Pediatrics 

> Diagnosis > Genitourinary > Renal Masses

Wilms Tumor

Hank Baskin, MD

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AP abdominal radiograph of a 3-year-old child with a firm, palpable mass shows leftward
displacement of bowel loops by a right-sided soft tissue mass . CT was performed next
in this child due to suspicion for a Wilms tumor.

Coronal CECT in the same patient confirms a large, heterogeneous, solid mass arising
from the right kidney. A residual "claw" of normal renal tissue is splayed along the upper
pole of this Wilms tumor. No contralateral lesions or venous invasion were identified.

Longitudinal US in a 5 year old with hypertension & abdominal fullness shows a round
heterogeneous mass extending out of the lower pole of the left kidney . Wilms tumor
was confirmed upon surgery.
Axial T2 (top) & T1 C+ (bottom) FS MR images show a large, well-defined, heterogeneous
mass arising from a splayed "claw" of the left kidney in a 6 year old with a Wilms
tumor. Note the presence of a T2 hypointense pseudocapsule around the tumor.

Axial CECT shows a large, poorly defined, heterogeneous Wilms tumor arising from the
left kidney in a 4-year-old girl. Note the enlarged lymph node displacing the aorta
anteriorly & to the right .

Sagittal (left) & coronal (right) CECTs show tumor thrombus extending through the left renal
vein, up the inferior vena cava, & into the right atrium from a Wilms tumor in the left
kidney. CECT has high sensitivity & specificity for cavoatrial tumor thrombus in children with
Wilms tumor.

Coronal CECT of a 3 year old with a palpable mass shows numerous round nonenhancing
lesions in both kidneys. These residual nephrogenic rests (nephroblastomatosis) will give
rise to a Wilms tumor in 30-40% of affected patients.
Axial CECT through the kidneys in the same patient shows a large, well-defined Wilms tumor
in the right kidney . The nephrogenic rests are seen as numerous, bland-appearing
cortical lesions in the periphery of both kidneys.

Coronal FDG PET/CT in a child with a history of left nephrectomy for Wilms tumor shows a
contralateral recurrence with metabolically active tumor in the right upper kidney. FDG
PET is useful in assessing chemotherapy response in children with relapsed Wilms tumor,
even when the tumor size is unchanged on other modalities.

Axial NECT through the lung apices in a 3 year old with Wilms tumor demonstrates a well-
defined, biopsy-confirmed metastatic lung nodule in the left upper lobe .

Additional Images
Axial CECT shows a low-density mass in the left flank several months after nephrectomy
for a Wilms tumor, consistent with local recurrence. Also note the numerous low-density
hepatic lesions of variable size, consistent with liver metastases.

Axial CECT shows a low-density, lobulated mass in the right kidney found to represent a
cystic Wilms tumor.

Axial CECT in lung algorithm shows round pulmonary metastases in the left lung base
from a Wilms tumor. Note the right pleural effusion & inferior vena cava thrombus in
this patient with stage IV disease.

Axial CECT shows a large, poorly enhancing Wilms tumor in the right flank replacing the right
kidney. Note the small, low-density focus in the contralateral kidney ; this small lesion
could be a precursor nephrogenic rest but proved to be a very small synchronous Wilms
tumor in this patient.
Coronal T1 MR shows a large, homogeneous mass occupying the right flank & displacing
the bowel & liver. This Wilms tumor compressed the inferior vena cava but did not exhibit
venous invasion.

Sagittal color Doppler ultrasound shows lack of color flow in the inferior vena cava in a
patient with Wilms tumor . Doppler ultrasound & MR are often used to assess venous
invasion by Wilms tumor.

Axial CECT in the same patient shows displacement of the aorta & mesenteric vessels by the
mass with low-density thrombus in the inferior vena cava.

Axial T2 FS MR shows a large, relatively homogeneous Wilms tumor extending out of the
right renal fossa, crossing the midline & displacing (rather than encasing) nearby vessels in
this case.

Coronal T2 FS MR in the same child shows whorled high signal intensity throughout the
Wilms tumor. The tumor fills the right side of the abdomen & extends across the midline.

