UI - 12116074
AU - Marx M; Langer T; Graf N; Hausdorf G; Stohr W; Ludwig R; Beck JD
Multicentre analysis of anthracycline-induced cardiotoxicity in children
following treatment according to the nephroblastoma studies SIOP
No.9/GPOH and SIOP 93-01/GPOH.
SO - Med Pediatr Oncol 2002 Jul;39(1):18-24
AD - Department of Immunology and Oncology, University Hospital for Children
and Adolescents Erlangen-Nuremberg, Germany.
BACKGROUND: To study cardiac function and the incidence of
anthracycline-induced cardiotoxicity in children following treatment
according to the nephroblastoma studies SIOP No.9/GPOH and SIOP
93-01/GPOH. PROCEDURE: Analysis of clinical status, echocardiography,
and ECG findings prior to administration of anthracyclines (median
cumulative doxorubicin dose: 250 mg/m(2) [range: 90-411 mg/m(2)] and
after a median posttherapeutic interval of 2.9 years [range: 0-10.2
years]. Data on cardiac function before and/or after therapy could be
obtained of 186 patients. RESULTS: Posttherapy left ventricular
fractional shortening was reduced in 4/157 (2.5%) patients. Out of the 4
children, 2 had clinically reduced tolerance to exercise and received
anticongestive therapy. Abnormal ECG findings that were not detectable
prior to therapy were found in 7/124 (5.6%) children. CONCLUSIONS: The
incidence of abnormal findings is low in our study group in comparison
to data from the literature and might be due to the comparably short
posttherapeutic interval. Copyright 2002 Wiley-Liss, Inc.
UI - 12116080
AU - Bernbeck B; Krauth KA; Scherer A; Engelbrecht V; Gobel U
Aseptic osteonecrosis in a child with nephroblastoma healed by
hyperbaric oxygen therapy.
SO - Med Pediatr Oncol 2002 Jul;39(1):47-8
AD - Department of Pediatric Hematology and Oncology,
Heinrich-Heine-University, Medical Center, Moorenstr. 5, 40225
Dusseldorf, Germany. email@example.com
UI - 12127397
AU - Stock C; Ambros IM; Lion T; Zoubek A; Amann G; Gadner H; Ambros PF
Genetic changes of two Wilms tumors with anaplasia and a review of the
literature suggesting a marker profile for therapy resistance.
SO - Cancer Genet Cytogenet 2002 Jun;135(2):128-38
AD - Children's Cancer Research Institute (CCRI), St. Anna Children's
Hospital, Kinderspitalgasse 6, A-1090 Vienna, Austria.
Cytogenetic data on Wilms tumors (WT) with anaplasia frequently
associated with an unfavorable outcome are scarce. We present
cytogenetic changes of two WT with anaplasia (primary tumor material)
from nonresponders with a synopsis of the literature. The WT were
investigated by cytogenetic analysis, comparative genomic hybridization,
fluorescence in situ hybridization, immunofluorescence, and flow
cytometric analyses. Both tumors exhibited characteristic genetic
changes. One tumor was hypodiploid due to loss of entire chromosome 11;
losses of 16p, 16q, 17p, chromosome 19 material, and loss of 22q12-qter.
The other tumor was hyperdiploid and triploid, and displayed gain of
1q12-q23 and chromosome 9 material. Moreover, two morphological and
genetically distinct cell lines have been established from both tumors,
demonstrating underrepresentation of chromosomes 13, 14, 16, and 19.
Karyotype descriptions of 120 WT with known clinical data together with
data of this report confirm: (1) inter- and intratumor heterogeneity
exists; (2) loss or underrepresentation of chromosome material at 11,
13, 14, 16, 17p, 19, and 22q in various combinations presents a new
marker profile of resistance to cytotoxic agents regardless of the
histological types; and (3) the prognostic impact of gain at 1q12-q23
sequences warrants further validation.
UI - 12019017
AU - Yu CC; Wong FH; Lo LJ; Chen YR
Hereditary cleft lip/palate and Wilms tumor: a rare association.
SO - Cleft Palate Craniofac J 2002 May;39(3):376-9
AD - Craniofacial Center, Department of Plastic and Reconstructive Surgery,
Chang Gung Memorial Hospital, Taipei, Taiwan.
OBJECTIVE: The association of cleft lip/palate (CLP) with other
anomalies is not uncommon, but its association with Wilms tumor (WT) is
very rare, especially in a familial pattern. In this report, we present
a family in which six members in two generations were affected with CLP,
WT, or both. PATIENTS AND RESULTS: A male patient presented with right
complete CLP. He had a family history of facial cleft and abdominal
tumor. Lip repair was performed at 3 months of age. An abdominal mass
was noticed at 12 months of age, which proved to be WT. Surgical
excision of the tumor and chemotherapy were conducted. He subsequently
underwent palate repair. His father had an unrepaired microform cleft
lip. Three of his aunts were known to have similar problems: one had
both facial cleft and WT, one had WT only, and the other had facial
cleft only. One of his cousins also was affected with WT. CONCLUSIONS:
This is a unique family affected with a rare association of CLP and WT.
