Ante situm liver resection with inferior vena cava replacement under hypothermic cardiopolmunary bypass for hepatoblastoma: Report of a case and review of the literature

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ContentslistsavailableatScienceDirect

International

Journal

of

Surgery

Case

Reports

jo u r n al ho me p a g e :w w w . c a s e r e p o r t s . c o m

Ante

situm

liver

resection

with

inferior

vena

cava

replacement

under

hypothermic

cardiopolmunary

bypass

for

hepatoblastoma:

Report

of

a

case

and

review

of

the

literature

Roberta

Angelico

a

_,

_Annalisa

_Passariello

b,c

_,

_Michele

_Pilato

d

_,

_Tommaso

_Cozzolino

b

_,

Marcello

Piazza

e

_,

_Roberto

_Miraglia

f

_,

_Paolo

_D’Angelo

g

_,

_Mariella

_Capasso

c

_,

Maria

Cristina

Safﬁoti

a

_,

_Daniele

_Alberti

h

_,

_Marco

_Spada

a,∗

a_Department_of_Abdominal_{Transplantation}_and_{Hepatobiliary}_and_Pancreatic_Surgery,_Bambino_Gesù_Children’s_Hospital_IRCCS,_Rome,_Italy b_Department_of_{Translational}_Medical_Science,_University_of_Naples_“Federico_II”,_Naples,_Italy

c_Department_of_Pediatric_Oncology,_Ospedale_Santobono-_Pausilipon,_Naples,_Italy

d_Cardiac_Surgery_and_Heart_{Transplantation}_Unit,_Department_for_the_Treatment_and_Study_of_{Cardiothoracic}_Diseases_and_{Cardiothoracic}_{Transplantation,}

IRCCS–ISMETT(MediterraneanInstituteforTransplantationandAdvancedSpecializedTherapies),Palermo,Italy

e_Department_of_Anesthesia_and_Intensive_Care,_IRCCS_–_ISMETT_{(Mediterranean}_Institute_for_{Transplantation}_and_Advanced_Specialized_Therapies),

Palermo,Italy

f_Radiology_Service,_Department_of_Diagnostic_and_Therapeutic_Services,_IRCCS_–_ISMETT_{(Mediterranean}_Institute_for_{Transplantation}_and_Advanced

SpecializedTherapies),Palermo,Italy

g_“Giovanni_Di_Cristina”_Children’s_Hospital,_Pediatric_Hematology_and_Oncology,_Palermo,_Italy h_Department_of_Pediatric_Surgery,_“Spedali_Civili”_Children’s_Hospital,_Brescia,_Italy

a

r

t

i

c

l

e

i

n

f

o

Articlehistory:

Received13February2017 Receivedinrevisedform4June2017 Accepted5June2017

Availableonline13June2017

Keywords: Hepatoblastoma

Inferiorvenacavatumoralthrombi Antesitumliverresection

Hypothermiccardiopolmunarybypass

a

b

s

t

r

a

c

t

INTRODUCTION:Hepatoblastomawith tumourthrombi extendingintoinferior-vena-cava andright atriumareoftenunresectablewithanextremelypoorprognosis.Thesurgicalapproachistechnically challengingandmightrequiremajorliverresectionwithvascularreconstructionandextracorporeal circulation.However,whichisthebestsurgicaltechniqueisyetunclear.

PRESENTATIONOFCASE:A11-months-oldboywasreferredforarighthepaticlobemass(90×78mm) suspiciousofhepatoblastomawithtumoralthrombiextendingintotheinferior-vena-cavaandtheright atrium,bilaterallunglesionsandserumalpha-fetoproteinlevelof50.795IU/mL.After8monthsof chemotherapy(SIOPEL2004-high-risk-Protocol),thelunglesionswerenolongerclearlyvisibleandthe hepatoblastomasizedecreasedto61×64mm.Thus,antesitumliverresectionwasplanned:afterhepatic parenchymaltransection,hypothermiccardiopulmonarybypasswasstartedandenblocresectionofthe extended-righthepaticlobe,theretro/suprahepaticcavaandthetumoraltrombiwasperformedwith concomitantcoldperfusionoftheremnantliver.Theinferior-vena-cavawasreplacedwithanaorticgraft fromablood-groupcompatiblecadavericdonor.Thepost-operativecoursewasuneventfulandafter8 monthsoffollow-upthechildhasnormalliverfunctionandanalpha-fetoproteinlevelandisfreeof diseaserecurrencewithpatentvasculargraft.