 Terminology
o Malignant tumor of primitive metanephric blastema
o Most common abdominal tumor in children 1-8 years old
 Imaging
o Ultrasound frequently 1st study performed; CT & MR better characterize
tumor & local extent
  Large, heterogeneous but predominantly solid hypoechoic
(US)/hypoenhancing (CT/MR) renal mass
 Careful evaluation of
 Adjacent soft tissues for tumor rupture, adenopathy
 Renal vein & inferior vena cava for tumor thrombus
 Contralateral kidney for synchronous tumor or nephrogenic
rests
o Chest radiograph or CT: Lung metastases in 10-20%
 Top Differential Diagnoses
o Neuroblastoma: Extrarenal, more often calcified
o Congenital mesoblastic nephroma: < 3-12 months
o Multilocular cystic nephroma: Multiseptated cystic mass
o Clear cell sarcoma: Look for skeletal metastases
o Renal cell carcinoma: Equal incidence to Wilms after age 12
o Nephroblastomatosis: Multiple bilateral nephrogenic rests
 Pathology
o Arises from primitive metanephric blastemal tissue
 Persistence after 34 weeks gestation termed "nephroblastomatosis"
→ 30-40% develop Wilms tumor
o Associated predisposing syndromes in 10%
 Quarterly screening renal ultrasounds until age 8
 Clinical Issues
o 80% of cases in children < 5 years old
o Typical presentation: Incidentally discovered palpable mass
o Preferred treatment: Up front complete surgical resection
 Preoperative chemotherapy for unresectable or bilateral tumors or
tumor thrombus above hepatic veins
 Postoperative chemotherapy ± radiation
o > 90% 5-year-survival for localized abdominal disease

 Synonyms
o Nephroblastoma, embryoma of kidney (archaic)
 Definitions
o Malignant tumor of primitive metanephric blastema
o Most common abdominal neoplasm ages 1-8 years

 General Features
o Best diagnostic clue
 Large, heterogeneous renal mass extending into renal vein & inferior
vena cava (IVC) in young child
o Location
 > 90% unilateral, 5-10% bilateral
o Size
 Typically quite large (mean diameter: 5-10 cm)
o Morphology
 Usually spherical; sometimes lobulated or multicentric
  Usually smooth contours; may have local extension
 Radiographic Findings
o Mass displacing adjacent bowel
o Ca²⁺ visible in 10%
 CT Findings
o NECT
 Ca²⁺ in 15%
 Lung metastases in 10-20% at time of diagnosis
 Well-defined solid nodules
o CECT
 Large, poorly enhancing, heterogeneous mass replacing most of
kidney
 "Claw" of residual renal tissue along tumor margin
 Often has well-defined margins or pseudocapsule
 Displaces adjacent organs, especially bowel
 ± adjacent adenopathy
 Predilection for invasion of renal vein & IVC
 May have local extension into perirenal fat or gross tumor rupture
with distant ascites
 MR Findings
o T1WI: Typically heterogeneous, predominantly intermediate to low signal but
may have foci of high T1 signal due to blood products
o T2WI: Typically heterogeneous, predominantly high signal
o T1 C+: Often poor, heterogeneous enhancement
o MRV: Useful in determining vascular invasion
 Ultrasonographic Findings
o Grayscale ultrasound
 Findings similar to CT & MR
 Large, hypoechoic, heterogeneous mass
 May see local invasion & adenopathy
 Often difficult to image entire tumor without extended field of view
o Color Doppler
 Can be useful to detect tumor thrombus vs. compression of veins by
bulky mass
 Echocardiographic Findings
o Used to assess intracardiac tumor thrombus
 Especially when preoperative chemotherapy used to "shrink" tumor
thrombus
 Intracardiac tumor thrombus may change surgical approach,
necessitate cardiothoracic surgical input
 Nuclear Medicine Findings
o Bone scan
 Not routinely used as bone metastases occur very late
o PET
 Increasing use in Wilms (& all pediatric tumors)
 Primarily has adjunctive, problem-solving role
  Differentiates scar from active tumor post chemotherapy
 May predict response to neoadjuvant therapy
 Imaging Recommendations
o Best imaging tool
 Ultrasound often performed initially for palpable mass
 CT or MR to further characterize tumor, local extent, adenopathy
 MR better for detection of contralateral lesions
 Chest radiograph or CT for staging
o Protocol advice
 Doppler US or contrast-enhanced CT or MR venography for
assessment of ipsilateral renal vein & IVC

