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NCI CANCERLIT® Search: Wilms' Tumor - September 2002
National Cancer Institute®
Ultima Vez Modificado: 1 de septiembre del 2002
- The occurrence of Wilms tumor in horseshoe kidneys: a report from the National Wilms Tumor Study Group (NWTSG).
- A surgical approach on an emergency basis for removal of Wilms' tumor with intracardiac neoplastic extension.
- Genomic imprinting and carcinogenesis.
- Wilms' tumor: a paradigm for the new genetics.
- Nephroblastoma (Wilms' tumour) in an adult.
- Changes in WT1 splicing are associated with a specific gene expression profile in Wilms' tumour.
- Bilateral nephroblastoma in familial Hay-Wells syndrome associated with familial reticulate pigmentation of the skin.
- [Nephroblastoma in adults: apropos of 2 cases]
- [Fetal rhabomyomatous nephroblastoma. Report of 2 cases and review of the literature]
- An 11-yr-old male with pneumonia and persistent airway obstruction.
- A developmental study of novH gene expression in human central nervous system.
- Chromosome 1q expression profiling and relapse in Wilms' tumour.
Table of Contents
1
UI - 12149688
AU - Neville H; Ritchey ML; Shamberger RC; Haase G; Perlman S; Yoshioka T
TI -
The occurrence of Wilms tumor in horseshoe kidneys: a report from the
National Wilms Tumor Study Group (NWTSG).
SO - J Pediatr Surg 2002 Aug;37(8):1134-7
AD - Department of Surgery, University of Texas, Houston Medical School,
Houston, TX, USA.
BACKGROUND/PURPOSE: An increased incidence of Wilms tumor has been noted
in patients with a horseshoe kidney. These represent a difficult
diagnostic and therapeutic challenge. The charts of all National Wilms
Tumor Study Group (NWTSG) patients with Wilms tumor occurring in a
horseshoe kidney were reviewed. METHODS: From 1969 to 1998, 8,617
patients were enrolled in the NWTSG. Forty-one patients were found to
have a Wilms tumor arising in a horseshoe kidney for an incidence of
0.48%. Their records were reviewed retrospectively. RESULTS: Horseshoe
kidney was not recognized preoperatively in 13 patients, 10 of whom were
evaluated with computed tomography (CT). Four of the 10 also had renal
ultrasonography and one an intravenous pyelogram (IVP). Two of the 13
were evaluated with an IVP only, and the last had no preoperative
imaging studies performed. Stage at presentation was stage I, 10 pts;
stage II, 10; stage III, 12; stage IV, 6; stage V, 3. Primary surgical
resection was performed in 26 patients, including 23 nephrectomies and 3
partial nephrectomies. Fifteen children were treated with preoperative
chemotherapy after initial biopsy of the tumor. The mean total remaining
renal parenchyma after all operations (excluding treatment of relapses)
was approximately 75%. Surgical complications occurred in 14.6% of
patients, including 2 urine leaks, 2 ureteral obstructions, and 1
ureteral injury. Two patients had transient renal failure. CONCLUSIONS:
The diagnosis of horseshoe kidney often was missed on preoperative
imaging. Accurate preoperative diagnosis is important in planning the
operative approach and may help to decrease complications related to
transection of the urinary collecting system. Although 37% of patients
with Wilms tumor arising in a horseshoe kidney were judged inoperable at
initial exploration, all were amenable to resection after chemotherapy.
Copyright 2002, Elsevier Science (USA). All rights reserved.
2
UI - 11876558
AU - Yilmaz M; Demircin M; Dogan R; Guvener M; Ozisik K; Celiker A; Pasaoglu
TI -
I
A surgical approach on an emergency basis for removal of Wilms' tumor
with intracardiac neoplastic extension.
