A critical review ' , Critical Reviews in Oncology / Hematology , vol. 110 , pp. 94-105 . https://doi.org/10.1016/j.critrevonc.2016.12.002

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https://helda.helsinki.fi

ECCO Essential Requirements for Quality Cancer Care : Soft Tissue Sarcoma in Adults and Bone Sarcoma. A critical review Andritsch, Elisabeth

2017-02

Andritsch , E , Beishon , M , Bielack , S , Bonvalot , S , Casali , P , Crul , M , Delgado-Bolton , R , Donatih , D M , Douis , H , Haas , R , Hogendoorn , P , Kozhaeva , O , Lavender , V , Lovey , J , Negrouk , A , Pereira , P , Roca , P , de Lempdes , G R , Saarto , T , van Berck , B , Vassal , G , Wartenberg , M , Yared , W , Costa , A & Naredi , P 2017 , ' ECCO Essential Requirements for Quality Cancer Care : Soft Tissue Sarcoma in Adults and Bone Sarcoma.

A critical review ' , Critical Reviews in Oncology / Hematology , vol. 110 , pp. 94-105 . https://doi.org/10.1016/j.critrevonc.2016.12.002

http://hdl.handle.net/10138/177883

https://doi.org/10.1016/j.critrevonc.2016.12.002

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CriticalReviewsinOncology/Hematology110(2017)94–105

ContentslistsavailableatScienceDirect

Critical Reviews in Oncology/Hematology

j ourna l h o m e pa g e : w w w . e l s e v i e r . c o m / l o c a t e / c r i t r e v o n c

ECCO Essential Requirements for Quality Cancer Care: Soft Tissue Sarcoma in Adults and Bone Sarcoma. A critical review

Elisabeth Andritsch

^a

, Marc Beishon

^b

, Stefan Bielack

^c

, Sylvie Bonvalot

^d

, Paolo Casali

^e

, Mirjam Crul

^f

, Roberto Delgado-Bolton

^g

, Davide Maria Donati

^h

, Hassan Douis

ⁱ

, Rick Haas

^j

, Pancras Hogendoorn

^k

, Olga Kozhaeva

^l

, Verna Lavender

^m

, Jozsef Lovey

ⁿ

,

Anastassia Negrouk

^o

, Philippe Pereira

^p

, Pierre Roca

^q

, Godelieve Rochette de Lempdes

^r

, Tiina Saarto

^s

, Bert van Berck

^t

, Gilles Vassal

^u

, Markus Wartenberg

^v

, Wendy Yared

^w

, Alberto Costa

^x

, Peter Naredi

^y,∗

aInternationalPsycho-OncologySociety(IPOS);ClinicalDepartmentofOncology,UniversityMedicalCentreofInternalMedicine,MedicalUniversityof Graz,Graz,Austria

bEuropeanSchoolofOncology,Milan,Italy

cEuropeanSocietyforPaediatricOncology(SIOPE);CentreforChild,YouthandWomen’sMedicine,StuttgartCancerCentre,ClinicStuttgart–Olgahospital, Stuttgart,Germany

dEuropeanSocietyforSurgicalOncology(ESSO);DepartmentofSurgery,InstitutCurie,PSLResearchUniversity,Paris,France

eEuropeanSocietyforMedicalOncology(ESMO);AdultMesenchymalTumourMedicalOncologyUnit,NationalCancerInstitute,Milan,Italy

fEuropeanSocietyofOncologyPharmacy(ESOP);OLVG,DepartmentofClinicalPharmacy,Amsterdam,TheNetherlands

gEuropeanAssociationofNuclearMedicine(EANM);DepartmentofDiagnosticImaging(Radiology)andNuclearMedicine,SanPedroHospitalandCentre forBiomedicalResearchofLaRioja(CIBIR),UniversityofLaRioja,Logro˜no,LaRioja,Spain

hEuropeanMusculo-SkeletalOncologySociety(EMSOS);RizzoliOrthopaedicInstitute,UniversityofBologna,Bologna,Italy

iEuropeanSocietyofRadiology(ESR);DepartmentofRadiology,UniversityHospitalBirmingham,Birmingham,UnitedKingdom

jEuropeanSocietyforRadiotherapyandOncology(ESTRO);NetherlandsCancerInstitute,Amsterdam,TheNetherlands

kEuropeanSocietyofPathology(ESP);LeidenUniversityMedicalCenter,Leiden,TheNetherlands

lEuropeanSocietyforPaediatricOncology(SIOPE);EuropeanCanCerOrganisation(ECCO),Belgium

mEuropeanOncologyNursingSociety(EONS);DepartmentofHealthandLifeSciences,OxfordBrookesUniversity,Oxford,UnitedKingdom

nOrganisationofEuropeanCancerInstitutes(OECI);NationalInstituteofOncology,Budapest,Hungary

oEuropeanOrganisationforResearchandTreatmentofCancer(EORTC),Belgium

pCardiovascularandInterventionalRadiologicalSocietyofEurope(CIRSE);ClinicforRadiology,Minimally-InvasiveTherapiesandNuclearMedicine, SLK-ClinicsHeilbronn,Karl-Ruprecht-UniversityofHeidelberg,Heilbronn,Germany

qEuropeanCanCerOrganisation(ECCO),Belgium

rInternationalSocietyofGeriatricOncology(SIOG);UnitéFonctionnelledeSoinsOncologiquesdeSupport,InstitutCurie-HôpitalRenéHuguenin,Saint Cloud,France

sEuropeanAssociationforPalliativeCare(EAPC);ComprehensiveCancerCenter,DepartmentofPalliativeCare,UniversityofHelsinkiandHelsinki UniversityHospital,Helsinki,Finland

tECCOPatientAdvisoryCommittee(PAC),UnitedKingdom

uEuropeanSocietyforPaediatricOncology(SIOPE);GustaveRoussyInstitute,Paris,France

vSarcomaPatientsEuroNet(SPAEN);ECCOPatientAdvisoryCommittee(PAC)

wAssociationofEuropeanCancerLeagues(ECL),Belgium

xEuropeanSchoolofOncology(ESO),Milan,Italy

yEuropeanCanCerOrganisation(ECCO);DepartmentofSurgery,InstituteofClinicalSciences,SahlgrenskaAcademy,UniversityofGothenburg, Gothenburg,Sweden

Contents

Preamble...96

Essentialrequirementsforqualitycancercare:sarcomasummarypoints...96

1. Introduction...96

1.1. Whyweneedqualityframeworks...96

2. Softtissuesarcomasinadultsandbonesarcomas:keyfactsandchallenges...97

2.1. Keyfacts...97

2.2. Diagnosisandtreatment...97

∗Correspondingauthor.

E-mailaddress:peter.naredi@gu.se(P.Naredi).

http://dx.doi.org/10.1016/j.critrevonc.2016.12.002

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2.3. Challengesinsarcomacare...97

2.3.1. Accesstospecialists...97

2.3.2. Diagnosis ... 97

2.3.3. Treatment...97

2.3.4. Inequalities...98

2.3.5. Youngpeople...98

2.3.6. Survivorship...98

3. Organisationofcare...98

3.1. Sarcomaunits/centres...98

3.2. Carepathwaysandtimelines...98

3.3. Europeannetworksandsocieties...99

3.4. Themultidisciplinaryteam...99

4. DisciplineswithinthecoreMDT ... 99

4.1. Radiology/imaging...99

4.2. Interventionalradiology...99

4.3. Pathology...100

4.4. Surgery...100

4.5. Radiotherapy...100

4.6. Medicalandpaediatriconcology...101

4.7. Nursing...101

5. DisciplineswithintheexpandedMDT...101

5.1. Nuclearmedicine...101

5.2. Geriatriconcology ... 101

5.3. Oncologypharmacy...102

5.4. Psycho-oncology...102

5.5. Palliativecare...102

5.6. Rehabilitationandsurvivorship...103

6. Otheressentialrequirements...103

6.1. Patientinvolvement,accesstoinformationandtransparency ... 103

6.2. Auditing,qualityassuranceandaccreditation...103

6.2.1. Countryexamples...104

6.3. Educationandtraining...104

6.4. Clinicalresearch ... 104

7. Conclusion...104

Conﬂictofinterest...104

References...104

a r t i c l e i n f o

Articlehistory:

Received4December2016 Accepted5December2016

Keywords:

Sarcoma Softtissuesarcoma Bonesarcoma Paediatriccancer Rarecancer Quality

EuropeanCanCerOrganisation Cancercentre

Cancerunit Europe Carepathways Multidisciplinary Cancerunits Cancercentres Organisationofcare Audit

Qualityassurance Patient-centred Multidisciplinaryteam Multidisciplinaryworking

a b s t r a c t

Background:ECCOessentialrequirementsforqualitycancercare(ERQCC)arechecklistsandexplanations oforganisationandactionsthatarenecessarytogivehigh-qualitycaretopatientswhohaveaspeciﬁc tumourtype.TheyarewrittenbyEuropeanexpertsrepresentingalldisciplinesinvolvedincancercare.

