imaging of renal neoplasms in infants and children
DESCRIPTION
Imaging of Renal Neoplasms in Infants and Children. Jason Coryell, MS4 March 31, 2006. Guiding principles. Ultrasound is a standard first-line modality ; CT and MRI often are used to delineate extent of disease - PowerPoint PPT PresentationTRANSCRIPT
Imaging of Renal Imaging of Renal Neoplasms in Infants Neoplasms in Infants
and Childrenand ChildrenJason Coryell, MS4Jason Coryell, MS4
March 31, 2006March 31, 2006
Guiding principlesGuiding principles Ultrasound is a standard first-line modalityUltrasound is a standard first-line modality; ;
CT and MRI often are used to delineate extent CT and MRI often are used to delineate extent of diseaseof disease
Different tumor types share radiographic Different tumor types share radiographic features; features; histologic diagnosis is the gold histologic diagnosis is the gold standardstandard
Trends based on age, distribution of metastases, Trends based on age, distribution of metastases, and appearance aid radiographic differentiationand appearance aid radiographic differentiation
Categorical approaches include age (infancy vs Categorical approaches include age (infancy vs childhood), severity (benign vs malignant), and childhood), severity (benign vs malignant), and structure (solid vs cystic)structure (solid vs cystic)
Distribution by ageDistribution by age InfancyInfancy
NephroblastomatosNephroblastomatosisis
Mesoblastic Mesoblastic nephromanephroma
Wilms' (less Wilms' (less frequent)frequent)
RhabdoidRhabdoid Ossifying renal Ossifying renal
tumor of infancytumor of infancy
ChildhoodChildhood Wilms'Wilms' Clear cell sarcomaClear cell sarcoma Renal cell Renal cell
carcinomacarcinoma Multilocular cystic Multilocular cystic
nephromanephroma AngiomyolipomaAngiomyolipoma Lymphoma (NHL)Lymphoma (NHL) Renal medullary Renal medullary
carcinomacarcinoma Rhabdomyosarcoma Rhabdomyosarcoma
(pelvis)(pelvis)
Distribution by severityDistribution by severity
BenignBenign NephroblastomatosNephroblastomatos
isis Mesoblastic Mesoblastic
nephromanephroma Multilocular cystic Multilocular cystic
nephromanephroma AngiomyolipomaAngiomyolipoma Ossifying renal Ossifying renal
tumor of infancytumor of infancy
MalignantMalignant Wilms'Wilms' Clear cell sarcomaClear cell sarcoma Renal cell Renal cell
carcinomacarcinoma RhabdoidRhabdoid LymphomaLymphoma Renal medullary Renal medullary
carcinomacarcinoma RhabdomyosarcomaRhabdomyosarcoma
Wilms' tumorWilms' tumor
a) US demonstrates exophytic tumor of lower pole
c) Contrast enhanced CT-well-circumscribed, lobulated, heterogeneously enhancing exophytic mass extending from superior pole of L kidney
d) CT Coronal reconstruction in same patient
e) MRI of same patient (Wilms' is variably hyperintense on T2 images)
Epidemiology of Wilms' Epidemiology of Wilms' tumortumor
Account for 90% of pediatric renal tumorsAccount for 90% of pediatric renal tumors Peak incidence = 3 years (<1-10)Peak incidence = 3 years (<1-10) Most often sporadic and unilateralMost often sporadic and unilateral
1% familial (16% of familial cases are 1% familial (16% of familial cases are bilateral)bilateral)
10% bilateral (2/3 synchronus, 1/3 10% bilateral (2/3 synchronus, 1/3 metachronus)metachronus)
Increased risk for Wilms' with persistence of Increased risk for Wilms' with persistence of embryonic renal tissue (nephrogenic rests = embryonic renal tissue (nephrogenic rests = nephroblastomas)nephroblastomas)
