Wilms Tumor Workup

  • Author: Arnold C Paulino, MD; Chief Editor: Robert J Arceci, MD, PhD   more...
 
Updated: Sep 23, 2011
 

Approach Considerations

The following studies are indicated in patients with Wilms tumor:

  • Complete blood count (CBC)
  • Chemistry profile - Including kidney function tests and routine measurements of electrolytes and calcium
  • Urinalysis
  • Coagulation studies
  • Cytogenetics studies, including 1p and 16q deletion

Results may reveal an 11p13 deletion, as in WAGR syndrome, or a duplication of the paternal allele 11p15, as in Beckwith-Wiedemann syndrome (BWS). Mutational analysis of the WT1 gene may be indicated when Denys-Drash syndrome (intersexual disorders, nephropathy, Wilms tumor) is suspected.

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Imaging Studies

Four-field chest radiography

Images may depict lung metastases. Patients with lung lesions on chest radiography have traditionally been given whole-lung radiation therapy.

Renal ultrasonography

Renal ultrasonography is often the initial study, because it does not expose children to the detrimental effects of radiation. Real-time ultrasonography is a relatively inexpensive way of helping determine the patency of the inferior vena cava. When a tumor is identified in the vessel, the proximal extent of the thrombus must be established prior to surgery, because of possible extension of the tumor to the right atrium.

CT scanning

Abdominal computed tomography (CT) scanning helps in determining the origin of the tumor, involvement of the lymph nodes, bilateral kidney involvement, invasion into major vessels (eg, inferior vena cava), and liver metastases. (See the images below.)[20]

CT scan in a patient with a right-sided Wilms tumoCT scan in a patient with a right-sided Wilms tumor with favorable histology. CT scan of child with a stage IV Wilms tumor with CT scan of child with a stage IV Wilms tumor with favorable histology. Note the bilateral pulmonary metastases.

If chest CT scan findings are positive and chest radiographic findings are negative, diagnostic biopsy of the lesions noted on the chest CT scan is recommended.

MRI scanning

Abdominal magnetic resonance imaging (MRI) is reportedly the most sensitive imaging modality for determination of caval patency and may be important in determining whether the inferior vena cava is directly invaded by the tumor. Wilms tumor demonstrates low signal intensity on T1-weighted images and high signal intensity on T2-weighted images.

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Surgical Examination and Biopsy

Histopathologic confirmation of Wilms tumor is essential. In North America, patients with suspected Wilms tumor undergo nephrectomy immediately (see the image below). During this procedure, the contralateral kidney is explored to ensure that the disease is indeed unilateral, and lymph node biopsy samples are obtained for staging purposes. Lymph node dissection is not indicated. (Immediate nephrectomy is not performed in patients with bilateral disease at presentation, when sparing of the renal tissue becomes important.)

Gross nephrectomy specimen shows a Wilms tumor pusGross nephrectomy specimen shows a Wilms tumor pushing the normal renal parenchyma to the side.

In contrast to immediate surgery, most European centers make a presumptive diagnosis of Wilms tumor based on imaging findings alone. Clinicians in Europe prefer to administer chemotherapy before nephrectomy without survival compromise.[21, 22]

Transcutaneous biopsy is not usually recommended and may in fact complicate treatment by causing preoperative tumor spill, requiring whole abdominal radiotherapy.

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Histologic Findings

The classic histologic pattern in Wilms tumor is triphasic and composed of epithelial, blastemal, and stromal elements. Approximately 90% of all renal tumors have favorable histology.

About 3-7% of Wilms tumors are characterized by anaplastic changes. If these changes are present diffusely throughout the tumor, they are predictive of a poor outcome. Wilms tumors with anaplastic changes have unfavorable histology.

Two tumor types previously included in the category with unfavorable histology are, in fact, clearly separate malignant entities: clear cell sarcoma of the kidney and rhabdoid tumor of the kidney.

The improved histopathologic classification of childhood renal tumors has not only helped to define appropriate treatment strategies for these patients but has also contributed to the understanding of the molecular genetic events underlying the Wilms tumor.

For instance, nephrogenic rests, dysplastic lesions of metanephric origin, are now believed to represent precursor lesions. These lesions are observed in approximately one third of kidneys affected by Wilms tumors.

