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March, 2006Harvard Mediical School, MS III

Gillian Lieberamn, MD

WilmsWilms Tumor: Imaging ofTumor: Imaging ofPediatric Renal MassesPediatric Renal Masses

Allison Young, HMS IIIAllison Young, HMS III

Gillian Lieberman, MDGillian Lieberman, MD

www.fraservalleymri.com/ gfx/doc4-5.jpg
http://images.google.com/imgres?imgurl=http://www.springfieldradiology.com/newtech/CTA%2520of%2520Kidneys.jpg&imgrefurl=http://www.springfieldradiology.com/technology.htm&h=402&w=576&sz=112&tbnid=M6v083BPV-rj_M:&tbnh=92&tbnw=132&hl=en&start=39&prev=/images%3Fq%3Dkidneys%26start%3D20%26svnum%3D10%26hl%3Den%26lr%3D%26sa%3DNhttp://images.google.com/imgres?imgurl=http://www.springfieldradiology.com/newtech/CTA%2520of%2520Kidneys.jpg&imgrefurl=http://www.springfieldradiology.com/technology.htm&h=402&w=576&sz=112&tbnid=M6v083BPV-rj_M:&tbnh=92&tbnw=132&hl=en&start=39&prev=/images%3Fq%3Dkidneys%26start%3D20%26svnum%3D10%26hl%3Den%26lr%3D%26sa%3DNhttp://images.google.com/imgres?imgurl=http://www.springfieldradiology.com/newtech/CTA%2520of%2520Kidneys.jpg&imgrefurl=http://www.springfieldradiology.com/technology.htm&h=402&w=576&sz=112&tbnid=M6v083BPV-rj_M:&tbnh=92&tbnw=132&hl=en&start=39&prev=/images%3Fq%3Dkidneys%26start%3D20%26svnum%3D10%26hl%3Den%26lr%3D%26sa%3DNhttp://images.google.com/imgres?imgurl=http://www.springfieldradiology.com/newtech/CTA%2520of%2520Kidneys.jpg&imgrefurl=http://www.springfieldradiology.com/technology.htm&h=402&w=576&sz=112&tbnid=M6v083BPV-rj_M:&tbnh=92&tbnw=132&hl=en&start=39&prev=/images%3Fq%3Dkidneys%26start%3D20%26svnum%3D10%26hl%3Den%26lr%3D%26sa%3DNhttp://images.google.com/imgres?imgurl=http://www.springfieldradiology.com/newtech/CTA%2520of%2520Kidneys.jpg&imgrefurl=http://www.springfieldradiology.com/technology.htm&h=402&w=576&sz=112&tbnid=M6v083BPV-rj_M:&tbnh=92&tbnw=132&hl=en&start=39&prev=/images%3Fq%3Dkidneys%26start%3D20%26svnum%3D10%26hl%3Den%26lr%3D%26sa%3DNhttp://images.google.com/imgres?imgurl=http://www.springfieldradiology.com/newtech/CTA%2520of%2520Kidneys.jpg&imgrefurl=http://www.springfieldradiology.com/technology.htm&h=402&w=576&sz=112&tbnid=M6v083BPV-rj_M:&tbnh=92&tbnw=132&hl=en&start=39&prev=/images%3Fq%3Dkidneys%26start%3D20%26svnum%3D10%26hl%3Den%26lr%3D%26sa%3DNhttp://images.google.com/imgres?imgurl=http://www.springfieldradiology.com/newtech/CTA%2520of%2520Kidneys.jpg&imgrefurl=http://www.springfieldradiology.com/technology.htm&h=402&w=576&sz=112&tbnid=M6v083BPV-rj_M:&tbnh=92&tbnw=132&hl=en&start=39&prev=/images%3Fq%3Dkidneys%26start%3D20%26svnum%3D10%26hl%3Den%26lr%3D%26sa%3DNhttp://images.google.com/imgres?imgurl=http://www.springfieldradiology.com/newtech/CTA%2520of%2520Kidneys.jpg&imgrefurl=http://www.springfieldradiology.com/technology.htm&h=402&w=576&sz=112&tbnid=M6v083BPV-rj_M:&tbnh=92&tbnw=132&hl=en&start=39&prev=/images%3Fq%3Dkidneys%26start%3D20%26svnum%3D10%26hl%3Den%26lr%3D%26sa%3DNhttp://images.google.com/imgres?imgurl=http://www.springfieldradiology.com/newtech/CTA%2520of%2520Kidneys.jpg&imgrefurl=http://www.springfieldradiology.com/technology.htm&h=402&w=576&sz=112&tbnid=M6v083BPV-rj_M:&tbnh=92&tbnw=132&hl=en&start=39&prev=/images%3Fq%3Dkidneys%26start%3D20%26svnum%3D10%26hl%3Den%26lr%3D%26sa%3DNhttp://images.google.com/imgres?imgurl=http://www.springfieldradiology.com/newtech/CTA%2520of%2520Kidneys.jpg&imgrefurl=http://www.springfieldradiology.com/technology.htm&h=402&w=576&sz=112&tbnid=M6v083BPV-rj_M:&tbnh=92&tbnw=132&hl=en&start=39&prev=/images%3Fq%3Dkidneys%26start%3D20%26svnum%3D10%26hl%3Den%26lr%3D%26sa%3DNhttp://images.google.com/imgres?imgurl=http://www.springfieldradiology.com/newtech/CTA%2520of%2520Kidneys.jpg&imgrefurl=http://www.springfieldradiology.com/technology.htm&h=402&w=576&sz=112&tbnid=M6v083BPV-rj_M:&tbnh=92&tbnw=132&hl=en&start=39&prev=/images%3Fq%3Dkidneys%26start%3D20%26svnum%3D10%26hl%3Den%26lr%3D%26sa%3DN