Wilms' tumor Wilms' tumor associationsassociations
Molecular Associations-2 known tumor suppressor genes on Molecular Associations-2 known tumor suppressor genes on chromosome 11chromosome 11 WT-1-assoc w/ WAGR (Wilms, aniridia, GU anomalies, retardation)WT-1-assoc w/ WAGR (Wilms, aniridia, GU anomalies, retardation) WT-2-loss of heterozygosity associated with Beckwith-WiedemannWT-2-loss of heterozygosity associated with Beckwith-Wiedemann
Associated conditions: Associated conditions: Beckwith-Wiedemann (omphalocele, gigantism, macroglossia)Beckwith-Wiedemann (omphalocele, gigantism, macroglossia) hemihypertrophy, hemihypertrophy, Denys-Drash (pseudohermaphroditism, glomerulonephritis)Denys-Drash (pseudohermaphroditism, glomerulonephritis) sporadic aniridia (1/3 develop Wilms')sporadic aniridia (1/3 develop Wilms') Sotos (cerebral gigantism),Sotos (cerebral gigantism), Bloom syndrome (facial telangiectasias, GI tumors)Bloom syndrome (facial telangiectasias, GI tumors) Perlman syndrome (visceromegaly, gigantism, cryptorchidism)Perlman syndrome (visceromegaly, gigantism, cryptorchidism)
Recommended US screening every 3-6 months for first 7 years; Recommended US screening every 3-6 months for first 7 years; however, of uncertain benefit in overall survivalhowever, of uncertain benefit in overall survival
15% of WT pts have other GU anomalies (cryptorchidism, 15% of WT pts have other GU anomalies (cryptorchidism, horseshoe kidney) horseshoe kidney)
Wilms' tumor Wilms' tumor morphologymorphology
Large, spherical masses (avg Large, spherical masses (avg 12 cm at dx) with 12 cm at dx) with pseudocapsule of pseudocapsule of compressed parenchymacompressed parenchyma
Heterogeneous density Heterogeneous density (hemorrhage, fat, necrosis, (hemorrhage, fat, necrosis, and/or calcification)and/or calcification)
US is reliable for identifying US is reliable for identifying tumor extension into renal tumor extension into renal vein or IVC (4-10% of cases)vein or IVC (4-10% of cases)
CT or MRI to delineate CT or MRI to delineate tumor extent; tumor tumor extent; tumor enhances to lesser extent enhances to lesser extent than surrounding than surrounding parenchymaparenchyma
CXR or CT to identify CXR or CT to identify pulmonary metastasespulmonary metastases
Wilms' in L kidney of 4 year old with hepatic mets
Tumor thrombus of portal v. in same patient
Wilms' tumor stagingWilms' tumor staging Stage I-confined to kidney without capsular or Stage I-confined to kidney without capsular or
IVC involvement; completely resectable with IVC involvement; completely resectable with capsule intactcapsule intact
Stage II-tumor extends beyond renal capsule Stage II-tumor extends beyond renal capsule or infiltrates vessels; resected tumor with or infiltrates vessels; resected tumor with confined local spillageconfined local spillage
Stage III-residual tumor confined to abdomen, Stage III-residual tumor confined to abdomen, including positive abdominal nodes, positive including positive abdominal nodes, positive surgical margins, diffuse peritoneal surgical margins, diffuse peritoneal contamination, or residual nonresected tumorcontamination, or residual nonresected tumor
Stage IV-hematogenous spread (lungs, nodes, Stage IV-hematogenous spread (lungs, nodes, liver)liver)
Stage V-bilateral diseaseStage V-bilateral disease
Don’t confuse Wilms' with Don’t confuse Wilms' with neuroblastoma!!neuroblastoma!!
