Summary
Definition
History and exam
Key diagnostic factors
- upper abdominal/flank mass or swelling
- congenital urogenital anomalies
- congenital syndromes
Other diagnostic factors
- abdominal distension
- abdominal pain
- hypertension
- hematuria
- poor appetite or weight loss
- fever
- pallor
- shortness of breath
- hepatomegaly
- varicocele
- family history of Wilms tumor
- hypoglycemia in infancy
- features of paraneoplastic syndrome
Risk factors
- age <5 years
- congenital urogenital anomalies
- congenital syndromes
- family history of Wilms tumor
- prenatal exposure to harmful environmental factors
Diagnostic tests
1st tests to order
- CBC
- renal function
- liver function tests
- urinalysis
- serum total protein/albumin
- coagulation studies
- serum calcium level
- abdominal ultrasound with Doppler
- CT or MRI abdomen and pelvis with and without contrast
- CT chest with and without contrast
- chest x-ray
Tests to consider
- tumor histology
- genetic testing
- loss of heterozygosity (LOH) studies
Treatment algorithm
COG criteria
SIOP criteria
tumor recurrence
Contributors
Authors
Sandeep Batra, MD
Clinical Assistant Professor
Riley Hospital for Children
Department of Pediatrics
Indiana University School of Medicine
Indianapolis
IN
Disclosures
SB declares that he has no competing interests.
Peer reviewers
Nadine Deannie Lee, MD
Pediatric Hematologist-Oncologist
Hematology/Oncology
Riley Children's Hospital
Indiana University Health
Indianapolis
IN
Disclosures
NDL declares that she has no competing interests.
Norbert Graf, MD
Direktor
Klinik für Pädiatrische Onkologie und Hämatologie
Universitätsklinikum des Saarlandes
Homburg
Germany
Disclosures
NG declares that he has no competing interests.
Zelig Tochner, MD
Associate Professor
Radiation Oncology
Children's Hospital of Philadelphia
Philadelphia
PA
Disclosures
ZT declares that he has no competing interests.
References
Key articles
Spreafico F, Fernandez CV, Brok J, et al. Wilms tumour. Nat Rev Dis Primers. 2021 Oct 14;7(1):75.Full text Abstract
Lopyan NM, Ehrlich PF. Surgical management of Wilms tumor (nephroblastoma) and renal cell carcinoma in children and young adults. Surg Oncol Clin N Am. 2021 Apr;30(2):305-23. Abstract
Vujanić GM, Parsons LN, D'Hooghe E, et al. Pathology of Wilms' tumour in International Society of Paediatric Oncology (SIOP) and Children's Oncology Group (COG) renal tumour studies: similarities and differences. Histopathology. 2022 Jun;80(7):1026-37. Abstract
van den Heuvel-Eibrink MM, Hol JA, Pritchard-Jones K, et al. Position paper: rationale for the treatment of Wilms tumour in the UMBRELLA SIOP-RTSG 2016 protocol. Nat Rev Urol. 2017 Dec;14(12):743-52.Full text Abstract
Dome JS, Mullen EA, Dix DB, et al. Impact of the first generation of Children's Oncology Group clinical trials on clinical practice for Wilms tumor. J Natl Compr Canc Netw. 2021 Aug 1;19(8):978-85.Full text Abstract
Reference articles
A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.
Differentials
- Neuroblastoma
- Clear cell sarcoma of the kidney
- Renal cell carcinoma
More DifferentialsGuidelines
- Wilms tumor (nephroblastoma)
- SIOP PODC: clinical guidelines for the management of children with Wilms tumour in a low income setting
More Guidelines
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