Imaging of Renal Neoplasms in Infants and Children

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Imaging of Renal Neoplasms in Infants and Children

  1. 1. Imaging of Renal NeoplasmsImaging of Renal Neoplasms in Infants and Childrenin Infants and Children Jason Coryell, MS4Jason Coryell, MS4 March 31, 2006March 31, 2006
  2. 2. Guiding principlesGuiding principles  Ultrasound is a standard first-line modalityUltrasound is a standard first-line modality; CT and; CT and MRI often are used to delineate extent of diseaseMRI often are used to delineate extent of disease  Different tumor types share radiographic features;Different tumor types share radiographic features; histologic diagnosis is the gold standardhistologic diagnosis is the gold standard  Trends based on age, distribution of metastases, andTrends based on age, distribution of metastases, and appearance aid radiographic differentiationappearance aid radiographic differentiation  Categorical approaches include age (infancy vsCategorical approaches include age (infancy vs childhood), severity (benign vs malignant), andchildhood), severity (benign vs malignant), and structure (solid vs cystic)structure (solid vs cystic)
  3. 3. Distribution by ageDistribution by age  InfancyInfancy  NephroblastomatosisNephroblastomatosis  Mesoblastic nephromaMesoblastic nephroma  Wilms' (less frequent)Wilms' (less frequent)  RhabdoidRhabdoid  Ossifying renal tumor ofOssifying renal tumor of infancyinfancy  ChildhoodChildhood  Wilms'Wilms'  Clear cell sarcomaClear cell sarcoma  Renal cell carcinomaRenal cell carcinoma  Multilocular cysticMultilocular cystic nephromanephroma  AngiomyolipomaAngiomyolipoma  Lymphoma (NHL)Lymphoma (NHL)  Renal medullaryRenal medullary carcinomacarcinoma  RhabdomyosarcomaRhabdomyosarcoma (pelvis)(pelvis)
  4. 4. Distribution by severityDistribution by severity  BenignBenign  NephroblastomatosisNephroblastomatosis  Mesoblastic nephromaMesoblastic nephroma  Multilocular cysticMultilocular cystic nephromanephroma  AngiomyolipomaAngiomyolipoma  Ossifying renal tumor ofOssifying renal tumor of infancyinfancy  MalignantMalignant  Wilms'Wilms'  Clear cell sarcomaClear cell sarcoma  Renal cell carcinomaRenal cell carcinoma  RhabdoidRhabdoid  LymphomaLymphoma  Renal medullaryRenal medullary carcinomacarcinoma  RhabdomyosarcomaRhabdomyosarcoma
  5. 5. 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)
  6. 6. Epidemiology of Wilms' tumorEpidemiology of Wilms' tumor  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 bilateral)1% familial (16% of familial cases are bilateral)  10% bilateral (2/3 synchronus, 1/3 metachronus)10% bilateral (2/3 synchronus, 1/3 metachronus)  Increased risk for Wilms' with persistence ofIncreased risk for Wilms' with persistence of embryonic renal tissue (nephrogenic rests =embryonic renal tissue (nephrogenic rests = nephroblastomas)nephroblastomas)
  7. 7. Wilms' tumor associationsWilms' tumor associations  Molecular Associations-2 known tumor suppressor genes on chromosome 11Molecular Associations-2 known tumor suppressor genes on chromosome 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; however, ofRecommended US screening every 3-6 months for first 7 years; however, of uncertain benefit in overall survivaluncertain benefit in overall survival  15% of WT pts have other GU anomalies (cryptorchidism, horseshoe kidney)15% of WT pts have other GU anomalies (cryptorchidism, horseshoe kidney)
  8. 8. Wilms' tumor morphologyWilms' tumor morphology  Large, spherical masses (avg 12 cmLarge, spherical masses (avg 12 cm at dx) with pseudocapsule ofat dx) with pseudocapsule of compressed parenchymacompressed parenchyma  Heterogeneous densityHeterogeneous density (hemorrhage, fat, necrosis, and/or(hemorrhage, fat, necrosis, and/or calcification)calcification)  US is reliable for identifying tumorUS is reliable for identifying tumor extension into renal vein or IVCextension into renal vein or IVC (4-10% of cases)(4-10% of cases)  CT or MRI to delineate tumorCT or MRI to delineate tumor extent; tumor enhances to lesserextent; tumor enhances to lesser extent than surroundingextent than surrounding parenchymaparenchyma  CXR or CT to identify pulmonaryCXR or CT to identify pulmonary metastasesmetastases Wilms' in L kidney of 4 year old with hepatic mets Tumor thrombus of portal v. in same patient
