Hepatoblastoma of the adult with pericardial metastasis: A case report

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InternationalJournalofSurgeryCaseReports20(2016)80–83

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

Hepatoblastoma

of

the

adult

with

pericardial

metastasis:

A

case

report

Andrea

Celotti

,

Gian

Luca

Baiocchi,

Marco

Ceresoli,

Michele

Bartoli,

Silvia

Ulinici,

Nazario

Portolani

DepartmentofMedicalandSurgicalSciences,SurgicalClinic,UniversityofBrescia,25123Brescia,Italy

a

r

t

i

c

l

e

i

n

f

o

Articlehistory:

Received10December2015 Accepted13January2016 Availableonline19January2016

Keywords: Hepatoblastoma

Hepatoblastomaoftheadult Livertumor

Diaphragmrepair Biologicmesh

a

b

s

t

r

a

c

t

BACKGROUND:Hepatoblastomaisthemostfrequentlivertumorinchildren,butveryrareintheadult andassociatedwithanunfavorableprognosis.Thediagnosisisalwayspostoperativeorpostmortemand biopsyisnotuseful.Surgeryistheonlyacceptedtreatment.

CASEPRESENTATION:Ourpatientunderwentsurgeryinthesuspectoflivermetastasisfromaprevious gastriccancer.Surgeryconsistedinleftlobectomywithpartialdiaphragmresectionandpartial peri-cardiectomyforapericardiallesion,foundaftertheopeningofthethorax.Thediaphragmdefectwas correctedwithabiologicalmesh.

RESULTS:Thehistopathologicalexaminationindicatedhepatoblastomaoftheadultwithpericardial metastases.Thepatientwasasymptomaticandwithoutrecurrenceafter21monthsoffollowup. CONCLUSION:Thehepatoblastomaoftheadultisrelatedtoapoorprognosiswithmediansurvivaltime lessthan5months.Surgeryistheonlycurativetreatment,butinmanycasestumorresectionrequires complexoperations.Vascularandthoracicexpertisecouldbeusefulinthemanagementof hepatoblas-toma.

©2016TheAuthors.PublishedbyElsevierLtd.onbehalfofIJSPublishingGroupLtd.Thisisanopen accessarticleundertheCCBY-NC-NDlicense(http://creativecommons.org/licenses/by-nc-nd/4.0/).

1. Introduction

Hepatoblastoma(HB)isararetumor,butthemostfrequentliver tumorinchildhood.

Itsusuallyoccursininfantsunder3yearsofageandthe5years overallsurvivalrateinchildrenwithnondisseminatedtumorsis closeto70%[1].

Hepatoblastomainadultisveryrare,withanunfavorable prog-nosis[2].Inliteratureaboutsixtycaseshavebeendescribed. In 1958Barnettetal.[3]reportedthefirstcaseinayoungman,who diedafteronemonthfromthediagnosisduetoacompleteliver replacebythetumorspreading.TheprognosisofHBintheadultis extremelypoor,becausemostcasesareunresectableatthe diag-nosisandnon-surgicaltreatmentshaveonlyapalliativerole[4].

Thisreportdescribesacaseofa68year-oldmanwho under-wenttotalgastrectomyforgastriccancer.Subsequently,thepatient underwent leftliver lobectomy after1 year forsuspected liver metastases, but the definitivehistological examination showed hepatoblastoma.

Abbreviations: HB,hepatoblastoma;MRI,magneticresonanceimaging;HCV, hepatitsCvirus;HBV,hepatitisBvirus;GOT,glutamicoxaloacetictransaminase; GPT,glutamicpyruvictransaminase;AFP,alpha-fetoprotein;PO,postoperative.

∗ Correspondingauthorat:DepartmentofMedicalandSurgicalSciences,Surgical Clinic,UniversityofBrescia,IIIChirurgia,SpedaliCivilidiBrescia,P.leSpedaliCivili 1,25123Brescia,Italy.Fax:+39030396011.

E-mailaddress:ceil85@hotmail.com(A.Celotti).

2. Casereport

This is the case of a 68 year-old man who underwent, in January2012atanotherInstitution,totalgastrectomywith Roux-en-YesophagojejunostomywithD2lymphadenectomyforgastric cancer.Histologicalexaminationshowedgastricadenocarcinoma, intestinaltype,withlowdifferentiation(G3),withmetastasisto theregionalnodes(pT3N3M0).