ERQCCpapersgiveoncologyteams,patients,policymakersandmanagersanoverviewoftheelements neededinanyhealthcaresystemtoprovidehighqualityofcarethroughoutthepatientjourney.Refer- encesaremadetoclinicalguidelinesandotherresourceswhereappropriate,andthefocusisoncarein Europe.

Sarcoma:essentialrequirementsforqualitycare

•Sarcomas–whichcanbeclassiﬁedintosofttissueandbonesarcomas–arerare,butallrarecan- cersmakeupmorethan20%ofcancersinEurope,andtherearesubstantialinequalitiesinaccessto high-qualitycare.Sarcomas,ofwhichtherearemanysubtypes,compriseaparticularlycomplexand demandingchallengeforhealthcaresystemsandproviders.Thispaperpresentsessentialrequirements forqualitycancercareofsofttissuesarcomasinadultsandbonesarcomas.

•High-qualitycaremustonlybecarriedoutinspecialisedsarcomacentres(includingpaediatriccancer centres)whichhavebothacoremultidisciplinaryteamandanextendedteamofalliedprofessionals,and whicharesubjecttoqualityandauditprocedures.AccesstosuchunitsisfarfromuniversalinallEuropean countries.

•Itisessentialthat,tomeetEuropeanaspirationsforhigh-qualitycomprehensivecancercontrol, healthcareorganisationsimplementtherequirementsinthispaper,payingparticularattentiontomul- tidisciplinarityandpatient-centredpathwaysfromdiagnosisandfollow-up,totreatment,toimprove survivalandqualityoflifeforpatients.

Conclusion:Takentogether,theinformationpresentedinthispaperprovidesacomprehensivedescription oftheessentialrequirementsforestablishingahigh-qualityserviceforsofttissuesarcomasinadultsand bonesarcomas.TheECCOexpertgroupisawarethatitisnotpossibletoproposea‘onesizeﬁtsall’

systemforallcountries,buturgesthataccesstomultidisciplinaryteamsisguaranteedtoallpatients withsarcoma.

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96 E.Andritschetal./CriticalReviewsinOncology/Hematology110(2017)94–105 Preamble

ECCOessential requirementsfor qualitycancercare(ERQCC) arechecklistsandexplanationsoforganisationandactionsthatare necessarytogivehigh-qualitycaretopatientswhohaveaspeciﬁc tumourtype.

Theyareprimarilyorganisationalrecommendations,notclinical guidelines,andareintendedtogivepolicymakersandmanagers, oncologyteamsandpatientgroupsanon-technicaloverviewofthe elementsneededinanyhealthcaresystemtoprovidehigh-quality carethroughoutthepatientjourney.Referencesaremadetoclinical guidelinesandotherresourceswhereappropriate,andthefocusis oncareinEurope.

ThefoundationofthisECCOrequirementsseriesistheconcept ofquality,whichhasbecomeincreasinglyimportantinallaspects ofhealthcare,asthepopulationhasanincreasingnumberofolder peopleneedingcare,asmanynewandcomplextreatmentscome intouse,andasmorepressureisputonusingresourceseffectively.

Policymakers and patients need to know that their healthcare workforce,technologyandfacilitiesareconﬁguredoptimallyfor eachillness.Inthiscontext,improvingqualitymeansdelivering cancercare thatis timely,safe,effectiveandefﬁcient; putsthe patientatthecentreofcare;andgivesallpeopleequalaccessto high-qualitycare.

Thestructure oftheECCOERQCCseriesisthesameforeach tumourtype:

•Introduction:whyweneedcancerqualityframeworks

•Keyfactsandchallengesassociatedwiththetumourtype,from diagnosistotreatment,tofollow-up

•Organisationofcare:anoverviewofthepatientpathwayand overallrequirementstodelivercare

•Multidisciplinaryworking:inmoredetail,therequirementsfor coreand‘expanded’teamsinvolvedinthepatientpathway

•Measurementandaccountability:qualityassuranceandaudit, patientinvolvementandaccesstoinformation.

Essentialrequirementsforqualitycancercare:sarcoma summarypoints

•Sarcomas–whichcanbeclassiﬁedintosofttissueandbonesar- comas–arerare,butallrarecancersmakeupmorethan20%of cancersinEurope,andtherearesubstantialinequalitiesinaccess tohigh-qualitycare.Sarcomas,ofwhichtherearemanysubtypes, comprisea particularlycomplexanddemandingchallengefor healthcaresystemsandproviders.Thispaperpresentsessential requirementsforqualitycancercareofsofttissuesarcomasin adultsandbonesarcomas.

•High-qualitycaremustonlybecarriedoutinspecialisedsarcoma centres(includingpaediatriccancercentres)whichhavebotha coremultidisciplinaryteamandanextendedteamofalliedpro- fessionals,andwhicharesubjecttoqualityandauditprocedures.

AccesstosuchunitsisfarfromuniversalinallEuropeancountries.

•Itisessentialthat,tomeetEuropeanaspirationsforhigh-quality comprehensivecancercontrol,healthcareorganisationsimplementtherequirementsinthispaper,payingparticularattention tomultidisciplinarityandpatient-centredpathwaysfromdiagnosisandfollow-up,totreatment,toimprovesurvivalandquality oflifeforpatients.

1. Introduction

1.1. Whyweneedqualityframeworks

Therehasbeenagrowingemphasis ondrivingupqualityin cancerorganisations,giventhatthereiswideagreementthatmuch careisnotcomprehensivelyaccessible,notwellcoordinatedand notbasedoncurrentevidence.Thisisthestartingpointofareport bytheUSInstituteofMedicine(IOM)in2013(Levitetal.,2013), whichisbluntindescribinga‘crisisincancercaredelivery’,asthe growingnumberofolderpeoplewillmeanrisingcancerincidence andnumbersofsurvivors,whiletherearepressuresonworkforces amidrisingcostsofcareandcomplexityoftreatments.

Notleast,theIOMnotesthatthefewtoolscurrentlyavailable forimprovingthequalityofcancercare−qualitymetrics,clinical practiceguidelinesandinformationtechnology−arenotwidely usedandallhaveseriouslimitations.

AnassessmentofthequalityofcancercareinEuropewasmade aspartoftheﬁrstEUJointActiononCancer,theEuropeanPart- nershipforActionAgainstCancer(EPAAC,http://www.epaac.eu), whichreportedin2014thatthereareimportantvariationsinser- vicedeliverybetweenandwithincountries,withrepercussionsin qualityofcare.Factorssuchaswaitingtimesandprovisionofopti- maltreatmentcanexplainaboutathirdofthedifferencesincancer survival,whilecancerplans,forexamplea nationalcancerplan thatpromotesclinicalguidelines,professionaltrainingandquality controlmeasures,mayberesponsibleforaquarterofthesurvival differences.

EPAACpaidparticularattentiontotheimportanceofproviding multidisciplinarycareforeachtumourtype,goingasfarasissuing apolicystatement(Borrasetal.,2014)thatemphasisedtheimpor- tanceofteamworking,ascancercareisundergoinga‘paradigm shift’fromadisease-basedapproachtoapatientcentredone,in whichincreasinglymoreattentionispaidtopsychosocialaspects, qualityoflife,patients’rightsandempowerment,comorbidities andsurvivorship.EPAACfurtherfocusedontheestablishmentof networksofexpertiseinregionswhereitisnotpossibletoestab- lishcomprehensivecentres.AnotherimportantoutcomeofEPAAC isthedevelopmentoftheEuropeanStandardsofCarefor Chil- drenwithCancer(EuropeanSocietyforPaediatricOncology,2009), whichsupportthispaperwherechildrenandadolescentsarecon- cerned.

TheEUJointActiononCancerControl(CANCON,http://www.

cancercontrol.eu),whichreplacedEPAACfrom2014,isalsofocus- ingonqualityofcancercareand isdue topublishin 2017the EuropeanGuideonQualityImprovementinComprehensiveCancer Control.

