eMedicine Specialties > Pediatrics: General Medicine > Oncology

WAGR Syndrome: Treatment & Medication

Author: Steven K Bergstrom, MD, Assistant to the Chairman, Department of Pediatrics, Division of Hematology-Oncology, Kaiser Permanente Medical Center of Oakland
Contributor Information and Disclosures

Updated: Oct 2, 2009

Treatment

Medical Care

  • Urology: After aniridia, GU abnormalities, and mental retardation (AGR) syndrome or Wilms tumor aniridia, GU abnormalities, and mental retardation (WAGR) syndrome is diagnosed, GU abnormalities must be evaluated immediately, and the patient should be referred to a pediatric urologist.
  • Genetics: Although the abnormality associated with WAGR syndrome may not be demonstrated by means of routine cytogenetic tests, the geneticist can help in assessing the 11p13 region using fluorescent in situ hybridization techniques. He or she can consult with the family regarding the likelihood of their having other affected offspring, and the geneticist can consult with patients regarding the risks to their offspring.
  • Oncology: Once a Wilms tumor is diagnosed in a patient with WAGR syndrome, the services of a pediatric oncologist are required for treatment and follow-up.

Surgical Care

  • Specific urologic intervention may be required early in patients with AGR syndrome. However, if a Wilms tumor develops, a multidisciplinary approach is required prior to surgery.

Consultations

  • A pediatric ophthalmologist should be consulted early in the course of the disease to evaluate the ocular deformity, and this ophthalmologist should follow-up with the patient for long-term consequences (eg, vision loss, potential cataract development).
  • The potential for developmental delay in children with AGR or WAGR syndrome requires early evaluation and intervention by a pediatric developmental specialist. On the basis of the findings from this evaluation, children with vision impairment and mental retardation can be appropriately referred to community resources.

Medication

Medical treatment of patients with Wilms tumor, aniridia, GU abnormalities, and mental retardation (WAGR) syndrome depends on the appearance of Wilms tumor. The histologic features and the stage of the tumor determine the appropriate chemotherapeutic course. Refer to a pediatric oncologist for the most current chemotherapeutic regimen.

More on WAGR Syndrome

Overview: WAGR Syndrome
Differential Diagnoses & Workup: WAGR Syndrome
Treatment & Medication: WAGR Syndrome
Follow-up: WAGR Syndrome
Multimedia: WAGR Syndrome
References
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References

  1. Han JC, Liu QR, Jones M, Levinn RL, Menzie CM, Jefferson-George KS, et al. Brain-derived neurotrophic factor and obesity in the WAGR syndrome. N Engl J Med. Aug 2008;359:918-927. [Medline].

  2. D'Angio GJ, Breslow N, Beckwith JB, et al. Treatment of Wilms' tumor. Results of the Third National Wilms' Tumor Study. Cancer. Jul 15 1989;64(2):349-60. [Medline].

  3. Breslow N, Olshan A Beckwith JB. Epidemiology of Wilms tumor. Med Ped Oncol. 1993;21:172-181.

  4. Robinson DO, Howarth RJ, Williamson KA, van Hyningen V, Beal SJ, Crolla JA. Genetic analysis of chromosome 11p13 and the PAX6 gene in a series of 125 cases referred with aniridia. Am J Med Genet A. Mar 2008;164A:558-569. [Medline].

  5. Termine C, Parigi G, Rossi M, Romano P, Balotin U. WAGR syndrome: is the 'R' always justified?. Clin Dysmorphol. Jan 2007;16:69-70. [Medline].

  6. Knudson AG, Strong LC. Mutation and cancer: a model for Wilms tumor of the kidney. J Natl Cancer Inst. Feb 1972;48:313-324.

  7. Call KM, Glaser T, Ito CY. Isolation and characterization of a zinc finger polypeptide gene at the human chromosome 11 Wilms tumor locus. Cell. Feb 1990;60:509-520.

  8. Ton CCT, Hirvonen H, Miwa H. Positional cloning and characterization of a paired box- and homeobox-containing gene from the aniridia region. Cell. 1991;67:1059-1074.

