History and exam

Key diagnostic factors

common

upper abdominal/flank mass or swelling

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

Usually painless, nontender, firm, smooth, and unilateral.

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

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

congenital urogenital anomalies

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

congenital syndromes

The risk for developing Wilms tumor 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 tumor, aniridia, genitourinary abnormalities, range of developmental delays) syndrome.[4][17]

Other diagnostic factors

common

abdominal distension

Due to rapidly growing tumor.

abdominal pain

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

hypertension

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

uncommon

hematuria

Occurs in approximately 25% of patients; most commonly, nonvisible hematuria.[5]​[48]​

Visible hematuria 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 tumor.[4][48]

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

pallor

Due to anemia, possibly secondary to tumor rupture and resulting hemorrhage.[4]

shortness of breath

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

hepatomegaly

Due to metastasis in the liver.[4]

varicocele

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

family history of Wilms tumor

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

hypoglycemia in infancy

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

features of paraneoplastic syndrome

Rarely, children may present with a paraneoplastic syndrome that affects the central and peripheral nervous system (e.g., generalized 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 tumor.[4][20][21][22]

congenital syndromes

The risk for developing Wilms tumor 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 tumor, aniridia, genitourinary abnormalities, range of developmental delays) syndrome.[4][17]

weak

family history of Wilms tumor

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

prenatal exposure to harmful environmental factors

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

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