Pedigree study revealed an autosomal dominant hereditary pattern.
UI - 12148958
AU - Yang CF; Lin CY
A 3-year old boy with recurrent hematuria.
SO - Acta Paediatr Taiwan 2002 May-Jun;43(3):119-21
AD - Department of Pediatrics, Taipei Veterans General Hospital, Taiwan.
UI - 12131349
AU - Ghanem MA; Van Steenbrugge GJ; Van Der Kwast TH; Sudaryo MK; Noordzij
MA; Nijman RJ
Expression and prognostic value Of CD44 isoforms in nephroblastoma
SO - J Urol 2002 Aug;168(2):681-6
AD - Department of Pediatric Urology, Josephine Nefkens Institute, The
Netherlands Institute for Health Sciences, Erasmus MC, Rotterdam, The
PURPOSE: CD44 is a group of transmembranous glycoproteins formed by
alternative splicing of a single messenger RNA. An abnormal pattern of
CD44 expression has been demonstrated in several human malignancies. We
evaluate the prognostic value of standard CD44 (CD44s) and some of its
isoforms in treating clinical Wilms tumor. MATERIALS AND METHODS: The
immunohistochemical expression of CD44 isoforms was studied in paraffin
material of 61 clinical Wilms tumors. Patients were treated
preoperatively with chemotherapy and mean followup was 5.7 years.
RESULTS: Generally CD44s, CD44v5 and CD44v10 were expressed in normal
kidney tissues and at variable levels in the 3 cell types of Wilms tumor
(blastemal, epithelial and stromal). No CD44v6 expression was found
neither in normal kidney or in tumor tissue. CD44s, CD44v5 and CD44v10
epithelial expression gradually decreased from stage T1 to T3. By
contrast the percentage of CD44 positive cells in the blastemal
component significantly increased from T1 to T3. CD44 immunoreactive
blastema cells were found in 62%, 44% and 41% for CD44s, CD44v5 and
CD44v10, respectively. Blastemal expression of CD44s and CD44v5
statistically significantly correlated with clinicopathological stage.
Univariate and multivariate analyses showed that blastemal CD44v5
expression was indicative of poor prognosis. CONCLUSIONS: Increased
expression of CD44v5 in the blastemal part of Wilms tumor correlated
with tumor stage, clinical progression and tumor related death.
Therefore, blastemal CD44v5 expression may be of value in identifying
patients with a high propensity for distant metastases. These patients
might benefit from adjuvant chemotherapy and/or radiotherapy.
UI - 12142789
AU - Kulkarni R; Wolf JS Jr; Padiyar N; Zuckerman L; Gera R; Scott-Emuakpor
Severe intrarenal fibrosis, infundibular stenosis, renal cysts, and
persistent perilobar nephrogenic rests in a patient with
Beckwith-Wiedemann syndrome 27 years after diffuse nephroblastomatosis
and Wilms tumor: natural progression or a consequence of treatment?
SO - J Pediatr Hematol Oncol 2002 Jun-Jul;24(5):389-93
AD - Michigan State University, East Lansing, Michigan, USA.
A27-year-old woman presented with back and abdominal pain. She was
diagnosed in infancy with Beckwith-Wiedemann syndrome and bilateral
multifocal perilobar nephrogenic rests that progressed to diffuse
nephroblastomatosis with neoplastic nephroblastomatous rests at 14
months of age and subsequently to a right Wilms tumor at 5 years of age.
Computed tomography of the abdomen during the current admission showed
multiple obstructed calices. Ureteroscopic inspection of the left kidney
revealed severe intrarenal scarring with multiple infundibular stenosis,
hydrocalices, and nephrocalcinosis. Renal biopsy showed sclerotic
glomeruli with calcification and scarring and persistent subcapsular
nodular renal blastema. Electrocautery incision and balloon dilatation
provided temporary pain relief. After discharge, the patient has had two
or three episodes of recurrent pain associated with new areas of
infundibular stenoses and renal cysts. Bilateral nephrectomy and renal
transplantation is being considered for management of progressive
disease and relief of intractable pain. The potential causes of
progressive and severe intrarenal fibrosis, infundibular stenosis and
nephrocalcinosis, and renal cysts in this patient may include abnormal
renal development secondary to Beckwith-Wiedemann syndrome itself,
radiation or chemotherapy damage, or a combination.
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