  9. Haber DA,. Oshn RL, Buckler AJ. Alternative splicing and alternative structure of the Wilms tumor gene WT1. Proc Natl Acad Sci USA. 1991;88:9618-9622.

  10. Beckwith JB. Precursor lesions of Wilms tumor: clinical and biological impications. Med Pediatr Oncol. 1993;21:158-168.

  11. Fischbach BV, Trout KL, Lewis J. WAGR syndrome: A clinical review of 54 cases. Pediatrics. Oct 2005;116:984-988.

  12. Dahan K, Kamal M, Noel LH, Jeanpierre C, Gubler MC, Brousse N, et al. Small glomeruli in WAGR syndrome (Wilms tumor, aniridia, genitourinary anomalies and mental retardation) syndrome. Am J Kidney Dis. June 2007;49:522-527.

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Further Reading

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Keywords

Wilms tumor, aniridia, genitourinary malformations, mental retardation, Wilms' tumor, adenomyosarcoma, embryoma of the kidney, mesoblastic nephroma, nephroblastoma, genitourinary malformations, GU malformations, WAGR syndrome, AGR syndrome, glaucoma, cataracts, cryptorchidism, hypospadias, renal malformations, ureteral malformations, streak ovaries, bicornuate uterus, pseudohermaphroditism, Denys-Drash syndrome, nephromegaly, hematuria, hypertension, varicocele, scanning nystagmus, Frasier syndrome, nephroblastomatosis 

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Contributor Information and Disclosures

Author

Steven K Bergstrom, MD, Assistant to the Chairman, Department of Pediatrics, Division of Hematology-Oncology, Kaiser Permanente Medical Center of Oakland
Steven K Bergstrom, MD is a member of the following medical societies: Alpha Omega Alpha, American Society of Clinical Oncology, American Society of Hematology, American Society of Pediatric Hematology/Oncology, Children's Oncology Group, and International Society for Experimental Hematology
Disclosure: Nothing to disclose.

Medical Editor

Stephan A Grupp, MD, PhD, Director, Stem Cell Biology Program, Department of Pediatrics, Division of Oncology, Children's Hospital of Philadelphia; Associate Professor of Pediatrics, University of Pennsylvania
Stephan A Grupp, MD, PhD is a member of the following medical societies: American Association for Cancer Research, American Society for Blood and Marrow Transplantation, American Society of Hematology, American Society of Pediatric Hematology/Oncology, and Society for Pediatric Research
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

Timothy P Cripe, MD, PhD, Professor of Pediatric Hematology/Oncology, University of Cincinnati; Director, Translational Research Trials Office, Department of Pediatrics, Cincinnati Children's Hospital Medical Center
Timothy P Cripe, MD, PhD is a member of the following medical societies: American Association for the Advancement of Science, American Pediatric Society, American Society of Hematology, American Society of Pediatric Hematology/Oncology, and Society for Pediatric Research
Disclosure: Nothing to disclose.

CME Editor

Helen SL Chan, MBBS, FRCP(C), FAAP, Senior Scientist, Research Institute; Professor, Division of Hematology/Oncology, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Canada
Helen SL Chan, MBBS, FRCP(C), FAAP is a member of the following medical societies: American Academy of Pediatrics, American Association for Cancer Research, American Society of Hematology, and Royal College of Physicians and Surgeons of Canada
Disclosure: Nothing to disclose.

Chief Editor

Max J Coppes, MD, PhD, MBA, Senior Vice President, Children's National Medical Center (Center for Cancer and Blood Disorders); Director, Center for Cancer and Immunology Research, Children's Research Institute, Children's National Medical Center; Professor of Medicine, Oncology, and Pediatrics, Georgetown University
Max J Coppes, MD, PhD, MBA is a member of the following medical societies: American Association for Cancer Research, American Society of Pediatric Hematology/Oncology, and Society for Pediatric Research
Disclosure: Nothing to disclose.

 
 
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