  9. 9. Wilms' tumor stagingWilms' tumor staging  Stage I-confined to kidney without capsular or IVCStage I-confined to kidney without capsular or IVC involvement; completely resectable with capsule intactinvolvement; completely resectable with capsule intact  Stage II-tumor extends beyond renal capsule orStage II-tumor extends beyond renal capsule or infiltrates vessels; resected tumor with confined localinfiltrates vessels; resected tumor with confined local spillagespillage  Stage III-residual tumor confined to abdomen,Stage III-residual tumor confined to abdomen, including positive abdominal nodes, positive surgicalincluding positive abdominal nodes, positive surgical margins, diffuse peritoneal contamination, or residualmargins, diffuse peritoneal contamination, or residual nonresected tumornonresected tumor  Stage IV-hematogenous spread (lungs, nodes, liver)Stage IV-hematogenous spread (lungs, nodes, liver)  Stage V-bilateral diseaseStage V-bilateral disease
  10. 10. Don’t confuse Wilms' withDon’t confuse Wilms' with neuroblastoma!!neuroblastoma!!  Neuroblastoma-arises from anywhere alongNeuroblastoma-arises from anywhere along sympathetic chain or adrenal medullasympathetic chain or adrenal medulla  Most common cancer of infancy and mostMost common cancer of infancy and most common extracranial solid tumor of childhoodcommon extracranial solid tumor of childhood (90% dx <5 years; median age at dx 2 years)(90% dx <5 years; median age at dx 2 years)  65% are intra-abdominal and they may invade65% are intra-abdominal and they may invade renal tissuerenal tissue  Determining adrenal vs renal origin is difficultDetermining adrenal vs renal origin is difficult when there is no clear plane of separationwhen there is no clear plane of separation
  11. 11. Wilms' tumor vs NeuroblastomaWilms' tumor vs Neuroblastoma  Wilms'Wilms'  <10% are calcified; more often<10% are calcified; more often a curvilinear patterna curvilinear pattern  Occasional local para-aorticOccasional local para-aortic adenopathy (less common thanadenopathy (less common than with neuroblastoma)with neuroblastoma)  IVC invasion has high positiveIVC invasion has high positive predictive valuepredictive value  Mets to lungs commonMets to lungs common  NeuroblastomaNeuroblastoma  Often calcified; scatteredOften 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 vesselsdisplacement of great vessels  Mets to liver, bone commonMets to liver, bone common  Elevated urine catecholaminesElevated urine catecholamines
  12. 12. Which is it?Which is it?
  13. 13. 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.
  14. 14. 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
  15. 15. Benign renal neoplasmsBenign renal neoplasms  NephroblastomatosisNephroblastomatosis  Mesoblastic nephromaMesoblastic nephroma  Multilocular cysticMultilocular cystic nephromanephroma  AngiomyolipomaAngiomyolipoma  Ossifying renal tumor ofOssifying renal tumor of infancyinfancy Hyperplastic perilobar nephroblastomatosis
  16. 16. NephroblastomatosisNephroblastomatosis  Diffuse or multifocalDiffuse or multifocal kidneykidney involvement of metanephric restsinvolvement of metanephric rests (embryonic renal tissue persisting(embryonic renal tissue persisting beyond 36 weeks)beyond 36 weeks)  Homogenous subcapsularHomogenous subcapsular hypodense regionshypodense regions on contrast CTon contrast CT  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 screeningresolve spontaneously ; serial screening recommended with assoc. syndromesrecommended with assoc. syndromes a) US of nephroblastomatosis (plusa) US of nephroblastomatosis (plus symbols)symbols) d) L kidney shows diffused) L kidney shows diffuse metanephric rests, while R kidneymetanephric rests, while R kidney shows 3 focal lesionsshows 3 focal lesions
  17. 17. Mesoblastic nephromaMesoblastic nephroma  Most common renal neoplasm inMost common renal neoplasm in 11stst three monthsthree months  Solid, uniform mass with possibleSolid, uniform mass with possible hypoechoic areas from cystic change orhypoechoic areas from cystic change or necrosis, thereby making it difficult tonecrosis, thereby making it difficult to distinguish from Wilms' on US (or CT)distinguish from Wilms' on US (or CT)  Has ill-defined margins and no capsule,Has ill-defined margins and no capsule, thus needs wide surgical marginsthus needs wide surgical margins  Does not invade vascular pedicle orDoes not invade vascular pedicle or metastasizemetastasize  US-large, solid mass with lowUS-large, solid mass with low echogenicityechogenicity  CT-soft tissue attenuation; enhancesCT-soft tissue attenuation; enhances less than surrounding parenchyma afterless than surrounding parenchyma after contrastcontrast 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
