1、Assessment and Diagnosis of Abdominal Masses in ChildrenResident Education Lecture SeriesGeneral approach to solid tumorsnWhat is it?nWhere is it?nWhere can it go?nThe answer to any one of these questions will help answer the other twoWork up two componentsStagingnX-ray of primary sitenCT chest,abdo
2、men,&pelvisnCXR(baseline)nbone scannSpecialty testsnGallium,MIBG,PETnBone marrownESREvaluate for complications of the tumornCBC with diffnTPN panelnLDH,uric acid tumor lysis,rapid cell growthnLytes,creatinine renal functionnTransaminases hepatic involvementnSpecialty testsnTumor markers nHCG,AFP nHV
3、A/VMA.nTissue diagnosisnIncisional biopsynExcisional biopsynSpecial casesnCalicified suprarenal mass+bone scan might consider getting dx from bone marrownFNA vs excisional biopsynBias towards excisional sufficient sample to be representative and to send for special research studies(histology,chromos
4、omes,special studies,research studies)nAbdominal MassesTrendsnAbdominal masses are most common in children under the age of 5 yearsnMost abdominal masses in neonates are retroperitoneal,of kidney origin and are not malignantnThe older the child the more likely the mass represents a malignant process
5、Possible Diagnoses of Abdominal Masses in Infancy and ChildhoodAtlas of Pediatric Physical Diagnosis,Fourth EditionAbdominal Masses in Older ChildrennRenal 55%nWilms(&other)25%nHydronephrosis 20%nCystic disease 5%nNon Renal Retroperitoneal 23%nNeuroblastoma 21%nTeratoma 1%nOther 1%nGastrointestinal
6、12%nAppendiceal AbscessnLymphomanHepatobiliary 6%nTumorsnHepatoblastomanHCCnGenital 4%nOvarian Cysts and TeratomaKirk et al.,1981 Radiol.Clin.North Am.,19:527-545Neonatal Abdominal MassesnRenal 55%nHydronephrosis35%nCystic disease10%nMulticystic dysplasticnPolycystic dysplasticnSolid Tumors10%nMeson
7、ephric nephromannephroblastomatosisnPelvic/Genital 15%nTeratomanOvarian CystsnHydrometrocolposnObstructed bladdernnon-Renal Retroperitoneal 10%nAdrenalnHemorrhagenneuroblastoma nGastrointestinal 15%nDuplicationnMesenteric omental cystnPseudocyst from complicated obstructionnMeconium ileusnHepatobili
8、ary 5%nHepatic tumorsnHemangioendotheliomanCystic mesenchymal hamartomanHepatoblastomanNeuroblastomanCholedochal cystKirk et al.,1981 Radiol.Clin.North Am.,19:527-545Examination of the Pediatric AbdomennHistory time the mass has been present,rapidity of growth,symptomsnUndress patient:evaluate for g
9、enetic or inherited predisposition as well as the bellynPalpate from the pelvis toward the thoraxnDescribe locationnSizenConsistencynAscitesnVenous congestion of surfaceGolden and Feusner,2002,Pediatr Clin N Am,49:1369-1392NeuroblastomauMalignancy in neural crest cells in sympathetic ganglia,adrenal
10、 medulla,chest,abdomen;small round blue tumor cellsuNonmalignant form is ganglioneuromauClinical effects r/t tumor size and locationuGenetic links/factors involved:N-myc oncogene,chromosome deletion NB Incidence/Etiology u4th peds cancer(7-10%)500-550 new US per yearuMost common cancer in infants ac
11、counts for 50%of cancer in NBs.M:F ratio:1.2:1uAverage age is 18 months;80%50%are retroperitoneal;head/neck,pelvis,posterior mediastinum;+/-spinal cord compression*uMetastatic to lymph nodes,bone,BM,liveruFever and malaise;catecholamine secretion:HTN,sweats,irritability;diarrhea;opsoclonus-myoclonus
12、;cerebellar ataxiaDiagnostic WorkupuHx:catecholamine related sx (htn,flushing,sweating,irritability);wt loss,pain,limpuPE:preorbital ecchymosis,cutaneous nodules;abd mass;weakness/paralysisuCT/MRI to locate tumor;bone scan;MIBG;PET?uLabs(urinary catecholamines);uBilateral BMA and bx;chromosome studi
13、esNeuroblastoma Staging1Localized tumor;complete excision2AUnilateral,incomplete gross resection;negative microscopic nodes2BUnilateral,positive ipsilateral nodes;negative contralateral3Across midline,or contralateral nodes4Dissemination:bone marrow,liver,skin,bones4S1y:local stage 1-2 with mets to
14、BM,liver,skinTreatment and PrognosisuSurgery:debulk or total removal;curative in low-stage disease;2nd-look after other RxuChemotherapy often platinum basedmulti-agent stageuRT:to primary tumor site;NB cells very radiosensitive;before or after surgery;emergency relief for cord compression,respirator
15、y compromise,proptosis NB Treatment contduBMT:uchildren with poor prognosis initially may be treated with high dose chemotherapy with autologous stem cell rescue(s);uBMT may be used with relapseuPrognosis:2 with stage IV disease(10-20%);Stage IV disease survival trendsp=0.034NEJM 341:1165-1173,1999p
16、=0.027NEJM 341:1165-1173,1999NEJM 341:1165-1173,1999p=0.02Tumors of the KidneyuPrimary tumors arising from the kidney,usually Wilms,rapidly growing vascular abdominal tumors;fragile gelatin capsuleuOthers:clear cell sarcoma,renal cell CA,lymphoma,PNET,rhabdoid,uWilms tumor pathology may be favorable
