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         Congenital Nephroblastoma:     more detail

41. TheFetus.net - Congenital Mesoblastic Nephroma -Andreas Rempen, MD,Thomas Kirchn
Sonography cannot provide a clear distinction between congenital mesoblasticnephroma and nephroblastoma (Wilms tumor) because both are essentially
http://www.thefetus.net/page.php?id=508

42. TheFetus.net - Neuroblastoma -Angela Regina Capelanes, MD*, Gloria Valero, MD&,
Prenatal diagnosis of congenital Wilms tumor (nephroblastoma) presenting asfetal hydrops. Ultrasound in Obstetrics and Gynecology 16 (1), 8083l.
http://www.thefetus.net/page.php?id=521

43. Nephroblastoma - Part A
nephroblastoma (Wilms Tumor) Clinical Features congenital Mesoblastic Nephroma Clear Cell Sarcoma Malignant Rhabdoid Tumor Renal Cell Carcinoma
http://sup.ultrakohl.com/uscap/abs-1998/nephr-A.htm
Back to Pediatric Renal Home Page
REVIEW OF PEDIATRIC RENAL NEOPLASMS
John Hicks
Texas Children's Hospital, Houston, TX
NEPHROBLASTOMA (WILMS TUMOR)
INDEX Nephroblastoma (Wilms Tumor): Epidemiology Incidence 5 to 6% of All Childhood Cancers n United States Cases per year in United States African-Americans Gender Ratio
(bilateral tumors) Mean Age for Unilateral Tumors
Male
Female Mean Age for Bilateral Tumors
Male
Female 90% Occur in Children
8.1 per million children
Highest Incidence
41 months 47 months 29 months 33 months Nephroblastoma (Wilms Tumor): Clinical Features Bilateral Tumors Abdominal Mass Abdominal Pain Acute Abdomen Hypertension Fever Hematuria Weight Loss Urinary Tract Infection Nausea/Vomiting Urogenital Anomalies (Renal Ectopia, Unilateral Agenesis, Horseshoe Kidney, Ureteral Duplication, Hypospadias, Cryptorchidism) Hemihypertrophy Aniridia Nephroblastoma (Wilms Tumor): Cytogenetics WT1 Tumor Suppressor Gene: A Constitutional Deletion Chromosome 11p13 WAGR Syndrome Nonhereditary Wilms Tumor (33%) 10 Exons Encoding for a 45-49 kd Protein Homology to Early Growth Response 1 (EGR1) Sequence Specific DNA Binding Regulate Transcription of Other Genes 30 to 40% of Wilms with Loss of Heterozygosity for region with WT1

44. Congenital Mesoblastic Nephroma
congenital Mesoblastic Nephroma Light microscopy (A) and Ultrastructure (B) nephroblastoma (Wilms tumor) Clear Cell Sarcoma
http://sup.ultrakohl.com/uscap/abs-1998/mesobl.htm
Back to Pediatric Renal Home Page
REVIEW OF PEDIATRIC RENAL NEOPLASMS
John Hicks
Texas Children's Hospital, Houston, TX
CONGENITAL MESOBLASTIC NEPHROMA
INDEX Congenital Mesoblastic Nephroma: Epidemiology Most Common Renal Tumor of Early Infancy 62% Occur in First 3 months of Life 90% in First Year of Life Mean Age = 2 months Gender Ratio 1.0M:1.0F Asymptomatic Renal Mass Some Detected During Prenatal Ultrasound
Delivery Hyper-reninism: Entrapped Normal Glomeruli With
Overproduction of Renin
Hypercalcemia: Prostaglandin E by Tumor Cells
Congenital Mesoblastic Nephroma: Pathology Arise Unicentrally within Deep Parenchyma Near Renal Sinus Most Unilateral Tumors Infiltration of Renal Parenchyma with Indistinct Tumor-Kidney
Interface - Requires Wide Resection Whorled Appearance Like Leiomyoma Hemorrhage, Necrosis and Cysts Unusual
Possibly Markers for Adverse Outcome Most Behave in Benign Fashion Composed of Spindle Cells (resembles infantile fibromatosis)
Entrapped Renal Tubules and Glomeruli
Cartilage Hematopoietic Cells Lymphocytes at Periphery Tortuous Large Vascular Spaces Congenital Mesoblastic Nephroma: Light microscopy (A) and Ultrastructure (B) Figure 2A: Inerface between normal kidney and spindle cells of a mesoblastic nephroma. Renal tubules frequently become entrapped within this spindle cell tumor.