Countrieshavebeenconcentratingexpertiseforcertaintumour typesindedicatedcentres,orunits,suchasforchildhoodandrare cancers,andmostcompreherehensivecancercentreshaveteams forthemaincancertypes.Forcommonadulttumours,however, attheEuropeanleveltherehasbeenwidespreadefforttoestablish universal,dedicatedunitsonlyforbreastcancer,followingseveral Europeandeclarationsthatsetatargetoftheyear2016forcareof allwomenandmenwithbreastcancertobedeliveredinspecialist multidisciplinarycentres.Whilethistargethasbeenfarfrommet (Cardosoetal.,2016),theviewofECCO’sessentialrequirements expertgroupisthatthedirectionoftravelisforalltumourtypes toadopttheprinciplesofsuchdedicatedcare.

Asa rare groupof cancers, manypeople withsarcomasare already referred tospecialistcentres, butthis again isfarfrom universal.Allpatientsmusthaveaccesstothecarepathwaysand

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multidisciplinaryteamsdescribedinthisdocument,andwhichare subjecttosameapproachtoauditing,qualityassuranceandaccred- itationofa‘unit’thatisemerginginbreastcancer.

2. Softtissuesarcomasinadultsandbonesarcomas:key factsandchallenges

2.1. Keyfacts

•Sarcomasarecancersthatareclassedas‘rare’,whichmeansthey haveaprevalence(peoplelivingwiththediseases)offewerthan ﬁvecasesinapopulationof10,000,andanincidenceoffewer thansixin100,000a year(Rare CancersEurope,http://www.

rarecancerseurope.org/About-Rare-Cancers).

•Sarcomasareamongthelargestgroupsofrarecancers.Thereare twomaincategories:softtissueandbonesarcomas.

Softtissuesarcomasarecancersthatoccurinmanypartsof thebody.Theyaremalignanciesofmesenchymal(supporting) tissuesandarenamedbythesiteortypeoftissueaffected.

Gastrointestinalstromaltumour(knownasGIST,andoneof themostfrequentsarcomas),affectsthewallofthegastroin- testinaltractandis usuallyputintoa separatecategoryto othersarcomas.Theincidenceofadultsofttissue sarcomas isabout4per100,000ayearinEuropeandtheycomprise morethan80%ofsarcomas.Theyaredistinctfromchildhood softtissuesarcomas− thelatterarecommontypesofrare paediatriccancers and havedifferent characteristics,treat- mentprotocolsandguidelinesandsoarenotincludedinthis document;seetheEuropeanSocietyforPaediatricOncology (SIOPE,https://www.siope.eu)andtheSIOPE strategicplan (Vassaletal.,2016)formoreinformation.

•Bonesarcomasareprimarycancersthatarisefrombone.Theyare lesscommonthanadultsofttissuesarcomas,comprisingabout 15%ofsarcomasinEurope.Themostcommontypesareosteosar- comaandEwingsarcoma,whichhavethehighestincidencein adolescentsandyoungadults,andareincludedinthisdocument astreatmentstrategiesaresimilartothoseforadults.Themost commonadultbonesarcomaischondrosarcoma.Otherbonesar- comasincludeundifferentiatedpleomorphicsarcomasofbone (UPS),chordomasandgiantcelltumoursofbone.TheEuropean StandardsofCareforChildrenwithCanceralsoapplytobone sarcomasinchildrenandadolescents.

•There are dozens of types of adult soft tissue sarcomas and adult/childbonesarcomas,withwidelydifferentpatternsofstage atdiagnosis,prognosisandtreatments.TheEurocare-5survival study(Bailietal.,2015)givesa60%5yearsurvivalforcancers classedasarisingfrom‘softtissue’andjustover50%forthose classiﬁedasarisingfrom‘bonesandcartilages’,indicatingthat aswhole,sarcomasareinthemid-toupper-levelin5yearsur- vivalrates.Detaileddatahavebeenpublishedfortheﬁrsttimein 2013byRARECARE(http://www.rarecare.eu),whichcarriesout surveillanceofrarecancersinEurope.Itfoundthattheincidence ofalltypeofsarcomaisabout6in100,000,with28,000newcases ayearinEurope;in2008,280,000peoplewereestimatedtobe alivefollowingadiagnosis.Detailsof5yearsurvivalofvarious typesandsitesofsarcomaaregiveninaRARECAREpaper(Stiller etal.,2013).

•Thecauseofmostsarcomasisunknown.Halfofpatientshavean excessofpathogenic(andpotentiallyaetiological)germlinevari- ants(Ballingeretal.,2016).Riskfactorsforsofttissuesarcomas includeage(aboutonethirdarediagnosedinpeopleaged65and older,andthisgrouphasthelowestsurvivalratesformostsar- comas),previousradiationtreatment,previouscancers,andrare geneticconditionsthatarepresentinfamilies.Kaposi’ssarcoma iscausedbyavirusandmainlyseeninpeoplewithHIVinfection,

andshouldbedistinguishedfromothersarcomas.Osteosarcoma inolderpeoplemaybeassociatedwithPaget’sdisease.

2.2. Diagnosisandtreatment

•Symptomsofadultsofttissuesarcomasincludelumpsandpain.

Diagnosisisbyimagingandbiopsy.Commonsymptomsofbone sarcomasarepain,swellingandproblemswithmovement.

•Adiverserangeoftreatmentsarecarriedoutforthemanytypes ofsarcoma(Casali,2016).Surgeryisthemaintreatmentformost sarcomas,andcanincludelimb-sparingoperationsoramputa- tionwhere,infrequently,thisistheonlyoptiontoeliminatethe cancer.Chemotherapyandradiotherapymayalsobeusedbefore surgery(todevitalisetumours)andafter(topreventrecurrence) dependingonthehistologyandtheriskofrelapse.Severaltar- getedtherapiesareusedinsarcomas,notablyimatinibtotreat GIST.

2.3. Challengesinsarcomacare 2.3.1. Accesstospecialists

•Anoverallchallengeforsarcomasistheavailabilityofexperts andmultidisciplinarygroupsandnetworks.Thisisoftenthecase withrarediseasessuchassarcomas,andsomesmallercountries mayevenlackaspecialisedsarcomaunit.

2.3.2. Diagnosis

•Therarityofsarcomas,thelargenumberoftypes,andoftenvague symptomsmeanthatmostprimarycaredoctorswillinfrequently encountera personwithsarcoma.Further,abenign diagnosis mayoutnumberthediagnosisofsarcomabyafactorof100.This canresultinlatediagnosesanddelayedreferrals.

•Radiologistsandpathologistsspecialisinginsarcomasplayacru- cialroleinthecorrectdiagnosisofsarcomas,butareusuallybased onlyinafewcentres.Surgicalbiopsiesnotperformedbyexperts canleadtocomplications,impairmentstosubsequenttreatments andpossiblytumourspread.Astudyfrom2012(Ray-Coquard etal.,2012)concludedthatmorethan40%ofﬁrsthistological diagnosesweremodiﬁedatsecondreading,possiblyresultingin differenttreatmentdecisions,andtheECCOexpertgroupstresses thatdiagnosismustonlytakeplaceinsarcomacentresorpaedi- atriccancercentreswithexpertiseintreatingsarcomas(Beishon, 2013).

•Insum,therecanbeprofoundimplicationsforapatientnotdiag- nosedatasarcomacentre,suchasmissingthechanceofatimely diagnosisofapotentiallycurabledisease,andbeingsparedmore extensivesurgery.

2.3.3. Treatment

•Surgeryforsarcomascanbedifﬁcultandneedshighlyexperi- encedsurgeonstoachievethebestoutcomes.Astudyfrom2004 (Ray-Coquardetal.,2004)showedthatmorethan50%ofsoft tissuesarcomapatientsarenotcorrectlyoperatedon.

•Aftersometimewithlittlechangeindrugtreatments(mainly chemotherapy)formetastaticsarcomas,therearenowseveral newsystemicandtargeteddrugsforadultsofttissuesarcomas, eitherapprovedorthatshowpromiseinclinicaltrials,following advancesinunderstandingthemolecularbiologyofsarcomas, and medical oncologistsface increasingly complex treatment choices.Asabouthalfofpatientswithintermediateandhigh- gradesarcomaswillhavearecurrence,theirbestmanagementis crucial.