CONCLUSIONS:Wereportfortheﬁrsttimeacaseofantesituliverresectionandinferior-vena-cava replacementassociatedwithhypothermiccardiopulmonarybypassinachildwithhepatoblastoma. Herein,weextensivelyreviewtheliteratureforhepatoblastomawiththumoralthrombiandwedescribe thetechnicalaspectsofantesitumapproach,whichisarealisticoptioninotherwiseunresectable hepa-toblastoma.

Abbreviations:HLB,hepatoblastoma;IVC,inferiorvenacava;FAP,familialadenomatouspolyposis;LT,livertransplantation;PV,portalvein;TVE,totalhepaticvascular exclusion;UVC,uppervenacava;PRE-TEXT,pretreatmentextentofdisease;POST-TEXT,posttreatmentextentofdisease;SIOPEL,SociétéInternationaled’Oncologie Pédiatrique-EpithelialLiverTumorStudyGroup.

∗ Correspondingauthorat:DepartmentofAbdominalTransplantationandHepatobiliaryandpancreaticSurgery,Bambino,GesùChildren’sHospitalIRCCS,Piazza Sant’Onofrio4,00146Rome,Italy.

E-mailaddresses:Roberta.angelico@gmail.com(R.Angelico),Annalisa.passariello@unina.it(A.Passariello),mpilato@ismett.edu(M.Pilato),Tom.cozzolino@gmail.com

(T.Cozzolino),mpiazza@ismett.edu(M.Piazza),rmiraglia@ismett.edu(R.Miraglia),oncoematoped@arnascivico.it(P.D’Angelo),Mariellacapasso1969@gmail.com

(M.Capasso),Mcristina.safﬁoti@opbg.net(M.C.Safﬁoti),daniele.alberti@unibs.it(D.Alberti),marco.spada@opbg.net(M.Spada).

http://dx.doi.org/10.1016/j.ijscr.2017.06.008

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R.Angelicoetal./InternationalJournalofSurgeryCaseReports37(2017)90–96 91 1. Introduction

Hepatoblastoma (HBL) is the most common paediatric liver tumouranditoccursusuallyintheﬁrst3yearsoflife.Theincidence ofHBLhasincreasedintherecentyearsupto1.5casespermillion, anditis frequentlyassociated withlowbirth weightorgenetic anomalies. Themanagement ofHBL hasimpressively improved duetocombinedneoadjuvantchemotherapyandliverresection orlivertransplantation(LT),increasingtheoverall5-yearssurvival

rateupto75%[1].Theriskstratiﬁcationwiththepre-treatment

extentofdisease(PRE-TEXT)system,theworldwidemulticentre

trialsexperienceandthemultidisciplinarymanagement,improved

HBLprognosisandsurgicalresectability[2].However,HBLwith

tumourthrombiextendedintotheinferiorvenacava(IVC),withor

withouttheinvolvementoftherightatrium,mayresultchallenging

todeﬁnethebestsurgicaltechnique.

Differentsurgicalprocedures,mainlyreportedinadults,have

beenproposedforlivertumourwithIVCinﬁltration,includingtotal

hepaticvascularexclusion(TVE)[3].Thesetechniquesareeffective

to control haemorrhage and air embolism during liver

resec-tion,but maycauseseverehepatic ischemia/reperfusioninjury,

hemodynamicinstabilityandpotentialrenalinjury.Recently,liver

resectionunderhypothermicliverperfusionwithcytoprotective

solution(includinginsitu,exsituorantesitumtechniques)hasbeen

proposedforpreventingischemicliverinjury [4].Yet,no

expe-rienceofantesitumliverperfusionassociatedwithhypothermic

cardiopulmonarybypasshasbeenreportedinchildren.

Hereinwereportasuccessfulcaseofantesituliverresection

andIVCreplacementunderhypothermiccardiopulmonarybypass

(CPB),performedina21months-oldmalewithHBLandtumour

thrombiintotheIVCandrightatrium.

ThecurrentcasehasbeenreportedinlinewiththeSCARE

cri-teria[5].

2. Casereport

The patient was a 11-months old child referred for right

upper quadrant abdominal mass. He was born on term (birth

weight of 2.470kg) and had a familial history of

adenoma-touspolyposis(FAP).Atthetime ofadmission,thepatientwas

asymptomaticwithnormalvitalsigns,butphysicalexamination

revealedhepatomegaly,abdominalbloatingandumbilicalhernia.