SO - J Exp Clin Cancer Res 2001 Dec;20(4):609-10
AD - Dept. of Thoracic and Cardiovascular Surgery, Hacettepe University
Faculty of Medicine, Ankara, Turkey. myilmazmd88@hotmail.com
Extension of Wilms' tumor through inferior vena cava into the heart two
years after the completion of therapy, is a very rare clinical aspect.
We successfully operated on a 7 year old girl who had a huge right
atrial mass which filled the right ventricle and right ventricular out
flow tract, on an emergency basis using cardiopulmonary bypass.
3
UI - 2893140
AU - Wilkins RJ
TI -
Genomic imprinting and carcinogenesis.
SO - Lancet 1988 Feb 13;1(8581):329-31
AD - Department of Biochemistry, University of Otago Medical School, Dunedin,
New Zealand.
Genomic imprinting might play an important part in the development of
several tumours. It is suggested that in Wilms' tumorigenesis,
imprinting normally renders inactive a transforming gene on the
maternally derived chromosome 11, leaving intact the paternally
inherited chromosome 11 and the Wilms' transforming gene that it
carries. A similar mechanism might account for the inheritance patterns
of other tumours.
4
UI - 11939407
AU - Malik K; Yan P; Huang TH; Brown KW
TI -
Wilms' tumor: a paradigm for the new genetics.
SO - Oncol Res 2000;12(11-12):441-9
AD - University of Bristol, Department of Pathology and Microbiology, School
of Medical Sciences, University Walk, UK. k.t.a.malik@bris.ac.uk
Carcinogenesis can be triggered by a diverse range of molecular lesions,
a variety of which can be illustrated by Wilm's tumor (WT), a pediatric
kidney cancer. Molecular defects observed in WTs include several
independent targets and mechanisms best exemplified by changes on the
short arm of chromosome 11. This article will review the molecular
pathology of WT and emphasize the broader ramifications for cancer
genetics. Consideration will be given to carcinogenic pathways, novel
cellular molecules, and technologies that will assist in the rapid
interpretation and assimilation of DNA sequence data arising from the
sequencing of the human genome.
5
UI - 12173886
AU - O'Malley P; Corcoran M
TI -
Nephroblastoma (Wilms' tumour) in an adult.
SO - Ir J Med Sci 2002 Apr-Jun;171(2):118
6
UI - 12165855
AU - Baudry D; Faussillon M; Cabanis MO; Rigolet M; Zucker JM; Patte C;
TI -
Sarnacki S; Boccon-Gibod L; Junien C; Jeanpierre C
Changes in WT1 splicing are associated with a specific gene expression
profile in Wilms' tumour.
SO - Oncogene 2002 Aug 15;21(36):5566-73
AD - INSERM U383, Hopital Necker-Enfants Malades, Universite Rene Descartes,
75743 Paris Cedex 15, France.
Wilms' tumour (WT) or nephroblastoma is the most frequent kidney cancer
in children. In a previous study, we reported alterations to WT1
transcription in 90% of WT tested, with decreased exon 5 +/- isoform
ratio being the most frequent alteration (56% of WT). We now report an
approach based on cDNA profiling of tumour pools to identify genes
likely to be dysregulated in association with a decreased WT1 exon 5 +/-
ratio. We compared the expression profiles of pools of tumours
classified according to whether this isoform imbalance was present (five
tumours) or not (four tumours), using Atlas Cancer cDNA expression
arrays. Fourteen of 588 genes tested displayed specific up-regulation
(CCND2, PCNA, N-MYC, E2F3, TOP2A, PAK1, DCC and PCDH2) or
down-regulation (VEGF, IGFBP5, TIMP3, ARHB, C-FOS and CD9) in the pool
of tumours with decreased exon 5 +/- ratio. These results were validated
by RT-PCR analysis of four genes (CCND2, PCNA, VEGF and IGFBP5). We
extended the analysis of VEGF expression to 51 tumours by real-time
RT-PCR and ascertained differential expression of this gene associated
with WT1 expression pattern. Moreover, our results suggest that the VEGF
expression level may be of prognosis relevance for relapsed patients.
