Pathologic Quiz Case: A Renal Tumor In A 62-Year-Old Woman no known personal or family history of either tumors or congenital syndromes . Pathologic Diagnosis Biphasic nephroblastoma in an Adult With Focal http://arpa.allenpress.com/arpaonline/?request=get-document&doi=10.1043/0003-998
MUMS List Of Disorders - N - O nephroblastoma (Benign Tumors on Kidneys) (3); nephroblastoma (kidney cancer) (1) Oppenheim s Disease (Benign congenital Hypotonia) (1) http://www.netnet.net/mums/mum_n-o.htm
Extractions: Dr Simon Attard Montalto History of presenting complaint Past history Family history Social history Medication Immunisations Review of systems Newborn respiratory limbs Children as per adults! non threatening patience Nutrition breast milk formulae weaning deficiency states normal growth centiles sexual maturation normal milestones gross motor fine motor special senses psychosocial educational Examination Prematurity, low birth weight Congenital anomalies Respiratory diseases Infections Jaundice Asphyxia, convulsions Surgical problems Infections - upper - lower Asthma Cystic fibrosis Congenital anomalies Examination - murmurs - femorals - BP Acyanotic - VSD - ASD - PDA - Coarctation of the aorta Cyanotic - transposition of the great arteries - Fallot tetralogy Acquired heart disease Heart failure Arrhythmias Abdominal pain Constipation Diseases - Coeliac - malabsorption - Crohns - ulcerative colitis Liver - jaundice - hepatitis Congenital anomalies Infection Seizures Headaches Neuromuscular diseases Congenital anomalies Infection Proteinuria - nephrotic syndrome - nephritis Haematuria - calculi Stature - short - excess Puberty - normal - abnormal Thyroid disease - hypo - hyper Adrenal disease - hypo - hyper IDDM - hypo - DKA DI Inheritance - recessive - dominant - X-linked - multifactorial
CancerLIT: 707455 CASE Following a right radical nephrectomy for adult nephroblastoma and two local Pirson Y TI Medullary sponge kidneypart of a congenital syndrome. http://cancerweb.ncl.ac.uk/cancernet/cancerlit/707455.html
Extractions: cancernet@cancerweb.org.uk 1 UI - 21119949 AU - Baskin LS TI - Case 1. Abdominal mass. A 1-year-old with a left abdominal mass and gross hematuria. SO - Tech Urol 2001 Mar;7(1):41; discussion 76 AD - University of California San Francisco, USA. 2 UI - 21285467 AU - Lin F; Krishnamurthy S TI - Fine needle aspiration cytology of a skeletal metastasis of adult Wilms' tumor. A case report. SO - Acta Cytol 2001 May-Jun;45(3):393-8 AD - Department of Pathology, University of Texas M. D. Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, Texas 77030, USA. AB - BACKGROUND: Nephroblastoma (Wilms' tumor) is the most common malignant tumor of the kidney in children but is rare in adults. The stage and histopathology of the tumor are the most important prognostic indicators. The common sites of metastasis are lung, liver and lymph nodes. Skeletal metastasis is exceedingly rare in both pediatric and adult nephroblastoma. We report an unusual case of a skeletal metastasis of adult nephroblastoma that developed nine years after the diagnosis of a typical nephroblastoma of favorable histology and that was diagnosed by computed tomography (CT)-guided fine needle aspiration cytology. CASE: Following a right radical nephrectomy for adult nephroblastoma and two local recurrences two and three years later, a 74-year-old woman presented with low back pain. CT and magnetic resonance imaging revealed lytic lesions in the 10th and 12th thoracic vertebrae. Smears prepared from specimens obtained through CT-guided fine needle aspiration biopsy were moderately cellular, with small, round cells arranged singly and in loosely cohesive clusters. These cells had inconspicuous nucleoli and scanty to moderate amounts of cytoplasm. The cells were also positive for cytokeratin and vimentin and appeared similar to areas of blastema in the original tumor. CONCLUSION: A definitive diagnosis of metastatic adult nephroblastoma in thoracic vertebrae was made possible by CT-guided fine needle aspiration cytology in conjunction with clinical and radiologic findings and by using ancillary modalities, such as immunohistochemical studies. 