 Neuroblastoma
o Suprarenal (adrenal gland) or paraspinal (sympathetic chain)
 Typically displaces rather than invades kidney
o Much more likely than Wilms to contain Ca²⁺, cross midline, & engulf or "lift"
adjacent vessels
 Congenital Mesoblastic Nephroma
o Solid or mixed solid & cystic tumor of infants
 > 90% diagnosed in 1st year of life
 Most common renal tumor < 3 months of age
 Multilocular Cystic Nephroma
o Entirely cystic mass with numerous thin septations
o Classically herniates into renal pelvis
o Pediatric cases typically boys 2-3 years of age
 Renal Cell Carcinoma
o Solid renal mass typically seen in 2nd decade of life
o Incidence equal to Wilms after 12 years of age
 Renal Rhabdoid Tumor
o Nonspecific solid renal mass in young child
o May have synchronous intracranial rhabdoid tumor
 Clear Cell Sarcoma
o Solid renal mass with skeletal metastases at diagnosis
 Angiomyolipoma
o Benign fat-containing, vascular tumors with variable enhancement
o Tuberous sclerosis complex in most pediatric angiomyolipomas (AMLs)
 AMLs typically multiple & accompanied by simple cysts
 Pyelonephritis
o Developing abscess can mimic cystic neoplasm but typically more infiltrative
o Patients often have clinical & laboratory features of upper urinary tract
infection
 Renal Medullary Carcinoma
o Poorly defined, infiltrating renal mass; can mimic phlegmonous
pyelonephritis
o Lymphangitic pulmonary spread common
o Typically in adolescents with sickle cell trait or disease
 Nephroblastomatosis
o Multiple nonenhancing foci of residual primitive nephrogenic rests, usually
bilateral
o Individual lesions relatively small & homogeneous
o May be perilobar or intralobar
 Perilobar: Well-defined peripheral lesions; often solitary but may be
confluent & encase entirety of both kidneys
 Intralobar: Randomly distributed through cortex &/or medulla
o High association with bilateral Wilms tumor, Beckwith-Wiedemann
syndrome, hemihypertrophy
 Lymphoma
o May have multifocal solid bland renal masses, typically in setting of other
confluent adenopathy ± splenic disease

 General Features
o Etiology
 Primitive metanephric blastema differentiates by 34 weeks gestation
 Persistence of metanephric blastema (nephrogenic rests) termed
"nephroblastomatosis"
 Found in 1% of infant autopsies, 4% of resected multicystic
dysplastic kidney
 Wilms tumor develops in 30-44%
o Genetics
 Numerous somatic & germ line mutations described
 10-20% have 11p13 (WT1) gene mutation
 Only 2% of Wilms tumors familial
o Associated abnormalities
 Genitourinary anomalies
 Overgrowth syndromes (Beckwith-Wiedemann, isolated
hemihypertrophy)
 WAGR syndrome: Wilms tumor, aniridia, genitourinary anomalies,
mental retardation
 Contiguous gene deletion syndrome involving Wilms tumor 1
gene (WT1), paired box gene 6 (PAX6), & possibly other genes
on chromosome 11p13
 Sporadic aniridia
 Denys-Drash syndrome
 Trisomy 18
 Sotos syndrome
 Bloom syndrome
 Staging, Grading, & Classification
o Similar systems used by Children's Oncology Group (COG)/National Wilms
Tumor Study Group (NWTSG) or Sociètè Internationale d'Oncologie
Pediatrique (SIOP)
 I: Confined to kidney, completely excised
 II: Local extension, completely resected
 III: Incomplete resection, no distant metastases
  IV: Distant metastases to lung, liver, brain, or bone
 V: Bilateral synchronous tumors
 Microscopic Features
o 4-10% have unfavorable (anaplastic) histology

 Presentation
o Most common signs/symptoms
 Asymptomatic flank mass, hematuria, vomiting, failure to thrive
o Other signs/symptoms
 Hypertension, fever from tumor necrosis, anemia
 Demographics
o Age
 80% of cases occur in children < 5 years old
 Peak: 3.6 years
 Syndromic tumor occurrence typically younger
o Gender
 M=F
o Epidemiology
 500 new cases each year in United States
 1/3 of patients with sporadic aniridia have Wilms; 1% of Wilms tumor
patients have aniridia
 Natural History & Prognosis
o Prognosis based on stage, tumor size, histology
o 5-year-survival for localized abdominal disease > 90%
 Treatment
o Up front complete surgical resection (nephrectomy) preferred
 Limited uses of renal-sparing resection: Predisposition to bilateral
tumors or preexisting solitary kidney
o Preoperative chemotherapy for unresectable or bilateral tumors or tumor
thrombus extending above hepatic veins
o Postoperative chemotherapy ± radiation
o Bone marrow transplant usually reserved for relapses

 Consider
o Children with predisposing syndromes require ultrasound screening every 3
months until 8 years of age
 Image Interpretation Pearls
o Careful assessment of ipsilateral renal vein & IVC, adjacent soft tissues &
lymph nodes, contralateral kidney, & lungs
o Look for free fluid that might suggest tumor rupture

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