  18. 18. Multilocular cystic nephromaMultilocular cystic nephroma  Tumor containsTumor contains cystic lesionscystic lesions lined by epithelial cells andlined by epithelial cells and fibrous septae (septae are onlyfibrous septae (septae are only solid component of tumor)solid component of tumor)  Lining may contain blastemaLining may contain blastema cells (cystic partiallycells (cystic partially differentiated nephroblastoma),differentiated nephroblastoma), which may have malignantwhich may have malignant potential. This can only bepotential. This can only be discerned histologically.discerned histologically.  Bimodal distribution (Bimodal distribution (boys < 4boys < 4 yearsyears; women 40-60 years); women 40-60 years) CT w/ contrast demonstrating cyst (arrow) within a cystic partially differentiated nephroblastoma
  19. 19. AngiomyolipomaAngiomyolipoma  Rare in children, unless pt hasRare in children, unless pt has tuberous sclerosistuberous sclerosis  80% of TS patients have renal80% of TS patients have renal angiomyolipomas by age 10 yearsangiomyolipomas by age 10 years  Hamartomatous lesions comprised ofHamartomatous lesions comprised of fat, smooth muscle, and blood vesselsfat, smooth muscle, and blood vessels  In TS, small, bilateral, multifocalIn TS, small, bilateral, multifocal nodules, typically corticalnodules, typically cortical  Tendency towardsTendency towards aneurysmaneurysm formationformation withwith spontaneousspontaneous bleedingbleeding in aneurysms >4 cm;in aneurysms >4 cm; treatment only indicated to managetreatment only indicated to manage hemorrhagehemorrhage  US-highly echogenic, non-shadowingUS-highly echogenic, non-shadowing foci correlating with fat contentfoci correlating with fat content  CT w/contrast diagnostic if high fatCT w/contrast diagnostic if high fat content; otherwise, unenhanced may becontent; otherwise, unenhanced may be necessarynecessary 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
  20. 20. Ossifying renal tumor of infancyOssifying renal tumor of infancy  Rare tumor, diagnosed inRare tumor, diagnosed in 11stst yearyear  CalcifiedCalcified renalrenal hamartomashamartomas  Soft tissue mass arisingSoft tissue mass arising from medulla, occupiesfrom medulla, occupies part of collecting ductpart of collecting duct  Can cause sx ofCan cause sx of obstruction, hematuriaobstruction, hematuria
  21. 21. Malignant renal neoplasmsMalignant renal neoplasms  Wilms'Wilms'  Clear cell sarcomaClear cell sarcoma  Renal cell carcinomaRenal cell carcinoma  RhabdoidRhabdoid  LymphomaLymphoma  Renal medullaryRenal medullary carcinomacarcinoma  RhabdomyosarcomaRhabdomyosarcoma Gross examination of Wilms' tumor exhibiting extension into the renal pelvis
  22. 22. Clear cell sarcomaClear cell sarcoma  4% of pediatric renal4% of pediatric renal neoplasmsneoplasms  Age distribution similar toAge distribution similar to Wilms' tumor (peak 3-5Wilms' tumor (peak 3-5 years)years)  No features that areNo features that are radiologically distinct fromradiologically distinct from WT (solid mass compressing,WT (solid mass compressing, distorting surroundingdistorting surrounding parenchyma +/- cystic areasparenchyma +/- cystic areas and necrosisand necrosis  20% have bony metastases20% have bony metastases (bone scan recommended)(bone scan recommended)  Mets also to lung, brain, liverMets also to lung, brain, liver CT in 13 month old with right renal mass showing extensive central necrosis (arrow) and hydronephrosis of adjacent calices (arrowheads)
  23. 23. Renal cell carcinomaRenal cell carcinoma  Mean age in children is 9 yearsMean age in children is 9 years  1% of pediatric renal tumors1% of pediatric renal tumors  WT is 30 times more frequent inWT is 30 times more frequent in children overall; however, WTchildren overall; however, WT incidence is equal to RCC in 2incidence is equal to RCC in 2ndnd decadedecade  More frequent withMore frequent with von Hippel-von Hippel- LindauLindau  GenerallyGenerally smaller than WTsmaller than WT atat presentation; otherwisepresentation; otherwise radiographically indistinct fromradiographically indistinct from WTWT  25% with calcifications (<10% in25% with calcifications (<10% in WT)WT)  Lumbar aortic adenopathyLumbar aortic adenopathy commoncommon Coronal T1 MRI of renal cell carcinoma Gadolinium enhanced MRI
  24. 24. RhabdoidRhabdoid  2-3% of pediatric renal2-3% of pediatric renal neoplasmsneoplasms  Occur in 1Occur in 1stst year of lifeyear of life  High risk for synchronus orHigh risk for synchronus or metachronus posterior fossametachronus posterior fossa tumors (esp. PNET)tumors (esp. PNET)  RadiographicallyRadiographically indistinguishable from WTindistinguishable from WT  Crescent sign-Crescent sign-peripheralperipheral crescentic fluid increscentic fluid in subcapsular or perinephricsubcapsular or perinephric space on CT (not a highspace on CT (not a high positive predictive value)positive predictive value)  Renal vein infiltrationRenal vein infiltration commoncommon Gross appearance of rhabdoid-round, lobulated mass CT with contrast shows heterogeneously enhancing mass of L kidney
