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         Congenital Nephroblastoma:     more detail

81. MUMS List Of Disorders - N - O
nephroblastoma (kidney cancer) (1); Nephrocalcinosis (3); Nephrogenic Diabetes NeuropathyCongenital Hypomyelination (5) *; Neuropathy Hereditary Type II (2
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  • Nephroblastoma (kidney cancer) (1)
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82. Results AFIP Wednesday Slide Conference - No. 28 10 May 1995
congenital diverticula contain all layers of the intestinal wall, while Rhabdomyocyticnephroblastoma, bilateral, kidney, swine Histologic examination of 4 æm
http://www.afip.org/vetpath/WSC/WSC94/94wsc28.txt

83. Congenital Mesoblastic Nephroma
TX. congenital MESOBLASTIC NEPHROMA. INDEX. congenital MesoblasticNephroma Epidemiology. Most Common Renal Tumor of Early Infancy 62
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.

84. Nephroblastoma - Part A
REVIEW OF PEDIATRIC RENAL NEOPLASMS John Hicks Texas Children s Hospital, Houston,TX. nephroblastoma (WILMS TUMOR). nephroblastoma (Wilms Tumor) Epidemiology.
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

85. Blackwell Synergy - Cookie Absent
19, Kobayashi Y Nagahara N. A pathological study of nephroblastoma with congenitalaniridia. Acta Pathologica Japonia 1990; 6 41724. 20, Palmer N Evans AE.
http://www.blackwell-synergy.com/links/doi/10.1046/j.1442-2042.1999.00075.x/full
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86. Archives Of The AFIP -- RadioGraphics 1995; 15: 653-669
BM, Mancer K. Magnetic resonance imaging of cystic, partially differentiated nephroblastoma. Congenitalmesoblastic nephroma of infancy report of a case with
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

Clinical Features
Pathologic Features ...
Source Information

References
References

87. NDT -- Rommel And Pirson 16 (3): 634
Teaching Point Medullary sponge kidney—part of a congenital syndrome. Anephroblastoma was diagnosed and total nephrectomy was performed.
http://ndt.oupjournals.org/cgi/content/full/16/3/634
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Nephrol Dial Transplant (2001) 16: 634-636
European Renal Association-European Dialysis and Transplant Association

Teaching Point
Denis Rommel and Yves Pirson Case A 25-year-old woman was referred to our department for recurrent acute pyelonephritis of the left kidney. A first episode had been treated 2 months earlier with amoxycillin for 3 weeks. Urinalysis showed 60 white blood cells per high-power field E. coli . Serum creatinine level was 1 mg/dl. Serum electrolytes were Na 139, K 4.2, HCO 23.5, Cl 105 mmol/l. C-reactive protein was elevated (14.3 mg/dl) as well as neutrophil count (9.600/mm ). The patient was successfully treated with norfloxacin for 3 weeks. Physical examination revealed a right body hemihypertrophy

88. Nephroblastom Review
Since that time the association of nephroblastoma and other different congenitalanomalies, notably aniridia hemihypertrophy and malformations of genitalia
http://www.uniklinik-saarland.de/kinderonkologie/Review/body_review.html
Wilms´tumour - the state of the art
Prof. Dr. N. Graf  
Wilms´tumour or nephroblastoma was first described as a renal neoplasm by Rance in 1814 (1). In 1899 Max Wilms, a surgeon, wrote a detailed monograph "Die Mischgeschwülste der Niere" (2), that gave later this tumour his name. It is a tumour, that was always incurable in former days, but that is today one of the best curable malignancies. The improvement in treatment is based mainly on the progress of surgery, radiotherapy and in the development of effective chemo- therapeutics. Interdisciplinary teamwork and prospective randomised multicenter studies are clues for the achieved increase in prognosis. 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).

89. Paediatric Surgery - Home
Morbidity of surgery and outcome in children with large pretreated nephroblastoma. Congenitaldiaphragmatic hernia the impact of delayed surgery and epidural
http://www.ukzn.ac.za/department/extra.asp?id=6&dept=paedsurgunm

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