Theultrasonography(US)showedalargehepaticmass(10cmin

diameter)intherightliver.Serumalpha-fetoprotein(AFP)level

was50.795 IU/mL.Liver function, coagulation,serum B-human

chorionicgonadotropin,blood cellcount,thyroidfunctionwere

withinnormallimits,exceptfortheevidenceofthrombocytosis

(805.000/UL).Computedtomography(CT)showedamassofthe

righthepaticlobe, 90×78mmin sizeextendingin segmentIV,

withdyshomogeneityandcalciﬁcations.Thetumourdisplaced

pos-teriorlytherightkidney,dislocatedtheaortaandtheIVCtothe

leftside,stretchingtheceliactrunkandthesuperiormesenteric

artery(Fig.1).Tumoralthrombiwaspresent,extendingfromthe

righthepaticveinintotheIVCuptotherightatrium.Bilaterallung

lesions,suspiciousforHBLmetastases,werefoundaswell.Heart

involvementwasconﬁrmedbyechocardiography,whichdetected

a2.6cmechoicmassthroughthetricuspidvalve.

APRE-TEXTIIIstaging(P0,V3,M1)withlungandatrium-cava

metastasisattheoutsetwasdeﬁned.Thechildunderwent

neoad-iuvantchemotherapy(SIOPEL2004highriskprotocol;cyclesA1-3

andcycleB)for 8months:3cycleswithcisplatin(70mg/m2_,₉

dosesadministered)anddoxorubicin(30mg/m2_,₆_doses);₄_cycles

withcarboplatin(6mg/Kg,4doses)anddoxorubicin(0.83mg/Kg,

10 doses); and 2 cycles with carboplatin (25mg/Kg, 2 doses),

vincristine(0.05mg/Kg,5doses)and5-ﬂuorouracil(33mg/Kg,6

doses).Duringthetreatment,thechildpresentedtransientsevere

thrombocytopeniaandoneepisodeofsepsissuccessfullytreated

withantibiotics.AfterneoadiuvanttherapyAFPdecreasedto879

IU/mL.CTscanshowedsizereductionoftheHBL(61×64mm),still

involvingtheIVCastherightandmiddlehepaticvein.Aleft

acces-soryhepaticarteryfromtheleftgastricarteryandareplacedright

hepaticarteryarisingfromthesuperiormesentericarterywere

documented;lunglesionswerenolongerclearlyvisible.

Cavogra-phydocumentedretrohepaticIVCinﬁltrationbyHBL(Fig.2).After

multidisciplinary team meeting (involvingsurgeons, oncologist,

anaesthesiologistandradiologist),thesmallpatientwasproposed

for anextended rightliverresection,withIVCand intracardiac

thrombusremoval,whichwasperformedbyaseniorliver

trans-plantationandhepato-biliary-pancreaticsurgeon.

2.1. Surgicalprocedure

Thepatientwasplaced in supinepositionand theabdomen

wasexploredthroughabilateralsub-costalincisionwithxyphoid

extension.Therewasnoevidenceofascitesorperitonealmetastasis

andintraoperativeUSdocumentedthatthetumourdidnotinvolve

theleftlateralsegmentoftheliver.TheArantius’ligamentwas

dis-sectedandthelefthepaticveinwaslooped.Aftercholecystectomy,

thecommonbileduct,therighthepaticarteryandtheanteriorand

posteriorbranchesoftherightportalvein(PV)wereligatedand

divided.TheleftPVandthelefthepaticarterieswereidentiﬁed

andlooped.TheRexrecesswasthenexposedandvesselsfor

seg-mentIVweredivided.Parenchymaltransection,alongthelineof

thefalciformligament,wasperformedviaananteriorapproach,

using thehangingmanoeuvre with“notouchapproach”of the

tumour.Biliaryandvascularstructuresweredividedbetweenclips

ortie.Pringlemanoeuvrewasnotused.Avessellooparoundthe

IVCabovetherenalveinswasthenplaced.

Thexiphoidincisionwasextendeduptothejugulumwitha

mediansternotomyandthepericardialsacwasopened.After

sys-temic heparinization,theascending aorta,the uppervena cava

(UVC)andtheinfra-renalIVCwerecannulatedandclamped,and

theextracorporealcirculationwithCPBwasstarted.Body

temper-ature wasreduced to28◦C,in ordertoprotect theorgans.The

diaphragmwasincisedverticallydowntowardthesuprahepatic

IVCandthediaphragmaticveinsweredivided.Afterclampingthe

lefthepaticarteriesandthePV,theleftPVwascannulatedthough

therightPV stump.The lefthepatic veinwasdivided andante

situhypothermicliverperfusionwithCelsiorsolution(4◦C)was

started.Theliverwasfurthercooledwithiceonhissurface.After

divisionoftherighttriangularligament,anen-blocresectionofthe

extended-righthepaticlobe(segmentsI+IV-VIII),oftheretro-and

supra-hepaticIVCandoftheneoplasticthrombus(extendingfrom

therighthepaticveintotherightatrium)wasperformed(Fig.3).