Neuroblastoma-arises from anywhere along Neuroblastoma-arises from anywhere along sympathetic chain or adrenal medullasympathetic chain or adrenal medulla
Most common cancer of infancy and most Most common cancer of infancy and most common extracranial solid tumor of common extracranial solid tumor of childhood (90% dx <5 years; median age at childhood (90% dx <5 years; median age at dx 2 years)dx 2 years)
65% are intra-abdominal and they may 65% are intra-abdominal and they may invade renal tissueinvade renal tissue
Determining adrenal vs renal origin is Determining adrenal vs renal origin is difficult when there is no clear plane of difficult when there is no clear plane of separationseparation
Wilms' tumor vs Wilms' tumor vs NeuroblastomaNeuroblastoma
Wilms'Wilms' <10% are calcified; <10% are calcified;
more often a more often a curvilinear patterncurvilinear pattern
Occasional local para-Occasional local para-aortic adenopathy (less aortic adenopathy (less common than with common than with neuroblastoma)neuroblastoma)
IVC invasion has high IVC invasion has high positive predictive positive predictive valuevalue
Mets to lungs commonMets to lungs common
NeuroblastomaNeuroblastoma Often calcified; scattered Often calcified; scattered
pattern throughout masspattern throughout mass Large regional adenopathyLarge regional adenopathy High predictive value: High predictive value:
encasement of great vessels, encasement of great vessels, spinal canal invasion, spinal canal invasion, paravertebral massparavertebral mass
Moderate predictive value: Moderate predictive value: extension across midline, extension across midline, displacement of great displacement of great vesselsvessels
Mets to liver, bone commonMets to liver, bone common Elevated urine Elevated urine
catecholaminescatecholamines
Which is it?Which is it?
NeuroblastomaNeuroblastoma
This contrast-enhanced CT shows a large retroperitoneal mass adjacent to the left kidney with scattered hyperintense signal (calcification). The tumor encases the descending aorta.
Wilms' tumor and neuroblastoma account for the majority of abdominal masses.
The remainder of the slides will provide an overview of the less common renal neoplasms.
They will be addressed in two groups: benign and malignant neoplasms
Benign renal neoplasmsBenign renal neoplasms
NephroblastomatosNephroblastomatosisis
Mesoblastic Mesoblastic nephromanephroma
Multilocular cystic Multilocular cystic nephromanephroma
AngiomyolipomaAngiomyolipoma Ossifying renal Ossifying renal
tumor of infancytumor of infancy Hyperplastic perilobar nephroblastomatosis
NephroblastomatosisNephroblastomatosis Diffuse or multifocalDiffuse or multifocal kidney kidney
involvement of metanephric rests involvement of metanephric rests (embryonic renal tissue persisting (embryonic renal tissue persisting beyond 36 weeks)beyond 36 weeks)
Homogenous subcapsular Homogenous subcapsular hypodense regionshypodense regions on contrast on contrast CTCT
Increased risk for Wilms' tumor. Increased risk for Wilms' tumor. Present in:Present in: 41% of unilateral casesof WT41% of unilateral casesof WT 94% of metachronus WT94% of metachronus WT 99% of synchronus bilateral WT99% of synchronus bilateral WT
Similar associations as with Wilms' Similar associations as with Wilms' Tumor (e.g. Beckwith-Wiedemann)Tumor (e.g. Beckwith-Wiedemann)
Treatment is controversial as 99% Treatment is controversial as 99% resolve spontaneously ; serial resolve spontaneously ; serial screening recommended with screening recommended with assoc. syndromesassoc. syndromes
a) US of a) US of nephroblastomatosis (plus nephroblastomatosis (plus symbols)symbols)d) L kidney shows diffuse d) L kidney shows diffuse metanephric rests, while R metanephric rests, while R kidney shows 3 focal lesionskidney shows 3 focal lesions