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Harvard Mediical School, MS III

Gillian Lieberamn, MDMarch, 2006

What isWhat is WilmsWilms Tumor?Tumor?~~defining featuresdefining features~~

First classified as anFirst classified as an embryonalembryonal sarcoma by Maxsarcoma by Max WilmsWilms inin1899 at the Institute of Pathology in Bonn, Germany.1899 at the Institute of Pathology in Bonn, Germany.

Also known asAlso known as nephroblastomanephroblastoma, it is the most common solid, it is the most common solid

renal tumor of childhood.renal tumor of childhood.

Usually bulky, may arise in any portion of the kidney, andUsually bulky, may arise in any portion of the kidney, and

expands within the renal parenchyma to displace and distortexpands within the renal parenchyma to displace and distortthethe pelvicalycealpelvicalyceal system.system.

Distinguished by vascular invasion and displacement ofDistinguished by vascular invasion and displacement ofsurrounding structures.surrounding structures.

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~~EpidemiologyEpidemiology

~~

Accounts for roughly 5% of childhood cancers andAccounts for roughly 5% of childhood cancers and

87% of pediatric renal masses.87% of pediatric renal masses. Incidence = 1:10,000. Approximately 500 new casesIncidence = 1:10,000. Approximately 500 new cases

annually.annually.

Peak incidence = 3Peak incidence = 3--4 years of age (80% present4 years of age (80% presentbefore age 5).before age 5).

55--10% present with bilateral10% present with bilateral WilmsWilms..

NationalNational WilmsWilms Tumor Study (NWTS)Tumor Study (NWTS) -- has 85% ofhas 85% ofall new cases diagnosed in North America enrolled inall new cases diagnosed in North America enrolled ingroup protocols.group protocols.

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Associated SyndromesAssociated Syndromes

~~ the minoritythe minority of casesof cases~~

WAGR SyndromeWAGR Syndrome

DrashDrash SyndromeSyndrome male pseudomale pseudo--hermaphroditismhermaphroditism,,

progressiveprogressive glomerulonephritisglomerulonephritis

Overgrowth SyndromesOvergrowth Syndromes BeckwithBeckwith WiedemannWiedemann,,

hemihypertrophyhemihypertrophy..