The IVC was reconstructed with a fresh aortic graft from

cadaveric donor withidentical blood group.The aortic conduit

wasend-to-endanastomosedwiththerightatrium(throughthe

diaphragmaticostium)andinferiorlywiththesupra-renalIVCby

5/0prolenecontinuousrunningsutures.Theneo-IVCwasopened

immediatelybelowthediaphragmaticostiumandend-to-side

tri-angularanastomosiswasperformedbetweenthelefthepaticvein

andtheneo-IVCby6/0prolene.After40minofhypothermicliver

perfusion,theportalﬂushwasinterrupted.UVC,IVC,aorta,left

hep-aticarteriesandPVwerede-clamped,andtheleftlateralsegment

wasreperfused.Thepatientwasgraduallyrewarmedand,once

hemodynamicstabilityandgoodhaemostasiswereconﬁrmed,the

CPB was weaned off, after a total time of 71min. Roux-and-Y

end-to-sidehepaticojejunostomywith6/0PDSwasperformedfor

biliaryreconstruction.Beforethoraco-abdominalclosure,

Doppler-USestablishedagoodﬂowthroughtheneo-IVC,lefthepaticvein,

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Fig.1.ComputerTomographyimagingatpresentation.

ComputerTomographyimagingatdiagnosisshowing:A)righthepaticlobemasswithcalciﬁcations(90×78mm);B)lungmetastasisandtumoralthrombiinvadingthe inferiorvenacavaandtherightatriumtroughtherighthepaticvein;c)tumourmassintherightextendedlobeoftheliver.

Fig.2.Tumoralstagingafterneoadjuvantchemotherapyandpreoperativeassessment.

Imagingofhepatoblastoma(HBL)afterneoadjuvantchemotherapy(SIOPEL2004HRprotocol):A-B)CTscanshowingHBLintheextended-rightlobeofliverwithtumoral thrombiintotherighthepaticveinandtherightatrium;C)cavographyshowingtumoralthrombiinﬁltratingandcompressingtheretrohepaticinferiorvenacavaand retroperitonealcollaterals.

10min,withabloodlossof200ml(videoofthesurgicaltechnique canbefoundinsupplementarymaterials).

Theresectedliverspecimenweighted210g.Thetumour mea-sured8×9cm. HistologicaldiagnosiswasHBL,mixedepithelial andmesenchymaltype,withteratoidfeatures,invadingthe hep-aticvenoussystemextensively.Thesurgicalmarginswereclear fromtumour.

2.2. Post-operativeoutcome

Thechildhadanuneventfulpost-operativecourseandwas dis-chargedafter23daysfromsurgery.

After4months,astagingCTscanshowedabsenceofdisease recurrenceandgoodliverperfusion,withpatentaorticgraft(Fig.4).

After12monthsoffollow-upthechildisingoodclinicalcondition

withnormalliverfunctiontestandanAFPlevelof1.1UI/mL.

3. Discussion

HBListhemostcommonprimarypaediatriclivertumour,with

greaterfrequencyamongmales.Themainsymptomsinclude

dis-comfortduetotheabdominalmassandlossofappetite,associated

withgeneralized fatiguesecondary toanaemia. Most HBLsare

sporadic,butsomeareassociatedwithgeneticabnormalitiesand

malformations, such as trisomy 18, Beckwith-Wiedemann

syn-drome, or FAP [1]. HBL should be suspected in patients aging

between6monthsand3yearsoldinthepresenceofanhepatic

tumourwiththrombocytosisandhighAFPlevels,whichwereall

presentinourcase.Histologically,HBLhasbeenclassiﬁesinthe

epithelialtype,whichisthemostcommonand presentswitha

combinationof mixedembryonaland fetalpatterns,and inthe

mesenchymaltype,whichoccurswithorwithoutteratoidfeatures.

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R.Angelicoetal./InternationalJournalofSurgeryCaseReports37(2017)90–96 93

Fig.3. Technicalaspectsofantesitumliverresectionandinferiorvenacavareplacement.