7
UI - 12116255
AU - Drut R; Pollono D; Drut RM
TI -
Bilateral nephroblastoma in familial Hay-Wells syndrome associated with
familial reticulate pigmentation of the skin.
SO - Am J Med Genet 2002 Jun 15;110(2):164-9
AD - Servicio de Patologia, Hospital de Ninos Superiora Sor Maria Ludovica,
La Plata, Argentina. patologi@netverk.com.ar
We report on a girl with maxillary hypoplasia, prominent ears, dry
sparse hair, palmar and plantar keratoderma, dystrophic nails, patchy
pigmented skin lesions in hands and feet and bilateral Wilms tumor. She
was born with bilateral ankyloblepharon. The mother and maternal
grandmother presented similar ectodermal defects. Skin biopsies of the
patient and her mother proved to contain cells overexpressing p63 by
immunohistochemistry. Karyotypes of the patient and her mother, and FISH
studies on lymphocytes and tumor cells of the girl demonstrated a mosaic
11p15.5 deletion. These findings suggest a relationship between familial
ankyloblepharon, ectodermal defects and cleft lip and palate (AEC)
syndrome (Hay-Wells syndrome) and familial reticulate pigmentation of
the skin. In addition the development of Wilms tumor and 11p15.5 region
involvement expand the genetic relationship between these conditions and
the enlarging group of genetic entities related to nephroblastoma.
Copyright 2002 Wiley-Liss, Inc.
8
UI - 12162187
AU - Ghadouane M; Alami M; Kasmaoui EH; Amhajji R; Ameur A; Abbar M
TI -
[Nephroblastoma in adults: apropos of 2 cases]
SO - Ann Urol (Paris) 2002 Jul;36(4):240-4
AD - Service d'urologie, hopital militaire d'instruction Mohammed V, Rabat,
Maroc. ghado1@caramail.com
Adult nephroblastoma is a rare tumor. The authors report two new cases
observed in a 30 and 34 years old men in whom the diagnosis was made by
histo-pathology. They were admitted for enormous kidney mass. We
performed nephrectomy in one case, and surgical biopsy in the other. No
adjuvant treatment was given. The prognosis was fatal. They study the
particular features of this tumour. Its prognosis appeared to be poorer
than that of renal adenocarcinoma.
9
UI - 12162188
AU - Mekki M; Belghith M; Krichene I; Zakhama A; Landolsi A; Chelly S; Nouri
TI -
A
[Fetal rhabomyomatous nephroblastoma. Report of 2 cases and review of
the literature]
SO - Ann Urol (Paris) 2002 Jul;36(4):245-9
AD - Service de chirurgie pediatrique, hopital Fattouma Bourguiba, 5000
Monastir, Tunisie. Mongi.Mekki@fmm.rnu.tn
OBJECTIVE: Fetal rhabdomyomatous nephroblastoma is a particular and very
rare histologic variety of nephroblastoma. The aim of this work is to
study the principal clinic, therapeutic and evolutive characteristics of
the fetal rhabdomyomatous nephroblastoma through two personal cases and
a review of the literature. PATIENTS AND METHODS: This is a
retrospective study of two observations of fetal rhabdomyomatous
nephroblastoma treated in the pediatric surgery departement of Monastir
(Tunisia) among 47 cases of nephroblastoma. The diagnosis was confirmed
in the two cases by the histologic examination. RESULTS: The two
patients were a six and a sixteen months old boy and girl. They were
admitted for a voluminous mass occupying the left half-abdomen. The
radiologic and biologic explorations load, in the two cases, to the
diagnosis of left nephroblastoma. After a first chemotherapy that did
not induce a reduction of the tumoral volume, a widened left nephrectomy
was performed for the two patients. The histologic examination of the
two pieces of nephrectomy concluded to a fetal rhabdomyomatous
nephroblastoma with existence in the second case of an extension of the
lesions to the renal pelvis and ureter in the form of a pseudo-botryoide
tumor. The tumor was classified stage I in the first case and stage II
N0 in the second. The treatment was completed by an adapted post
operative chemotherapy according to the SIOP 9 protocol. The two
patients are currently in complete remission with an overview of six
years and half. CONCLUSION: The fetal rhabdomyomatous nephroblastoma is
a special histologic form of nephroblastoma that is characterized by the
paucity of pulmonary metastasis, the absence of response to chemotherapy
and the possibility of tumoral extension in the renal pelvis and ureter.
