Most common renal malignancy in children.
Presents as a unilateral, painless, abdominal/flank mass; rarely presents bilaterally.
Metastatic disease occurs in <10% of patients and needs to be carefully excluded using ultrasound and CT/MRI.
Long-term survival approaches 90% in localised disease.
Treatment is with nephrectomy, chemotherapy, and radiotherapy.
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. Approximately 500 children and adolescents <20 years of age are diagnosed each year in the US. The risk for developing Wilms' tumour is increased in certain congenital overgrowth syndromes, congenital non-overgrowth syndromes, and children with congenital urogenital anomalies. Long-term survival approaches 90% in localised disease.
History and exam
Key diagnostic factors
- presence of risk factors
- upper abdominal/flank mass or swelling
Other diagnostic factors
- abdominal distension
- abdominal pain
- hypoglycaemia in infancy
- poor appetite or cachexia
- shortness of breath
- bone tenderness or pain
- features of paraneoplastic syndrome
- age 2 to 5 years
- congenital urogenital anomalies
- congenital syndromes
- antenatal exposure to radiation or chemicals
- paternal occupation
1st investigations to order
- renal function
- serum total protein/albumin
- coagulation studies
- serum calcium level
- abdominal ultrasound with Doppler
- CT or MRI abdomen and pelvis with and without contrast
- CT or MRI chest with and without contrast
Investigations to consider
- genetic testing
- loss of heterozygosity (LOH) studies
first tumour recurrence without multiple adverse prognostic factors
multiple adverse prognostic factors or multiple relapses
Sandeep Batra, MD
Clinical Assistant Professor
Riley Hospital for Children
Department of Pediatrics
Indiana University School of Medicine
SB declares that he has no competing interests.
Nadine Deannie Lee, MD
Riley Children's Hospital
Indiana University Health
NDL declares that she has no competing interests.
Norbert Graf, MD
Klinik für Pädiatrische Onkologie und Hämatologie
Universitätsklinikum des Saarlandes
NG declares that he has no competing interests.
Zelig Tochner, MD
Children's Hospital of Philadelphia
ZT declares that he has no competing interests.
- Clear cell sarcoma of the kidney (CCSK)
- Renal cell carcinoma
- SIOP PODC: clinical guidelines for the management of children with Wilms tumour in a low income setting
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