IntraoperativeviewofA)hepatichilumdissection;B)parenchymaltransection,onthelineofthefalciformligamentviaanteriorapproach;C)antesituhypothermicliver perfusionwithCelsiorsolution(4◦C)throughtherightportalveinstumpandinferiorvenacavareplacementwithdonoraorticconduit(notethediaphragmaticostium resectedandreconstructed);D)ﬁnalviewofen-blocresectionoftheextended-righthepaticlobe(segmentsI+IV-VIII),theinferiorvenacavawithtumuralthrombiandthe diaphragmaticostium.

histologicalcomponents,andonlyrarelycomposedofasingle his-tologicaltype.Mesenchymalelementshavebeenassociatedwith animprovedprognosisinpatientswithadvanceddisease,asitwas inourcase[2].

ThePRE-TEXTsystemallowstostageandstratifytheriskof

HBLandtodeﬁneitsprognosisandsurgicalresectability.Although

60%of tumoursare unresectableat presentation,HBLis highly

chemosensitive and up to85% of cases become operable after

neoadjuvantchemotherapy[2].

Thebestchemotherapyforadvanced tumoursis still

contro-versial.Theplatinum-basedchemotherapeuticregimenshavebeen

essentialinimprovingpatientsurvivalinadvancedHBL.The

Chil-dren’sOncologyGroup(COG)recommendscisplatin,5-ﬂuoruracil

andvincristine,associatedwithdoxorubicinforintermediateand

high-risk patients, while the SociétéInternationale d’Oncologie

Pédiatrique-EpithelialLiverTumorStudyGroup(SIOPEL)

recom-mendsinveryhigh-riskpatientscisplatinintensiﬁcationtherapy

(SIOPEL-4protocol)[2].

In thecurrent case the SIOPEL4 protocolwas used

accord-ingly with presence of metastatic disease and major vascular

invasion. Since tumour remained unresectable at the ﬁrst CT

re-evaluation withhighAFP levels,he receivedadditional

pre-operativechemotherapybeforesurgerywasattempted(datanot

shown).

CompletesurgicalremovalofHBL,byresectionorLT,remains

theonlytreatmentachievinglong-termsurvival.LT playsa key

roleinthemanagementofchildrenwithlargeandmultifocalHBL,

butequivalentlong-termdisease-freesurvivalhavebeenrecently

achievedwithlargenon-anatomicorextendedliverresection,

pro-videdthatcompletemacro-andmicro-scopictumourresectioncan

beachieved[6].

Although it must becarefully considered on a case-by-case

basis, multidisciplinaryposttreatmentextentofdisease

(POST-TEXT)tumourevaluationandintraoperativeliverinspectionare

essentialtodeﬁnethebesttherapeuticmanagement.Inthissense,

promptreferraltoacenterwithexpertiseinbothpaediatricLTand

extremeresectionmustbeconsideredthegoldstandardincare

giving.POST-TEXTtumoursthatspareatleast1branchofthe

por-talveinand1hepaticveinshouldalwaysbeevaluatedforliver

resection.Whenvenousobstruction,encasement,and/orinvasion

ofthemainportalveinorbifurcationortheIVCorall3hepaticveins

arepresent,thetumourisclassiﬁedunresectableandintendedto

transplantation[2].

InthiscaseweoptedforamajorliverresectionwithIVC

recon-structionbecauseofevidenceoflungmetastasisandpresenceof

leftlateralliverfreeofdiseasewithadequateremnantlivervolume.

Hepaticresectionavoided exposingtheyoungboytolong-term

immunosuppression.

TumourthrombiinthehepaticveinsandIVCwithan

exten-sionuptotheatriumareassociatedwithhighriskofpulmonary

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Fig.4.ComputerTomographyimagingaftersurgery.

ComputerTomographyscanafter4monthsfromsurgeryshowingpatentlefthepaticveinanastomosis(A)andretrohepaticcavareplacementwithaorticgraftfromcadaveric donor(B,C).

congestiveheartfailureandspreadofsystemicmetastasisandare mainlyreportedinadultswithhepatocellularcarcinoma[3].

Despitesurgicaltreatmentseemstoremaintheonlyeffective

therapeuticoption,thereisnoestablishedmanagementforsuch

cases.In1966,Heaneyetal.ﬁrstlyproposedtheTVEoftheliver[7].

TVEiseffectiveincontrollinghaemorrhageandairembolism,but

causesseverehemodynamicdisturbancescharacterizedby>30%

decreaseinmeanarterialpressure,>50%decreaseofcardiacindex

andsevereischemicliverdamage,inparticularinsmallremnant

liverafterneoajuvantchemotherapy[3].Evenitisnotmandatory,it

isadvisabletouseTVEincombinationwithCPBinordertoreduce

hemodynamicinstabilityandpotentialrenalinjury,inparticular

whenprongedTVEisrequired.

