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Wilms Tumor Clinical Presentation

  • Author: Arnold C Paulino, MD; Chief Editor: Jennifer Reikes Willert, MD  more...
 
Updated: Apr 27, 2016
 

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.

 
 
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: Radiological Society of North America, Children's Oncology Group, American Society of Clinical Oncology, International Society of Paediatric Oncology, American Medical Association, American Radium Society, American Society for Radiation Oncology

Disclosure: Received royalty from Elsevier, Inc for author of book.

Coauthor(s)

Max J Coppes, MD, PhD, MBA Executive Vice President, Chief Medical and Academic Officer, Renown Heath

Max J Coppes, MD, PhD, MBA is a member of the following medical societies: American College of Healthcare Executives, American Society of Pediatric Hematology/Oncology, Society for Pediatric Research

Disclosure: Nothing to disclose.

Specialty Editor Board

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, Children's Oncology Group, American Society of Clinical Oncology, International Society for Experimental Hematology, American Society of Hematology, American Society of Pediatric Hematology/Oncology

Disclosure: Nothing to disclose.

Chief Editor

Jennifer Reikes Willert, MD Associate Clinical Professor, Department of Pediatrics, Division of Pediatric Hematology/Oncology, Section of Stem Cell Transplantation, Stanford University Medical Center, Lucile Packard Children's Hospital

Jennifer Reikes Willert, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Hematology, American Society for Blood and Marrow Transplantation, Children's Oncology Group, American Society of Pediatric Hematology/Oncology

Disclosure: Nothing to disclose.

Additional Contributors

Kathleen M Sakamoto, MD, PhD Shelagh Galligan Professor, Division of Hematology/Oncology, Department of Pediatrics, Stanford University School of Medicine

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

Disclosure: Nothing to disclose.

References
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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.[28] Postoperative radiotherapy is started within 14 days of nephrectomy.[29] 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.[30] 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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