History and exam

Key diagnostic factors

common

presence of risk factors

Key risk factors include age <5 years, family history of Wilms' tumour, presence of congenital overgrowth or non-overgrowth syndromes and congenital urogenital anomalies.

upper abdominal/flank mass or swelling

Most commonly presents with an asymptomatic abdominal mass.[4]

Usually painless, non-tender, firm, smooth, and unilateral.

Retroperitoneal ('ballotable'), and does not move with respirations.[4]

Bilateral tumours occur in around 5% to 10% of patients.[4][6]

Other diagnostic factors

common

abdominal distension

Due to rapidly growing tumour.

abdominal pain

A minority of children present with symptoms. Localised or diffuse abdominal pain may be due to intra-abdominal spread and/or tumour rupture.

hypertension

Present in approximately 25% of patients and is secondary to compression of renal vasculature or renin hypersecretion.[5][47]​​

uncommon

haematuria

Occurs in approximately 25% of patients; most commonly, non-visible haematuria.[5]​[48]​

Visible haematuria is less common and may be suggestive of ureteric involvement.[60]

poor appetite, weight loss, and/or fever

Constitutional symptoms such as fever, anorexia, and weight loss occur in approximately 20% of patients with Wilms' tumour.[4]​[48]

Fever may result from release of pyrogens from the tumour, host immune response, or due to bacterial infection in the lung or urinary tract.

pallor

Due to anaemia, possibly secondary to tumour rupture and resulting haemorrhage.[4]

shortness of breath

May occur due to lung metastasis, abdominal compression on the diaphragm, or severe anaemia.

hepatomegaly

Due to metastasis in the liver.[4]

varicocele

Due to inferior vena cava or renal vein involvement by tumour thrombus.[46]

hypoglycaemia in infancy

Patients with Beckwith-Wiedemann syndrome may have transient or persistent hypoglycaemia in infancy due to a hyperinsulinaemic state.[45]

features of paraneoplastic syndrome

Rarely, children may present with a paraneoplastic syndrome that affects the central and peripheral nervous system (e.g., generalised weakness, fatigue, ptosis, hypokinesis, dysarthria, urinary retention, facial diplegia, ophthalmoplegia, and autonomic dysfunction).[10]

Risk factors

strong

age <5 years

Most commonly occurs in the first 5 years of life (median age at diagnosis is 4 years).[2][3]

congenital urogenital anomalies

Children with congenital urogenital anomalies such as hypospadias, atypical genitalia, fused (horseshoe) kidney, or cryptorchidism are predisposed to developing Wilms' tumour.[4][20][21][22]

congenital syndromes

The risk for developing Wilms' tumour is increased among children with certain congenital syndromes.[12]

These include overgrowth syndromes, such as Beckwith-Wiedemann syndrome, Perlman syndrome, and Simpson-Golabi-Behmel syndrome, and other congenital syndromes, such as Denys-Drash syndrome and WAGR (Wilms' tumour, aniridia, genitourinary abnormalities, range of developmental delays) syndrome.[4][17]

weak

family history of Wilms' tumour

Approximately 1% to 2% of patients have a positive family history of Wilms' tumour.[12][17][35]​​​[36]

antenatal exposure to harmful environmental factors

Parental pesticide exposure during the preconception or pregnancy period may increase the risk for Wilms' tumour.[23][24]

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