Andersons pediatric cardiology 1367
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Abstract
Cardiactumorsininfantsandchildrenarerare.Theyaremostfrequently
benignandrarelymalignantormetastatic.Themajorityofprimarycardiac
tumorscanbemanagedconservatively.Signsandsymptomsmaybedueto
hemodynamicdisturbances,arrhythmias,embolization,orsystemiceffects.
Inthecurrentera,pharmacologic,interventional,andsurgicaloptionsfor
managementexist.
Keywords
CardiacTumor;Rhabdomyoma;TuberousSclerosisComplex;Fibroma;
Myxoma;Teratoma;Hemangioma
Cardiactumorsininfantsandchildrenarerare.Previously,theiratypicalclinical
presentationpreventedtimelydiagnosis,andthediagnosiswasoften
postmortem.Currently,thewidespreaduseofechocardiographyandother
noninvasivediagnosticimagingmodalitieshasresultedinamarkedincreasein
thedetectionofcardiactumorsduringfetallifeandchildhood,whenthepatients
areoftenasymptomatic.1,2Inturn,earlyrecognitionofcardiactumorshas
resultedinbetterunderstandingoftheirnaturalhistoryand,combinedwith
advancesinsurgicalandinterventionaltechniques,animprovedoverall
outcome.1,3–5
Itisdifficulttoascertainthetrueincidenceofcardiactumorsbecauseofthe
tendencytobaseestimatesonpostmortemstudies,casereports,andexperiences
ofsingleinstitutions.Thelackofnoninvasivediagnosticimaginginearlier
reportswasanotherlimitingfactor.Basedonthedataof22largeautopsyseries,
theincidenceofcardiactumorsinallagegroupshasbeenreportedat
approximately0.02%.6Largepediatricstudiesperformedintheeraof
echocardiographyhavereportedanincidenceof0.17%to0.2%inchildrenand
0.14%infetuses.7–9
Themajorityofcardiactumorsinchildrenarebenign.Malignanttumorsare
exceedinglyrare,withmostbeingmetastaticratherthanprimary.Overall,the
mostcommonpediatriccardiactumorisrhabdomyoma.Fibromasandmyxomas
tendtobethenextmostcommontumorsinlargerstudies.Presentationis
dependentonage,withmyxomasbeingmorecommoninolderpatientsand
rhabdomyomasandteratomaspredominatinginfetusesandinfants.Table52.1
showsthebreakdownof158patientsupto18yearsolddiagnosedwithcardiac
tumorsatfourChinesehospitalsbetween1998and2014.10Table52.2showsthe
breakdownof173patientsupto21yearsolddiagnosedwithprimarycardiac
tumorsatChildren'sHospitalBostonbetween1968and2010.5Table52.3shows
thebreakdownof224fetuses,stillborns,andneonatescompiledfromametaanalysisoftheliteratureperformedin2004.1
Table52.1
AllCardiacTumorsIdentifiedinPatients0–18YearsOldinShanghai
andShandongProvince,China,1998–2014
TypeofTumor
Rhabdomyoma
Fibroma
Myxoma
Vascular
Lipoma
Papillaryfibroma
Pericardialcyst
Primarymalignant
Metastatic
Unknown
Overall
No.(%)
100(60.2)
21(12.7)
15(9)
6(3.6)
5(3)
2(1.2)
1(0.6)
8(4.8)
6(3.6)
2(1.2)
166(100)
%Male
69
57
73
67
60
50
100
Age(Median)
4mo
9mo
10y
2.5mo
9y
67
Surgeries
16
11
13
5
3
2
Deaths
4
1
8
2
3
4
2
13
59
Primarymalignanttumors:fibrosarcoma(n=2),rhabdomyosarcoma(n=1),malignant
mesothelioma(n=2),lymphoma(n=2),andundifferentiatedsarcoma(n=1).
Metastatic:adrenocorticalcarcinoma(n=1),renalclearcellsarcoma(n=1),Wilmstumor(n=1),
yolksactumor(n=1),squamouscellcarcinoma(n=1),andhepatoblastoma(n=1).
ModifiedfromShiL,WuL,FangH,etal.Identificationandclinicalcourseof166pediatriccardiac
tumors.EurJPediatr.2017;176:253–260.
Table52.2
PrimaryCardiacTumorsIdentifiedinPatients0–21YearsOldat
Children'sHospitalBoston,1968–2010
Typeof
Tumor
N
(%)
Rhabdomyoma 106
(61)
Fibroma
25
(14)
%
MedianAgeat
Male Diagnosis(Range)
Asymptomatic Hemodynamic
(%)
Changes(%)
46
2mo(PN-18y)
76(72)
18(17)
Significant
Arrhythmias
(%)
17(16)
64
1y(PN-10y)
4(16)
5(20)
16(64)
Deaths
(%)
3(3)
0
Myxoma
Vascular
Teratoma
Lipoma
Other
Overall
14
(8)
6(4)
4(2)
3(2)
15
(9)
173
(100)
43
9y(3wk–21y)
5(36)
6(43)
1(7)
0
67
25
67
67
11y(PN-17y)
20days(PN-3y)
13y(1day–16y)
1y(1day–16y)
4(66)
0
3(100)
1(7)
0
4(100)
0
2(13)
1(17)
0
0
7(47)
2(33)
1(25)
0
0
55
7mo(PN-21y)
93(54)
35(20)
42(24)
6(3)
Other:foregutcyst(n=1),papillaryfibroelastoma(n=2),inflammatorypseudotumor(n=1),
spindlecellsarcoma(n=1),inflammatorymyofibroblastictumor(n=1),plexiformneurofibroma
(n=1),pericardialcyst(n=1),bloodcyst(n=2),Purkinjecelltumor(n=1),paraganglioma(n=
1),andunknown(n=3).
PN,Prenatal.
ModifiedfromMiyakeCY,DelNidoPJ,AlexanderME,etal.Cardiactumorsandassociated
arrhythmiasinpediatricpatients,withobservationsonsurgicaltherapyforventriculartachycardia.
JAmCollCardiol.2011;18:1903–1909.
Table52.3
CardiacTumorsIdentifiedinFetuses,Stillborns,andNeonatesa
TypeofTumor
Rhabdomyoma
Teratoma
Fibroma
Purkinjecelltumor
Vasculartumors
Myxoma
Malignant
Overallnumber
No.(%)
120(53.8)
40(17.8)
28(12.4)
15(6.6)
13(5.8)
6(2.7)
2(0.9)
224(100)
No.Alive
72
30
8
1
11
1
0
123
%Survival
60
75
29
7
85
17
0
55
aAsreviewedbyIsaacsHJr.Fetalandneonatalcardiactumors.PediatrCardiol.2004;25:252–
273.
