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.[2]

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

Retroperitoneal ('ballotable'), and does not move with respirations.[3]

Bilateral tumours occur in around 10% of patients.[3][5]

Other diagnostic factors

common

abdominal distension

Due to rapidly growing tumour.

abdominal pain

Approximately 20% to 30% of children present with symptoms.[2] Localised or diffuse abdominal pain may be due to intra-abdominal spread and/or tumour rupture.[3]

hypertension

Present in approximately 25% of patients and is secondary to compression of renal vasculature or renin hypersecretion.[4][37]​​

uncommon

haematuria

Visible haematuria occurs in about 18% of children and non-visible haematuria in 24% of patients.[17]

poor appetite or weight loss

Constitutional symptoms such as fever, anorexia, and weight loss occur in 10% of patients with Wilms' tumour.[17]

fever

Constitutional symptoms such as fever, anorexia, and weight loss occur in 10% of patients with Wilms' tumour.[17] 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 secondary to tumour rupture and haemorrhage blood loss from haematuria.[3][17]

shortness of breath

May occur due to lung metastasis or severe anaemia.

hepatomegaly

Due to metastasis in the liver.[3]

varicocele

Due to inferior vena cava or renal vein involvement by tumour thrombus.[36]

hypoglycaemia in infancy

Patients with Beckwith-Wiedemann syndrome may have transient or persistent hypoglycaemia in infancy due to a hyperinsulinaemic state.[35]

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).[9]

Risk factors

strong

age <5 years

Most commonly occurs in the first 5 years of life.[2]

congenital urogenital anomalies

Children with congenital urogenital anomalies such as hypospadias, atypical genitalia, fused (horseshoe) kidney, or cryptorchidism are also predisposed to developing Wilms' tumour.[16][29][30]

congenital syndromes

The risk for developing Wilms' tumour is increased among children with certain congenital overgrowth syndromes such as Perlman's syndrome, Beckwith-Wiedemann syndrome, and Simpson-Golabi-Behmel syndrome, and also among children with certain congenital non-overgrowth syndromes, such as Denys-Drash syndrome and WAGR (Wilms' tumour, aniridia, genitourinary abnormalities, range of developmental delays) syndrome.[3][17]

weak

family history of Wilms' tumour

Approximately 2% of patients have a positive family history of Wilms' tumour.[11]

antenatal exposure to harmful environmental factors

Studies have investigated a potential link between Wilms' tumour and antenatal exposure to harmful environmental factors or maternal lifestyle risk factors; most studies are inconclusive or require further investigation. However, one meta-analysis of case-control studies identified a link between parental pesticide exposure during the preconception or pregnancy period and an increased risk for Wilms' tumour.[19]

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