3 UI - 21310635 AU - Chen CW; Huang SP; Li YC; Chou YH; Huang CH TI - Adult Wilms' tumor associated with polycythemiaa case report. SO - Kaohsiung J Med Sci 2001 Feb;17(2):107-11 AD - Department of Urology, Kaohsiung Medical University, No. 100, Shih-Chuan 1st Road, Kaohsiung 807, Taiwan. AB - Both Wilms' tumor (nephroblastoma) in adults and polycythemia in Wilms' tumor are rare. Herein we report an extremely rare case of adult Wilms' tumor associated with polycythemia. A 41-year-old female was incidentally found to have right renal mass by abdominal sonography in a routine health examination. Laboratory examination revealed polycythemia (hemoglobin 20.2 g/dL). Although physical examination was unremarkable, CT scan revealed an homogeneous mass at the middle pole of right kidney, and chest x-ray revealed no metastatic lesions. Right radical nephrectomy was performed smoothly. Grossly, the tumor of 5 x 4.5 x 4.5 cm in size was well circumscribed, and had no vascular structure or collecting system involvement. Microscopic features were consistent with adult nephroblastoma. The post-operative course was uneventful. At follow-up, the patient was well, showed no evidence of recurrence and her hemoglobin level had returned to normal (hemoglobin 14.5 g/dl). We suggest that the relationship between polycythemia and Wilms' tumor should be carefully evaluated before surgical treatment. 4 UI - 21301299 AU - Sandoval C; Ozkaynak MF; Tugal O; Jayabose S TI - Hyperdiploidy in a case of favorable histology Wilms tumor. SO - Cancer Genet Cytogenet 2001 May;127(1):89-90 AD - Department of Pediatrics, New York Medical College, Munger Pavilion Room 110, Valhalla, NY 10595, USA. AB - Hyperdiploidy is useful in defining histologic variants of Wilms tumor and prognosis in other childhood cancers. We describe a case of hyperdiploid favorable histology Wilms tumor (50,XY,+6,+7,+8,+10,+12,-21[2]/51,idem,+9[6]/46,XY[12]) in a 3-year-old boy, and review the literature for other hyperdiploid childhood renal lesions. 5 UI - 21137121 AU - Rommel D; Pirson Y TI - Medullary sponge kidneypart of a congenital syndrome. SO - Nephrol Dial Transplant 2001 Mar;16(3):634-6 AD - Cliniques Universitaires St-Luc, Department of Nephrology, Universite Catholique de Louvain, Brussels, Belgium. 6 UI - 21324305 AU - Cui H; Niemitz EL; Ravenel JD; Onyango P; Brandenburg SA; Lobanenkov VV; Feinberg AP TI - Loss of imprinting of insulin-like growth factor-II in Wilms' tumor commonly involves altered methylation but not mutations of CTCF or its binding site. SO - Cancer Res 2001 Jul 1;61(13):4947-50 AD - Institute of Genetic Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA. AB - Loss of imprinting (LOI) is the most common molecular abnormality in Wilms' tumor (WT), other embryonal cancers, and most other tumor types. LOI in WT involves activation of the normally silent maternal allele of the insulin-like growth factor-II (IGF2) gene, silencing of the normally active maternal allele of the H19 gene, and aberrant methylation of a differentially methylated region (DMR) upstream of the maternal copy of H19. Recently, the transcription factor CTCF, which binds to the H19 DMR, has been implicated in the maintenance of H19 and IGF2 imprinting. Here, we show that mutations in the CTCF gene or in the H19 DMR do not occur at significant frequency in WT, nor is there transcriptional silencing of CTCF. We also confirm that methylation of the H19 DMR in WT with LOI includes the CTCF core consensus site. However, some WTs with normal imprinting of IGF2 also show aberrant methylation of CTCF binding sites, indicating that methylation of these sites is necessary but not sufficient for LOI in WT. 7 UI - 21319785 AU - Korones DN; Brown MR; Palis J TI - "Liver function tests" are not always tests of liver function. SO - Am J Hematol 2001 Jan;66(1):46-8 AD - Department of Pediatrics, University of Rochester, School of Medicine and Dentistry, Children's Hospital at Strong, New York 14642, USA. david-korones@urmc.rochester.edu AB - A child with Wilm's tumor and a child with immune thrombocytopenic purpura (ITP) were each noted to have persistent elevations of aspartate aminotransferase (AST), alanine aminotransferase (ALT), and lactate dehydrogenase (LDH). Both children underwent thorough evaluation for liver disease and, as a result, experienced delays in treatment of the Wilm's tumor and ITP. Eventually both