Scottish Referral Guidelines for Suspected Cancer · Scottish Referral Guidelines for Suspected...

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Scottish Referral Guidelines for Suspected Cancer January 2019 First published October 2013. Updated May and August 2014. Refreshed 2018 You can copy or reproduce the information in this document for use within NHSScotland and for educational purposes. You must not make a profit using information in this document. Commercial organisations must get our written permission before reproducing this document. SCOTTISH PRIMARY CARE CANCER GROUP SCOTTISH PRIMARY CARE CANCER GROUP

Transcript of Scottish Referral Guidelines for Suspected Cancer · Scottish Referral Guidelines for Suspected...

Page 1: Scottish Referral Guidelines for Suspected Cancer · Scottish Referral Guidelines for Suspected Cancer January 2019 First published October 2013. Updated May and August 2014. Refreshed

Scottish Referral Guidelines for

Suspected CancerJanuary 2019

First published October 2013. Updated May and August 2014. Refreshed 2018

You can copy or reproduce the information in this document for use within NHSScotland and for educational purposes. You must not make a profit using information in this document. Commercial organisations must get our written permission before reproducing this document.

SCOTTISH PRIMARYCARE CANCER GROUP

SCOTTISH PRIMARYCARE CANCER GROUP

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CONTENTS

1 Introduction 4

1.1 Background 51.2 Purpose 61.3 Development of the 2014 Guidelines 61.4 Guideline refresh 2019 61.5 FormatoftheGuidelines 71.6 Referraltimelines 71.7 Referralpathways 81.8 DisseminationoftheGuidelines 81.9 AuditandreviewoftheGuidelines 8

2 Commonissuesforcancerreferrals 9

2.1 PatientIssues 102.1.1 Patients’andcarers’needs 10

2.1.2 Demographicfactors 11

2.1.3 Comorbidity 11

2.1.4 Safetynetting 11

2.1.5 Followup 11

2.2 ReferralProcess 122.2.1 UseoftheGuidelines 12

2.2.2 Purposeofreferral 12

2.2.3 Clinicaldecisionsupporttoolsandstructured documentation/proformasforreferral 12

2.2.4 Downgradingofurgentsuspectedcancerreferrals 12

2.2.5 Feedbackwherenocancerisfound 13

2.2.6 Opportunityforhealthpromotion 13

2.2.7 Generalpointsaboutsuspectedcancer 13

3 ReferralGuidelines 15

3.1 LungCancer 163.2 BreastCancer 173.3 LowerGastrointestinalCancer 203.4 Oesophago-gastric,hepatobiliaryandpancreaticcancers 223.5 UrologicalCancers 25

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3.6 SkinCancers 273.7 Gynaecologicalcancers 283.8 Haematologicalcancers 313.9 Headandneckcancers 333.10 Brainandcentralnervoussystemcancers 343.11 Sarcomasandbonecancers 353.12 Children,teenagersandyoungadultcancers 373.13 Malignantspinalcordcompression 40

APPENDICES 42

Appendix1:Methodologyused–2014version 43Appendix2:MembershipofGuidelineSteeringGroup2018 43Appendix3:MembershipoftheGuidelineDisseminationGroup2018 44Appendix4:MembershipofGuidelineSubgroups 45Appendix5:RegionalGeneticsCentres 50Appendix6:EqualityandDiversity 50Appendix7:KeyReferences 51

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1 INTRODUCTION

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1.1 BACKGROUND

Althoughcancerisacommonconditionwith31,331newcasesbeingdiagnosedin2016inScotland(excludingnon-melanomaskincancers),anindividualgeneralpractitioner(GP)practiceislikelytoseeonlyabout35newcasesperannum.TheaveragenumberofnewcasesperannumofindividualcancertypesforaGPpracticewithalistsizeof5,881patientsisshowninTable1.AGPpracticeislikelytoseeonaveragefourorfivenewcasesperannumofpeoplewitheachofthemostcommoncancers(lung,breastandcolorectal)andonlyapproximatelyonenewcancerofthebladder,kidneyandoesophagus.AnindividualGPmightseeonlyonenewcancerinachildunder15yearsina35yearcareer(oneeverysevenoreightyearsinanaveragesizedGPpractice)andyetaGPwillseechildrenwithsymptomsandsignsthatcouldconceivablybecancereverysingleweek.