In1981,Einetal.describedtheﬁrstsuccessfullyuseofCPB

asso-ciatedwithhypothermiccardiocirculatoryarrestin6childrenwith

rightatrialtumoralthrombi[8].However,theprocedureswere

associatedwithhighpost-operativehaemorrhageandmicroscopic

residualtumour(R1).Later,furtherreportsofmajorHBL

resec-tionsusingtheCPBhavebeenreported,assummarizedinTable1.

Manycaseshavebeenassociatedwithmajorcomplicationssuchas

post-operativedeadforpulmonaryembolism(possiblyrelatedto

tumoralthrombispreadingduringlivermobilization)[9];ischemic

cholangiopathyrequiring subsequent LT [10]; residual tumoral

thrombiinmajorvessels[6].

To reduce ischemic damage related to TVE and cellular

metabolismduringthisphase,theconceptofhypothermic

preser-vation,byliverperfusionwithcytoprotectivesolutionscombined

withcoolingoftheorgan’ssurface,hasbeeninvestigated.In1974,

Forneretal.describedtheﬁrstinsituhypothermicliverperfusion

duringmajorliverresection,wherehypothermiawasinducedby

liverperfusionviathearterialandportalsystemwithcoldRinger’s

solution(4◦C)[11].However,incaseoftumourslocatedonthe

posteriorsideoftheliverandinvadingtheIVC,insituhypothermic

liverpreservationmaynotbesufﬁcienttoexposetheretro-hepatic

venacava.Consequently,in1990Pichlmayretal.proposedtheex

situliverperfusion[12],wheretheliveriscompletelyremoved

fromthepatient, cooledwithice and perfusedwithcold

solu-tiononthebacktable;afterthebenchsurgerytheremnantliver

isreimplantedorthotopically.Later,in1991Hannounetal.[13]

introducedtheantesitumliverresectioncharacterizedbyno

hepa-toduodenalligamentdivision,coldliverperfusion,TVEanddivision

ofthesupra-hepatic IVC, whichallows therotationoftheliver

aroundthecoronaryaxiswithoptimalexposureofthehepaticveins

conﬂuenceandtheretro-hepaticIVC.Belghitietal.describedthe

modiﬁedantesitumtechniqueinwhichtheIVCiscutaboveand

belowtheliver,permittingabettermobilizationoftheliver[14].A

recentreviewofhypothermicantesituresectionintumourofthe

hepatocavalconﬂuencesuggeststhatthis approachiseaserand

saferthentheexsitutechnique,withanacceptablemorbidityand

mortalityrate[4].

Tothebestofourknowledge,thecurrentreportistheﬁrstcase

ofantesitumliverresectionandIVCreplacementwithhypothermic

CPBforHBLinayoungchild.Sincethetumourwasinvolvingthe

extendedrightlobeoftheliverwiththeretro-hepaticIVCandthe

rightatrium,theCPBwasneeded.Anteriorapproachtotheliver

wasadoptedperformingparenchymaltransectionwith“notouch

technique”ofthelesion,toavoidtumoralembolization.Moreover,

toreducetheriskofbleedingduringthisphase,liverresectionwas

completedbeforeeparinizationandCPB,differingfromprevious

reports.Themodiﬁedantesitumtechniquepermittedtoexpose

optimallytheretro-hepaticcavabycuttingtheIVCaboveandbelow

theliver,tomobilizetheliveranteriorlyandtoreducetheischemia

liverinjuryoftheremnantsegmentsbyPVperfusion.Furthermore,

wedidn’tdividetheliverhilum,avoidingtheriskofhepaticartery

thrombosis.

Sofar,thelargestseriesoftheantesitumliverresectionwas

reportedbyRaabetal.:outof24,oneadultpatienthadHBL[15].

AuthorsadoptedanormothermicveinbypassandIVC

reconstruc-tionwasperformedwithautologoussaphenous;however,details

regardingtheoutcomeoftheHBLpatientarenotavailable.