GU abnormalitiesGU abnormalities hypospadiashypospadias,, cryptorchidismcryptorchidism,,

horseshoe kidney.horseshoe kidney.

}}Abnl. WT 1

gene

}} Abnl. WT 2gene

Patients with associated syndromes should be screened

starting at 6 months of age, with initial CT and follow up US

every 3 months up to 7 years of age.

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Wilms is an abnormal embryonal renal neoplasm,presumed to develop from abnormal histiogenesis.

Precursor cells = renal blastemal tissue(nephrogenic rests)

Normal nephrogenesis is complete at 36 weeksgestation. Kidneys of normal full-term newbornscontain no foci of renal blastema.

Wilms is thought to arise from metanephric precursortissue that persists in the developing child.

~~ Pathogenesis of Wilms tumor ~~

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~~NephrogenesisNephrogenesis ~~ Ureteric buds penetrate metanephric tissue

that is molded around the distal ends like a

cap. Buds gives rise to collecting system

(ureter, calcyes, renal pelvis).

Epithelium of the ureteric bud from the

MESONEPHROS interacts with

mesenchyme ofMETANEPHRIC blastema.

Metanephric blastemal tissue expresses WT1

(transcription factor) which enables

metanephric tissue to respond to induction by

ureteric buds. Nephrons are formed from

metanphros through molecular signaling.

Metanephric

blastema

Beginning at week 5 of development:

Langmans Medical Embryology, 9th Ed., T.W. Sadler, PhD. Lippincott, Williams & Wilkins, 2004.

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Gross PathologyGross Pathology

Usually shows wellUsually shows well--differentiated renal tissuedifferentiated renal tissuewith embryonicwith embryonic glomeruliglomeruli and tubule formationand tubule formationsurrounded by spindle cellsurrounded by spindle cell stromastroma.. TriphasicTriphasicpattern =pattern = stromalstromal,, blastemalblastemal and tubularand tubular

elements.elements.

10% of10% ofWilmsWilms tumors have unfavorabletumors have unfavorablehistology withhistology with anaplasiaanaplasia (atypical mitoses or(atypical mitoses orhyperchromatichyperchromatic cells with large nuclei).cells with large nuclei).

Histology

*most important prognostic factor

Solid multi-lobulated, gray/tan

intrarenal mass with a pseudocapsule

distorting the renal parenchyma and

collecting system. Spreads by directextension but does not encase the aorta.

Lowe et al. Pediatric Renal Masses: Wilms Tumor and

Beyond. Radiographics. 2000 Nov-Dec;20(6):1585-603.

Stromal Elements

Blastemal elements

Tubular elements

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~~ Renal AnatomyRenal Anatomy ~~

Grays Anatomy Online

http://www.bartleby.com/107/253.html

Renal vein

Renal artery

Left kidney andsuprarenal gland in situPositions of Urinary Organs

Rohen, Johannes. Color Atlas of Anatomy, 5th Edition. 1998.

Renal pelvis

Renal calyx

Fibrous

capsule

Cortex

Medulla
http://www.bartleby.com/107/illus1122.html

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AnatomyAnatomy~Axial View ~~Axial View ~

http://radiology.med.sc.edu/%20Portalveinliver.htm

Pancreas

Kidney

Stomach

AbdominalAorta

Renal Vein

Color Atlas of Anatomy, 5th Ed., Johannes Rohen.

Lippincott, Williams & Wilkins 1998.

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Clinical Presentation ofClinical Presentation ofWilmsWilms tumortumor

Common: Patient presents with an asymptomaticCommon: Patient presents with an asymptomatic

abdominal mass noted by patient, physician or parent.abdominal mass noted by patient, physician or parent.

Uncommon: Patient can present with abdominal pain,Uncommon: Patient can present with abdominal pain,anorexia,anorexia, hematuriahematuria and hypertension due toand hypertension due to reninrenin

production by tumor.production by tumor.