45. Department Of Paediatric Surgery
congenital anomalies like Cleft Lip and Palate, Cystic Hygroma, Thyroglossal Cystand Hydronephrosis, Ectopia Vesicae and tumours like nephroblastoma
http://www.aimshospital.org/paedsurg/Paedsurg.html

Services
Support Services Staffing Contact Department of Paediatric Surgery The Department of Paeditaric Surgery specialises in the following disciplines:
  • Minimal Access Endoscopic surgeries
  • Paediatric Urology
  • Congenital anomalies like Cleft Lip and Palate, Cystic Hygroma, Thyroglossal Cyst and other problems of head and neck
  • Thoracic Surgical repairs such as Oesophageal Atresia Diaphragmatic Hernia, Eventeration and Lobar Emphysema
  • Abdominal Surgeries like Atresia of Intestine, Imperforate Anus, Hirschsprung's Disease, Biliary Atresia, Choledochal Cysts, tumours of liver and adrenals. Single Stage Transanal pull through, etc.
  • Congenital urinary tract repairs like Hypospadiasis, Posterior Urethral Valves, Vesico-Ureteric reflux, Hydronephrosis, Ectopia Vesicae and tumours like Nephroblastoma
  • Paediatric trauma is also managed efficiently in the department
Top Modalities available to our doctors include:
  • Newborn and paediatric cystoscopes that enable us to do the surgeries without causing trauma to the urethra
  • Newborn and paediatric bronchoscopes are utilized for many diagnostic procedures and also for removal of foreign bodies like peanuts
  • Paediatric Laparoscopes are utilized for minimally invasive abdominal surgeries. Laparoscopic accessories include both 5mm and 3mm sizes with which the surgeries are being performed virtually scarless and painless

46. Advances In Anatomic Pathology - UserLogin
A few undergo malignant transformation and advance to nephroblastoma. Nagahara N. A pathological study of nephroblastoma with congenital aniridia.
http://www.anatomicpathology.com/pt/re/aapath/fulltext.00125480-200109000-00005.
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47. Uhrad.com - Pediatric Imaging Teaching Files
Discussion Wilm s tumor (nephroblastoma) is the most common abdominal malignancy, Associated conditions include congenital genitourinary malformations,
http://www.uhrad.com/pedsarc/peds048.htm

48. Nephroblastoma - Wilms Tumor - Information Page With HONselect
Scientific articles from MEDLINE for nephroblastoma Diseases congenital,Hereditary, and Neonatal Diseases and Abnormalities
http://www.hon.ch/HONselect/RareDiseases/EN/C04.557.435.595.html
InitBulle("navy","#F8F8F8","#000066",1); HONcode sites All Web sites HONselect News ... Images HONselect Search English French German Spanish Portuguese
the word the part of word in MeSH term in MeSH term and description Information on "Nephroblastoma": Medical hierarchy and definition Research Articles Web resources Medical Images Medical News Medical Conferences Clinical Trials Hierarchy English French German Spanish Portuguese
Nephroblastoma Definition: A malignant kidney tumor made up of three cell types: blastemal, stromal, and epithelial, but not all present in every case.
Synonym(s): Wilms Tumor / Wilms' Tumor / Nephroblastomas / Tumor, Wilms /
Narrow term(s): Denys-Drash Syndrome WAGR Syndrome
See also: Genes, Wilms Tumor WT1 Proteins
Browse
New search
Web resources for "Nephroblastoma" English French German Spanish Portuguese = Site with HON description - = Site with a robot description info: enter the site: (click below) domain of the site: HONcode - eMedicine - Wilms Tumor : Article by Arnold C Paulino, MD www.emedicine.com Wilms Tumor - Grundlagen www.med-rz.uni-sb.de HONcode - www.meb.uni-bonn.de

49. Wilms' Tumor And Congenital Hemihypertrophy: Report Of Five Cases And Review Of
Wilms tumor and congenital hemihypertrophy report of five cases and review The possibility that Wilms tumor (nephroblastoma) has prenatal origins is
http://pediatrics.aappublications.org/cgi/content/abstract/40/5/886
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This Article P Rs: Submit a response Alert me when this article is cited Alert me when P ... Alert me if a correction is posted Services E-mail this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal ... Cited by other online articles PubMed PubMed Citation Articles by Fraumeni, J. F. Articles by Manning, M. D. Pediatrics, Nov 1967, 886-899, Vol 40, No. 5
Wilms' tumor and congenital hemihypertrophy: report of five cases and review of literature
JF Fraumeni, CF Geiser and MD Manning
Epidemiology Branch, National Cancer Institute, Bethesda, Maryland 20014. The possibility that Wilms' tumor (nephroblastoma) has prenatal origins is based on the histologic appearance suggesting embryonic maldevelopment, a peak incidence in early childhood, its detection among newborn children and in the fetus, and reported associations with congenital malformations. Among the anomalies found to occur excessively in a multi-hospital survey of children with Wilms' tumor were aniridia, genitourinary defects

50. Large Muscles
Adrenal; nephroblastoma; Hepatoblastoma; Rhabdomyosarcoma Onset congenital;Occurs early in development during branchial arch formation; Clinical
http://www.neuro.wustl.edu/neuromuscular/mother/mlarge.html

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51. Archives Of The AFIP -- RadioGraphics 1995; 15: 653-669
Benign cystic differentiated nephroblastoma in an infant. congenital mesoblasticnephroma of infancy report of a case with unusual clinical behavior.
http://www.rsna.org/REG/publications/rg/afip/privateM/1995/0015/0003/0653/10.htm
RadioGraphics
Multilocular Cystic Renal Tumor in Children
Radiologic-Pathologic Correlation
Abstract
Introduction and Historical Background

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Pathologic Features ...
Source Information

References
References

52. OBGYN.net - Ultrasound Informal Case
there was no tumor (ie. nephroblastoma, or congenital mesoblastic nephroma)or cystic structures in the parenchym, and the pelvis renalis visible but
http://www.obgyn.net/us/us.asp?page=/us/present/0302/has_kidney

53. Wilms Tumor - Grundlagen
Wilms´tumour or nephroblastoma was first described as a renal neoplasm by the association of nephroblastoma and other different congenital anomalies,
http://www.med-rz.uni-sb.de/med_fak/kinderklinik/wilms2.htm
Therapieoptimierungsstudie zur Behandlung von Kindern und Jugendlichen mit einem Nephroblastom
SIOP 93-01 / GPOH
kingra@med-rz.uni-sb.de
Basics: Wilms´tumour - the state of the art
Epidemiology, Incidence
Nephroblastoma, being 6 % of all malignancies in children, is the most common childhood renal tumour. The annual incidence rate of Wilms´tumour is 8 / 1.000.000 children under the age of 15 years, meaning that about 1 out of 100.000 children will suffer of this neoplasm. In Germany more than 100 new cases occur every year (3). The highest incidence rates are reported among United States blacks, in Finland and regions of France, the lowest rates are reported from Asia (4). World-wide the sex ratio is 1:1. The distribution of age at diagnosis peaks at 2 to 3 years in unilateral cases and is lower in children with a bilateral tumour. Bilateralisation will occur in 5 % of nephroblastoma (5).
Aetiology and genetics
In 1964 Miller et al. (6) reported for the first time of an association of Wilms´tumour and aniridia. Since that time the association of nephroblastoma and other different congenital anomalies, notably aniridia hemihypertrophy and malformations of genitalia (cryptorchidism, hypospadias, pseudohermaphroditism and gonadal dysgenesis) is well known. Aniridia and hemihypertrophy are extremely rare in the general population, and children with either of these conditions should be screened carefully for Wilms´tumour. The disease occurs in conjunction with neurofibromatosis, WAGR (Wilms´tumour, aniridia malformations of genitalia, retardation) Beckwith-Wiedemann (BWS), Drash (pseudohermaphroditism, glomerulopathy, and Wilms´tumour) and Perlman familial nephroblastomatosis (bilateral renal hamartomas, macrosomia, islet cell hypertrophy, unusual facial) malformation syndromes (7).

54. INTERNATIONAL PEDIATRIC UROLOGY NEPHROLOGY UPDATE Post-graduate
16.0016.20 congenital anomalies of the urinary bladder Epidemiology,diagnosis of nephroblastoma, vesico-prostatic rhabdomyosarcoma and genital
http://www.epsa.org.eg/Heidelberg.html