Inthecurrentreportasimpletrombectomywasnotﬂeasible

sincethetumoralthrombiinﬁltratedtheIVCwall.Therefore,

retro-hepaticIVCresectionandinterposition ofgraftwereneededto

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Table 1 Literature reports of liver resection and cardiopolmunary bypass for hepatoblastoma with inferior vena cava tumoral thrombi. Report Year Cases Age (months)/Gender Type of vascular infiltration Metastatic disease* Neoadjuvant chemotherapy Adjuvant chemotherapy Type of liver resection IVC reconstruction CPB (type, min) Outcome Ein et al. [8] 1981 6** 8–15 yrs/4M, 2F NA None None Yes (5/6) NA None Hypothermic 20 ◦C, 43–75 min 2 died/2 Alive NED, 2 alive with lung metastasis Mestres et al. [9] 1991 1 36/M RA None DOXO + CIS None Right hepatectomy Transatrial trombectomy Hypothermic 20 ◦C, 146 min Died for polmunary embolism (day 23) Lautz et al. [10] 2011 1 96/F RA None VCR, CIS, 5FU VCR, CIS, 5FU Non anatomical resection Transatrial trombectomy Yes Alive, NED (LT for ischemic cholangiopathy) Fuchs et al. [6] 2016 2 NA IVC-RA None Platinum- based Yes NA 1:prothesis;1:pericardial patch Yes Died for tumoral thrombi recurrence Current case 2016 1 11/M IVC-RA Lungs CBCDA, 5FU, VCR, DOXO – Ante situm liver resection (right hepatectomy) Fresh aortic graft from cadaveric compatible donor Hypothermic, 71 min Alive, NED Abbreviations : CBCDA , Carboplatin; CIS , Cisplatin; CPB , cardiopolmunary bypass; DOXO , doxorubicin ; IVC , inferior vena cava; NA , non available; NED , non evidence of disease; RA , right atrium; VCR , vincristine; 5FU , F-fluoro-uracil. *Distant metastatic disease with the exception of vascular infiltration of IVC and right atrium. **In this case series, indications for surgery included: hepatoblastoma (n = 4), rabdomyosarcoma (n = 1), hepatocarcinoma (n = 1).

maryrepairorpatchingwithbovine’spericardiumorautologous peritoneumincaseoflimitedIVCinvolvement.Extensivevenous involvement requiressubstitutionwithsynthetic,autologousor heterologousfreshorcriopreservatedgraftconduit.Nodeﬁnitive dataexistsinfavourofonetechniquecomparedtotheothers[16].

Inouryoungpatient,wepreferredtheinterpositionofagraftfrom

acompatiblecadavericdonortoreducetheriskofvesselcollapse

andtoavoidlong-termanticoagulation.

Inconclusion,althoughthesigniﬁcanceofourdataislimitedby

thecase-reportnatureofoursurgicalpractice,thecurrentcase

sug-geststhatantesitumliverresectionisfeasibleinchildrenwithliver

tumoursconsideredunresectablebyconventionalsurgeryorwhen

LT is contraindicatedor consideredas a secondline-treatment.

TheuseofhypothermicCPBissafeandallowsexpandingsurgical

indicationswhenprolongedTVEwithcomplexvessel

reconstruc-tionareneeded.Patientselection,preoperativeevaluationofthe

liverfunctionandanatomyaswellasintraoperativeassessment

areessential toachievegood outcome.Our ﬁndingsjustify

fur-therinvestigationstoidentifytheoptimalsurgicalmanagement

forchildrenwithlargeandcentrallylocatedlivertumours.

Conﬂictsofinterest

Noconﬂictsofinterest

Funding

Thisresearchdidnotreceiveanyspeciﬁcgrantfromthe

found-ingagenciesinthepublic,commercial,ornot-for-proﬁtsectors.

Ethicalapproval

Notapplicable

Consentdeclaration

Writteninformedconsentwasobtainedfromthepatientfor

publicationofthiscasereportandaccompanyingimages.Acopy

ofthewrittenconsentisavailableforreviewbytheEditor-in-Chief

ofthisjournalonrequest.

Authorcontribution

-RobertaAngelico–Datacollection,analysis,reviewofthe

litera-tureandwrotemanuscript

-AnnalisaPassariello–Oncologicalmanagement,intellectual

con-tent,reviewoftheliterature

-PilatoMichele–Performedthecardiothoracicsurgery,

intellec-tualcontent

-CozzolinoTommaso–Oncologicalmanagement,datacollection,

reviewoftheliterature

-PiazzaMarcello–Oncologicalmanagement,analysisand

inter-pretation,intellectualcontent

-MiragliaRoberto–Datacollection,analysisandinterpretation

-D’AngeloPaolo–Oncologicalmanagement,analysisand

inter-pretation

-CapassoMariella–Analysisandinterpretation,intellectual

con-tent

-SafﬁotiMariaCristina–Datacollection,analysisand

interpreta-tion

-AlbertiDaniele–Planningofsurgicalstrategy,intellectual

conti-nent

-SpadaMarco–Performedtheabdominalsurgery,draftthework,

(7)

Guarantor MarcoSpada.