Rare: Patient presents withRare: Patient presents with dysuriadysuria and renal failure.and renal failure.

* Discovered after coincidental trauma in up to 10% of cases.* Discovered after coincidental trauma in up to 10% of cases.

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Index PatientIndex Patient

KG is a 2 yearKG is a 2 year--old girl whoold girl whopresented in January 2006 with leftpresented in January 2006 with left

upper quadrant pain and a left flankupper quadrant pain and a left flankmass detected by her parents.mass detected by her parents.

She underwent CT scan directly,She underwent CT scan directly,which showed.which showed.

d dii l h l

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: 20 OP 320 30CC20 OP 320 30CC320 30CC320 30CC

ANDARDNDARD

DOB: 6/9/2DOB: 6/9/21/19/21/19/2

a very large multi-lobulated,

heterogenously enhancing mass

replacing the pole of the left

kidney. CLAW SIGN

MDCT Abdomen with Oral & Intravenous Contrast

The mass shows smooth margins. It

measured 12 x 7 cm in its largest

dimension and extended fromapproximately the iliac crest up to the

diaphragm.

The lesion itself appears to be comprised

of either multiple confluent masses, or

single large septated mass. It is exerting mass effect on the

surrounding abdominal structures pushing

the pancreatic tail and splenic vein, and

the stomach superiorly and anteriorly.

The left renal artery and vein are widelypatent.

Courtesy of Dr. Mara Barth Boston Childrens Hospital Boston

Normal enhancing renal parenchyma

Tumor mass

H d M dii l S h l MS III

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ed Pro 16 SYS#CT02_OC0ed Pro 16 SYS#CT02_OC04

OP 320 30CCOP 320 30CC30CC30CCRDRD

C: 50 Z: 1C: 50 Z: 1MODE /15:05:16MODE /15:05:16

512X512512X512

00

Acc Num: 1Acc Num: 10

002Y F002Y F 2DOB: 6DOB: 6

1/1/

Page: 7Page: 7CompresCompress

HH

FF

o 16 SYS#CT02_OC0o 16 SYS#CT02_OC0

0 30CC0 30CC

Z: 1Z: 1E /15:05:16/15:05:161212

Acc NumAcc Num

002Y002YDODO

Page:Page:CompComp

HH

FF

Coronal andCoronal and SagittalSagittal ReconstructionsReconstructions

Courtesy of Dr. Mara Barth Boston Childrens Hospital Boston

Tumor Mass

heterogeneous, claw

formation.

H d M dii l S h l MS III

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Differential diagnosisDifferential diagnosis

of a Renal Massof a Renal Mass*varies depending on clinical presentation + imaging features**varies depending on clinical presentation + imaging features*

Malignant renal massMalignant renal mass Clear Cell SarcomaClear Cell Sarcoma

Leukemia; LymphomaLeukemia; Lymphoma

Metastasis ( e.g.Metastasis ( e.g.neuroblastomaneuroblastoma))

*Renal Cell CA**Renal Cell CA*

RhabdoidRhabdoid tumor;tumor;

rhabdomyosarcomarhabdomyosarcoma

WilmsWilms tumortumor

* Far more common in older age groups.*

Har ard Mediical School MS III

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KGs differentialKGs differential

Renal Mass Clinical and imaging features

Wilms Tumor Large solid mass, often with vascular

invasion. Most common solid renal

mass of childhood. Congenital Mesoblastic Nephroma Most common solid renal mass in newborns

and infants. Tends to be a large infiltrative

mass with ill-defined margins and no capsule.

Multilocular Cystic Nephroma. Multicystic mass with little solid tissue. Septaare the only solid components distinguished

from Wilms by absence of expansile solid

masses.

Clear Cell Sarcoma Non-specific presentation. Manifests asabdominal mass. Distinguished by histology.

Commonly shows skeletal mets.

Rhabdoid Tumor Rare, highly aggressive. Imaging features can

closely resemble Wilms. Usually diagnosed in

infancy. Associated with brain malignancies.