Mesoblastic nephromaMesoblastic nephroma Most common renal neoplasm Most common renal neoplasm
in in 11stst three months three months Solid, uniform mass with Solid, uniform mass with
possible hypoechoic areas from possible hypoechoic areas from cystic change or necrosis, cystic change or necrosis, thereby making it difficult to thereby making it difficult to distinguish from Wilms' on US distinguish from Wilms' on US (or CT) (or CT)
Has ill-defined margins and no Has ill-defined margins and no capsule, thus needs wide capsule, thus needs wide surgical marginssurgical margins
Does not invade vascular Does not invade vascular pedicle or metastasizepedicle or metastasize
US-large, solid mass with low US-large, solid mass with low echogenicityechogenicity
CT-soft tissue attenuation; CT-soft tissue attenuation; enhances less than surrounding enhances less than surrounding parenchyma after contrastparenchyma after contrast
Prenatally diagnosed destructive renal mass; rim of capsule-like parenchyma; dx made after nephrectomy
CT w/ contrast in same patient; rim of renal parenchyma ventromedially; well-defined mass surrounded by fat
Multilocular cystic Multilocular cystic nephromanephroma
Tumor contains Tumor contains cystic cystic lesionslesions lined by epithelial lined by epithelial cells and fibrous septae cells and fibrous septae (septae are only solid (septae are only solid component of tumor)component of tumor)
Lining may contain Lining may contain blastema cells (cystic blastema cells (cystic partially differentiated partially differentiated nephroblastoma), which nephroblastoma), which may have malignant may have malignant potential. This can only be potential. This can only be discerned histologically.discerned histologically.
Bimodal distribution (Bimodal distribution (boys boys < 4< 4 yearsyears; women 40-60 ; women 40-60 years)years)
CT w/ contrast demonstrating cyst (arrow) within a cystic partially differentiated nephroblastoma
AngiomyolipomaAngiomyolipoma Rare in children, unless pt has Rare in children, unless pt has
tuberous sclerosis tuberous sclerosis 80% of TS patients have renal 80% of TS patients have renal
angiomyolipomas by age 10 angiomyolipomas by age 10 yearsyears
Hamartomatous lesions Hamartomatous lesions comprised of fat, smooth muscle, comprised of fat, smooth muscle, and blood vesselsand blood vessels
In TS, small, bilateral, multifocal In TS, small, bilateral, multifocal nodules, typically corticalnodules, typically cortical
Tendency towards Tendency towards aneurysm aneurysm formationformation with with spontaneous spontaneous bleedingbleeding in aneurysms >4 cm; in aneurysms >4 cm; treatment only indicated to treatment only indicated to manage hemorrhagemanage hemorrhage
US-highly echogenic, non-US-highly echogenic, non-shadowing foci correlating with shadowing foci correlating with fat contentfat content
CT w/contrast diagnostic if high CT w/contrast diagnostic if high fat content; otherwise, fat content; otherwise, unenhanced may be necessaryunenhanced may be necessary
Acute hemorrhage of angiomyolipoma on contrast CT
Selective digital subtraction angiography; increased irregular vascularity in area of angiomyolipoma (arrow); leaked contrast defines area of hemorrhage in pole
Ossifying renal tumor of Ossifying renal tumor of infancyinfancy
Rare tumor, Rare tumor, diagnosed in diagnosed in 11stst year year
CalcifiedCalcified renal renal hamartomashamartomas
Soft tissue mass Soft tissue mass arising from medulla, arising from medulla, occupies part of occupies part of collecting ductcollecting duct
Can cause sx of Can cause sx of obstruction, obstruction, hematuriahematuria
Malignant renal Malignant renal neoplasmsneoplasms
Wilms'Wilms' Clear cell sarcomaClear cell sarcoma Renal cell Renal cell
carcinomacarcinoma RhabdoidRhabdoid LymphomaLymphoma Renal medullary Renal medullary
carcinomacarcinoma RhabdomyosarcomRhabdomyosarcom
aa
Gross examination of Wilms' tumor exhibiting extension into the renal pelvis
Clear cell sarcomaClear cell sarcoma 4% of pediatric renal 4% of pediatric renal
neoplasmsneoplasms Age distribution similar to Age distribution similar to
Wilms' tumor (peak 3-5 Wilms' tumor (peak 3-5 years)years)
No features that are No features that are radiologically distinct radiologically distinct from WT (solid mass from WT (solid mass compressing, distorting compressing, distorting surrounding parenchyma surrounding parenchyma +/- cystic areas and +/- cystic areas and necrosisnecrosis
20% have bony 20% have bony metastases (bone scan metastases (bone scan recommended)recommended)
Mets also to lung, brain, Mets also to lung, brain, liverliver
CT in 13 month old with right renal mass showing extensive central necrosis (arrow) and hydronephrosis of adjacent calices (arrowheads)
Renal cell carcinomaRenal cell carcinoma Mean age in children is 9 Mean age in children is 9
yearsyears 1% of pediatric renal tumors1% of pediatric renal tumors WT is 30 times more WT is 30 times more
frequent in children overall; frequent in children overall; however, WT incidence is however, WT incidence is equal to RCC in 2equal to RCC in 2ndnd decade decade
More frequent with More frequent with von von Hippel-LindauHippel-Lindau
Generally Generally smaller than WTsmaller than WT at presentation; otherwise at presentation; otherwise radiographically indistinct radiographically indistinct from WTfrom WT
25% with calcifications 25% with calcifications (<10% in WT)(<10% in WT)
Lumbar aortic adenopathy Lumbar aortic adenopathy commoncommon
Coronal T1 MRI of renal cell carcinoma
Gadolinium enhanced MRI
RhabdoidRhabdoid 2-3% of pediatric renal 2-3% of pediatric renal
neoplasmsneoplasms Occur in 1Occur in 1stst year of life year of life High risk for synchronus High risk for synchronus
or metachronus posterior or metachronus posterior fossa tumors (esp. PNET)fossa tumors (esp. PNET)
Radiographically Radiographically indistinguishable from WTindistinguishable from WT
Crescent sign-Crescent sign-peripheral peripheral crescentic fluid in crescentic fluid in subcapsular or subcapsular or perinephric space on CT perinephric space on CT (not a high positive (not a high positive predictive value)predictive value)
Renal vein infiltration Renal vein infiltration commoncommon
Gross appearance of rhabdoid-round, lobulated mass
CT with contrast shows heterogeneously enhancing mass of L kidney
LymphomaLymphoma Non-Hodgkin’s lymphoma (esp. Non-Hodgkin’s lymphoma (esp.
Burkitt’s) commonly has renal Burkitt’s) commonly has renal involvement involvement 8-12% seen on CT8-12% seen on CT 64% on autopsy 64% on autopsy
Usually Usually bilateralbilateral dz--spread dz--spread hematogenously or by direct hematogenously or by direct extensionextension
Common appearances:Common appearances: Multiple parenchymal Multiple parenchymal
masses/nodules that distort the renal masses/nodules that distort the renal contour and displace the collecting contour and displace the collecting systemsystem
Invasion from retroperitoneal mass or Invasion from retroperitoneal mass or adjacent lymph nodesadjacent lymph nodes
CT-homogenous, hypodense on both CT-homogenous, hypodense on both enhanced and unehancedenhanced and unehanced
Multifocal, nodular dz-may appear Multifocal, nodular dz-may appear like multiple renal cystslike multiple renal cysts
Diffuse dz-general renal enlargementDiffuse dz-general renal enlargement Associated Associated splenomegaly, splenomegaly,
abdominal adenopathyabdominal adenopathy, elevated , elevated LDHLDH
Gadolinium enhanced MRI of patient shows lymphomatous infiltration of the lower pole in both the coronal (b) and transverse (c) image; coronal also shows small focus in upper pole
Renal medullary Renal medullary carcinomacarcinoma
Aggressive tumorAggressive tumor Quickly fills renal pelvisQuickly fills renal pelvis Invades vessels, lymphaticsInvades vessels, lymphatics Unfavorable prognosisUnfavorable prognosis
Most common in Most common in adolescents, young adolescents, young adults withadults with sickle cell sickle cell traittrait (SA, SC); no increased (SA, SC); no increased prevalence in SS diseaseprevalence in SS disease
Centrally located, Centrally located, infiltrative lesion invading infiltrative lesion invading renal sinusrenal sinus Peripheal caliectasisPeripheal caliectasis Reniform enlargementReniform enlargement
Adjacent adenopathy Adjacent adenopathy commoncommon
US demonstrating lack of corticomedullary differentiation; hydronephrosis in calices
CT with contrast shows heterogeneous, infiltrating mass (curved areas); hypodense areas reflecting hydronephrosis; extenseive paravertebral and para-aortic adenopathy (straight arrows)
RhabdomyosarcomaRhabdomyosarcoma Most common pediatric Most common pediatric
malignancy of the malignancy of the pelvispelvis GU tract is the 2GU tract is the 2ndnd most most
common site after head & common site after head & neckneck
MRI preferred over CTMRI preferred over CT MRI, CT both show MRI, CT both show
excellent contrast excellent contrast enhancementenhancement
Solid mass of muscle Solid mass of muscle density with areas of density with areas of necrosisnecrosis
Botyroid subtype accounts Botyroid subtype accounts for 5% (grape-like, polypoid for 5% (grape-like, polypoid mass most common in mass most common in vagina)vagina)
Pelvic rhabdomyosarcoma
BibliographyBibliographyGrainger & Allison’s Diagnostic Radiology: A Textbook of Grainger & Allison’s Diagnostic Radiology: A Textbook of
Medical Imaging, 4Medical Imaging, 4thth ed. Churchill Livingstone, 2001; ed. Churchill Livingstone, 2001; pp. 1757-1762. pp. 1757-1762.
Golden CB, Feusner JH. Malignant abdominal masses in Golden CB, Feusner JH. Malignant abdominal masses in children: quick guide to evaluation and diagnosis. children: quick guide to evaluation and diagnosis. Pediatr Clin N AmPediatr Clin N Am, 2002; 49:1369-1392. , 2002; 49:1369-1392.
Lowe LH, Hernanz-Schulman M et al. Pediatric Renal Lowe LH, Hernanz-Schulman M et al. Pediatric Renal Masses: Wilms Tumor and Beyond. Masses: Wilms Tumor and Beyond. RadioGraphicsRadioGraphics, , 2000; 20: 1585-1603.2000; 20: 1585-1603.
Owens CM, Dicks-Mireaux C et al. Role of Chest Owens CM, Dicks-Mireaux C et al. Role of Chest Computed Tomography at Diagnosis in the Computed Tomography at Diagnosis in the Management of Wilms’ Tumor: A Study by the United Management of Wilms’ Tumor: A Study by the United Kingdom Children’s Cancer Study Group. Kingdom Children’s Cancer Study Group. J Clin OncJ Clin Onc, , 2002; 20: 2768-2773. 2002; 20: 2768-2773.
Riccabona M. Imaging of Renal Tumours in Infancy and Riccabona M. Imaging of Renal Tumours in Infancy and Childhood. Childhood. Eur RadiolEur Radiol, 2003; 13:L116-L129., 2003; 13:L116-L129.
Scott DJ, Wallace WHB, Hendry GMA. With Advances in Scott DJ, Wallace WHB, Hendry GMA. With Advances in Medical Imaging Can the Radiologist Reliably Diagnose Medical Imaging Can the Radiologist Reliably Diagnose Wilms’ Tumours? Wilms’ Tumours? Clinical RadiologyClinical Radiology, 1999; 54:321-327. , 1999; 54:321-327.
Images obtained from above sources as well as Google Images