(4B):2915-20
AD - University of Gottingen, Germany. efferth@vcrp.de
Nephroblastomas (Wilms' tumors) are curable with survival rates above
80%. Nevertheless, some tumors fail to respond to therapy and those
patients have a poor prognosis. Prognostic factors for nephroblastomas
have still not been satisfactorily explored. In an effort to unravel
molecular markers for non-responding nephroblastomas, we investigated by
means of immunohistochemistty the expression of heat-shock protein 70
(HSP70) in formalin-fixed and paraffin-embedded tissue samples from 32
children afflicted with nephroblastoma. The results were validated using
real-time RT-PCR. HSP70 expression was confined to blastemal and
epithelial components, while the tumor stroma was negative. HSP70
expression was greater, if the tumors had been chemotherapeutically
treated prior to operation, indicating that cytostatic drugs induce
HSP70. Furthermore, high HSP70 expression was confined to tumors from
children who survived, whereas tumors from dead patients were negative
or weakly-positive for HSP70. Though the number of cases analyzed was
small, they provide an indication that HSP70 expression may be of
prognostic value.
20
UI - 11717330
AU - Ravenel JD; Broman KW; Perlman EJ; Niemitz EL; Jayawardena TM; Bell DW;
TI -
Haber DA; Uejima H; Feinberg AP
Loss of imprinting of insulin-like growth factor-II (IGF2) gene in
distinguishing specific biologic subtypes of Wilms tumor.
SO - J Natl Cancer Inst 2001 Nov 21;93(22):1698-703
AD - Institute of Genetic Medicine, The Johns Hopkins University School of
Medicine, Baltimore, MD 21205, USA.
BACKGROUND: Loss of imprinting (LOI) of the insulin-like growth
factor-II (IGF2) gene, an epigenetic alteration associated with
expression of the normally silent maternal allele, was observed first in
Wilms tumor. Although LOI has subsequently been detected in most adult
tumors, the biologic role of LOI in cancer remains obscure. We analyzed
the imprinting status of Wilms tumors with respect to pathologic
subtype, stage, and patient's age at diagnosis and examined the
expression of genes potentially affected by LOI. METHODS: Of 60 Wilms
tumors examined, 25 were informative for an ApaI polymorphism in the
IGF2 gene, allowing analysis of allele-specific gene expression, and
could be classified by pathologic subtype. Gene expression was measured
quantitatively by real-time polymerase chain reaction, and pathologic
analysis was blinded for genetic status. All statistical tests were
two-sided. RESULTS: We observed LOI of IGF2 in nine (90%) of 10 Wilms
tumors classified as having a pathologic subtype associated with a later
stage of renal development and in only one (6.7%) of 15 Wilms tumors
with a pathologic subtype associated with an earlier stage of renal
development (P< .001). LOI was associated with a 2.2-fold increase (95%
confidence interval [CI] = 1.6-fold to 3.1-fold) in IGF2 expression (P<
.001). Children whose Wilms tumors displayed LOI of IGF2 were
statistically significantly older at diagnosis (median = 65 months;
interquartile range [IQR] = 47-83 months) than children whose tumors
displayed normal imprinting (median = 24 months; IQR = 13-35 months; P<
.001). CONCLUSIONS: These data demonstrate a clear relationship between
LOI and altered expression of IGF2 in Wilms tumors and provide a
molecular basis for understanding the divergent pathogenesis of this
cancer. Analysis of LOI could provide a valuable molecular tool for the
classification of Wilms tumor.
21
UI - 11735802
AU - Kawase E; Tanaka K; Honna T; Azuma N
TI -
A case of atypical WAGR syndrome with anterior segment anomaly and
microphthalmos.
SO - Arch Ophthalmol 2001 Dec;119(12):1855-6
AD - Department of Ophthalmology, National Children's Hospital, 3-35-31
Taishido, Setagaya-ku Tokyo, 154-8509 Japan.