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98 E.Andritschetal./CriticalReviewsinOncology/Hematology110(2017)94–105

•Osteosarcoma,EwingsarcomaandboneUPShaveahighriskof metastaticspread,particularlytothelungsandtodistantbones, andtreatmentaimedatlocalcontroloftheprimarytumouris rarelycurativeunlessintegratedintoamultidisciplinarytreat- mentconceptwithmulti-agentchemotherapy.Uptotwothirds ofpatientsmaybecomelong-term,disease-freesurvivors,pro- videdtheyreceivehigh-qualitymultidisciplinarycare.

2.3.4. Inequalities

•PeoplewithsarcomasinCentralandEasternEuropehavelower 5yearsurvivalratesthanthoseinothercountries.Thisispartic- ularlytrueofbonesarcomasandGIST,andtheRARECAREpaper notesthatoutcomesforbonesarcomas,inparticular,dependon multidisciplinaryteams,whichmaybelackinginanumberof countries,andnotonlyinCentralandEasternEurope(Stilleretal., 2013).

2.3.5. Youngpeople

•Whilepaediatriccancerunitsareavailableinmanycountries, unitswithexpertiseandappropriatefacilitiestomeettheneeds ofadolescentsand youngadults(AYA)arefewer,butarealso required.

2.3.6. Survivorship

•AlthoughthenumberofpeopleinEuropewhohavehadtreat- mentforsarcomaissmallcomparedwiththosewhohavehad commoncancers, survivors can have a wide range of needs, includingrehabilitationandsurveillanceforlatetoxicities.

3. Organisationofcare

Essentialrequirementsfortheorganisationofsarcomacareare:

•Cancercarepathwaysthatcovertheentirepatientjourney

•Timelinessofcare

•Minimumcasevolumesforsarcomacentres

•Multidisciplinary team working including core and extended groupsofprofessionals,indedicatedsarcomacentresorunits

•Auditandqualityassuranceofoutcomesandcareprocesses

•Education,policiestoenrolpatientsinclinicaltrials,patientinformation.

Thesetopicsareoutlinedinthefollowingsections, withref- erencetonationaland Europeanresourcesandclinicalpractice guidelines,whereappropriate.

3.1. Sarcomaunits/centres

•Itisessentialthattreatmentisorganisedinunitsorcentresthat specialiseinsarcomas,oftentermed‘referencecentres’,which arealsooftenpartofnetworksatanappropriategeographical level(regional,nationalandsupranational).Diagnosisandmany treatmentproceduresmustonlybeperformedinthesarcoma centre,althoughprofessionalsatacentrecanalsobepartofan extendedmultidisciplinaryteam(MDT)coveringotherinstitutes andnetworks.

•Treatmentofchildhoodsarcomasisusuallyorganisedinpaedi- atriccancercentresthatalsotreatotherpaediatriccancers.For thepurposeofthispaper,theterm‘sarcomacentre’alsoapplies topaediatriccancercentres.

•Itisessentialthatthesarcomacentreandthemembersofthe MDThaveasigniﬁcantannualnumberofcasesandthatthecore MDThasmemberswithsarcomasastheironly,oroneoftheir primary,interest(s).Onthebasisofexistingevidence,theexpert

grouprecommendsthatforaninstitutiontobeconsideredasa sarcomacentreitshouldtreatatleast100newsarcomapatients (both soft tissue and bone) a year,although a thresholdwill dependonthestructureofsarcomanetworksinaregionorcoun- tryandthedistributionofexpertise.GuidancefromtheNational InstituteforHealthandCareExcellence(NICE)inEnglandand Walessaysthat MDTsmanagingeithersoft tissuesarcomaor bonesarcomashouldmanagethecareofatleast100newpatients ayear(100softtissueand50bonesarcomasiftheMDTmanages bothtypes),reﬂectingthemorecentralizednatureoftheUK’s healthsystem(NationalInstituteofHealthandCareExcellence, 2006).Notethatowingtotherarityofpaediatriccanceringeneral andbonesarcomainparticular,minimumcasevolumesarenec- essarilydifferentbetweenadultandpaediatrictreatmentcentres.

•RareCareNet,aEuropeanUnioninformationnetworkonrarecan- cers,hassetoutcriteriaforasarcomareferralcentre,andwhich arediscussedina paper,‘Accreditationforcentresofsarcoma surgery’(Sandruccietal.,2016).

3.2. Carepathwaysandtimelines

•Careforsarcomapatientsmustbeorganisedinpathwaysthat coverthepatient’sjourneyfromtheirpointofviewratherthan thatofthehealthcaresystem,andpathwaysmustcorrespondto currentnationalandEuropeanevidence-basedclinicalpractice guidelines ondiagnosis,treatment and follow-up. (TheEuro- peanPathwayAssociationdefinesacarepathwayas“acomplex interventionfor themutual decisionmakingand organisation ofcareprocessesforawell-definedgroupofpatientsduringa well-definedperiod”.Thisbroaddefinitioncoverstermssuchas clinical,critical,integrated andpatientpathwaysthatarealso oftenused.Seehttp://e-p-a.org/care-pathways).Onesourceof informationoncareorganisationisagainNICE–ithaspublished documents including a manual on improving sarcoma outcomes(NationalInstituteofHealthandCareExcellence,2006),a pathway(http://pathways.nice.org.uk/pathways/sarcoma),anda qualitystandard(seesectiononauditing,qualityassuranceand accreditation).Pathwaysforsofttissueandbonesarcomasare different,andthereareexamplesofsuchpathways(e.g.NHSLon- donandSouthEastSarcomaNetwork,http://www.lsesn.nhs.uk/

sarcoma.html).

•Primarycarepractitioners,generalsurgeonsandmedicaloncol- ogistsareoftenreferrersofthosewithsuspectedsarcomaand needtimelyaccesstoreferencecentres.Themaximumtimefor anappointmentforsuspectedadultcancerinEnglandandWales is2 weeks,forexample. NICEalsorecommendsthat children andyoungpeoplewithsuspectedbonesarcomaonanx-rayare referredwithin48hforanappointmentwithaspecialist,andalso within48hforunexplainedbonepainorswelling.

•Reasonabletimestoreportadiagnosisofsarcomaandtheoppor- tunitytostarttreatmentarecrucialtotimelytreatmentandtothe wellbeingofpatients.ForexampleguidelinesintheNetherlands statethatthemaximumtimefordiagnosticandstagingproce- duresis3weeks,andthemaximumtimefromﬁrstappointment toﬁrsttreatmentis6weeks,butshortertimesshouldbeaimed for.

•Afteradiagnosis,itmustbecleartothepatientwhichprofes- sionalisresponsibleforeachstepinthetreatmentpathwaysand whoisfollowingthepatientduringthejourney(usuallycalleda casemanagerorpatientnavigator)(Albrehtetal.,2015).Inmany countries,casemanagersduringthemainstagesoftreatmentare cancernurses.

•Follow-upandsurvivorshiparemajorissuesinsarcoma.Typi- cally,carepathwaysincludesurveillanceforcancerrecurrence butpatientsoftenhave toseekhelpelsewherefor longterm side-effectsof treatment,bygoingtobothacuteand commu-

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nityfacilities.Continuityandintegrationofallcarebyspecialists mustbeimplementedasgapsinlong-termcarecancausemuch distress.

3.3. Europeannetworksandsocieties

SarcomacentresmustalsoparticipateinEuropeansarcomacare andresearchnetworksandsocieties.Suchorganisationsplayacru- cialroleinpoolingexpertiseinallrarecancers.Insarcoma,research groupsincludetheSoftTissueandBoneSarcomaGroupatEORTC (EuropeanOrganisation for Research and Treatmentof Cancer), andtheEuroEwingConsortium;andprofessionalsocietiesinclude theEuropeanMusculo-SkeletalOncologySociety(EMSOS)andthe ConnectiveTissueOncologySociety(CTOS).

Achallengeissustainabilityofnetworks,andtherarecancer communityhasbeenlobbyingforfunding,includingfromthenew EuropeanReferenceNetworks(ERNs)(Wagstaff,2016)(Blayetal., 2016a).ApplicationsforERNsonrarecancers,includingadultsar- comasandchildhoodsarcomas,arecurrentlybeingreviewedby theEuropeanCommission;qualityofcarerequirementswillbean importantpartoftheworkofthesenetworks.TheEUJointAction onRareCancerswillalsosupportthecreationofERNsintheEU.