Harvard Mediical School MS III

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Diagnostic Imaging of suspectedDiagnostic Imaging of suspected WilmsWilms Tumor:Tumor:

low performance modalitieslow performance modalities

~~Plain film, IVUPlain film, IVU~~

No longer commonly used:No longer commonly used:

Plain filmPlain film minimal contribution;minimal contribution;

may show typical changes of ribsmay show typical changes of ribs

caused by compression by a slowlycaused by compression by a slowly

growing tumor. (Modality of choicegrowing tumor. (Modality of choice

to evaluate for presence of lung mets.)to evaluate for presence of lung mets.)

*Common imaging challenge = stating the renal origin of the*Common imaging challenge = stating the renal origin of the

mass.*mass.*

Kioumehr et al .Wilms Tumor in the Adult Patient. American Journal of

Roentgenology 1989: 152 (2); 299.Courtesy of Dr. Mike Geary, Boston Childrens Hospital

IVUIVU shows distortion ofshows distortion ofthethe pyelocalicealpyelocaliceal systemsystem notnot

sufficient for diagnosis andsufficient for diagnosis and

staging and now rarelystaging and now rarely

performed.performed.


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Diagnostic Imaging of SuspectedDiagnostic Imaging of Suspected WilmsWilms Tumor:Tumor:

high performance modalitieshigh performance modalities

US, CT, MRUS, CT, MR USUS DemonstratesDemonstrates intrarenalintrarenal massmass

withwith heterogenousheterogenous echogenicityechogenicity..bursts the normal kidney with a spurbursts the normal kidney with a spurof normal parenchyma surroundingof normal parenchyma surroundingthe tumor.the tumor. Color Doppler StudyColor Doppler Study may help to bettermay help to better

define and depict tumor extent and necroticdefine and depict tumor extent and necroticarea, as well as vascular invasion.area, as well as vascular invasion.

CTCT Demonstrates heterogeneousDemonstrates heterogeneousmass with slightly lower attenuationmass with slightly lower attenuationthan normal kidney. Allows exam ofthan normal kidney. Allows exam ofcontralateralcontralateral kidney.kidney. IV contrastIV contrast mandatory. Shows nodal,mandatory. Shows nodal,

hepatic mets, and tumor extension intohepatic mets, and tumor extension intorenal vein or IVC.renal vein or IVC.

MRIMRI HeterogeneouslyHeterogeneously hypointensehypointenseon T1, hypo/on T1, hypo/isoiso. intense on T2. Most. intense on T2. Mostsensitive modality for determinationsensitive modality for determinationofofcavalcaval patentcypatentcy. [requires sedation. [requires sedation not routinely done]not routinely done]

Contrast enhanced CT & MR: Abnormal tissueappears heterogeneously less enhancing.

US (dorsolateral):Exophytic Wilms tumor

on lower pole of kidney

Axial US: Showsthrombus in IVC.

Riccabona, Michael. Imaging of renal tumours in infancy and childhood. European Radiology 2003, 13:L116-L129.


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Back to KGBack to KG

KG underwent leftKG underwent left nephroureterectomynephroureterectomy andand

adrenalectomyadrenalectomy with complete resection of tumor.with complete resection of tumor.

Pathology showed multifocalPathology showed multifocal WilmsWilms tumor withtumor withdiffusediffuse anaplasiaanaplasia, intact renal capsule, and focal, intact renal capsule, and focalinvasion of renal sinus and renal sinus vessels.invasion of renal sinus and renal sinus vessels.MultipleMultiple nephrogenicnephrogenic rests were present.rests were present. RResectedlymph nodes were negative for tumor involvement.

There was no evidence of right kidney involvementor abdominal lymph node disease in preoperativescans. Chest CT was negative for metastases.


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StagingStaging Staging of Wilms Tumor: by imaging, surgery, and pathology Stage Description

I Limited to the kidney andcompletely resectable with renalcapsule intact; renal sinus may beinflitrated but not beyond hilum.

II Tumor infiltrates beyond kidneybut completely resected.