22
UI - 11766686
AU - Yanagidate F; Dohi S; Iizawa A
TI -
Anaesthetic management for a patient with WAGR syndrome.
SO - Anaesthesia 2001 Dec;56(12):1215-6
23
UI - 11197266
AU - Ben-Neriah Z; Ackerman Z
TI -
WAGR syndrome in a baby--the result of 6-MP treatment in a father
affected by Crohn's disease?
SO - Am J Gastroenterol 2001 Jan;96(1):251
24
UI - 11745874
AU - Sredni ST; de Camargo B; Lopes LF; Teixeira R; Simpson A
TI -
Immunohistochemical detection of p53 protein expression as a prognostic
indicator in Wilms tumor.
SO - Med Pediatr Oncol 2001 Nov;37(5):455-8
AD - Pathology Department, Hospital do Cancer, Sao Paulo, Brazil.
BACKGROUND: Mutations of the tumor suppressor gene p53 are commonly
found in several kinds of human cancer. In some types of neoplasms,
accumulation of p53 protein has been reported to correlate with more
aggressive clinical behavior. The role of p53 expression in Wilms tumors
(WT) is not clear yet, but most studies have confirmed its correlation
with anaplasia and advanced stage disease. PROCEDURE: Ninety-seven WT
were evaluated for p53 expression by immunohistochemistry in
formalin-fixed paraffin-embedded tissue and correlated with outcome.
Tumors were classified as p53-Negative (p53-N) when no positivity was
observed or only few cells showed weak positivity (0/1+) and
p53-Positive (p53-P) when there was a diffuse and strong nuclear
positivity (2+/3+). RESULTS: p53-P was detected in 13 out of 97 tumors
and was associated with disease relapse (39 vs.17%; P = 0.06) but not
with anaplasia. Among p53-N patients only 5% had metastatic disease
compared with 31% of the p53-P sample. (P = 0.038). Overall survival was
94% for patients with p53-N vs. 85% for patients with p53-P at 1 year (P
= 0.34). CONCLUSIONS: p53 expression in Wilms tumor detected by
immunohistochemistry seems to be associated with advanced disease and
relapse. Copyright 2001 Wiley-Liss, Inc.
25
UI - 11745884
AU - Indolfi P; Di Tullio MT; Casale F; De Rosa E; Polito C; Cioce F
TI -
Kidney size and function after unilateral nephrectomy for Wilms tumor: a
longitudinal study.
SO - Med Pediatr Oncol 2001 Nov;37(5):485-6
26
UI - 11745420
AU - Nakadate H; Yokomori K; Watanabe N; Tsuchiya T; Namiki T; Kobayshi H;
TI -
Suita S; Tsunematsu Y; Horikoshi Y; Hatae Y; Endo M; Komada Y; Eguchi H;
Toyoda Y; Kikuta A; Kobayashi R; Kaneko Y
Mutations/deletions of the WT1 gene, loss of heterozygosity on
chromosome arms 11p and 11q, chromosome ploidy and histology in Wilms'
tumors in Japan.
SO - Int J Cancer 2001 Nov 1;94(3):396-400
AD - Saitama Cancer Center Hospital, Saitama, Japan.
Incidence rates of Wilms' tumor (WT) markedly differ in East Asian and
Caucasian children. In the present study, we examined WT1
deletions/mutations and loss of heterozygosity (LOH) on 11p and 11q in a
large number of WTs and compared our findings with those from 4 series
of Caucasian WTs. Incidence rates of the subtle WT1 mutation in 3 of the
5 series of sporadic and unilateral WTs including ours were 4.3-6.2% and
similar. However, gross homozygous WT1 deletion was more frequent in our
series than in some others. In addition, our series tended to show a
higher incidence of LOH limited to 11p13 and a lower incidence of LOH
including 11p15 than the Caucasian one. These findings indicate some
genetic differences in WT between the 2 regions. One of the 4 Caucasian
series reported a correlation of germinal WT1 mutation with the
predominantly stromal histology. The present study not only confirms the
correlation of germinal WT1 deletion/mutation with predominant stromal
histology but also establishes a correlation with somatic WT1
deletion/mutations with predominant stromal histology. While WTs with
WT1 abnormalities usually showed pseudodiploidy and predominant stromal
histology, those without WT1 abnormalities showed various chromosome
numbers and histologic subtypes. Copyright 2001 Wiley-Liss, Inc.