17、 or unfavorable depending on degree of anaplasia present;prognosis and treatment r/t pathologyIncidence and EtiologyuRenal tumors represent 5-6%of peds cancer;460 new US cases/yruHigher in AA,lower in AsiansuPeak age at 2-3;rare in kids 5;M:F 0.9:1.0(unilateral)0.6:1.0(bilateral)males younger age at
18、 diagnosisu1.5%familial in origin;associated with aniridia,hemihypertrophy,GU malformsuGenetic factors,deletion or translocationsWhat is this syndrome?nOmphalocelenMacroglossianGigantismnExophthalmosnHypoglycemianBeckwith-WiedemannHemi-hypertrophyClinical PresentationuAsymptomatic abdominal mass fou
19、nd by family or on routine PEuPain,malaise,hematuria in 20-30%;25%with HTN;rare subcapsular hemorrhage,with rapid increase in size,anemia,HTNuMets to lungs,liver,regional nodesu7%bilateral,at dx or laterDiagnostic WorkupuH and P uLabs,renal and hepatic functionuImaging studies:US to determine size a
20、nd shape,vessel involvement,thrombi in major vessels;chest film/CT to check for metsuLiver,brain,and bone mets not routinely assessed unless indicated by S/SPrognosisuHistology is most important prognostic factor(favorable histology vs.anaplastic)uStage at diagnosis also crucialuGenetic factorsuAgeS
21、taging of Wilms TumorsILimited to kidney;complete resectionIIExtent beyond kidney,but complete RIIIResidual tumor,confined to abdomenIVHematogenous mets(lung,liver,bone,brain)or lymph nodes outside abdomenVBilateral renal involvement at diagnosisTumor spill at time of surgery considered stage IIITre
22、atment and PrognosisuSurgery initially,with exam of contralateral kidney;uPreop chemotherapy if intravascular spread or very large invasive tumors;if bilateral;uNA argument:Preop Chemo prevents adequate assessment of staginguConsidered Stage III if imaged onlyTreatment and Prognosis contduBilateral:
23、preop Chemo;nephrectomy of worse side,partial on otheruChemotherapy:regimens based in national groupsuRT:port extended across midline to prevent scoliosis;if favorable histology,RT only for Stage III and IV;post lung RT,adjust ChemouRecurrence:worse if 1 year;on chemouPrognosis:50%-100%(stage/histol
24、ogy)Malignant Hepatic TumorsuHepatoblastoma;median age of 1 yr;uHepatocellular carcinoma,median age of 12 yrs,associated with hepatitis B 65%)uHepatocellular Ca:Difficult to resect and difficult to cure even with complete resection(20%)uRT of little benefit Chemo-embolization?Orthotopic liver transp
25、lant?PrognosisnHepatoblastomanResectable tumorsnAt diagnosis(stage I&II)-90%nFollowing chemo-reduction(III)80%nUnresectable tumors-50%nMetastases at diagnosis-10%PrognosisnHepatocellular CarcinomanChildren with initially resectable HCC have a good prognosis and may benefit from adjuvant chemotherapy
26、.nThe outcome for children with unresectable or metastatic HCC continues to be dismal with current therapies.Intergroup Study for the Treatment of Childhood Hepatocellular Carcinoma00.20.40.60.8101234567Time from Study Entry(years)ProbabilityStage I(N=8)Stage III(N=25)Stage IV(N=13)P0.0001Event-Free
27、 Survival by StageDifferential diagnosis of Thoracic Masses(malignant)EXTRA-THORACICnSoft tissue massnSoft tissue sarcomanPNET/EwingsnLymphoma(much less common)nBony MassnEwingsnNeuroblastomanOsteosarcoma(much less common)INTRA-THORACICnAnterior mediastinum(the 4“Ts”)nTeratoma(or germ cell tumor)nTh
28、ymomanThyroid carcinomanT-cell leukemia or other lymphoma(adenopathy+/-effusion)nPosterior mediastinumnNeuroblastoma,Ewings,other soft tissue sarcomanPulmonary parenchymanMetastatic diseasenLymphomanPrimary pulmonary malignancy(rare,usually embryonal type)nHilarnLymphomanRare soft tissue sarcoma or
29、angiosarcomaDifferential diagnosis of extremity and/or soft tissue masses(malignant)nBonenOsteosarcomanEwingsnSoft tissuenRhabdomyosarcomanPNET/EwingsnFibrosarcomanotherFrom ABP Certifying Exam Content OutlinenFormulate a differential diagnosis for an abdominal massnKnow that multicystic dysplastic
30、kidneys and hydronephrosis are the most common causes of palpable abdominal masses in infantsnRecognize that children with hemihypertrophy and somatic overgrowth syndromes should be periodically evaluated for the development of associated embryonal tumorsFrom ABP Certifying Exam Content Outline,cont
31、inuednUnderstand that a neuroblastoma usually presents as a nontender abdominal massnUnderstand that urinary catecholamine excretion is increased in most patients with a neuroblastoma and that tests of urine for VMA and VHA are appropriate screening tests for the tumornKnow that Wilms tumor is assoc
32、iated with hemihypertrophy and aniridia,somatic overgrowth,and/or genitourinary abnormalities nUnderstand that Wilms tumor usually presents as an abdominal mass and may cause hypertensionnRecognize the tumors that may produce precocious puberty(eg,in liver,CNS,ovary,testes,adrenal glands)CreditsnMichael Kelly MD PhDAnne Warwick MD MPH