3.4. Themultidisciplinaryteam

Treatment strategies for all patients must be decided on, plannedanddeliveredasaresultofconsensusamongacoremul- tidisciplinary team (MDT)that comprises themost appropriate membersfortheparticulardiagnosisandstageofcancer,patient characteristicsandpreferences,andwithinputfromtheextended communityofprofessionals.Theheartofthisdecision-makingpro- cessisnormallyaweeklyormorefrequentMDTmeetingwhere allpatientsarediscussedwiththeobjectiveofbalancingtherec- ommendationsof clinicalguidelines with theoften formidable complexityoftheindividualsarcomapatient.

ToproperlytreatsarcomasitisessentialtohaveacoreMDTof dedicatedhealthprofessionalsfromthefollowingdisciplines:

•Radiology/imaging

•Interventionalradiology

•Pathology

•Surgery

•Radiotherapy

•Medicalandpaediatriconcology

•Nursing.

ThiscoreMDTmeetstodiscuss:

•Allcasesafterdiagnosisandstagingtodecideonoptimaltreat- ment

•All cases prior to local treatment (surgery, radiotherapy or chemotherapy(Gronchietal.,2016))

•Patientsaftermajortreatment,usuallysurgery,todecideonfur- thertreatmentandfollow-up

•Patientswitharecurrenceduringfollow-up,orwherechangesto treatmentprogrammesareindicatedandhavemultidisciplinary relevanceand/orplanneddeviationsfromclinicalpracticeguide- lines.

Inaddition,sarcomaradiologistsshouldparticipateinmeetings wherediscrepanciesbetweenradiologyandhistology,aswellas mistakes,arediscussed.Whenthereisadiscrepancybetweena radiologistnotbasedatthecentreandtheﬁnaldiagnosis,feed- backshouldbeprovidedinanopenandnon-judgmentalmanner, helpingtoraisestandardsamongnon-sarcomaradiologists.

Healthcareprofessionals fromthefollowingdisciplinesmust also be available whenever their expertise is required (the

‘expanded’MDT):

•Nuclearmedicine

•Oncologypharmacy

•Geriatriconcology

•Psycho-oncology

•Palliativecare

•Rehabilitationandsurvivorship.

Thereisalsoanincreasingsub-groupofsarcomasthathavea geneticpredisposition.It maybenecessarysoontoadda clini- calgeneticisttotheexpandedMDTtodiscussoptionsforgenetic testinganditsresultswithpatientsandtheirfamilies.

Alldecisionshavetobedocumentedinanunderstandableman- ner, and should becomepart of thepatient records. It is good practicefordecisionstakenduringMDTmeetingstobemonitored, anddeviationsreportedbacktotheMDTwherethereareproblems.

Itisessentialthatallrelevantpatientdata,suchaspathology reports,meetqualitystandardsandareavailableatthetimeofthe MDTmeeting.

4. DisciplineswithinthecoreMDT

4.1. Radiology/imaging

Radiology/imagingplaysacriticalroleindiagnosing,stagingand follow-upofsarcomasandpersonalisedtreatment.Theroleofthe radiologististoperformandinterpretrelevantimagingprocedures aspartofthediagnosisofsarcomas.

Essentialrequirements:

•Sarcoma centresmust have radiologists who have signiﬁcant expertiseinthediagnosis,stagingandfollow-upofsarcomas

•Radiologistsmusthaveaccesstoimagingmodalitiesrequiredfor diagnosingandstagingofsarcomas(e.g.ultrasound,radiographs, CT,MRI)

•Theradiologistmustknowwhen torefer apatienttonuclear medicine.Inthatcase(referralforbonescintigraphy,SPECT/CT orPET/CT),nuclearmedicinephysiciansand radiologistsmust liaisetoallowjointpatientmanagement,readingandreporting

•Sarcomaradiologistsmustcollaboratewithotherspecialistradi- ologists (e.g. ENT radiologists and paediatric radiologists), as sarcomasaffectawidevarietyoforgansandages

•Imaging and histopathology ﬁndings should be discussed togetherbeforemakingadiagnosis,tominimisediagnosticdis- crepancies.Thisisofparticularimportanceinbonetumoursand tumour-likelesionswhereconditionssuchasmyositisossiﬁcans maybemisinterpretedasosteosarcomaonhistopathology,orin caseswheretheobtainedbiopsymaynotberepresentativeof theentirelesion(Nuovoetal.,1992)(Noebauer-Huhmannetal., 2015)(SLICED,2007)

•Forbonesarcomasandothersarcomasinchildren,adolescents andyoungadults,radiologistsneedexperiencewiththeseage groups.

4.2. Interventionalradiology

Interventionalradiologyplaysanimportantroleinthediagno- sisofsarcomas.Indeed,image-guidedpercutaneouscoreneedle biopsyis crucial inthedeliveryof asafeand efﬁcientsarcoma service,andisthepreferredbiopsytechniqueinthediagnosisof sarcomas.Theroleoftheinterventionalradiologististo:

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skull,spineandsacrum),radiotherapyisrarelyusedinbonesarco- masastheonlycurativetreatment,owingtoradioresistancewhich requireshigherdosesthatincreaseside-effects.Itisimportantto notethatthequalityofradiotherapyissigniﬁcantlyassociatedwith localcontrol,andqualityassuranceismandatoryforthesetypesof cancer(Donaldsonetal.,1998).

•Radiationoncologistsmusthaveexpertiseinsarcomasubtypes andespeciallytheprobabilityoflocalrecurrencepersubtype

•They must know the indications and contra-indications for (neo)adjuvantanddeﬁnitiveradiotherapy,counselpatientsprior tosurgeryonthechoiceofneoadjuvantoradjuvanttherapy,and informpatientsaboutacuteandlateside-effects,andinterven- tionstopreventthemfromhappeningorworsening

•ThecentremusthaveaccesstolatesttechnologiessuchasIMRT, IMATandstereotactic(body)radiotherapy,withastateoftheart mouldroomtomakepersonalisedimmobilisationdevices

•Thecentremustbeabletoperform(daily)onlinesetupveriﬁ- cationprotocolsandtoreactaccordingtodeviationsobserved.

Prospectivequalityassuranceprotocolsmustbeinplace

•Thecentremustorganisetreatmentataproton/heavyionradio- therapycentreifneeded(DeLaneyandHaas,2016)

•Forbonesarcomasandothersarcomasinchildren,adolescents andyoungadults,radiationoncologistsneedsarcomaexperience withtheseagegroups.

4.6. Medicalandpaediatriconcology

Medicaltherapyisneededinmostpatientswithadvanceddis- easeforallsarcomas,invirtuallyallpatientswithosteosarcoma andEwingsarcoma,andinmanyhigh-risksofttissuesarcomaand GISTpatientswithlocalizeddisease(TheESMO/EuropeanSarcoma NetworkWorkingGroup,2014b)(Neuvilleetal.,2014).Itisalso oftenusedasfront-linetherapybeforesurgery.Medicaltherapyis becominghighlyvariabledependingonthepathologicandmolec- ularcharacteristicsof thepatient. Tumourresponse tomedical therapymaypresentpeculiarpatterns,especiallyinbonesarco- mas,GISTandwithsomemolecularlytargetedtherapies insoft tissuesarcomas.

Giventherarityofsarcomas,and thatmedicaltherapyoften takesmanymonthstoadminister,medicaloncologistsshouldbe preparedtoworkinhealthnetworksthatcareforadultpatients closetotheirhome.

•Medicaltherapymustbeplannedandadministeredbyamedi- caloncologist,orapaediatriconcologistforyoungpatients,from thebeginningofthepatient’sjourney,incollaborationwiththe MDTandcloselyfollowingimaging(withregardalsotouncon- ventionalpatternsoftumourresponse)

•Themedical/paediatriconcologistmusthavespecialisedexper- tise in sarcomaswithexperiencefrom workingin a sarcoma referencecentreand/orasarcomareferencenetwork.Sarcomas mustbeamajorcomponentoftheirwork

•Medical/paediatric oncologists must be involved in sarcoma clinicalresearchcollaborativegroupsatanationaland/orinter- nationallevel.

4.7. Nursing

Nursesaretheprofessionalswhospendmosttimecaringfor peoplewithsarcoma,andrequirearangeofroles,owingtothe diversityof tumourtypes andcontextsof care.Theyneed spe-

cialisedknowledgeandskillstonursepeoplereceivingcomplex, multi-modalsarcomatreatments,whichhaveahighdegreeofmor- bidity(Samuel,2018).