Table1:TenmostcommoncancersinScotlandin20161

Cancer ICD-10codeTotalnewcasesperannum

No.casesper5,881populationper

annumTrachea,bronchusandlung C33-C34 5,045 5.49Breast C50 4,636 5.04Colorectal C18-C20 3,700 4.03Prostate C61 3,167 3.45Malignantmelanomaofskin C43 1,383 1.5

Headandneck C00-C14,C30-C32 1,240 1.35

Non-Hodgkin’slymphoma C82-C85 1,022 1.11Kidney C64-C65 980 1.07Bladder C67 870 0.95Oesophagus C15 858 0.93

Thetaskfortheclinicianistodifferentiatebetweenpeoplewhosesymptomsmaybeduetocancerandthemuchlargernumberofpeoplewithsimilarsymptomsarisingfromothercauses.Forcertainsymptoms,itmaybeentirelyappropriateforacliniciantowaittoseeifitresolves.Persistenceorworseningofthesymptommayalertthecliniciantothepossibilityofcancer.Whereverpossiblethesefactorshavebeentakenintoaccountinthedevelopmentoftheseguidelines.

1 BasedontotalScottishpopulationof5,404,700asat30June2016:NationalRecordsofScotlandmid2016populationestimatesScotland,ScottishCancerRegistry,InformationServicesDivision(ISD),April2018,GPWorkforce&PractisePopulations

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CancerremainsanationalclinicalpriorityfortheScottishGovernmentandNHSScotland.TheScottishGovernment’sCancerStrategy‘BeatingCancer:AmbitionandAction’2waslaunchedinMarch2016.Thisstrategyandaccompanying£100millioncommitment,servesasablueprintforthefutureofcancerservicesinScotland,improvingtheprevention,detection,diagnosis,treatmentandaftercareofthoseaffectedbythedisease.

Increasingearlydiagnosisofcancercanreduceprematuredeathsfromcancerandsubsequentlyhaveapositiveeffectonoveralllifeexpectancy.OneoftheobjectivesoftheScottishGovernmentDetectCancerEarlyProgrammeistoworkwithcliniciansandthewiderprimarycareteamtopromotereferralorinvestigationattheearliestreasonableopportunityforpeoplewhomaybeshowingasuspicionofcancer,whilemakingthemostefficientuseofNHSresourcesandavoidinganadverseimpactonaccesstoservices.

1.2 PURPOSE

TheScottishReferralGuidelinesforSuspectedCancerwerefirstpublishedin2002andsubsequentlyrevisedin2007and2014.Therecommendationsheresupersedethoseinpreviousguidelines.

TheguidelinesshouldhelpGPs,thewiderprimarycareteam,otherclinicians,patientsandcarerstoidentifythosepeoplewhoaremostlikelytohavecancerandwhothereforerequireurgentassessmentbyaspecialist.Equally,itishopedthattheguidelineswillhelpclinicianstoidentifypeoplewhoareunlikelytohavecancerandwhomayappropriatelybemanagedinaprimarycaresettingorwhomayrequirenon-urgentreferraltoaspecialist.

1.3 DEVELOPMENTOFTHE2014GUIDELINES

Amultidisciplinarysteeringgroupwasconvenedin2012toproducearelevant, evidence-based,clinicallyusefulanduser-friendlydocumentforcliniciansinprimarycare.ThemethodologyandscopeoftheguidelinesisdetailedinAppendix 1.

1.4 GUIDELINEREFRESH2019

TheScottishPrimaryCareCancerGroupreviewedthe2014guidelinesin2018andidentifiedchangesthatwererequiredasaresultofnewevidenceandguidelines.Itwasthereforedecidedtoundertakeafurtherupdate.HealthcareImprovementScotlandidentifiedevidencepublishedsincetheoriginalguidelines.OfparticularnotewasthepublicationofNICEguidelineNG12Suspectedcancer:recognitionandreferral(June2015updatedJuly2017)whichusesariskthresholdvalueof3%positivepredictivevalueoffindingcancerforanyspecificpresentingsymptomsorsigns.AswithNICE,wehaveincludedexceptionstothe3%PPVthreshold,inparticular,forchildren’scancer.3

2 BeatingCancer:AmbitionandActionhttps://www.gov.scot/publications/beating-cancer-ambition-action/ 3 NICEGuideline[NG12]Suspectedcancer:recognitionandreferral(June2015lastupdatedJuly2017)https://www.nice.org.

uk/guidance/ng12

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Asteeringgroupwasestablishedtooverseetheupdateprocess,underthechairmanshipofDrPeterHutchison(membershipinAppendix 2).Membersoftheprevioustumourspecificgroupswereaskedtoparticipateintherevisionoftheguidelines.Wherethepreviousmemberswereunabletodoso,theywereaskedtorecommendspecialiststobeinvolved.Membershipofthegroupswasratifiedbythesteeringgroup.Subgroupswereconvenedforthevariouscancerswheretheevidencesuggestedthatrecommendationsshouldchange.Wheretheevidencedidnotsupporttheneedforafullupdate,viewsweresoughtfromtheoriginalmembersoftheindividualtumoursubgroups.Epidemiologicaldatawereupdatedthroughouttheguidelines.