27
UI - 11745239
AU - Felgenhauer JL; Barce JM; Benson RL; Nan B; Olson JM; Breslow NE
TI -
No excess of early onset cancer in family members of Wilms tumor
patients.
SO - Cancer 2001 Sep 15;92(6):1606-12
AD - Department of Pediatrics, University of Washington, Seattle, Washington,
USA. felgenj@empirehealth.org
BACKGROUND: Wilms tumor is one of the few pediatric cancers with
well-defined familial and genetic components. The authors assessed the
risk of early-onset cancers in first- and second-degree relatives of
patients enrolled by the National Wilms Tumor Study Group. METHODS:
Using a stratified sampling scheme that targeted 530 families of
patients who were believed a priori to have a genetic contribution to
their disease, the authors conducted interviews regarding cancer
occurrence in 4258 family members from 296 families of patients with
Wilms tumor. Reports of malignant neoplasms that occurred before 55
years of age were confirmed by review of medical records wherever
possible. A period of risk was defined for each family member based on
calendar time and his or her relationship to the proband. RESULTS:
Ninety-nine cancers were observed, whereas 126.8 were expected by
applying standard cancer rates for age and calendar period to the
120,885 person-years at risk. The standardized incidence ratio (SIR) was
O-E = 0.78 with 95% confidence interval (CI) of (0.64, 0.95). In
subgroup analyses, the highest relative risks were observed for parents
of the index case (O/E = 21/13.0 = 1.6, 95% CI = 1.0, 2.5) and for
leukemia (O/E = 9/4.9 = 1.9, 95% CI= 0.85,3.5). CONCLUSIONS: The results
of this study may provide reassurance to families of children who have
had Wilms tumor. Potential sources of bias included the low (56%) rate
of participation of targeted families. In general, the biases might have
led to the underreporting of some cancers, especially in more distant
relatives. The possibility of a slight excess of cancer in parents of
Wilms tumor patients could not be excluded. Copyright 2001 American
Cancer Society.
28
UI - 11754052
AU - Rapley EA; Hargrave D; Persinguhe N; Barfoot R; Moore I; Radford M;
TI -
Stratton MR; Rahman N; Pritchard-Jones K
Case of interstitial 12q deletion in association with Wilms tumor.
SO - Am J Med Genet 2001 Dec 1;104(3):246-9
AD - Institute of Cancer Research, Section of Cancer Genetics, Haddow Labs,
15 Cotswold Road, Sutton, Surrey, SM6 8RW, UK. lizr@icr.ac.uk
A 14-month-old boy presenting with Wilms tumor (WT) was found to have a
small de novo deletion of the long arm of chromosome 12
(12q11-12q13.11). Microsatellite analysis of this region from
constitutional DNA showed that the paternal allele was absent between
the markers D12S331 and D12S1713 (inclusive). In the WT there was no
evidence of loss of the maternal chromosome. Constitutional chromosome
abnormalities can often point to the presence of genes that are
important in disease, and the deletion of chromosome 12 in this patient
may indicate a gene involved in WT. To determine whether a WT
predisposition locus exists at 12q we examined the region in two
familial Wilms tumor (FWT) pedigrees unlinked to the known FWT genes on
chromosomes 17q (FWT1), 19q (FWT2), and 11p (WT1). In both families WT
did not segregate with chromosome 12q markers located within the
deletion boundaries. Copyright 2001 Wiley-Liss, Inc.
29
UI - 2172947
AU - Sheng WW; Jacobs D; Soukup S; Gates R
TI -
Comparison of chromosome analysis to DNA content by flow cytometry for
pediatric tumors.
SO - Pediatr Pathol 1990;10(5):671-9
AD - National Yang Ming Medical College, Taipei, Taiwan, Republic of China.
Chromosome analyses in a series of 50 pediatric tumor samples showed
abnormal chromosome number, ranging from hypodiploid to tetraploid.
Aliquots of the same solid tumor samples were analyzed by flow cytometry
(FCM). Material from spontaneous abortions and 12 tumor samples with
normal chromosomes were also compared in a control series of 83 samples.
Tumors or abortuses with 44-48 chromosomes could not be differentiated
from material with normal diploid complement by FCM. However, greater
than or equal to 3-4 extra chromosomes produced detectable differences
in mean DNA index. Triploidy and tetraploidy were readily identified by
FCM. It is concluded that FCM could identify an important group of
hyperdiploid pediatric tumors, as well as 3N and 4N tumor complements.
30
UI - 1657374
AU - Kaneko Y; Homma C; Maseki N; Sakurai M; Hata J
TI -
Correlation of chromosome abnormalities with histological and clinical
features in Wilms' and other childhood renal tumors.
SO - Cancer Res 1991 Nov 1;51(21):5937-42
AD - Department of Laboratory Medicine, Saitama Cancer Center Hospital,
Japan.
Chromosomes and histology were successfully studied in 33 childhood
renal tumors. Thirty-one tumors were classified as one of four subtypes
of Wilms' tumor. Of 24 typical Wilms' tumors, 12 had hyperdiploidy with
nonrandom trisomies, mostly including +6 and/or +12. Three typical
Wilms' tumors with an 11p13 deletion or a pericentric inversion with a
break in 11p13 were not associated with aniridia. Two other typical
Wilms' tumors with the 11p13 deletion and one fetal rhabdomyomatous
nephroblastoma with an 11p13 translocation were associated with
aniridia. Two cystic partially differentiated nephroblastomas showed
hyperdiploidy with +12. Of four clear cell sarcomas of the kidney, three
had normal diploidy and the other had a 2;22 translocation. Two
congenital mesoblastic nephromas had hyperdiploid karyotype with trisomy
11, which was never seen in the 31 Wilms' tumors. Our findings and a
review of data on 102 reported Wilms' tumors revealed 11p13
abnormalities in 24 tumors, 11p15 abnormalities in five tumors, and
partial deletions of 1p, 7p, 11q, 12q, 16q, or 17p or monosomy of No. 21
or No. 22 each in four or more tumors. These findings suggest that
increased copy number of genes on the nonrandom trisomic chromosomes
might contribute to the genesis of many Wilm's tumors and that deletion
of various tumor suppressor genes other than a Wilms' tumor gene, WT1 in
11p13, might also play a critical role in the development of some
tumors.
31
UI - 1681381
AU - Grundy P; Telzerow P; Paterson MC; Haber D; Berman B; Li F; Garber J
TI -
Chromosome 11 uniparental isodisomy predisposing to embryonal neoplasms.
SO - Lancet 1991 Oct 26;338(8774):1079-80
32
UI - 8094715
AU - Velasco S; D'Amico D; Schneider NR; Timmons C; Chappell E; Lee D; Nisen
TI -
PD
Molecular and cellular heterogeneity of Wilms' tumor.
SO - Int J Cancer 1993 Feb 20;53(4):672-9
AD - Department of Pediatrics, University of Texas Southwestern Medical
Center, Dallas 75235-9063.
We developed a Wilms' tumor-cell culture system to investigate the
molecular basis of nephrogenesis and oncogenesis. Several distinct
fractions of cells were isolated and characterized from the same tumor
s
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