•Nurses must conduct holistic nursing assessments to ensure safe,personalisedand age-appropriatenursing care,and pro- vide patientinformation andsupporttopromote self-efﬁcacy throughoutthepatientjourney

•Nursesmustprovideintensivecarefollowingsurgery;carefor patientswhohavehadtissueconservation,boneﬁxation,limb salvage or surgicalreconstruction; care for patientsreceiving high-dosechemotherapy;andcareforpatientsreceivingadju- vantradiationtherapy,includingbrachytherapy(Lahletal.,2008)

•They must alleviatesymptoms of sarcoma (e.g. pain, fatigue, spinal cord compression); prevent or manage side-effects of treatment (e.g. radiation-induced skin injury, change in body appearanceand/orfunction);and carefor patientswith treatment-related complications (e.g. wound infection, ﬂap necrosis,neutropenicsepsis,acutekidneyinjury)

•Whenactingascasemanagers,nursesmustcoordinatecarewith healthprofessionalsoutsidethecoreMDT,includingrehabilita- tion,psychosocial,fertilityandpalliativecareservices(Prades etal.,2015).

5. DisciplineswithintheexpandedMDT

5.1. Nuclearmedicine

Bone scintigraphy, SPECT/CT with various radiotracers, and PET/CTwith¹⁸FFDGand¹⁸F-FNamaybeindicatedincertainsar- comas(musculoskeletalsofttissuesarcomas,bonesarcomasand GIST)forprognosis,staging,treatmentresponseevaluation,and restaging(toconﬁrmlimitedorresectablediseasebeforecurative intenttherapy,and forlocalrecurrenceandmetastases)(Nanni etal.,2009)(GabrielandRubello,2016).

The role of thenuclearmedicine physicianis to oversee all aspectsofbonescintigraphy,SPECT/CTandPET/CTforpatientswho requiretheseprocedures,includingindications,multidisciplinary algorithmsandmanagementprotocols.

•NuclearmedicinephysicianswithexpertiseinPETmustbeavail- abletotheMDT.In2016,mostEuropeanhospitalshaveaccessto PET/CTtechnologybutitshouldpreferablybeon-site,belessthan 10yearsoldandreadyforintegrationinradiationtreatmentplan- ning,andhaveintegratedPACS/RISandupdatedworkstations

•Conventionalnuclearmedicinemustalsobeavailable

•Nuclearmedicinemustbeabletoperformdailyveriﬁcationpro- tocolsandtoreactaccordingly.Quality-assuranceprotocolsmust beinplace.AnoptionforensuringthehighqualityofPET/CTscan- nersisprovidedbytheEuropeanAssociationofNuclearMedicine (EANM)throughEARLaccreditation(Boellaardetal.,2015)

•Forbonesarcomasandothersarcomasinchildren,adolescents and youngadults, nuclearmedicine physicians needsarcoma experiencewiththeseagegroups.

5.2. Geriatriconcology

Asathirdofsofttissuesarcomapatientsareaged65ormore, theMDTmusthaveaccesstogeriatricianswithoncologyexperi- ence.Whilechronologicalageshouldnotbeareasontowithhold

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102 E.Andritschetal./CriticalReviewsinOncology/Hematology110(2017)94–105 effectivetherapy,goalsmayvarysigniﬁcantlyaccordingtoageand

requiresexpertgeriatricinput.

Theroleofthegeriatriconcologististo:

•Ensurethatolderpatientsarescreenedforfrailty

•Coordinaterecommendationstootherspecialistsabouttheneed forpersonalisedtreatmentforfrailpatients.

•Geriatriconcologistsmustensureallolderpatientsarescreened witha simplerisk-assessmentfrailtyscreeningtool (Decoster etal.,2015)(Huismanetal.,2014)withwheneverpossiblean estimationof life expectancytohelp prioritisemedicalinter- ventions (e.g. ePrognosis colorectal screening survey. http://

cancerscreening.eprognosis.org/screening)

•Ageriatriconcologyteam(includinggeriatriciansandotherspe- cialists)mustbeavailableforallfrailpatientsandtheirevaluation discussedinMDTmeetingstoofferpersonalisedtreatment

•Geriatriconcologistsmustensuretheearlyintegrationofpal- liativecareplansorgeriatricinterventions,especiallyfor frail patients.

5.3. Oncologypharmacy

Oncologypharmacyplaysacriticalroleinthecareofsarcoma patients,giventheimportanceofsystemictreatment.Theroleof theoncologypharmacististo:

•Liaisewiththemedicaloncologistand/orpaediatriconcologistto discusspharmaceuticaltreatment

•Supervisethepreparationofoncologydrugs.

•Oncologypharmacistsmustworkcloselywithmedical/paediatric oncologists.Theymusthaveexperiencewithinteractionswith otherdrugs;experiencewithdoseadjustmentsbased onage, liverandkidneyfunction;andknowledgeofcomplementaryand alternativemedicines.Oncologypharmacistsmustcomplywith theEuropeanQuapoSguidelines(EuropeanSocietyofOncology Pharmacy,2014)

•Oncologydrugsmustbepreparedinthepharmacyordesignated areawhichmeetsthecriteriapharmaciesmustcomplywithand dispensingmusttakeplaceunderthesupervisionoftheoncology pharmacist.

5.4. Psycho-oncology

About30%ofsarcomapatientssufferfromanxietyatdiagno- sisandduringtreatmentand20%sufferfromclinicaldepression (ParedesandCanavarroSimões,2010)(Paredesetal.,2012).Treat- mentcanseriouslyaffectqualityoflife,especiallyforthosewho havemajoroperationsonlimbs.Concernsaboutbodyimageare particularlyhighinyoungpeoplewithbonesarcomas.Appropri- atepsychologicalinterventionssuchasmindfulnesstrainingand psycho-educativeprogrammesareneededforthemainsarcoma agegroups.Supportingfamilymembersisalsoessential,particu- larlyforrelationsofchildren,adolescentsandyoungadults.

Theroleofthepsycho-oncologististo:

•Ensure that psychosocial distress (National Comprehensive CancerNetwork,2003),and otherpsychologicaldisordersand psychosocialneeds,areidentiﬁedbyscreening,andareconsid- eredbytheMDT

•Promote effective communication between patients, family membersandhealthcareprofessionals.

•Supportpatientsandfamilymemberstocopewithmultifaceted diseaseeffects

•Facilitatethereintegrationofsarcomasurvivorsinschool,work, social and family environments through evidence-based psy- choeducationalinterventions.

•Patientsmusthaveaccesstoaself-administeredpsychological assessmenttool(‘distressthermometer’)

•Psychosocialcaremustbeprovidedatallstagesofthediseaseand itstreatmentforpatientsandtheirfamiliesandmustbepresent toensurecomprehensivecancercare

•Inpaediatriccancer,itisrecommendedthatpsychosocialsupport includesplaytherapyandaccesstoschooling.

5.5. Palliativecare

About30–50%of patientswithsarcomasdie within5 years fromdiagnosis,andthereisanincreasingneedforpalliativecare throughoutthediseasetrajectory,notonlyat end-of-lifebutat diagnosisandduringcancertreatmentstomanagedistressingclin- icalcomplicationsandsymptomsandtoimprovethequalityoflife ofpatientsandtheirfamilies(Temeletal.,2010)(Huietal.,2015) (QuillandAbernethy,2013)(Coindreetal.,2001).Palliativecare,as deﬁnedbytheWorldHealthOrganization,appliesnotonlyatend oflifebutthroughoutcancercare(seehttp://www.who.int/cancer/

palliative/deﬁnition/en).

Theroleofthepalliativespecialististo:

•Beresponsible forspecialist palliativecare andrecommenda- tionstootherspecialistsregardinggeneralpalliativecare(e.g.

symptomcontrol)

•Beavailableatdiagnosisandtheearlyphaseoftreatment

•Identifypatientsinneedforpalliativecarethroughsystematic assessment of distressing physical, psychosocial and spiritual problems

•Provideearly palliative care in conjunction with cancer spe- ciﬁctreatments,treatdiseaseandtreatment-relateddistressing symptomssuchaspainanddyspnoea,andofferpsychosocialand spiritualcare

•Providesupportforfamilymembers

•Provideend-of-lifecaretogetherwithprimarycarepalliativecare providers.