Adisseminationgroup(membershipinAppendix 3)wasestablishedtooverseetheimplementationoftheguidelines.

Theupdatedguidelinesweresubmittedforpeerreviewacrossclinical,thirdsectorandpatientrepresentativenetworksinScotlandandthesteeringgroupconsideredandrespondedtoeachcommentreceived.

1.5 FORMATOFTHEGUIDELINES

Thereisnotcompleteuniformityinthelayoutoftheguidelinesasmembersofspecificsubgroupsadvisedslightlydifferentformatsthatreflectthedistinctnatureofsymptomsandpatternsofdisease.However,foreachtumourgrouptheguidelinesincludeinformationonkeypointsaboutthepatternoftherelevantcancerandguidelinesforreferral.

1.6 REFERRALTIMELINES

Thereferraltimelinesusedintheguidelinesinclude:

• urgentsuspicionofcancer:Patientsreferredviatheurgentsuspectedcancerpathwayshouldreceivefirsttreatmentwithin62daysofreceiptofreferral.Thesereferralswillbeprioritisedandtracked.

• emergencyreferral:tobeseenonthesameday

• urgent(notsuspectedcancer):nottrackedorcountedinthetargetforcancerreferrals–notusedintheseguidelines

• routine:allotherreferrals,and• primarycaremanagement

Allstaffinvolvedinthereferralprocessshouldbeawareofthedifferenceandimportanceofusingtheseterms.Inparticular,labellinganurgentreferralasbeingforsuspectedcancerensuresthatthesecasescanbespecificallyidentified,trackedandauditedundertheScottishGovernmenttarget.

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1.7 REFERRALPATHWAYS

NHSboardshavewell-establishedurgentsuspicionofcancerreferralpathwaystofacilitatepromptdiagnosisofcancer.Thesepathwaysfunctionparticularlywellincaseswheresymptomsandsignsaresuspiciousofaspecifictumourtype.However,forpeoplewithvaguesymptoms(suchasunexplainedweightlossandfatigue)thereispotentialfordelayinreachingadiagnosis.Tominimisethisrisk,directaccesstoimagingforprimarycarepractitionersenablesthedifferentialdiagnosistobenarrowedandreferraltotheappropriatesecondarycarespecialtytobemade,therebyreducingdelays.TheavailabilityofsuchaccesstoimagingvariesacrosstheNHSboards.

1.8 DISSEMINATIONOFTHEGUIDELINES

TheguidelineswillbewidelydisseminatedinavarietyofformatstoallclinicianstowhomsomeonemayfirstpresentwithsymptomsofpossiblecancerincludingGPs,AdvancedNursePractitionersandothernursingstaff,pharmacists,dentists,optometrists,NHS24,paramedicsandA&Edepartments.Theywillalsobebroughttotheattentionofsecondarycarecliniciansofallgradesinordertoencourageequityofaccesstoinvestigationandtofacilitateinterdepartmentalreferrals.

Thecurrentguidelinesareavailableathttp://www.cancerreferral.scot.nhs.uk/andadesktopQuickReferenceGuidehasbeendevelopedbytheScottishPrimaryCareCancerGroupwhichhasbeenusedasthebasisforanAppforuseonmobiledevices.

LinktoWebsite:http://www.cancerreferral.scot.nhs.uk

LinktoboththeAppleAppstore:https://itunes.apple.com/gb/app/cancer-referral-guidelines-quick-reference-guide/id1049728177?mt=8andtotheAndroidAppstore:https://play.google.com/store/apps/details?id=com.scet.cancercareguidelines

1.9 AUDITANDREVIEWOFTHEGUIDELINES

Auditandmonitoringoftheguidelinesinpracticeshouldgenerateavaluableamountofnewinformationwhichwillbeusedtorevisetheguidelinesinthefuture.ItisstronglyrecommendedthattheRegionalCancerNetworksundertakeprospectiveauditoftheguidelines.

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2 COMMONISSUESFORCANCERREFERRALS

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2.1 PATIENTISSUES

2.1.1 Patients’andcarers’needsAllhealthcareprofessionalsshouldbesensitivetotheneedsofpatients,carersandrelativeswhencancerissuspected.RealisticMedicine4istheScottishGovernment’sinitiativetoputthepersonatthecentreofdecision-makingandencouragesapersonalisedapproachtotheircare.Goodcommunicationiskeyandfivequestionstobeconsideredbyallinvolvedcanhelpleadtoinformeddecision-making:

• Isthisactionreallyneeded?

• Whatarethebenefitsandrisks?

• Whatarethepossiblesideeffects?