Patient received adjuvant chemotherapy with 10 cycles of FOLFOX.InDecember 2012,during theoncologicalfollow-up, a suspected2cmlivermetastaseswasdiscovered.Forthisreason, the patient started 8 cycles of FOLFIRI. In October 2013, new MRIrevealed anincreased livermetastases upto 5cm (Fig.1). Noothercancerlocalizationswerefound.Patientwasreferredto ourhospital.Bloodexaminationsrevealednegativeserologyfor HCVandHBV,normallevelsofGOT,GPT,bilirubin,albumin.AFP levelwas1231UI/mL.Hehad nohistory ofalcohol intake.MRI wasre-evaluatedbyhepato-biliarydedicated-radiologistsandthe malignant natureofthelesion, withthefeatures of metastases wasconfirmed.InNovember2013thepatientunderwentsurgery. Peritonealexplorationrevealedalargesolidlesionofthesecond liversegment,completelyexophytic,withdiaphragminfiltration (Fig.2).Intraoperativeliverultrasoundwasnegativeforoccultliver metastasesandnoperitonealcarcinomatosisorothercancer local-izationswerefound.Theoperationconsistedofleftlobectomyand diaphragmresection.

http://dx.doi.org/10.1016/j.ijscr.2016.01.010

2210-2612/©2016TheAuthors.PublishedbyElsevierLtd.onbehalfofIJSPublishingGroupLtd.ThisisanopenaccessarticleundertheCCBY-NC-NDlicense(http://

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A.Celottietal./InternationalJournalofSurgeryCaseReports20(2016)80–83 81

Fig.1.RMNimaging:typicalradiologicalaspectofalivermetastasis.NootherlocalizationsareevidentintheRMN.

Fig.2. ExophiticlesionoftheIIsegmentwithdiaphragminfiltration.

Thefirststepwastheincisionofthediaphragmsurroundingthe liverlesion,untilacompletedetachmentofthetumoren-blocwith apieceofdiaphragm.Duringthesurgery,throughthe diaphrag-maticdefect,apericardialnoduleofabout4cm wasdiscovered alongtheinfero-lateralcardiacmargin(Fig.3).

Althoughthepresenceofasecondneoplasticlocalization,aR0 surgerywasconsideredfeasible.Inferiorcavaveinwasisolated until rightathrium and anextended pericardiectomy was per-formed,fromtheupperrightathriumtotheinferiorcardiacmargin. Topreventcardiactamponade,theparietalpleurawassuturedto thepericardialmargin.Finallyleftliverlobectomywascompleted. Thedefectofthediaphragmwasrepairedusinga 10×10cmof bovinepericardium(Fig.4),suturedwithEthyleneTerephthalate. Abdominalandthoracicdrainswereplaced.Postoperativecourse wasuneventfulanddrainswereremovedon7thpostoperativeday (PO).Thepatientwasdischargedon13thPO,withoutproblems. AFPlevelwasremarkablydecreasedat290UI/mLatthedischarge. Themacroscopicpathologyreportshoweda6×4cmlivermass anda3.5_×3cmpericardialnodewiththesamehistological fea-tures. Bothlesions werecomposed by double cell-populations: columnarandvacuolated cellsandpleomorphic cells.Extensive

Fig.3. Pericardialnodule.TheHarringtonretractorholdsthehearttopermitthe exposureoftheinferiorcavavein.

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Fig.5.(A,B)Hematoxylineosinofthelivertumor;(C,D)anti-hepatociticantigenandalphaphetoproteinpositivityrespectivelyofthelivertumor;(E,F)hematoxylineosin pericardialmetastasis.

necrosisandcalcificareaswerefound.Immunohistochemistrywas positivefor:anti-hepatociticantigen,alphaphetoprotein,vimentin, CD10andcytokeratin8e19andnegativeforcytokeratin7, cytoker-atin20,CDX-2,PLAP,betaHCG,OCT4,TCL1,CD30,calretinin.Final diagnosiswasadulthepatoblastomawithpericardialmetastasis (Fig.5).Wediscussedthecasewiththeoncologistsandno adju-vanttherapywasstarted.After21monthspatientisdisease-free andasymptomatic.

3. Discussion

Hepatoblastomaisthemostcommonlivertumorinchildren, andoccursespeciallyinpatientsunder5yearsofage[1].

Nowadaysinchildren,theadvancesinchemotherapy,especially toallowcompletesurgicalresectioneveninadvancedtumors,have increasedtheoverallsurvivalfrom30%toover80%[5].Inadult, HBisveryrareandinliteratureonly62casesaredescribedand presentsanextremelypoorprognosis.

PatientswithHBusuallyhaverightupperquadrantpain,often inpresenceofabdominalmass.Insomecasesacutepresentation withhemoperitoneumisreported[6–8].

Imagingisnotusefulfor thediagnosis,becauseHBpresents similarcharacteristicstootherscommonlivertumorssuchas hep-atocarcinomaormetastases[8].