The relationship between the pathology of the nephrogenic rests, the tumor, and the congenital disorders is of particular interest. These associations have been helpful in evaluating a potential correlation between a Wilms tumor phenotype in one regard and molecular genetic events leading to the development of that same tumor in another.

Children younger than age 12 months diagnosed with perilobar nephrogenic rests have a markedly increased risk of developing a contralateral Wilms tumor.[23]

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Contributor Information and Disclosures
Author

Arnold C Paulino, MD  Professor of Radiation Oncology, Methodist Hospital and Weill-Cornell Medical College; Associate Professor of Pediatrics, Baylor College of Medicine

Arnold C Paulino, MD is a member of the following medical societies: American Medical Association, American Radium Society, American Society for Therapeutic Radiology and Oncology, American Society of Clinical Oncology, Children's Oncology Group, International Society of Paediatric Oncology, and Radiological Society of North America

Disclosure: Nothing to disclose.

Coauthor(s)

Max J Coppes, MD, PhD, MBA  Senior Vice President, Center for Cancer and Blood Disorders, Children's National Medical Center; Professor of Medicine, Oncology, and Pediatrics, Georgetown University School of Medicine; Clinical Professor of Pediatrics, George Washington University School of Medicine and Health Sciences

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.

Specialty Editor Board

Kathleen M Sakamoto, MD, PhD  Professor and Chief, Division of Hematology-Oncology, Vice-Chair of Research, Mattel Children's Hospital at UCLA; Co-Associate Program Director of the Signal Transduction Program Area, Jonsson Comprehensive Cancer Center, California Nanosystems Institute and Molecular Biology Institute, University of California, Los Angeles, David Geffen School of Medicine

Kathleen M Sakamoto, MD, PhD is a member of the following medical societies: American Society of Hematology, American Society of Pediatric Hematology/Oncology, International Society for Experimental Hematology, Society for Pediatric Research, and Western Society for Pediatric Research

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Steven K Bergstrom, MD  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.

Chief Editor

Robert J Arceci, MD, PhD  King Fahd Professor of Pediatric Oncology, Professor of Pediatrics, Oncology and the Cellular and Molecular Medicine Graduate Program, Kimmel Comprehensive Cancer Center at Johns Hopkins University School of Medicine

Robert J Arceci, MD, PhD is a member of the following medical societies: American Association for Cancer Research, American Association for the Advancement of Science, American Pediatric Society, American Society of Hematology, and American Society of Pediatric Hematology/Oncology

Disclosure: Nothing to disclose.

References

  1. Coppes MJ, Pritchard-Jones K. Principles of Wilms' tumor biology. Urol Clin North Am. Aug 2000;27(3):423-33, viii. [Medline].

  2. Knudson AG, Strong LC. Mutation and cancer: a model for Wilms' tumor of the kidney. J Natl Cancer Inst. Feb 1972;48(2):313-24. [Medline].

  3. Coppes MJ, Haber DA, Grundy PE. Genetic events in the development of Wilms' tumor. N Engl J Med. Sep 1 1994;331(9):586-90. [Medline].

  4. Coppes MJ, Huff V, Pelletier J. Denys-Drash syndrome: relating a clinical disorder to genetic alterations in the tumor suppressor gene WT1. J Pediatr. Nov 1993;123(5):673-8. [Medline].

  5. Grundy PE, Breslow NE, Li S, et al. Loss of heterozygosity for chromosomes 1p and 16q is an adverse prognostic factor in favorable-histology Wilms tumor: a report from the National Wilms Tumor Study Group. J Clin Oncol. Oct 10 2005;23(29):7312-21. [Medline].

  6. Breslow N, Olshan A, Beckwith JB, Green DM. Epidemiology of Wilms tumor. Med Pediatr Oncol. 1993;21(3):172-81. [Medline].

  7. Green DM. The treatment of stages I-IV favorable histology Wilms' tumor. J Clin Oncol. Apr 15 2004;22(8):1366-72. [Medline].

  8. Montgomery BT, Kelalis PP, Blute ML, et al. Extended followup of bilateral Wilms tumor: results of the National Wilms Tumor Study. J Urol. Aug 1991;146(2 ( Pt 2)):514-8. [Medline].