• Aretherealternativeoptions?

• And,importantly,whatwouldhappenifwedidnothing?

Goodpracticeincludes:

• Beingsensitivetotheperson’swishestobeinvolvedindecisionsabouttheircare

• Providingunderstandableinformationatalevelappropriatetotheperson’swishestobeinformed

• Beingawareof,andofferingtoprovideaccessto,sourcesofinformationinvariousformats

• Usingtheword“cancer”asareasonforinvestigationorreferralunlessthereisseriousconcernaboutcausingunwarranteddistress

• Providinginformationaboutanyreferraltootherservicesinformat(s)mostsuitablefortheperson,includinghowlongtheymighthavetowait,whotheyarelikelytosee,andwhatislikelytohappentothem

• Consideringcarefullytheneedforemotionalandphysicalsupportwhileawaitinganappointmentwithaspecialistand,whereappropriate,providingakeycontact

• Consideringanycarers’needsforsupportandinformation,takingissuesofconfidentialityintoconsideration

• Takingtheindividual’sparticularcircumstancesintoaccount,forexampleage,family,workandculture

• Recognisingthatthereareoccasionswhenintrusiveinterventionisnotinaperson’sbestinterests.Thereshouldbefulldiscussionaboutalternativeapproaches,includingwithrelevantothersifapersonlackscapacity,complyingwiththeAdultswithIncapacity(Scotland)Act2000

• Maintainingahighstandardofcommunicationskills,including,forexample,intheprocessofbreakingbadnews

4 PractisingRealisticMedicinehttps://www.gov.scot/publications/summary-practising-realistic-medicine/

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2.1.2 DemographicfactorsDeprivationaffectstheincidenceofandmortalityassociatedwithcancers.Italsoimpactsontheabilityofpeopletoaccesshealthcareservices.Itisessentialthatanyconsultationorotheropportunitywhereapersonfromadeprivedareapresentswithsymptomssuggestiveofpossiblecancerisusedtofulladvantage.Somecancersoccurmorefrequentlyincertaincommunities,e.g.thelifetimeriskofprostatecancerinblackmenistwicethatofallmencombined.

2.1.3 ComorbidityTheincreasingnumberofpeoplewithlong-termconditionsandco-morbidityposemajorclinicalchallengesandaffectboththeincidenceofandmortalityfromcancer.Chronicdiseasemanagementprogrammesaffordanopportunitytoidentifysymptomssuggestiveofpossiblecancer.

2.1.4 SafetynettingItisnotalwaysappropriateforacliniciantorefersomeoneimmediatelywithnewsymptomsorsignswhichcouldbecancer(forexample,oneweekofdiarrhoeaorasorethroatfor10days)andaninitial‘watchandwait’strategymaybeappropriate.Itisalsoimportantforclinicianstoprovidea‘safetynet’andensurepeopleknowwhatsymptomstomonitorandwhentoreturniftheirconditiondoesnotimproveorchange.Insomecases,howeverpeoplemaybeunwillingtowatchandwaitduetohighlevelsofanxiety.Insuchcases,thereferringclinicianshouldensurethatthisisdetailedinthereferraldocumentation.

Notethatinchildren,repeatpresentations(threeormoretimes)ofanysymptomswhichdonotappeartoberesolvingorfollowinganexpectedpatternshouldbeconsideredforreferralforasecondopinion,takingintoaccountparental/carerandchild concerns.

2.1.5 FollowupItisgoodpracticeforthereferrertoconsiderwaysofsupportingthepersontoattendinvestigations,consultationsorreviewsandaddressinganyconcernstheymayhaveabouttheirreferral.Forexample,aleafletsuchasCancerResearchUK’s“YourUrgentReferralExplained”5canbegiventothematthetimeofreferral.Othersimilarresourcesareavailable.Systemsshouldbeinplacetoensurepeoplearenotlosttofollowup.

5 CancerResearchUK:YourUrgentReferralExplainedhttps://publications.cancerresearchuk.org/categories/your-urgent-referral

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2.2 REFERRALPROCESS

2.2.1 UseoftheGuidelinesTheguidelinesaredesignedforuseinanyprimarycaresetting,byanymemberoftheclinicalteam.LocalarrangementsshouldbeinplaceineachNHSboardareaforadvancednursepractitionersandothernursingstaff,pharmacists,dentists,optometrists,NHS24,paramedicsandotherstoensurerapidreferralisarranged.Thismaybebydirectreferral(withsimultaneousnotificationoftheGP)orbymakingarrangementsforthepersontoseetheirGPurgently,clearlynotifyingtheconcernaboutsuspectedcancer.

Theguidelineswillalsobebroughttotheattentionofsecondarycarecliniciansofallgradesinordertoencourageequityofaccesstoinvestigationandtofacilitateinterdepartmentalreferrals.

2.2.2 PurposeofreferralThe‘urgentsuspicionofcancer’referralpathwayisdesignedtoallowtherapidassessmentandinvestigationofapersontodeterminethecauseoftheirsymptoms.Forpeoplewhosepresentingsymptomspersist,itisnotacceptabletosimplyexcludecancerwithoutprovidinganassessmentofthelikelyunderlyingcause.Thismayinvolveindividualhospitalspecialtiesmakinginternalreferralstotheircolleaguestohelpdeterminethenatureandcauseofthepresentingsymptoms.Theseinternalreferralsshouldbeundertakenwiththeminimumofdelayandwithgoodcommunicationtoboththepatientandreferringclinician.Wherediagnostictestsareundertaken,theclinicianrequestingthetesthasaresponsibilityforactingontheresultandensuringthatthepatientreceivesthis.

NHSboardsmaywishtoconsidertowhichdiagnosticservicesprimarycarecliniciansshouldhavedirectopenaccess.Inthesesituationstheclinicianwouldberesponsibleforcommunicatingtheresulttothepatientandarranginganysubsequentfollowup.

2.2.3 Clinicaldecisionsupporttoolsandstructureddocumentation/proformas forreferral

Toachieveconsistency,clinicaldecisionsupportsystemsandstructuredproformasforreferralcanbehelpfulforuseinallclinicalsettings.ScottishCareInformation(SCI)Gatewayprovidesthemeansforelectronicreferralsincorporatingstructuredproformas,butclinicaldecisionsupportsystemsvaryacrossNHSScotland.

2.2.4 DowngradingofurgentsuspectedcancerreferralsOnrareoccasionsitmaybeacceptableforthereceivinghospitalspecialtytodowngradeanurgentsuspicionofcancerreferraltourgentorroutine.ThisshouldneveroccurwithoutnotifyingthereferringGPpracticetimeously.Theclinicianshouldhavetheopportunitytoexplainwhyanurgentsuspectedcancerreferralwasrequested.Vitalinformationmayhavebeenomittedfromthereferralormayhavebecomeavailablesincethereferralwasmade.Itisessentialthatthepersoniskeptinformedaboutanychangeinreferralpriority.

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2.2.5 FeedbackwherenocancerisfoundThereferringclinicianshouldreceivetimelyfeedbackontheoutcomesforallpeoplewithanurgentsuspicionofcancerreferral.Wherenegativeresultsarefound,andconcernsstillexist,thespecialistshouldconsiderdirectonwardreferraltoanotherspecialty.Informationaboutinappropriatereferralsshouldbefedbacktothereferringcliniciandetailingwhyitwasfelttobeinappropriateandsuggestinganalternativecourseofaction.

2.2.6 OpportunityforhealthpromotionSuspicionofcancer,whetherwarrantingreferralornot,isanopportunitytoconsiderhealthpromotionsuchassmokingcessation,alcohol,diet,obesity,exerciseandengagingwithnationalscreeningandimmunisationprogrammes.Peopleshouldbeinformedthat4in10cancersarepreventable6,andthataddressingriskfactorscanhelpreducetheiroverallcancerrisk.

2.2.7 Generalpointsaboutsuspectedcancer• Canceroftenpresentswithvaguesymptomsthatdonothelpidentifywhichpathwayof

investigationtofollow.Inparticular,ovarianandpancreaticcanceroftenpresentverylateso,inunwellpeoplewithnothingotherthanmalaiseandsignificantunexplainedweightloss,mostNHSBoardshavepathwaysinplaceforPrimaryCareaccesstoCTchest,abdomenandpelvisasfirstinvestigation

• Recentevidencehasidentifiedthrombocytosisasastrongriskmarkerformalignancy,inparticularlung,endometrial,gastric,oesophagealandcolorectalcancer(acronym“LEGO-C”).Withacancerincidenceof11.6%and6.2%inmalesandfemalesrespectively,thesefigureswellexceedthe3%thresholdtowarrantinvestigation7

• Metastaticdiseaseiscommonlythefirstpresentationofanewcancer.Thepossibilityofanunderlyingprimarycancershouldbeconsideredespeciallywithsymptomsandsignssuggestinglung,liver,boneorbraincancer.Forexample,bonemetastasesarecommonlyduetoprostate,breastandlungcancer.Metastaticdiseaseshouldbeborneinmindwhenanybodywithaprevioushistoryofcancerpresentswithnewsymptoms

• Tumourmarkershavealimitedplaceinthedecisiontoreferforsuspectedcancer:onlyPSAforprostatecancerinmen,CA125forovariancancerinwomen,andserumandurineparaproteinsformyelomashouldberoutinelyusedinPrimaryCare8

6 StatisticsonPreventableCancershttps://www.cancerresearchuk.org/health-professional/cancer-statistics/risk/preventable-cancers

7 Clinicalrelevanceofthrombocytosisinprimarycare:BrJGenPract2017;67(659):e405-e413.DOI:https://doi.org/10.3399/bjgp17X691109

8 CancerWorkingGroupoftheScottishClinicalBiochemistryManagedDiagnosticNetworkhttp://www.mcns.scot.nhs.uk/scbmdn/wp-content/uploads/sites/10/2018/09/Tumour-Marker-bookmark-NHS-download.pdf

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• Itisgoodpracticetoincludegeneralfitnessorperformancestatusinthereferral(e.g.ECOG/WHOscale)inordertofacilitatediscussionaboutthemostappropriatepathway

Grade ECOG/WHOPerformanceStatus0 Fullyactive,abletocarryonallpre-diseaseperformancewithoutrestriction1 Restrictedinphysicallystrenuousactivitybutambulatoryandabletocarry

workofalightorsedentarynature,e.g.,lighthousework,officework2 Ambulatoryandcapableofallself-carebutunabletocarryoutanywork

activities;upandaboutmorethan50%ofwakinghours3 Capableofonlylimitedself-care;confinedtobedorchairmorethan50%of

wakinghours4 Completelydisabled;cannotcarryonanyself-care;totallyconfinedtobedor

chair5 Dead

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3 REFERRALGUIDELINES

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3.1 LUNGCANCER

Morethan90%ofpeoplewithlungcanceraresymptomaticatthetimeofdiagnosis.Manysymptomsoflungcancer(particularlycoughandfatigue),however,arecommonpresentationsinprimarycare,oftenassociatedwithchronicdiseasessuchasgastricrefluxorchronicobstructivepulmonarydisease.Itisthereforeimportantthatchangesinsymptomsareidentifiedandactedupon.

ChestX-rayfindingsareabnormalinover96%ofsymptomaticpeoplewithlungcancer.Inmostcaseswherelungcancerissuspected,itisappropriatetoarrangeanurgentchestX-raybeforeurgentreferraltoachestphysician.However,anormalchestX-raydoesnotexcludeadiagnosisoflungcancer.IfthechestX-rayisnormalbutthereisahighsuspicionoflungcancer,peopleshouldbeofferedurgentsuspicionofcancerreferraltoarespiratoryphysician.

Inpeoplewithahistoryofasbestosexposure,mesothelioma,aswellaslungcancer,shouldbeconsidered.Approximately80to90%ofpeoplewithmesotheliomawillhaveahistoryofoccupationalorclosecontactexposure.Morecommonpresentationsincludechestpain,dyspnoeaandunexplainedsystemicsymptoms.

UrgentsuspicionofcancerchestX-ray(CXR)

• Anyunexplainedhaemoptysis

• Unexplainedandpersistent(morethanthreeweeks)

• changeincoughornewcough

• dyspnoea

• chest/shoulderpain

• lossofappetite

• weightloss

• chestsigns

• hoarseness(ifnoothersymptomspresenttosuggestlungcancerreferviaHead&Neckpathway)

• fatigueinasmokeragedover40years• Newornotpreviouslydocumentedfingerclubbing

• Persistentorrecurrentchestinfection

• Cervicaland/orpersistentsupraclavicularlymphadenopathy*

• Thrombocytosiswheresymptomsandsignsdonotsuggestotherspecificcancer**

• AnypersonwhohasconsolidationonchestX-rayshouldhavefurtherimagingnomorethansixweekslatertoconfirmresolution

*ifCXRnormal,referviaHeadandNeckpathway

**ifCXRnormal,consideralternativediagnosisincludingothercancers

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Urgentsuspicionofcancerreferral

• Anyunexplainedsymptomsorsignsdetailedonpreviouspagepersistingforlongerthansixweeks,despiteanormalchestX-ray(otherthanisolatedthrombocytosisorcervicaland/orpersistentsupraclavicularlymphadenopathy)

• ChestX-raysuggestive/suspiciousoflungcancer(includingpleuraleffusion,pleuralmassandslowlyresolvingconsolidation)

• Persistenthaemoptysisinsmokers/ex-smokersover40yearsofage

Goodpracticepoints

• ThereshouldbealocallyagreedpathwayforradiologytonotifytherespiratoryteamofanabnormalchestX-raysuggestiveofcancer

• Itisgoodpracticeforthereferrertoconsidertakingbloods,includingfullbloodcountandanassessmentofrenalfunctionifnotdoneinprecedingthreemonths,inordertoexpeditefurtherimaging

• Inpeoplewithfeatures,suggestiveofcancerincludingsuspectedmetastaticdisease,butnoothersignstosuggesttheprimarysource,considerCTchest,abdomenandpelvisinaccordancewithlocalguidelinesabouttheinvestigationofanunknownprimarycancer

3.2 BREAST CANCER

Itisestimatedthatbetween0.35%and0.6%ofallGPconsultationsinScotlandareforbreastsymptoms.Manyofthesewillbeforyoungwomen,whereasthebiggestriskfactor,aftergender,isincreasingage,withmorethan80%ofbreastcancersoccurringinwomenovertheageof50.

Breastcanceraccountsfor30%ofcancersinwomenandaround4,500peoplearediagnosedwithbreastcancerinScotlandeachyear;approximately25ofthesearemen.Incasesofgenderreassignment,caremustbetakentoprovidesensitiveandclinicallyappropriatecaredependingonindividualcircumstancesandtakingintoaccountanyhormonetherapyinvolved.

Guidanceaboutreferraltoregionalgeneticscentres(seeAppendix5)forthosewithafamilyhistoryofbreastcancerisavailable.9

9 HISFamilialBreastCancerReportwww.healthcareimprovementscotland.org/our_work/cancer_care_improvement/programme_resources/familial_breast_cancer_report.aspx

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Urgentsuspicionofcancerreferral

Routinereferral Primarycaremanagement-issuerelevantadviceleaflet(ifavailable)

Lump • Anynewdiscretelumpinpatients30yearsandover

• Newasymmetricalnodularitythatpersistsatreviewaftertwotothreeweeks(inpatientsover35years)

• Unilateralisolatedaxillarylymphnodeinwomenpersistingatreviewaftertwotothreeweeks

• Recurrentlumpatthesiteofapreviouslyaspiratedcyst

• Anynewdiscretelumpinpatientsunder30yearswithnoothersuspiciousfeatures

• Newasymmetricalnodularitythatpersistsatreviewaftertwotothreeweeks(inpatientsunder35years)

• Womenwithlongstandingtenderlumpybreastsandnofocallesion

• Tenderdevelopingbreastsinadolescents

NippleSymptoms • Visiblybloodstaineddischarge

• Newunilateralnippleretraction

• Nippleeczemaifunresponsivetomoderatelypotenttopicalsteroidsafteraminimumoftwoweeks

• Persistentunilateralspontaneousdischargesufficienttostainouterclothes

• Transientnippledischargewhichisnotbloodstained

• Checkprolactinlevelsinpersistentbilateraldischarge

• Longstandingnippleretraction

• Nippleeczemaifeczemapresentelsewhere

Skinchanges • Skintethering

• Fixation

• Ulceration

• Peaud’orange

• Obvioussimpleskinlesionssuchasepidermoid(sebaceous)cysts

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Urgentsuspicionofcancerreferral

Routinereferral Primarycaremanagement-issuerelevantadviceleaflet(ifavailable)

Abscess/infection • Mastitisorbreastinflammationwhichdoesnotsettleorrecursafteronecourseofantibiotics

• Abscessorinflammation–tryonecourseofantibioticsasperlocalguidelines

• Anyacuteabscessrequiresimmediatediscussionwithsecondarycare

Breastpain • Unilateralpainpersistingoverthreemonthsin post-menopausalwomen

• Intractablepainthatinterfereswiththeperson’slifestyleorsleep

• Womenwithmoderatedegreesofbreastpainandnodiscretepalpablelesion

Gynaecomastia • Exceptionalaestheticsreferraltoplasticsurgerypathwayifappropriate(i.e.NOTtothebreastservice)

• Excludeortreatanyendocrinecausepriortoreferral

• Examineandexcludeabnormalitiessuchaslymphadenopathyorevidenceofendocrineconditionwithbloodtestsasperlocalguidelines

• Reviewtoexcludedrugcauses

Breastimplants • Ifappropriate,refertotheservicethatfirstinsertedtheimplant(usuallyplasticsurgery)

• Reassuranceisoftenappropriateifsymptomsrelatetotheimplantaloneandnottounderlyingbreasttissue

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3.3 LOWERGASTROINTESTINALCANCER

Lowergastrointestinalsymptomsarecommonpresentationsinprimarycare.Rectalbleedingisestimatedtoaffect14,000per100,000populationeachyear.Therearelargedifferencesinthepredictivevalueofrectalbleedingforcanceraccordingtoitsassociationwithothersymptomsandsignsandtheageoftheperson.

Differentmanagementstrategiesshouldbeadoptedaccordingtocancerrisk,sothatthosepeoplewithtransientlow-risksymptomscausedbybenigndiseaseavoidunnecessaryinvestigation.

