Wilms Tumor Clinical Presentation

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

History and Physical Examination

History

The most common manifestation of Wilms tumor is an asymptomatic abdominal mass; an abdominal mass occurs in 80% of children at presentation. Abdominal pain or hematuria occurs in 25%. Urinary tract infection and varicocele are less common findings than these. Hypertension, gross hematuria, and fever are observed in 5-30% of patients. A few patients with hemorrhage into their tumor may present with hypotension, anemia, and fever. Rare patients with advanced disease may present with respiratory symptoms related to lung metastases.

Physical examination

Examination often reveals a palpable abdominal mass. Pay special attention to features of those syndromes (WAGR syndrome and Beckwith-Wiedemann syndrome [BWS]) associated with Wilms tumor (ie, aniridia, genitourinary malformations, and signs of overgrowth).

The abdominal mass should be carefully examined. Palpating a mass too vigorously could lead to the rupture of a large tumor into the peritoneal cavity.

Proceed to Differential Diagnoses
 
 
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

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  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].

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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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