III Residual tumor confined toabdomen and without hematogenousspread.

IV Hematogenous metastases to lung (mostcommon), bone, liver or brain.

V Bilateral renal involvement; eachside staged separately.


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KG was found to have Stage IIKG was found to have Stage II anaplasticanaplastic WilmsWilms TumorTumor*Most recent trial showed that*Most recent trial showed that stage II disease did not have

a worse prognosis with diffuse anaplasia than without.

Multifocal disease shows

hyperchromatic cells with atypical

mitoses and large nuclei. Each focus isat a slightly different stage of disease.

Courtesy of Dr. Mara Barth, Boston Childrens Hospital

Gross Pathology shows multilobular

encapsulated tumor, originating from

the kidney.


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PrognosisPrognosis

5 year survival rates, based on recent5 year survival rates, based on recentstudies:studies:

Favorable histologyFavorable histology survival approaches 90%survival approaches 90%

Most important negative prognostic factors areMost important negative prognostic factors are

unfavorableunfavorable histologichistologic subtypes.subtypes.

Recent NWTS data suggests 3Recent NWTS data suggests 3--year survivalyear survivalrates for bilateralrates for bilateral WilmsWilms tumors = 82%tumors = 82%


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,


KGKG multimulti--modal treatmentmodal treatment

*Based on data from National*Based on data from National WilmsWilms Tumor Study TrialsTumor Study Trials

Surgery: Radical nephrectomy via transabdominal incision is procedure ofchoice with biopsy of regional lymphatics and careful examination ofopposite kidney for staging and prognosis. Major emphasis placed onavoiding spillage of tumor as this increases abdominal recurrence.

Chemotherapy: Adjuvant therapy planned based on staging. Currentstudies are focusing on minimizing toxicity of therapy.

Radiation: Complicated by potential for growth disturbance and organtoxicities. Only used for patients with III or IV unless unfavorablehistology seen.

Follow-up imaging: Ultrasound

Based on the anaplastic features of her Stage II tumor, KG is currentlyundergoing chemotherapy and radiation.


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Companion CaseCompanion Case~~ Patient # 2Patient # 2 ~~

JC is a 7 year old boy, s/p resection forJC is a 7 year old boy, s/p resection for

stage IIIstage III WilmsWilms tumor who presents fortumor who presents for

restaging due to recurrence of palpablerestaging due to recurrence of palpable

abdominal mass.abdominal mass.


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JCsJCs preoperative CT Scan: dated April, 2005preoperative CT Scan: dated April, 2005

CT abdomen with contrast: showsCT abdomen with contrast: shows

heterogeneously enhancing solidheterogeneously enhancing solidmass arising from the left kidneymass arising from the left kidney

with adjacent soft tissue probablywith adjacent soft tissue probably

lymphadenopathylymphadenopathy, and thrombus, and thrombus

in the left renal vein.in the left renal vein.

Venous extension ofVenous extension ofWilmsWilms tumortumor

follows the rule of 10s: 10%follows the rule of 10s: 10%

extend into renal vein; 10% ofextend into renal vein; 10% of

that group extend into IVC; 10%that group extend into IVC; 10%

of the latter further extend into theof the latter further extend into the

right atrium.right atrium.

Thrombus inleft renal vein.Soft tissue: likely

LAD

Courtesy of Dr. Melissa Gerlach, Boston Childrens Hospital


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Restaging October 2005:Restaging October 2005: status post Wilms resection~Abdomen, Pelvis CT with contrast~

The left kidney is absent.

There is a lobulated softtissue mass in the left renalfossa and lower

retroperitoneum associatedwith multiple vascular clips.It is bilobed with a largemass at the level of the

SMA and another at thelevel of the IMA. The lowercomponent demonstratescystic degeneration in its

inferior and lateral aspects.


Superior

component

Inferior componentwith cystic

degeneration.


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RestagingRestaging~~Chest CT with contrastChest CT with contrast~~

Multiple poorly enhancingmasses seen throughout the

lungs. Largest in the anterior

aspect of the apical posterior

segment of the left upper lobe,

measures approximately 3 .0 x

1.8 cm.

Left upper mediastinaladenopathy.


Given the lung findingssuggestive of hematogenous spread,

JC likely has Stage IV disease.


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Potential diagnostic confusionPotential diagnostic confusion~~ Patient #3Patient #3 ~~

KO is a 5-year-old girl from Puerto Rico, whopresented to her physician last November witha reactive airway disease exacerbation. On

chest X-ray, an incidental calcified right upperquadrant mass was discovered.


Gilli Li b MD

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Biopsy was performed and preliminary

pathology was consistent withganglioneuroma.

Due to size of the massKO wasreferred to Boston for repeat metastatic

workup and resection of the mass.


Gilli Li b MD

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~Abdominal CT with contrast~

From OSH

Show large right-sided adrenalmass with coarse central

calcifications. Similar sized mass

was seen in the left adrenal.

Complete encasement of the

IVC and partial encasement of

the left renal vein and SMA were

noted.

Courtesy of Dr. Melissa Gerlach Boston Childrens Hospital

calcification

Crossing the

midline


Gilli Li b MD

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Final diagnosis s/p resection:Final diagnosis s/p resection:NeuroblastomaNeuroblastoma

Pathologically distinct fromPathologically distinct from WilmsWilms tumortumor but frequentlybut frequentlypresents in the abdomen as a mass arising from adrenal glandspresents in the abdomen as a mass arising from adrenal glandsoror paraspinalparaspinal ganglion.ganglion.

22ndnd most common abdominal malignancy in childrenmost common abdominal malignancy in children

occurring as frequently asoccurring as frequently as WilmsWilms.. RadiographicallyRadiographically indistinguishable fromindistinguishable from WilmsWilms tumors.tumors.

Features that aid in diagnosis:Features that aid in diagnosis: NeuroblastomasNeuroblastomas usually cross the midline whereasusually cross the midline whereas WilmsWilms is confinedis confined

to one side.to one side. NeuroblastomasNeuroblastomas may cause an outward and downward displacement ofmay cause an outward and downward displacement of

the kidney (drooping lily) whereasthe kidney (drooping lily) whereas WilmsWilms tumors aretumors are intrarenalintrarenal masses,masses,rarely causing a change in axis of the kidney.rarely causing a change in axis of the kidney.

NeuroblastomasNeuroblastomas are more likely to present with mets, and tend toare more likely to present with mets, and tend tocalcify at higher frequency. Tumor markers include VMA and othercalcify at higher frequency. Tumor markers include VMA and othercatecholaminescatecholamines..


Gillian Lieberamn MD

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WilmsWilms Tumor vs.Tumor vs. NeuroblastomaNeuroblastoma~Axial CT~~Axial CT~

Contrast-enhanced axial scan at the level

of the renal hilum: Large, well-defined tumor

seen on the right attenuates similarly to the

renal parenchyma which displaces the right

kidney (double arrow) medially.

Contrast-enhanced axial scan at the level

of the upper pole of the kidneys: Large,

irregularly-calcified tumor seen in theretroperitoneum, displacing left kidney

dorsally (double arrow).

http://www.szote.u-szeged.hu/radio/a13.htm



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ReviewReview take home pointstake home points WilmsWilms is anis an embryonalembryonal renal neoplasm accounting for greatestrenal neoplasm accounting for greatest

percentage of pediatric renal masses (peak age 3percentage of pediatric renal masses (peak age 3--4). Can be4). Can be

difficult to distinguishdifficult to distinguish radiographicallyradiographically from other renalfrom other renalmasses &masses & neuroblastomaneuroblastoma..

Diagnosis of a renal mass is made by confronting elements ofDiagnosis of a renal mass is made by confronting elements ofclinical presentation with imagingclinical presentation with imaging definitive diagnosis oftendefinitive diagnosis often

awaits path.awaits path. Steps in diagnosis: radiologist localizes mass, analyses tumorSteps in diagnosis: radiologist localizes mass, analyses tumor

features, searches for regional/distant spread.features, searches for regional/distant spread.