His prognosis is similar to the classical nephroblastoma.
10
UI - 11936541
AU - Niggemann B; Gerstner B; Guschmann M; Paul K; Wit J; Mau H; Wahn U
TI -
An 11-yr-old male with pneumonia and persistent airway obstruction.
SO - Eur Respir J 2002 Mar;19(3):582-4
AD - Dept of Paediatric Pneumology and Immunology, University Hospital
Charite, Humboldt University, Berlin, Germany. bodo.niggemann@charite.de
11
UI - 9879467
AU - Su BY; Cai WQ; Zhang CG; Su HC; Perbal B
TI -
A developmental study of novH gene expression in human central nervous
system.
SO - C R Acad Sci III 1998 Nov;321(11):883-92
AD - Department of Histology and Embryology, Third Military University,
Chongqing, P.R. China.
The expression pattern of the human nephroblastoma overexpressed (novH)
gene in the fetal human central nervous system was examined by in situ
hybridization using digoxigenin-labeled novH-specific riboprobes. In the
spinal cord, the nov-expressing neurons were first detected both in the
ventral region at 16 weeks of gestation (G16W) and in the dorsal region
at G38W. In the medulla, nov-expressing neurons were detected in the
principal nucleus of the inferior olive, the hypoglossal nucleus and the
dorsal motor nucleus of vagus at G16W. Nov-positive neurons were
detected at G28W in the nucleus of the spinal tract of the trigeminal
and cuneate nucleus, and at G38W in the abducens nucleus of pons, the
red nucleus and the substantia nigra of the midbrain, the ventral
posterolateral and the mediodorsal thalamic nucleus. A strong labeling
was also detected in the striatum of the cerebrum and the cerebral
cortex of the parietal lobe. These data established that novH is mainly
expressed in somato-motor neurons in the lower central nervous system at
early developmental stages and in the higher central nervous system at
later stages, suggesting that nov may play an important role in neuronal
differentiation.
12
UI - 12241781
AU - Lu YJ; Hing S; Williams R; Pinkerton R; Shipley J; Pritchard-Jones K; UK
TI -
Children's Cancer Study Goup Wilms' tumor group
Chromosome 1q expression profiling and relapse in Wilms' tumour.
SO - Lancet 2002 Aug 3;360(9330):385-6
AD - Section of Paediatrics, Institute of Cancer Research and Royal Marsden
Hospital NHS Trust, Sutton, UK.
Gene-expression patterns have proved useful for tumour classification
and prediction of prognosis. We investigated a new gene-expression
profiling method that targets individual chromosomes--comparative
expressed sequence hybridisation (CESH)--to establish if any expression
pattern was associated with relapse, and if this pattern could
accurately predict outcome. We studied 18 cases of Wilms' tumour with
favourable histological findings. Relative overexpression of genes on
the long arm of chromosome 1 was shown in all tumours that subsequently
relapsed but in none of those from patients in remission (odds ratio
357, 95% CI 6.4-19960). This finding shows the value of CESH analysis to
rapidly screen for chromosomal regions that carry genes whose
differential expression is important in establishment of tumour
behaviour.
The above citations and abstracts reflect those newly added to CANCERLIT for the month and topic listed in the title. The citations have been retrieved from CANCERLIT using a predefined search strategy of indexed subject terms. Although the search strategy has been refined as best as possible, citations may appear that are not directly related to the topic, and occasionally relevant references may be omitted.
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