•Theremustbeapalliativecareteamthatprovidesexpertoutpa- tientandinpatientcare

•Thepalliativecareteammustincludespecialistphysiciansand nurses,workingwithsocialworkers,chaplains,psychotherapists, physiotherapists,occupational therapists, dieticians,pain spe- cialistsandthepsycho-oncologyteam

•Thepalliativecareunitmustcollaboratewithcommunitypallia- tivecareteams

•Allpatientswithseveresymptomsorsuffering,orpatientswith metastaticdiseaseandshortlifeexpectancy(underayear),irre-

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spectiveofthecancertreatmentplan,mustalsobeinthecareof palliativecareteam

•Forbonesarcomasandothersarcomasinchildren,adolescents andyoungadults,thepalliativecareteamneedsexperiencewith theseagegroups.

5.6. Rehabilitationandsurvivorship

Rehabilitationandsurvivorshipplansareoftenomittedinthe planningof clinicaland psychosocialcareforpatientsand their families.Theymustbeintegratedintopathwaystoensurethebest possiblecarecontinuesbeyondinitialtreatment(Stubbleﬁeldetal., 2013)(Bergetal.,2016)(Scottet al.,2013).Amultidisciplinary teaminvolvingclinicians,nurses,psychologistsandphysiothera- pistsmustdiscusswithpatientshowtheirfunctioningwillchange duringandaftertreatment,andoptionsforimprovement.

•Manycancerpatientsarelivinglongeraftertheirtreatment,but theyareoftennotwell-informedaboutlate-effectsandhowtheir livescouldbeaffected.Patientsandtheirfamiliesmustbebet- terinformedaboutpotentiallate-effectsandhowthesecanbe monitoredandtackled

•Wherethelimbsareinvolved,itisimportanttoidentifyrehabil- itationneedsrelatedtomovementanddailyactivities,andplan physicaltrainingaccordingly

•Returntoschoolorworkisimportantformanycancerpatients andhasbothfinancialandwellbeingbenefits.Employersmust haveearlydiscussionsaboutflexibilityinreturningtowork,such aschangingjobdutiesandworkinghours,includingforfamilies ofpatientswithpaediatriccancer

•Rehabilitationandsurvivorshipofcancermustbeintegratedinto nationalcancerplans.

6. Otheressentialrequirements

6.1. Patientinvolvement,accesstoinformationandtransparency

•Patientsmustbeinvolvedineverystepofthedecision-making process. Their satisfaction with their care must be assessed throughoutthepatientcarepathway.Patientsmustbeoffered relevantandunderstandableinformationtohelpthemappreci- atetheprocessthatwillbefollowedwiththeirtreatmentfrom thepointofdiagnosis.Theymustbesupportedandencouragedto engagewiththeirhealthteamtoaskquestionsandobtainfeed- backontheirtreatmentwhereverpossible.Childrenneedtobe involvedinanage-appropriatemannerandtheirparents/carers shouldbeincludedintheprocessasappropriate.

•Itis alsoessentialthat sarcomapatientsupportorganisations areinvolvedwheneverrelevantthroughoutthepatientpathway.

Thesegroupsworkto:

Improvepatients’knowledgeandabilitytotakedecisions Secureaccesstoinnovativetherapiesandimprovequalityof

treatment

Supportsarcomaresearch,suchasbybeinginvolvedinthe designofclinicaltrials

Advocateatnationalhealthpolicylevel.

The Sarcoma Patients EuroNet Association (SPAEN) (www.

sarcoma-patients.eu) is an international network of national sarcomasupportandadvocacygroups.ChildhoodCancerInterna- tional(CCI)isthelargestpatientsupportorganisationforchildhood cancerandhasaEuropeancommittee,CCIEurope(http://www.

childhoodcancerinternational.org/cci-global-network/europe).

•Conclusionsoneachcasediscussionmustbemadeavailableto patientsandtheirprimarycarephysician.Adviceonseekingsec- ondopinionsmustbesupported.

•Cancerhealthcareprovidersmustpublishonawebsite,ormake availabletopatientsonrequest,dataoncentre/unitperformance, including:

Informationservicestheyoffer Waitingtimestoﬁrstappointment Pathwaysofcancercare

Numbersofpatientsandtreatmentsatthecentre Numberofoperatedpatientsatthecentre Clinicaloutcomes

Patientexperiencemeasurements Incidents/adverseevents.

6.2. Auditing,qualityassuranceandaccreditation

•TheexpandedMDTmustmeetatleastonceayeartoreviewthe activityofthepreviousyear,discusschangesinprotocolsand procedures,andimprovetheperformanceoftheunit/centre.

•Toproperlyassessqualityofsarcomacare,threecategoriesof outcomesmustbemeasuredandcollectedinadatabaseatthe level ofthespecialisedsarcoma centre,and regionally and/or nationally:

Clinicaloutcomes Processoutcomes

Patient-reportedoutcomes(PROs).

•Datameasuredandcollectedvariesfromonecountrytoanother butitisrecommendedthatthefollowingoutcomedataaresys- tematicallymeasuredandcollected:

5yearsurvivalrate 5yearlocalrecurrencerate 5yearlocalcontrolrate Complications

%ofpatientsdiscussedintheMDTbeforeanytreatment %ofpostoperativepatientsdiscussedintheMDT.

Theexpertgroupalsorecommendsthatcentresdevelopperfor- mancemeasurementmetricsbasedontheessentialrequirements inthispaper.

•TheECCOexpertgrouprecommendsthatfurtherattentionmust begiventopatientreportedoutcomemeasures(PROMs),tonot onlyagreeonwhichtoolsshouldbeused,butalsotousePROs moresystematicallyaspartofdiscussionsandevaluationwithin theMDT.

Toensureappropriate,timelyandhigh-qualitycare,aquality managementsystem(QMS)mustbeinplace.Itmustinvolveclini- calcare,strategicplanning,humanresourcemanagement,training etc.TheQMSmustbeaccountableataninstitutionalmanagement levelandbebasedonwrittenandagreeddocumentationsuchas guidelines,protocols,patientpathways,structuredreferralsystems andstandardoperatingprocedures(SOPs).

TheQMSmustensurethecontinuityofcareforpatients,the involvementofpatientsincancercarepathways,andthereport- ing of patient outcomes and experience. Aspart of a QMS,an effectivedatamanagementandreportingsystem,andaninternal auditsystem,arenecessities.Whereavailable,externalnational audit and certiﬁcation systems are to be followed. The ECCO expert also strongly recommends participation in international accreditationprogrammes(e.g.OrganisationofEuropeanCancer Institutes(OECI)accreditation,http://oeci.selfassessment.nu/cms) (Windetal.,2016).

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104 E.Andritschetal./CriticalReviewsinOncology/Hematology110(2017)94–105

6.2.1. Countryexamples

•TheNationalInstituteofHealthand CareExcellence(NICE)in EnglandandWaleshaspublishedaqualitystandardforsarcoma (NationalInstituteofHealthandCareExcellence,2015).Itaimsto ensurethatpeoplewithsarcomaaretreatedbyhealthcarepro- fessionalswithexperienceandexpertiseintreatingsarcoma,and thatpeoplewithsarcoma areinformedabouttheircondition, receiveappropriateandtimelyadvice,andcanaccessrelevant services.Statementsinthestandard coverreferralsandtreat- mentbyMDTs,amongothers.

•The German Cancer Society operates a certiﬁcation system for cancer centres that includes sarcomas (see https://www.

krebsgesellschaft.de/gcs/german-cancer-society/certiﬁcation.

html).

•Francehasclinicalandpathologynetworks(NetSarcandRRePS) thatofferpatientsa meanstomakea systematicdiagnosisof softtissuesarcomaandhelptoaccesstreatmentinaspecialised centre(Honoréetal.,2015).

6.3. Educationandtraining

Itisessentialthateachsarcomacentreprovidesprofessional clinicalandscientiﬁceducationonthediseaseandthatatleastone personisresponsibleforthisprogramme.Healthcareprofession- alsworkinginsarcomamustalsoreceivetraininginpsycho-social oncology,palliativecare,rehabilitationandcommunicationskills, tailoredtopatientagewhererelevant.Suchtrainingmustalsobe incorporatedintospecialistpostgraduateandundergraduatecur- riculumsfor physicians, nursesand other professionals. Nurses workinginsarcomacentresshouldundertakepost-qualiﬁcation educationand training aboutproviding holistic carefor people beingtreatedforsarcomathroughoutthepatientjourney.