children were found to have extremely elevated serum creatine kinase (CK). Muscle biopsy confirmed diagnoses of Duchenne's muscular dystrophy in one child, and Becker's muscular dystrophy in the second. Hematologists/oncologists should consider obtaining a serum CK to rule out muscle disease in patients with unexplained elevations of AST, ALT, and LDH. 8 UI - 21345515 AU - Hogeboom CJ; Grosser SC; Guthrie KA; Thomas PR; D'Angio GJ; Breslow NE TI - Stature loss following treatment for Wilms tumor. SO - Med Pediatr Oncol 2001 Feb;36(2):295-304 AD - Department of Biostatistics, University of California-San Francisco, USA. AB - BACKGROUND: The study was designed to estimate reduction in adult stature induced by megavoltage radiation therapy (RT) of the spine in children treated for Wilms tumor and to ascertain whether the dose reduction in successive National Wilms Tumor Study Group (NWTSG) trials has mitigated late effects of RT in these children. PROCEDURE: Effects of RT dose, age at treatment, and chemotherapy on stature of 2,778 children with Wilms or another solid tumor of the kidney were analyzed using statistical models accounting for the dependence of height on gender and advancing age. Model predictions were validated by descriptive analysis of heights measured at 17 to 18 years of age for 205 patients. RESULTS: Radiation-induced reductions below normal height depended on dose, portal size, and age at treatment and were not augmented by doxorubicin or cyclophosphamide. Younger children were more strongly affected. Predicted height deficit at age 18 years was 1.8 cm for a child treated with 10 Gy to the flank at age 4 years. Observed height deficits at age 1 7 to 18 years were 4.1 cm for 57 patients who received 15-24 Gy at a mean age of 55 months and zero for 16 children who received RT doses under 15 Gy at a mean age of 83 months. CONCLUSIONS: Reduction in stature following RT to the pediatric spine is dose- and age-dependent, persists into adulthood, and is not exacerbated by doxorubicin or cyclophosphamide. Average height deficits observed at maturity for children receiving doses currently recommended by the NWTSG are clinically nonsignificant. 9 UI - 21325336 AU - Papezova M; Mares J; Goetz P TI - [Molecular genetics of Wilms' tumor] SO - Cas Lek Cesk 2001 Jun 7;140(11):323-7 AD - Ustav biologie a lekarske genetiky 2. LF UK, Praha. AB - Molecular genetics of the Wilms' tumor plays an important role in the elucidation of the genetic etiology of the tumor disease generally. Contrary to the genesis of retinoblastoma, where a single gene is inactivated by two hits, the biological signalling pathways determining the origin of the Wilms' tumor are more complex and several genes in several loci may participate. Formation of the Wilms' tumor is accompanied with the most frequent genetic alteration, which is the loss of heterozygosity on the short arm of chromosome 11. It indicates inactivation of one or several tumor suppressor genes located at 11p region. The most studied gene of the Wilms' tumor is WT1 gene, which has been cloned and sequenced. Biological function of WT1 protein is complex one and it requires probably an interaction with other proteins, DNA and also RNA. The development of the tumor determines not only the genetic changes, but also epigenetic changes, e.g., hypermethylation of promoter and genome imprinting. 10 UI - 20342050 AU - Ishida Y; Kato K; Kigasawa H; Ohama Y; Ijiri R; Tanaka Y TI - Synchronous occurrence of pleuropulmonary blastoma and cystic nephroma: possible genetic link in cystic lesions of the lung and the kidney. SO - Med Pediatr Oncol 2000 Jul;35(1):85-7 AD - Division of Hematology, Kanagawa Children's Medical Center, Yokohama, Japan. 11 UI - 21327969 AU - Costelloe CM; Neitzschman H TI - Radiology case of the month. Asymptomatic abdominal mass in a child. Wilms' tumor (Nephroblastoma). SO - J La State Med Soc 2001 May;153(5):229 AD - Tulane University School of Medicine, New Orleans, Louisiana, USA. 12 UI - 21359809 AU - Ishikawa K; Toyoda Y; Fukuzato Y; Kato K; Ijiri R; Tanaka Y TI - Maturation in the primary and metastatic lesions of fetal rhabdomyomatous nephroblastoma. SO - Med Pediatr Oncol 2001 Jul;37(1):62-3 AD - Division of Oncology, Kanagawa Children's Medical Center, Yokohama, Japan.
CancerLIT: 703455 nephroblastoma (Wilms tumour) with diffuse anaplasia and lymph metastasis. Eleven of 63 evaluable children (17.5%) had a congenital anomaly. http://cancerweb.ncl.ac.uk/cancernet/cancerlit/703455.html
School Of Human Development University Of Nottingham Vadeyar S, Ramsay M, James D, ONeill D. Prenatal diagnosis of congenital Wilmstumor (nephroblastoma) presenting as fetal hydrops. http://www.nottingham.ac.uk/human-development/staff/James.htm
Extractions: Fetal development: The main emphasis of my work has been in the area of fetal neurobehavioural development. The methods used include ultrasound recording of fetal behaviour (both passive and stimulated) and cerebral blood flow together with functional MRI. In utero observations are related to postnatal behaviour and long term follow-up. Recent work has focused on fetal learning.
School Of Human Development University Of Nottingham Vadeyar S, Ramsay M, James D, ONeill D. Prenatal diagnosis of congenital Wilmstumor (nephroblastoma) presenting as fetal hydrops. http://www.nottingham.ac.uk/human-development/staff/Ramsay.htm
Extractions: Publications (2000-2003) Brackley KJ, Ramsay MM, Broughton Pipkin F, Rubin PC. The maternal cerebral circulation in pre-eclampsia: investigations using Laplace transform analysis of Doppler waveforms. Br J Obstet Gynaecol 2000; 107: 492-500. Ramsay M. Management of pre-eclampsia. Chapter 14 in Handbook of Hypertension, vol 21: Hypertension in Pregnancy. Editor: PC Rubin. Elsevier Science, Amsterdam, 2000: pp279-295. Ramsay MM, James DK, Steer PJ, Weiner CP, Gonik B. Normal Values in Pregnancy 2nd Edition. WB Saunders, London, 2000.
Extractions: This Article Extract FREE Full Text (PDF) Alert me when this article is cited ... Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in ISI Web of Science Similar articles in PubMed ... Request Permissions PubMed PubMed Citation Articles by Rommel, D. Articles by Pirson, Y. Nephrol Dial Transplant (2001) 16: 634-636 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
The EyePathologist Disease - N nephroblastoma Klintworth, Gordon K. Night blindness - congenital stationarywith myopia - Klintworth, Gordon K. NINCL - Klintworth, Gordon K. http://www.eyepathologist.com/LIST.ASP?Title=N
Vet Pathol -- Table Of Contents (May 1989, 26 [3]) congenital hypomyelinating polyneuropathy in two golden retriever a dog abenign variant of nephroblastoma T. Takeda; T. Makita; N. Nakamura; H. Horie http://www.vetpathology.org/content/vol26/issue3/index.shtml
Extractions: [Search ALL Issues] To see an article , click its [Full Text] link. To review many abstracts , check the boxes to the left of the titles you want, and click the 'Get All Checked Abstract(s)' button. To see one abstract at a time , click its [Abstract] link. A necrotizing meningoencephalitis of pug dogs D. R. Cordy; T. A. Holliday
256300 NEPHROSIS 1, CONGENITAL, FINNISH TYPE; NPHS1 A number sign ( ) is used with this entry because Finnish congenital and Soave (1964) observed nephrosis in association with nephroblastoma in 2 sibs. http://srs.sanger.ac.uk/srsbin/cgi-bin/wgetz?[omim-ID:256300] -e
Ophthalmic Plastic And Reconstructive Surgery - UserLogin congenital Orbital and Disseminated Extrarenal Malignant Rhabdoid Tumor tumor of the kidney) have a distinct genetic origin from nephroblastoma. http://www.op-rs.com/pt/re/oprs/fulltext.00002341-200501000-00017.htm
The American Journal Of Surgical Pathology - UserLogin Heterotopic nephrogenic rests in the colon and multiple congenital anomalies Imaging of a spinal nephroblastoma in a dog. Vet Radiol Ultrasound. http://www.ajsp.com/pt/re/ajsp/fulltext.00000478-200410000-00017.htm
Untitled Document Management of various childhood tumours (nephroblastoma, neuroblastoma, teratoma, Harvest and utilisation of homografts in congenital heart surgery http://www.wacs-coac.org/surger.htm
Extractions: INTRODUCTION The training programme shall be for a minimum of 4 years. A candidate may be exempted from part of the training programme and examination following an application and approval by Council, on the recommendation of the Credentials Committee through the Faculty Board. QUALIFICATIONS FOR ACCEPTANCE INTO THE PROGRAMME Candidates must possess a basic medical degree or qualification registrable by the local Medical Council. Candidates must have served the pre-registration year/years in their own country or in any other country accepted by the local Medical Council and must have been fully registered. EXAMINATIONS The examinations for the Fellowship are held in April and October and shall be in three Parts: Primary Examination in the Basic Sciences
Indian Pediatrics - Editorial The malignant tumors reported in full blown BWS are nephroblastoma adrenal cortical congenital hepatoblastoma and schizencephaly in an infant with http://www.indianpediatrics.net/mar2002/mar-299-304.htm
Extractions: L.S. Arya From the Division of Pediatric Oncology, Department of Pediatrics and *Rajendra Prasad Center for Ophthalmic Sciences, All India Institute of Medical Sciences, Ansari Nagar, New Delhi 110 029, India. Correspondence to: Dr. V. Thavaraj, Department of Pediatrics, All India Institute of Medical Sciences, Ansari Nagar, New Delhi 110 029, India. E-mail: sowmyam@mantraonline.com Manuscript received: May 31, 2001; Revision accepted: September 5, 2001. Beckwith-Wiedemann syndrome (BWS) was first recognized as a syndrome by Beckwith in 1963, when he reported autopsy findings on three unrelated children with omphalocele, macroglossia, cytomegaly of adrenal cortex, renal medullary hyperplasia and hyperplastic visceromegaly(1). In 1964 Wiedemann published an article on the same syndrome in three siblings with omphalocele, macrosmia and neonatal hypoglycemia(2). The incidence of BWS has been estimated to be 1:13700 live births(3). The increased risk of tumor formation in BWS patients is estimated to be 7.5%(4) and the risk is further increased to 10% if hemihypertrophy is present(4). It has been suggested that BWS can be inherited by the homozygous state of an autosomal recessive mutation(5). We describe a rare case report of partial expression of BWS associated with orbital rhabdomyosarcoma (RMS).
Birth Disorder Information Directory - W Westerhof Beemer Cormane Syndrome (Macules, Hereditary congenital Hypopigmented Wilms Tumor (nephroblastoma). List of Sites; with Pseudohermaphroditism http://www.bdid.com/defectw.htm
Extractions: HOME W Syndrome Waaler Aarskog Syndrome (Hydrocephalus with Costovertebral Dysplasia and Sprengel Anomaly) Waardenburg Syndrome -Shah Syndrome with Hirschsprung Disease Wagner Syndrome (Erosive Vitreoretinopathy, Hyaloideoretinal Degeneration of Wagner, Wagner Vitreoretinal Degeneration) Wagner-Stickler Syndrome WAGR Syndrome Walbaum Titran Durieux Crepin Syndrome (Fibular Hypoplasia with Scapulo Pelvic Dysplasia and Absent 5th Fingers) Walker Dyson Syndrome (Aniridia-Mental Retardation Syndrome) Walker Warburg Syndrome (Cerebroocular Dysgenesis; Cerebroocular Dysplasia-Muscular Dystrophy Syndrome; Chemke Syndrome; COD-MD Syndrome; Hard +/- E Syndrome; H ydrocephalus
Results AFIP Wednesday Slide Conference - No. 28 10 May 1995 They may be congenital, such as Meckel s diverticulum, or acquired. Most casesof nephroblastoma in swine are diagnosed based on gross findings at http://www.afip.org/vetpath/WSC/WSC94/94wsc28.txt
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