High performance imaging modalities commonly used:High performance imaging modalities commonly used: USUS

CTCT

MRMR



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ReviewReview imaging ofimaging ofWilmsWilms

USUS 11stst imaging test forimaging test for WilmsWilms tumortumor excellent forexcellent fordepicting the mass, identifying adjacent organ invasion anddepicting the mass, identifying adjacent organ invasion andtumor thrombus extension in renal vein and IVC.tumor thrombus extension in renal vein and IVC.

CT (contrast & nonCT (contrast & non--contrast)contrast) Preferred modality for furtherPreferred modality for furtherstaging and crossstaging and cross--sectional imagingsectional imaging enablesenables evaleval. of lung. of lung

mets and view of both kidneys. Controversy surrounding usemets and view of both kidneys. Controversy surrounding useof lung CT vs. plain film for mets. at initial diagnosis.of lung CT vs. plain film for mets. at initial diagnosis.

MRMR Similar imaging benefit to CT. Most sensitive modalitySimilar imaging benefit to CT. Most sensitive modalityfor determination offor determination ofcavalcaval patentcypatentcy, but requires sedation., but requires sedation.


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AcknowledgementsAcknowledgementsMany thanks toMany thanks to

MelissaMelissa GerlachGerlach, MD, MD

Mara Barth, MDMara Barth, MD

Mike Geary, MDMike Geary, MD

Edward Lee, MDEdward Lee, MD

Gillian Lieberman, MDGillian Lieberman, MD

Pamela LepkowskiPamela Lepkowski

Larry Barbaras our WebmasterLarry Barbaras our Webmaster


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ReferencesReferences Kirks, Donald R, Editor. Practical Pediatric Imaging:Kirks, Donald R, Editor. Practical Pediatric Imaging: DiagnositcDiagnositc Radiology of Infants andRadiology of Infants and

Children, 3Children, 3rdrd Edition. Lippincott Williams & Wilkins 1998; 1111Edition. Lippincott Williams & Wilkins 1998; 1111-- 1126.1126.

KioumehrKioumehr,, FarhadFarhad et. al.et. al. WilmsWilms Tumor (Tumor (NehproblastomaNehproblastoma) in the Adult Patient: Clinical and) in the Adult Patient: Clinical andRadiologic Manifestations. American Journal ofRadiologic Manifestations. American Journal ofRoentgenologyRoentgenology 1989; 152(2):299.1989; 152(2):299.

Lowe, Lisa H. et. al. Pediatric Renal Masses:Lowe, Lisa H. et. al. Pediatric Renal Masses: WilmsWilms Tumor and Beyond.Tumor and Beyond. RadioGraphicsRadioGraphics2000; 20: 15852000; 20: 1585--1603.1603.

Reeder, Maurice.Reeder, Maurice. GamutsGamuts In Radiology (Reeder &In Radiology (Reeder & FelsonsFelsons) Comprehensive List of Roentgen) Comprehensive List of Roentgen

Differential Diagnoses, 4Differential Diagnoses, 4thth Ed. 2003.Ed. 2003. RiccabonaRiccabona, M. Imaging of renal, M. Imaging of renal tumourstumours in infancy and childhood. European Radiology 2003;in infancy and childhood. European Radiology 2003;

13:L11613:L116--L129.L129.

RohanRohan, Johannes. Color Atlas of Anatomy: A Photographic Study of the, Johannes. Color Atlas of Anatomy: A Photographic Study of the Human Body, 5Human Body, 5thth

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Sadler, T.W.Sadler, T.W. LangmansLangmans Medical Embryology, 9Medical Embryology, 9

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TanaghoTanagho, Emil and Jack, Emil and JackMcAnichMcAnich. Smiths General Urology, 16. Smiths General Urology, 16thth Edition. Lange 2004; 358Edition. Lange 2004; 358--362.362.

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