Summary
Definition
Wilms' tumour, or nephroblastoma, is the most common form of renal malignancy in childhood. It usually occurs in the first 2 to 5 years of life.[1][2] Approximately 500 children and adolescents <20 years of age are diagnosed each year in the US.[3][4] The risk for developing Wilms' tumour is increased in certain congenital overgrowth syndromes, congenital non-overgrowth syndromes, and children with congenital urogenital anomalies.[5][6][7][8][9][10] Long-term survival approaches 90% in localised disease.[Figure caption and citation for the preceding image starts]: Resected Wilms' tumourUniversity of Utah Health Sciences Center, Salt Lake City, Utah; used with permission [Citation ends].
History and exam
Key diagnostic factors
- presence of risk factors
- upper abdominal/flank mass or swelling
Other diagnostic factors
- abdominal distension
- abdominal pain
- pallor
- hypoglycaemia in infancy
- hypertension
- lymphadenopathy
- varicocele
- hepatomegaly
- cholestasis
- fatigue
- poor appetite or cachexia
- fever
- haematuria
- shortness of breath
- irritability
- bone tenderness or pain
- features of paraneoplastic syndrome
Risk factors
- age 2 to 5 years
- FHx
- congenital urogenital anomalies
- congenital syndromes
- antenatal exposure to radiation or chemicals
- paternal occupation
Diagnostic investigations
1st investigations to order
- FBC
- renal function
- LFTs
- urinalysis
- serum total protein/albumin
- coagulation studies
- serum calcium level
- abdominal ultrasound with Doppler
- CXR
- CT or MRI abdomen and pelvis with and without contrast
- CT or MRI chest with and without contrast
- biopsy
Investigations to consider
- genetic testing
Emerging tests
- loss of heterozygosity (LOH) studies
Treatment algorithm
NWTSG/COG criteria
SIOP/UKCCSG criteria
first tumour recurrence without multiple adverse prognostic factors
multiple adverse prognostic factors or multiple relapses
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.
Differentials
- Neuroblastoma
- Clear cell sarcoma of the kidney (CCSK)
- Renal cell carcinoma
More DifferentialsGuidelines
- SIOP PODC: clinical guidelines for the management of children with Wilms tumour in a low income setting
More Guidelines
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