6.4. Clinicalresearch

Centres treating sarcoma must have clinical research pro- grammes (either their own research or as a participant in programmesledbyothercentres).Theresearchportfolioshould have both interventional and non-interventional projects and includeacademicresearch.

TheMDTmustassessallnewpatientsforeligibilitytotakepart inclinical trialsat thecentre orin researchnetworks. For sarcoma,centresshouldhave at least10%of allpatientsincluded intheirresearchprojectsorinresearchperformedinothercen- tres.Researchersatothercentresshouldbeconsideredaspartof theexpandedMDTforatleastannualdiscussionofclinicaltrial participation.

In paediatric oncology, participation in therapy-optimising studies is a standard of care in mostcountries. Children, adolescents, and young adults in all countries should have access tonationalorinternationalmulticentrestudies,and accelerated accesstoinnovativetherapiesiftheirdiseaseprogresses.

Olderadults are currently underrepresented in cancer clinical trials despite having a disproportionate burden of disease (Ka ´zmierska,2013).Strategiestoincreasetheparticipationofolder adults, adolescents and young adults in clinical trials must be implementedandtrialsdesignedtotaketheirneedsintoaccount.

7. Conclusion

Takentogether,theinformationpresentedinthispaperpro- videsacomprehensivedescriptionoftheessentialrequirements forestablishingahigh-qualityserviceforsofttissuesarcomasin adultsandbonesarcomas.TheECCOexpertgroupisawarethatit isnotpossibletoproposea‘onesizeﬁtsall’systemforallcountries,

buturgesthataccesstomultidisciplinaryteamsisguaranteedtoall patientswithsarcoma.

Conﬂictofinterest

Theauthorsdeclarenoconﬂictsofinterest.

References

Albreht,T.,Martin-Moreno,J.M.,Jelenc,M.,Gorgojo,L.,Harris,M.Eds:European GuideforQualityNationalCancerControlProgrammes.p30.European PartnershipActionAgainstCancer(EPAAC).2015.http://www.epaac.eu/

images/WP10/EuropeanGuideforQualityNationalCancerControl ProgrammesEPAAC.pdf.

Baili,P.,DiSalvo,F.,Mancos-Gragera,R.,Siesling,S.,Mallone,S.,Santaquilana,M., etal.,2015.Ageandcasemix-standardisedsurvivalforallcancerpatientsin Europe1999–2007:resultsofEUROCARE-5,apopulation-basedstudy.Eur.J.

Cancer51(15),2120–2129,http://dx.doi.org/10.1016/j.ejca.2015.07.025.

Ballinger,M.L.,Goode,D.L.,Ray-Coquard,I.,James,P.A.,Mitchell,G.,Niedermayr, E.,InternationalSarcomaKindredStudy,etal.,2016.Monogenicandpolygenic determinantsofsarcomarisk:aninternationalgeneticstudy.LancetOncol.17 (9),1261–1271,http://dx.doi.org/10.1016/S1470-2045(16)30147-4.

Beishon,M.,2013.Whenindoubt,askanexpert.CancerWorld(May/June)http://

www.cancerworld.org/pdf/2385pagina3034Systems&Services.pdf.

Berg,L.,Nolbris,M.J.,Koinberg,I.,Melin-Johansson,C.,Möller,A.,Ohlén,J.,2016.

Characterisationofcancersupportandrehabilitationprogrammes:aSwedish multiplecasestudy.OpenNurs.J.8,1–7,http://dx.doi.org/10.2174/

1874434601408010001.

Blay,J.-Y.,Coindre,J.-M.,Ducimetière,F.,Ray-Coquard,I.,2016a.Thevalueof researchcollaborationsandconsortiainrarecancers.LancetOncol.17(2), e62–e69,http://dx.doi.org/10.1016/S1470-2045(15)00388-5.

Blay,J.-Y.,LeCesne,A.,Penel,N.,Bompas,E.,Chevreau,C.,Duffaud,F.,etal.,2016b.

Thenationwidecohortof26,883patientswithsarcomastreatedinNETSARC referencenetworkbetween2010and2015inFrance:majorimpactof multidisciplinaryboardpresentationpriorto1sttreatment.Ann.Oncol.27 (Suppl6),http://dx.doi.org/10.1093/annonc/mdw388.03http://annonc.

oxfordjournals.org/content/27/suppl6/1397O.

Boellaard,R.,Delgado-Bolton,R.,Oyen,W.J.,Giammarile,F.,Tatsch,K.,Eschner PET/CT,W.F.D.G.,2015.EANMprocedureguidelinesfortumourimaging:

version2.0.Eur.J.Nucl.Med.Mol.Imaging42(2),328–354,http://dx.doi.org/

10.1007/s00259-014-2961-x.

Borras,J.M.,Albreht,T.,Audisio,R.,Briers,E.,Casali,P.,Esperou,H.,etal.,2014.

Policystatementonmultidisciplinarycancercare.Eur.J.Cancer50(3), 475–480,http://dx.doi.org/10.1016/j.ejca.2013.11.012.

Cardoso,F.,Cataliotti,L.,Costa,A.,Knox,S.,Marotti,L.,Rutgers,E.,etal.,2016.

EuropeanBreastCancerConferencemanifestoonbreastcentres/units.Eur.J.

Cancer,http://dx.doi.org/10.1016/j.ejca.2016.10.023.

Casali,P.,2016.Managingadultsofttissuesarcomasandgastrointestinalstromal tumours.CancerWorld(September/October)http://cancerworld.net/e- grandround/managing-adult-soft-tissue-sarcomas-and-gastrointestinal- stromal-tumours.

Coindre,J.M.,Terrier,P.,Guillou,L.,LeDoussal,V.,Collin,F.,Ranchère,D.,etal., 2001.Predictivevalueofgradeformetastasisdevelopmentinthemain histologictypesofadultsofttissuesarcomas:astudyof1240patientsfromthe FrenchFederationofCancerCentersSarcomaGroup.Cancer91(10), 1914–1926,http://dx.doi.org/10.1002/1097-0142(20010515)91:10<1914:AID- CNCR1214>3.0.

DeLaney,T.F.,Haas,R.L.,2016.Innovativeradiotherapyofsarcoma:protonbeam radiation.Eur.J.Cancer62,112–123,http://dx.doi.org/10.1016/j.ejca.2016.04.

015.

Decoster,L.,VanPuyvelde,K.,Mohile,S.,Wedding,U.,Basso,U.,Colloca,G.,etal., 2015.Screeningtoolsformultidimensionalhealthproblemswarrantinga geriatricassessmentinoldercancerpatients:anupdateonSIOG recommendations.Ann.Oncol.26(2),288–300,http://dx.doi.org/10.1093/

annonc/mdu210.

Donaldson,S.S.,Torrey,M.,Link,M.P.,Glicksman,A.,Gilula,L.,Laurie,F.,etal., 1998.AmultidisciplinarystudyinvestigatingradiotherapyinEwing’ssarcoma:

endresultsofPOG#8346.PediatricOncologyGroup.Int.J.Radiat.Oncol.Biol.

Phys.42(1),125–135,http://dx.doi.org/10.1016/S0360-3016(98)00191-6.

EuropeanSocietyforPaediatricOncology,2009.EuropeanStandardsofCarefor ChildrenwithCancer.https://www.siope.eu/european-research-and- standards/standards-of-care-in-paediatric-oncology.

EuropeanSocietyofOncologyPharmacy,2014.QualityStandardfortheOncology PharmacyService(QuapoS5).http://www.esop.li/activities.php.

Falk,A.T.,Moureau-Zabotto,L.,Ouali,M.,Penel,N.,Italiano,A.,Bay,J.O.,etal.,2015.

Effectonsurvivaloflocalablativetreatmentofmetastasesfromsarcomas:a studyoftheFrenchsarcomagroup.Clin.Oncol.R.Coll.Radiol.27(1),48–55, http://dx.doi.org/10.1016/j.clon.2014.09.010.

Gabriel,M.,Rubello,D.,2016.18F-FDGPET-CTinsofttissuesarcomas:staging, restagingandprognosticvalue?Nucl.Med.Commun.37(1),3–8,http://dx.doi.

org/10.1097/MNM.0000000000000407.

Gronchi,A.,Miceli,R.,Allard,M.A.,Callegaro,D.,LePéchoux,C.,Fiore,M.,etal., 2015.Personalizingtheapproachtoretroperitonealsofttissuesarcoma: