UI - 11807873
AU - Gul D; Ogur G; Tunca Y; Ozcan O
Third case of WAGR syndrome with severe obesity and constitutional
deletion of chromosome (11)(p12p14).
SO - Am J Med Genet 2002 Jan 1;107(1):70-1
UI - 11685715
AU - Gow KW; Hoffer F; Lasater O; Shochat SJ
Intraureteral Wilms tumor.
SO - J Pediatr Surg 2001 Nov;36(11):1729-30
AD - Department of Surgery & Radiology, St Jude Children's Research Hospital,
Memphis, TN, USA.
UI - 11793111
AU - Rigolet M; Faussillon M; Baudry D; Junien C; Jeanpierre C
Profiling of differential gene expression in Wilms tumor by cDNA
SO - Pediatr Nephrol 2001 Dec;16(12):1113-21
AD - U383 INSERM, Hopital Necker-Enfants Malades, Universite Rene Descartes,
149 rue de Sevres, 75015 Paris, France. firstname.lastname@example.org
In order to identify genes or pathways involved in Wilms tumor etiology,
we used the Atlas Cancer cDNA expression array to compare the gene
expression profiles of five tumors, one Wilms tumor cell line (SK-NEP1),
and normal mature and fetal kidneys. Of 588 genes tested, 153 had a
different expression pattern in tumors compared with mature kidney.
Ninety-six genes were differentially expressed in tumors compared with
both normal mature and fetal kidney, and 57 genes had expression
profiles similar to that of fetal kidney, which may reflect the
developmental stage of the tumor cells. Comparison of the expression
patterns of tumors shows that only 13% of the differentially expressed
genes are constantly up- or downregulated in the five tumors tested, and
this provides molecular evidence of tumor heterogeneity. We then
confirmed the differential expression by an independent method, using
quantitative reverse transcriptase polymerase chain reaction for two of
the differentially expressed genes, MMP-14 and cyclin D2. Analysis of
expression levels in a panel of 40 tumors showed that 30% overexpressed
MMP-14 and 80% overexpressed cyclin D2. Profiling of gene expression
using cDNA arrays in a large tumor panel will ultimately lead to the
molecular classification of tumors, the identification of prognosis
markers, and the design of targeted therapy.
UI - 11869017
AU - Skotnicka-Klonowicz G; Kobos J; Los E; Trejster E; Szymik-Kantorowicz S;
Prognostic value of proliferating cell nuclear antigen in Wilms' tumour
SO - Eur J Surg Oncol 2002 Feb;28(1):67-71
AD - Clinic of Surgery and Paediatric Oncology, II Clinic of Children's
Diseases, Institute of Paediatrics, Medical University of Lodz, ul.
Sporna 36/50, 91-738 Lodz, Poland.
AIMS: To evaluate the prognostic value of index Proliferating Cell
Nuclear Antigen (PCNA) in Wilms' tumour in children. METHODS: The study
comprised 64 children aged from 2 days to 13 years treated according to
the SIOP (Society International of Oncology Paediatric) and accepted by
the PPGGL (Polish Paediatric Group for the Treatment of Solid Tumours).
The studies were conducted on tumour tissue removed during surgery,
fixed in formalin and embedded in paraffin blocks. Sections (4 microns)
were evaluated by immunohistochemistry, using the peroxidase method to
determine the expression of PCNA in Wilms' tumour cells by primary
monoclonal antibody NCL-PCNA from Novocastra. RESULTS: The percentage of
immunopositive cells in particular fragments of the tumour ranged from
0--93%, mean 30.5%, median 25.5%. Mean and median values enabled
division of children into two groups: Group A, where the percentage of
cells staining with anti-PCNA was <30% and Group B, where this
percentage was >30%. The expression of PCNA was evaluated in various
stages of advancement, various histological types and depending on the
course of disease. The studies revealed the correlation between index
PCNA and stage of advancement P<0.01, index PCNA and histological type
of Wilms' tumour P<0.025. Moreover we observed that deaths were found
more frequently in tumours with index PCNA >30%, P<0.001. CONCLUSIONS:
PCNA is a useful prognostic factor in Wilms' tumour in children.
Copyright Harcourt Publishers Limited.
UI - 11813327
AU - Li P; Perle MA; Scholes JV; Yang GC
Wilms' tumor in adults: aspiration cytology and cytogenetics.
SO - Diagn Cytopathol 2002 Feb;26(2):99-103
AD - Department of Pathology, New York University School of Medicine, New
York, New York, USA.
The fine-needle aspiration cytologic findings of Wilms' tumor occurring
in a 20-yr-old female patient and a 35-yr-old male patient showing
blastemal, spindled sarcomatous and rare epithelial components are
reported. The male patient had the typical presentation of renal mass
with metastasis to lung and pleura, whereas the female patient had an
unusual presentation with the tumor originated from the subcapsular
nephrogenic zone of the kidney, extending into the liver without
invasion into the renal cortex. Cytogenetic analysis of this case
identified: 90, XXXX, +2x3-4, -5, -15, -16, -17, -17, i (17)(q10) x2.
This finding may represent a genetic change associated with Wilms' tumor
of older pediatric and young adult patients. To the best of our
knowledge, this case is the sixth case with cytogenetic study and the
first case revealing isochromosome 17q of an adult Wilms' tumor.
UI - 11880727
AU - Dome JS; Coppes MJ
Recent advances in Wilms tumor genetics.
SO - Curr Opin Pediatr 2002 Feb;14(1):5-11
AD - Department of Hematology and Oncology, St. Jude Children's Research
Hospital, Tennessee 38105-2794, USA. email@example.com
The past decade has witnessed substantial growth in our knowledge of the
genes and loci that are altered in Wilms tumor. Although Wilms tumor was
one of the original paradigms of Knudson's two-hit model of cancer
formation, it has become apparent that several genetic events contribute
to Wilms tumorigenesis. Recent research has identified targets and
regulators of the first Wilms tumor gene, WT1, has uncovered several
candidate genes at the second Wilms tumor locus, WT2, and has identified
two familial Wilms tumor loci, FWT1 and FWT2. The recent discovery of
activating beta-catenin mutations in some Wilms tumors has also
implicated the Wnt signaling pathway in this neoplasm. Recurrent
abnormalities of other loci, including 16q, 1p, and 7p, have indicated
that these sites may harbor Wilms tumor genes. An enhanced understanding
of these and other genetic lesions will provide the foundation for novel
targeted Wilms tumor therapies.
UI - 11826367
AU - Jain D; Hui P; McNamara J; Schwartz D; German J; Reyes-Mugica M
Bloom syndrome in sibs: first reports of hepatocellular carcinoma and
Wilms tumor with documented anaplasia and nephrogenic rests.
SO - Pediatr Dev Pathol 2001 Nov-Dec;4(6):585-9
AD - Department of Pathology, Yale University School of Medicine, 310 Cedar
Street, New Haven, CT 06520-8023, USA.
The triad of small body size, immunodeficiency, and sun-sensitive facial
erythema characterizes the phenotype Bloom syndrome (BS), a rare
autosomal recessive disorder with a striking predisposition to multiple
types of cancers that arise earlier than expected in the general
population. Here we report two sibs with BS. The older, a
15-year-old-girl, developed a hepatocellular carcinoma, a neoplasm not
yet reported in association with BS. Her younger brother developed an
anaplastic Wilms tumor (WT) associated with nephrogenic rests at the age
of 31/2 years, and this was followed by a myelodysplastic syndrome.
Complex cytogenetic abnormalities were identified in all three
neoplasms. These examples expand the spectrum of malignancies occurring
in BS to include liver cell neoplasms, and confirm the association of
nephrogenic rests with WT, even in the setting of BS.
UI - 11888090
AU - Camassei FD; Arancia G; Cianfriglia M; Bosman C; Francalanci P; Rava L;
Jenkner A; Donfrancesco A; Boldrini R
Nephroblastoma: multidrug-resistance P-glycoprotein expression in tumor
cells and intratumoral capillary endothelial cells.
SO - Am J Clin Pathol 2002 Mar;117(3):484-90
AD - Department of Pathology, Bambino Gesu Children's Hospital-Research
Institute, Rome, Italy.
The development of chemoresistance in a variety of cancers seems related
to overexpression of the P-glycoprotein (P-gp) drug pump.
Nephroblastoma, the most common malignant renal tumor of childhood,
usually is responsive to treatment, and prognosis is favorable in most
cases. However, the disease in a subset of patients is refractory to
treatment, and the disease follows an aggressive course. To study P-gp
expression in this tumor and its correlation with outcome, tumor samples
from 93 patients were examined by immunohistochemical analysis. P-gp
expression was determined separately in both tumor cells and
intratumoral capillary endothelium. The likelihood ratio test, the
Kaplan-Meier method, and the log-rank test were used to evaluate its
association with clinical course, grade, stage, and administration of
preoperative chemotherapy. The results for the majority of
nephroblastomas were variably positive; in 43 (46%) of them, newly
formed capillary endothelial cells also stained positive. While no
association of P-gp expression in tumor cells with clinical course,
stage, and grade could be demonstrated, positivity in endothelial cells
correlated significantly with unfavorable outcome, suggesting that
chemoresistance depended on an active blood-tumor barrier. Previous
chemotherapy induced P-gp overexpression in tumor cells.
UI - 11858031
AU - Dombrovskii V
[Wilm's tumor. Diagnostic capacities of magnetic resonance imaging.
SO - Vestn Rentgenol Radiol 2001 Nov-Dec;(6):29-43
AD - Rostov State Medical University, Ministry of Health of the Russian
The accuracy of magnetic resonance imaging (MRI) in the diagnosis of
Wilms' tumor (WT) and in the evaluation of preoperative chemotherapy
(PCH) efficiency was investigated and compared with histopathological
data of 56 children and infants with proven retroperitoneum neoplasma
(WT--49, neuroblastoma--6, congenital mesoblastic nephroma--1). The
author described the WT MRI-semiotics in general and in particular for
its changes during the preoperative chemotherapy. The formula for
calculation of tumor reduction index is suggested. The MRI sensitivity
(100%), specificity (77.8%) and accuracy (91.1%) are detected. The high
positive correlation level between the MRI and pathologic findings,
concerning WT dimensions, pseudocapsule presence and safety, tumor
structure secondary alterations and tumor spreading was found. At the
same time, the specific MRI criteria for the different histological
types of WT were not found. MRI is confirmed to be an accurate tool for
diagnostic monitoring of patients with WT and other retroperitoneum
UI - 11878776
AU - Toretsky J A; Zitomersky N L; Eskenazi A E; Voigt R W; Strauch E D; Sun
C C; Huber R; Meltzer S J; Schlessinger D
Glypican-3 expression in Wilms tumor and hepatoblastoma.
SO - J Pediatr Hematol Oncol 2001 Nov;23(8):496-9
AD - Department of Pediatrics, and Greenebaum Cancer Center, University of
Maryland School of Medicine and Baltimore VA Medical Center, USA.
BACKGROUND: Glypican-3 (GPC3) is a heparan sulfate proteoglycan. When it
is disrupted, it causes the X-linked gigantism-overgrowth
Simpson-Golabi-Behmel syndrome. Its involvement in growth control is
consistent with recent reports that it can bind to growth factors,
possibly including insulin-like growth factor 2. Further, it has been
hypothesized that it may function as a tumor suppressor gene in breast
and ovarian carcinomas and mesotheliomas. PATIENTS AND METHODS: RNA and
protein were extracted from Wilms tumor and hepatoblastoma tissue
samples and GPC3 levels were measured in these extracts by Northern
blotting, reverse transcription polymerase chain reaction, and
immunoblotting. RESULTS: In contrast to published results with
carcinomas, high levels of GPC3 expression were found in Wilms tumor and
hepatoblastoma. Low or undetectable expressions of this gene were found
in normal tissue surrounding the tumor. CONCLUSIONS: Increased
expression of GPC3 in Wilms tumor and hepatoblastoma suggests a
growth-promoting or neutral activity for this gene product rather than a
UI - 11836726
AU - Pumberger W; Pomberger G; Wiesbauer P
Postoperative intussusception: an overlooked complication in pediatric
SO - Med Pediatr Oncol 2002 Mar;38(3):208-10
AD - Division of Pediatric Surgery, University of Vienna, Vienna / Wien,
UI - 11673715
AU - Lambert AW; Nathan M; Jones PL; Huddart SN
A case of fatal tumor embolus following trauma in a patient with
undiagnosed Wilms' tumor.
SO - Pediatr Emerg Care 2001 Oct;17(5):356-7
AD - Department of Paediatric Surgery, University Hospital of Wales.
Involvement of the inferior vena cava with tumor thrombus has been
reported in 5 to 10% of patients with Wilms' tumor. Preoperative imaging
usually alerts the surgeon to the extent of the intravascular extension.
We present a case report of trauma to a previously undiagnosed Wilms'
tumor that resulted in a fatal intraoperative pulmonary tumor embolus.
UI - 11782005
AU - Oner U U; Tokar B; Acikalin MF; Ilhan H; Tel N
Wilms' tumor of the ovary: A case report.
SO - J Pediatr Surg 2002 Jan;37(1):127-9
AD - Osmangazi Universitesi Tip Fakultesi Patoloji Anabilim Dal, 26480,
The occurrence of true extrarenal Wilms' tumor is extremely rare. The
most frequently noted extrarenal sites are the retroperitoneal and
inguinal regions. In the female genital tract, the occurrence of Wilms'
tumor has been documented in the uterus, endocervix, and ovary in
isolated case reports. In this article the authors describe a case of
ovarian Wilms' tumor in a 3.5-year-old girl. Her abdominal ultrasound
scan and computed tomography scan showed a solid mass with cystic
components on the left lower quadrant. Total excision was performed with
left salpingo-oophorectomy. There was no other mass and also no evidence
of metastasis. To the best of the authors' knowledge, this patient is
the first reported case of primary ovarian Wilms' tumor arising in
childhood. Copyright 2002 by W.B. Saunders Company.
UI - 11781987
AU - McNeil DE; Langer JC; Choyke P; DeBaun MR
Feasibility of partial nephrectomy for Wilms' tumor in children with
Beckwith-Wiedemann syndrome who have been screened with abdominal
SO - J Pediatr Surg 2002 Jan;37(1):57-60
AD - Division of Cancer Epidemiology and Genetics, Genetic Epidemiology
Branch, National Institutes of Health, Bethesda, Maryland, USA.
BACKGROUND: Children with Beckwith-Wiedemann syndrome (BWS), a
congenital syndrome associated with Wilms' tumor commonly are screened
with abdominal sonography resulting in detection of tumor at a lower
stage. Wilms' tumors have been traditionally treated with complete
nephrectomy; however, smaller tumors are amenable to nephron-sparing
surgery. Because Wilms' tumors may be metachronous and nonmalignant
disease may compromise renal function in BWS, nephron-sparing approaches
may be desirable as the first option. METHODS: Seven patients with BWS
and Wilms' tumor underwent nephrectomy. The preoperative computed
tomography (CT) or ultrasound scan were evaluated by a pediatric surgeon
to assess whether partial nephrectomy would have been feasible. The
determining criteria included tumor involving one third or less of the
kidney and no involvement of either hilar or vascular structures.
RESULTS: Seven patients underwent complete nephrectomies. The remaining
patient, who had undergone a left nephrectomy before the initiation of
screening had salvage chemotherapy after biopsy results showed right
kidney involvement with Wilms' tumor. CONCLUSIONS: Nephron-sparing
surgery is reasonable to consider in children with Beckwith-Wiedemann
syndrome who are screened at intervals of 4 months or less. The relative
benefits of partial nephrectomy for children with Wilms'
tumor-predisposing conditions only can be assessed in the setting of a
cooperative clinical trial. Copyright 2002 by W.B. Saunders Company.
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