InthiscaseMRIwastypicalforamalignantlesionwiththe fea-turesof asecondary livertumor,especially thepresenceofthe T1-hyperintensityperipheralringwasstronglysuspectedfor sec-ondarism.BloodexaminationofthepatientrevealedanAFPlevel of1231UI/mL,withoutliverdiseaseandhepresentedahistoryof gastriccancer.Thisdatacouldberelatedtothepreviousoncologic history,becauseinliteraturearereportedcasesofgastriccancer alpha-fetoproducing[9–11].AnywayYamazakietal.[12]declared thatAFPlevelswereoftenelevatedinHB,soitcouldbeusedasa serologicalmarkerlikeforthehepatocarcinoma.

Consideringitsrarity,thelackofparticularradiologicalfeatures andtheuncertainroleofAFP,thepreoperativediagnosisofHBin theadultisquiteimpossiblewithoutsurgeryorbiopsy.

Anywaytheuseofthebiopsyisdebatableinliterature,because inthepresenceofapotentiallyresectablelivermalignancy,surgery shouldbeperformedandthepostoperativediagnosisconsidered adequate.Mondragonetal.[13]reportedacaseofapatientdied afterpercutaneousbiopsyformultifocalHB.

The core treatment of this tumor is surgery as in pedi-atricpatients. Inchildrensurgeryisusuallyperformedafterpre

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operativechemotherapy, butin theadultthere is no standard-izedmanagement ofHB.In many cases,in ordertoachieve an R0resection,majorhepatectomiesoren-blocresectionsof adja-centorganssuchasdiaphragm,stomach,esophagus,pericardium, orspleenareneeded.Andersonetal.[14]reportedacaseofan extensiveresectionoftheleftliverlobe,withdemolitionofleft diaphragm, partialpericardiectomy, distal esophagectomy, par-tialgastrectomy,splenectomyand omentectomy.Regardingthe chemotherapytreatment,thereisnoagreementinliteratureonits use.InthereviewofYamazakietal.[12].noresponseto chemother-apywasobservedinanycase andtheauthorconcludedthatan excisionofthetumorshouldbeconsidered,withoutpreoperative chemotherapyandtumorpuncture.

InsteadNakamura etal.[4]reportedacaseofapatientwith a4yearssurvivalaftermultimodaltreatmentwithneoadjuvant chemotherapy,liverresectionandpostoperativechemotherapy.

HBpresentsapoorprognosisintheadultduetoitsmetastatic behaviororthelocalaggressiveness.InthereviewofWangetal. themediansurvivaltimefor27patientswithavailablefollowup was4months,with1-yearsurvivalof29.6%[2].Rougemontetal. [6]reported34patientswithamediansurvivaltimeof2months andwitha1-yearsurvivalof24%.Likeinourcase,inliteratureare reportedjusttwocasesofdiaphragmresectionduetothe inva-sionofthetumor,butnoinformationsaboutreconstructionare discussed[14,15].

Inthiscasethediaphragmdefectwastoobiggertoallowadirect closure,soabiologicalmeshofbovinepericardiumwasused.This methodisgenerallyadopted,inourDivision,inthecasesoflarge hepatocarcinomasoftheposteriorsegmentswithgoodresults.

Inliteraturetheuseofbovinepericardialmeshseemtohave goodoutcomeintermsofbiocompatibility,high-tensilestrength and easy use, so it could be recommended in many cases of diaphragmreconstruction[16–18].

Inthecaseofourpatienttheuseofthismaterialwas particu-larlyappropriatebecauseafterpericardiectomy,theheartwould be directly into contact of a soft biological mesh, but with a high-tensilestrength.Inthiscase,thepericardialmetastasectomy allowedacurativesurgery.Thepatient,after21monthsoffollow up,wasasymptomaticandwithoutrecurrence.Thisisthecaseof metastatichepatoblastomawiththelongerdisease-freesurvivalin theliterature.

4. Conclusion

Theprognosisofthehepatoblastomaintheadultis unfavor-ablewithmediansurvivaltimelessthan5months.ManyHBare unresectablesatdiagnosis,becauseoflocalaggressivebehavioror metastaticspread.Surgeryrepresentsthegoldstandardtreatment, butinmanycasestumorresectionrequirescomplexoperations. Vascularandthoracicexpertisecouldbeusefulinthemanagement ofhepatoblastoma.

Conflictofinterest

Theauthorsdeclarenopotentialconflictsofinterestrelevantto thisarticle.

Funding

Nofundingareemployed.

Ethicalapproval

Thisisnotaresearchstudy. Consent

Weobtainedawrittenandsignedconsenttopublishthiscase report.

Authorcontribution

AlltheAuthorsmadesubstantialcontributionstoconception anddesignofthestudy,CelottiA.andCeresoliM.conceivedthe studyand wrotethepaper; BartoliM.andUlinici S. performed Literaturesearch;BaiocchiG.L.andPortolaniN.reviewedthepaper. Guarantor

AndreaCelotti. References

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