  25. 25. LymphomaLymphoma  Non-Hodgkin’s lymphoma (esp. Burkitt’s)Non-Hodgkin’s lymphoma (esp. Burkitt’s) commonly has renal involvementcommonly has renal involvement  8-12% seen on CT8-12% seen on CT  64% on autopsy64% on autopsy  UsuallyUsually bilateralbilateral dz--spreaddz--spread hematogenously or by direct extensionhematogenously or by direct extension  Common appearances:Common appearances:  Multiple parenchymal masses/nodules thatMultiple parenchymal masses/nodules that distort the renal contour and displace thedistort the renal contour and displace the collecting systemcollecting system  Invasion from retroperitoneal mass orInvasion from retroperitoneal mass or adjacent lymph nodesadjacent lymph nodes  CT-homogenous, hypodense on bothCT-homogenous, hypodense on both enhanced and unehancedenhanced and unehanced  Multifocal, nodular dz-may appear likeMultifocal, nodular dz-may appear like multiple renal cystsmultiple renal cysts  Diffuse dz-general renal enlargementDiffuse dz-general renal enlargement  AssociatedAssociated splenomegaly, abdominalsplenomegaly, abdominal adenopathyadenopathy, elevated LDH, elevated LDH 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
  26. 26. Renal medullary carcinomaRenal medullary carcinoma  Aggressive tumorAggressive tumor  Quickly fills renal pelvisQuickly fills renal pelvis  Invades vessels, lymphaticsInvades vessels, lymphatics  Unfavorable prognosisUnfavorable prognosis  Most common inMost common in adolescents,adolescents, young adults withyoung adults with sickle cellsickle cell traittrait (SA, SC); no increased(SA, SC); no increased prevalence in SS diseaseprevalence in SS disease  Centrally located, infiltrativeCentrally located, infiltrative lesion invading renal sinuslesion invading renal sinus  Peripheal caliectasisPeripheal caliectasis  Reniform enlargementReniform enlargement  Adjacent adenopathy commonAdjacent adenopathy common 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)
  27. 27. RhabdomyosarcomaRhabdomyosarcoma  Most common pediatricMost common pediatric malignancy of themalignancy of the pelvispelvis  GU tract is the 2GU tract is the 2ndnd most commonmost common site after head & necksite after head & neck  MRI preferred over CTMRI preferred over CT  MRI, CT both show excellentMRI, CT both show excellent contrast enhancementcontrast enhancement  Solid mass of muscle density withSolid mass of muscle density with areas of necrosisareas of necrosis  Botyroid subtype accounts for 5%Botyroid subtype accounts for 5% (grape-like, polypoid mass most(grape-like, polypoid mass most common in vagina)common in vagina) Pelvic rhabdomyosarcoma
  28. 28. BibliographyBibliography Grainger & Allison’s Diagnostic Radiology: A Textbook of MedicalGrainger & Allison’s Diagnostic Radiology: A Textbook of Medical Imaging, 4Imaging, 4thth ed. Churchill Livingstone, 2001; pp. 1757-1762.ed. Churchill Livingstone, 2001; pp. 1757-1762. Golden CB, Feusner JH. Malignant abdominal masses in children:Golden CB, Feusner JH. Malignant abdominal masses in children: quick guide to evaluation and diagnosis.quick guide to evaluation and diagnosis. Pediatr Clin N AmPediatr Clin N Am, 2002;, 2002; 49:1369-1392.49:1369-1392. Lowe LH, Hernanz-Schulman M et al. Pediatric Renal Masses:Lowe LH, Hernanz-Schulman M et al. Pediatric Renal Masses: Wilms Tumor and Beyond.Wilms Tumor and Beyond. RadioGraphicsRadioGraphics, 2000; 20: 1585-1603., 2000; 20: 1585-1603. Owens CM, Dicks-Mireaux C et al. Role of Chest ComputedOwens CM, Dicks-Mireaux C et al. Role of Chest Computed Tomography at Diagnosis in the Management of Wilms’ Tumor:Tomography at Diagnosis in the Management of Wilms’ Tumor: A Study by the United Kingdom Children’s Cancer Study Group.A Study by the United 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 andRiccabona 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 MedicalScott DJ, Wallace WHB, Hendry GMA. With Advances in Medical Imaging Can the Radiologist Reliably Diagnose Wilms’Imaging Can the Radiologist Reliably Diagnose Wilms’ Tumours?Tumours? Clinical RadiologyClinical Radiology, 1999; 54:321-327., 1999; 54:321-327. Images obtained from above sources as well as Google Images

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