HEIDELBERG UNIVERSITY
In Cooperation with
European Pediatric Surgeons Association
(EUPSA)
15-17 September, 2005
Organisation:
Prof. Dr. Karl Ludwig Waag
Prof. Dr. Ahmed Hadidi
Prof.Dr. Jean Michel Guys
Prof. Dr. Burkhardt T.nshoff Prof. Dr. M Hohenfellner Prof. Dr. Alaa El Ghoneimi PD Dr. Stuart Hosie Dr. S Holland- Cunz Date: 15-17 September, 2005 Venue: Der Europ.ische Hof Hotel Europa Tel: (0049) 6221 515-0 69117 Heidelberg, Germany Official Language: English Congress Secretary: Mrs. D Ihrig Tel : 0049 621 383 2709 Fax: 0049 621 383 3823 e-mail: Kinderchirurgie@kch.ma.uni-heidelberg.de www.cvb-heidelberg.de info@cvb-heidelberg.de Tel: +49(0)6221/19433 Registration Fees EUPSA member Non EUPSA member Up to August 30th 170 € 200€ After August 30th Bank Account: Dresdner Bank- Mannheim Konto Nr. 658 786 000 Bankleitzahl : 670 800 50 Sonderkonto Kinderchirurgie 784 061/Pediatric Urology course Registration fees include: - Welcome reception - Course bag including - Coffee breaks for 3 days, lunches for 2 days

55. Dreddyclinic.com - Wilms' Tumor
Also known as nephroblastoma, it s the most common malignant tumor of the kidney He or she will ask if there s a family history of cancer or congenital
http://www.dreddyclinic.com/findinformation/ww/wilmstumor.htm
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Wilms' tumor - nephroblastoma A B C D ... Z CANCER Urinary Tract
  • Prostate cancer Testicular cancer Bladder cancer Kidney cancer ... Wilms' tumor
  • Wilms' tumor is is a cancerous tumor of the kidney that occurs
    in children.

    56. Fetal Turmors - DIAGNOSIS OF CONGENITAL ABNORMALITIES - THE 18-23 WEEKS SCAN
    Examples include congenital neuroblastomas and hepatoblastomas in the first yearof life, while Wilms’ tumor (nephroblastoma) is extremely rare.
    http://www.centrus.com.br/DiplomaFMF/SeriesFMF/18-23-weeks/chapter-11/tumors.htm
    Fetal Tumors Handbook of Fetal Abnormalities Algorithms Appendix
    Fetal tumors
    With the collaboration of Israel Goldstein
    Introduction
    Etiology and mechanisms of carcinogenesis

    Classification

    Intracranial
    ...
    Authors

    Introduction
    Etiology and mechanisms of carcinogenesis
    Developmental errors during embryonic and fetal maturation may result in embryonic tumors. One hypothesis is that more cells are produced than are required for the formation of an organ or tissue and the origins of embryonic tumors rest in developmental errors in these surplus embryonic rudiments. Embryonic tumors developing after infancy are explained by the persistence of cell rests or developmental vestiges. Developmentally anomalous tissue (such as hamartomas and dysgenic gonads) is a source of neoplasms in older children and adults. When any of this developmentally abnormal tissue is present at birth, it is inferred that the cells failed to mature, migrate or differentiate properly during intrauterine life.
    Benignity of fetal and infantile neoplasms
    Some neonatal and infantile tumors have a benign clinical behavior despite histological evidence of malignancy. Examples include congenital neuroblastomas and hepatoblastomas in the first year of life, and congenital and infantile fibromatosis, and sacrococcygeal teratomas in the first few months of life. The factors responsible for this ‘oncogenic period of grace’, which starts in utero and extends through the first few months of extrauterine life, are uncertain.

    57. Conditions & Diseases - By Type
    congenital Kidney Malformations Diabetes Insipidus nephroblastoma Nephrotic Syndrome Potter Syndrome Tuberous sclerosis Urethral meatal stenosis
    http://www.pediatrix.com/body.cfm?id=568

    58. Conditions & Diseases - Alphabetical
    nephroblastoma Neural Tube Defect (NTD, Spina Bifida) Nephrotic Syndrome Retinopathy of Prematurity (ROP) Rubella (German Measles) congenital
    http://www.pediatrix.com/body.cfm?id=569

    59. Nephroblastoma
    nephroblastomas may be associated with a variety of congenital malformations nephroblastoma has a strong tendency to metastasize in lungs, liver,
    http://www.orpha.net/static/GB/nephroblastoma.html
    Orphanet database access
    Nephroblastoma
    Direct access to data Summary
    Update : 04/09/2005
    Orphanet database access

    60. Protocol For The Examination Of Specimens From Patients With Wilms Tumor (Nephro
    This protocol applies to Wilms tumor (nephroblastoma) or other renal tumors There is a growing appreciation that congenital mesoblastic nephroma (CMN),
    http://arpa.allenpress.com/arpaonline/?request=get-document&doi=10.1043/1543-216

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