AppendixA. Supplementarydata

Supplementarydataassociatedwiththisarticlecanbefound,in theonlineversion,athttp://dx.doi.org/10.1016/j.ijscr.2017.06.008. References

[1]D.C.Aronson,R.L.Meyers,Malignanttumorsoftheliverinchildren,Semin. Pediatr.Surg.25(5)(2016)265–275.

[2]P.Czauderna,B.Haeberle,E.Hiyama,A.Rangaswami,M.Krailo,R.Maibach, etal.,TheChildren’sHepatictumorsInternationalCollaboration(CHIC):novel globalraretumordatabaseyieldsnewprognosticfactorsinhepatoblastoma andbecomesaresearchmodel,Eur.J.Cancer52(2016)92–101.

[3]A.W.Hemming,K.L.Mekeel,I.Zendejas,R.D.Kim,J.K.Sicklick,A.I.Reed, Resectionoftheliverandinferiorvenacavaforhepaticmalignancy,J.Am. Coll.Surg.217(1)(2013)115–124(Discussion124–125).

[4]A.Mehrabi,H.Fonouni,M.Golriz,S.Hofer,M.Hafezi,N.N.Rahbari,etal., Hypothermicantesitumresectionintumorsofthehepatocavalconﬂuence, Dig.Surg.28(2)(2011)100–108.

[5]R.A.Agha,A.J.Fowler,A.Saetta,I.Barai,S.Rajmohan,OrgillDPandtheSCARE group.theSCAREstatement:consensus-basedsurgicalcasereportguidelines, Int.J.Surg.34(2016)180–186.

[6]J.Fuchs,S.Cavdar,G.Blumenstock,M.Ebinger,J.F.Schäfer,B.Sipos,etal., POST-TEXTIIIandIVhepatoblastoma:extendedhepaticresectionavoidsliver transplantationinselectedcases,Ann.Surg.(August(5))(2016)(Epubahead ofprint).

[7]J.P.Heaney,W.K.Stanton,D.S.Halbert,J.Seidel,T.Vice,Animproved techniqueforvascularisolationoftheliver:experimentalstudyandcase reports,Ann.Surg.163(1966)237–241.

[8]S.H.Ein,B.Shandling,W.G.Williams,G.Trusler,Majorhepatictumor resectionusingprofoundhypothermiaandcirculationarrest,J.Pediatr.Surg. 16(3)(1981)339–342.

[9]C.A.Mestres,K.Prabhakaran,O.A.Adebo,C.K.Kum,C.N.Lee,Combined resectionofhepatoblastomaandintracavalrightatrialextensionwith profoundhypothermiaandcirculatoryarrest,Eur.J.Cardiothorac.Surg.5(12) (1991)657–659.

[10]T.B.Lautz,T.Ben-Ami,N.Tantemsapya,Y.Gosiengﬁao,R.A.Superina, SuccessfulnontransplantresectionofPOST-TEXTIIIandIVhepatoblastoma, Cancer117(9)(2011)1976–1983.

[11]J.G.Fortner,M.H.Shiu,D.W.Kinne,D.K.Kim,E.B.Castro,R.C.Watson,etal., Majorhepaticresectionusingvascularisolationandhypothermicperfusion, Ann.Surg.180(1974)644–652.

[12]R.Pichlmayr,H.Grosse,J.Hauss,G.Gubernatis,P.Lamesch,H.J.Bretschneider, Techniqueandpreliminaryresultsofextracorporealliversurgery(bench procedure)andofsurgeryontheinsituperfusedliver,Br.J.Surg.77(1990) 21–26.

[13]L.Hannoun,Y.Panis,P.Balladur,E.Delva,J.Honiger,E.Levy,etal.,Exsitu in-vivoliversurgery,Lancet337(1991)1616–1617.

[14]J.Belghiti,B.Dousset,A.Sauvanet,E.Lipinska,J.Aschehoug,F.Fekete, Preliminaryresultswith‘exsitu’surgeryforhepatictumors:analternative betweenpalliativetreatmentandlivertransplantation?Gastroenterol.Clin. Biol.15(1991)449–453.

[15]R.Raab,H.J.Schlitt,K.J.Oldhafer,A.Bornscheuer,H.Lang,R.Pichlmayr, Ex-vivoresectiontechniquesintissue-preservingsurgeryforliver malignancies,LangenbecksArch.Surg.385(3)(2000)179–184.

[16]N.N.Vladov,V.I.Mihaylov,N.V.Belev,V.M.Mutafchiiski,I.R.Takorov,S.K. Sergeev,etal.,Resectionandreconstructionoftheinferiorvenacavafor neoplasms,WorldJ.Gastrointest.Surg.27(4)(2012)96–101(4).

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