  9. Paulino AC, Wilimas J, Marina N, et al. Local control in synchronous bilateral Wilms tumor. Int J Radiat Oncol Biol Phys. Oct 1 1996;36(3):541-8. [Medline].

  10. Paulino AC, Thakkar B, Henderson WG. Metachronous bilateral Wilms' tumor: the importance of time interval to the development of a second tumor. Cancer. Jan 15 1998;82(2):415-20. [Medline].

  11. Dome JS, Cotton CA, Perlman EJ, et al. Treatment of anaplastic histology Wilms' tumor: results from the fifth National Wilms' Tumor Study. J Clin Oncol. May 20 2006;24(15):2352-8. [Medline].

  12. Green DM, Cotton CA, Malogolowkin M, et al. Treatment of Wilms tumor relapsing after initial treatment with vincristine and actinomycin D: a report from the National Wilms Tumor Study Group. Pediatr Blood Cancer. May/ 2007;48:493-9. [Medline].

  13. Malogolowkin M, Cotton CA, Green DM, et al. Treatment of Wilms tumor relapsing after initial treatment with vincristine, actinomycin D, and doxorubicin. A report from the National Wilms Tumor Study Group. Pediatr Blood Cancer. Feb 2008;50(2):236-41. [Medline].

  14. Green DM, Donckerwolcke R, Evans AE, D'Angio GJ. Late effects of treatment for Wilms tumor. Hematol Oncol Clin North Am. Dec 1995;9(6):1317-27. [Medline].

  15. Egeler RM, Wolff JE, Anderson RA, Coppes MJ. Long-term complications and post-treatment follow-up of patients with Wilms' tumor. Semin Urol Oncol. Feb 1999;17(1):55-61. [Medline].

  16. Evans AE, Norkool P, Evans I, et al. Late effects of treatment for Wilms' tumor. A report from the National Wilms' Tumor Study Group. Cancer. Jan 15 1991;67(2):331-6. [Medline].

  17. Lange J, Peterson SM, Takashima JR, et al. Risk Factors for End Stage Renal Disease in Non-WT1-Syndromic Wilms Tumor. J Urol. Aug 2011;186(2):378-86. [Medline]. [Full Text].

  18. Paulino AC, Wen BC, Brown CK, et al. Late effects in children treated with radiation therapy for Wilms' tumor. Int J Radiat Oncol Biol Phys. Mar 15 2000;46(5):1239-46. [Medline].

  19. van den Heuvel-Eibrink MM, Grundy P, Graf N, et al. Characteristics and survival of 750 children diagnosed with a renal tumor in the first seven months of life: A collaborative study by the SIOP/GPOH/SFOP, NWTSG, and UKCCSG Wilms tumor study groups. Pediatr Blood Cancer. Jun 2008;50(6):1130-4. [Medline].

  20. Refaie HD, Sarhan M, Hafez A. Role of CT in assessment of unresectable Wilms' tumor response after preoperative chemotherapy in pediatrics. ScientificWorldJournal. Jul 13 2008;8:661-9. [Medline].

  21. Mitchell C, Pritchard-Jones K, Shannon R, et al. Immediate nephrectomy versus preoperative chemotherapy in the management of non-metastatic Wilms' tumour: results of a randomised trial (UKW3) by the UK Children's Cancer Study Group. Eur J Cancer. Oct 2006;42(15):2554-62. [Medline].

  22. Reinhard H, Semler O, Burger D, et al. Results of the SIOP 93-01/GPOH trial and study for the treatment of patients with unilateral nonmetastatic Wilms Tumor. Klin Padiatr. May-Jun 2004;216(3):132-40. [Medline].

  23. Coppes MJ, Arnold M, Beckwith JB, et al. Factors affecting the risk of contralateral Wilms tumor development: a report from the National Wilms Tumor Study Group. Cancer. Apr 1 1999;85(7):1616-25. [Medline].

  24. 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].

  25. Kalapurakal JA, Li SM, Breslow NE, et al. Influence of radiation therapy delay on abdominal tumor recurrence in patients with favorable histology Wilms' tumor treated on NWTS-3 and NWTS-4: a report from the National Wilms' tumor Study Group. Int J Radiat Oncol Biol Phys. 2003;57:495-9. [Medline].

  26. Green DM, Breslow NE, Beckwith JB, et al. Treatment with nephrectomy only for small, stage I/favorable histology Wilms tumor: a report from the National Wilms Tumor Study Group. J Clin Oncol. Sep 1/ 2001;19:3719-24. [Medline].

  27. Meisel JA, Guthrie KA, Breslow NE, Donaldson SS, Green DM. Significance and management of computed tomography detected pulmonary nodules: a report from the National Wilms Tumor Study Group. Int J Radiat Oncol Biol Phys. Jun 1 1999;44(3):579-85. [Medline].

  28. Hamilton TE, Ritchey ML, Haase GM, Argani P, Peterson SM, Anderson JR, et al. The management of synchronous bilateral wilms tumor: a report from the national wilms tumor study group. Ann Surg. May 2011;253(5):1004-10. [Medline].

  29. D'Angio GJ, Rosenberg H, Sharples K, et al. Position paper: imaging methods for primary renal tumors of childhood: costs versus benefits [published erratum appears in Med Pediatr Oncol 1993;21(9):695]. Med Pediatr Oncol. 1993;21(3):205-12. [Medline].

 
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CT scan in a patient with a right-sided Wilms tumor with favorable histology.
CT scan of child with a stage IV Wilms tumor with favorable histology. Note the bilateral pulmonary metastases.
Gross nephrectomy specimen shows a Wilms tumor pushing the normal renal parenchyma to the side.
Table 3. Survival Rates in Patients with Favorable-Histology Wilms Tumor
StageRelapse-Free Survival, %Overall Survival, %
I9298
II8596
III9095
IV8090
Table 1. Current Approach to Favorable Histology Wilms Tumor by Stage
Stage and HistologySurgeryChemotherapyRadiation Therapy*
Stage I or II favorable histology without loss of heterozygosity (LOH) 1p and 16q†NephrectomyVincristine, dactinomycinNo
Stage I or II favorable histology with LOH 1p and 16qNephrectomyVincristine, dactinomycin, doxorubicinNo
Stage III and IV favorable histology without LOH 1p and 16qNephrectomyVincristine, dactinomycin, doxorubicinYes
Stage III and IV favorable histology with LOH 1p and 16qNephrectomyVincristine, dactinomycin, doxorubicin, cyclophosphamide, etoposideYes
* The current dose for radiation therapy for favorable histology Wilms tumor is approximately 1080 cGy for the abdomen and 1200 cGy for the lung.[24] Postoperative radiotherapy is started within 14 days of nephrectomy.[25] Patients with stage IV favorable histology Wilms tumor and lung metastases whose pulmonary lesions do not disappear after 6 weeks of chemotherapy receive whole-lung radiation therapy.



† Some evidence suggests that certain children with stage I disease and favorable histology do well with nephrectomy alone.[26] Children younger than 24 months with small (< 550 g) Wilms tumors with favorable histology are noted in the current COG protocol.



Table 2. Recommended Follow-Up Imaging Studies in Children with Wilms Tumor Without Metastasis at Diagnosis*
Stage and Type of Wilms TumorImaging StudiesOff-Treatment Schedule
Stages I, II, and III with favorable histology; stages I, II, and III with anaplastic histologyChest radiography6 wk and 3 mo after surgery, then every 3 mo (5 times), then every 6 mo (3 times), then yearly (2 times)
All stages in patients aged < 48 mo at diagnosis with nephrogenic restsAbdominal ultrasonographyEvery 3 mo for 6 y
All stages in patients aged >48 mo at diagnosis with nephrogenic restsAbdominal ultrasonographyEvery 3 mo for 4 y
Stages I and II with favorable histologyAbdominal ultrasonographyYearly (6 times)
Stage III with favorable histologyAbdominal ultrasonography6 wk and 3 mo after surgery, then every 3 mo (5 times), then every 6 mo (3 times), then yearly (2 times)
All stages with unfavorable histologyAbdominal ultrasonographyEvery 3 mo (4 times), then every 6 mo (4 times)
* Subsequent imaging studies should be performed as clinically indicated.
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