Theriskofcolorectalcancerisincreasedifthereisapasthistoryofulcerativecolitis,colorectalpolypsorcancer,orifthereisafamilyhistoryofcolorectalcancerorLynchsyndrome.Guidanceforreferraltoregionalgeneticscentres(seeAppendix5)forthosewithsuchafamilyhistoryisavailableinSIGN126.10

Inpeoplewithulcerativecolitis,aplanforfollowupshouldbeagreedinlinewithcurrentnationalguidelines.

Anabdominalandrectalexaminationplusbloodteststoassessrenalfunction(incaseoftriagestraighttoCTcolonography),liverfunctiontestsandtoexcludeanaemiaandthrombocytosisshouldbeperformedonallpeoplewithsymptomssuggestiveofcolorectalcancer.Thereisemergingevidencethatthrombocytosisisariskmarkerforunderlyingcancer,includingcolorectal,andthiscanfacilitateappropriatetriageinsecondarycare.Anegativerectalexamination,orarecentnegativebowelscreeningtest,shouldnotruleouttheneedtorefer.Thecarcinoembryonicantigentestshouldnotbeusedasascreeningtool.Quantitativefaecalimmunochemicaltesting(qFIT)isbeingusedforsymptomaticpatientsinpilotprojectsinmanyNHSBoards.Insomeitisusedbysecondarycareasatriagetooltodeterminemostappropriateinitialinvestigationandinothersbyprimarycaretohelpdecideontheneedforreferral.Eachpilothasitsownreferralguidancewhichmustbeusedwhereavailable.Inallpilotsthesereferralsandtheiroutcomeswillbeformallyauditedinordertodeterminethemostappropriateuseofthetest.UltimatelyitisexpectedthatanationallyagreedsystemforusingqFITtosupporttheinvestigationofgastrointestinaldiseasewillbeimplemented.Thisislikelytoradicallychangeourapproachtothereferralprocess.Thisguidelinewillbefurtherreviewedatthatpoint.

10 SIGN126-Diagnosisandmanagementofcolorectalcancer http://www.sign.ac.uk/sign-126-diagnosis-and-management-of-colorectal-cancer.html

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Urgentsuspicionofcancerreferral-highriskfeatures

Bleeding • Repeatedrectalbleedingwithoutanobviousanalcause

• Anybloodmixedwiththestool

Bowelhabit • Persistent(morethanfourweeks)changeinbowelhabitespeciallytolooserstools-notsimpleconstipation

Mass • Unexplainedabdominalmass

• Palpableano-rectalmass

Pain • Abdominalpainwithweightloss(alsoconsiderupperGIcancer)

Irondeficiencyanaemia

• Unexplainedirondeficiencyanaemia

Goodpracticepoints

• Considerthepossibilityofovariancanceraspergynaecologicalcancersguideline

• Anabdominalpalpationshouldbeundertaken,CA125bloodserumlevelmeasuredandurgentpelvicultrasoundscancarriedoutin:

• anywomanover50yearswhohasexperiencednewsymptomswithinthelast12monthsthatsuggestirritablebowelsyndrome,or

• women(especiallythoseover50years)withoneormoreunexplainedandrecurrentsymptoms(mostdays)of:

• abdominaldistensionorpersistentbloating

• feelingfullquicklyordifficultyeating

• lossofappetite

• pelvicorabdominalpain

• increasedurinaryurgencyand/orfrequency

• changeinbowelhabit

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Primarycaremanagement

• Lowriskfeatures:

• transientsymptoms(lessthanfourweeks)

• patientsunder40yearsinabsenceofhighriskfeatures

• Watchandwait(fourweeks):

• Assessmentandreview

• Considerboweldiary

• Appropriateinformation,counsellingandagreedplanforreviewwithGP

• Referifsymptomspersistorrecur

3.4 OESOPHAGO-GASTRIC,HEPATOBILIARYANDPANCREATICCANCERS

Approximately2,900peoplearediagnosedwithaprimaryoesophago-gastric,hepatobiliaryorpancreaticcancerinScotlandeveryyear11andtheseoftenpresentlatewithconsequentpooroutcomes,earlysignsbeingnotoriouslyvague.

Combinationsofsymptomsandsignsincreasethelikelihoodofcancer,asdoesincreasingage.

Theriskofdevelopinganoesophago-gastriccancerishigherinpeopleofEastAsianorigin.OtherriskfactorsforupperGIcancers(includinghepatobiliaryandpancreatic)aresmoking,alcohol,obesityandfamilyhistory.

Investigationofoesophago-gastriccancerscommonlystartswithupperGIendoscopy,whereastheinvestigationofhepatobiliaryandpancreaticcancerstartswithCTimaging.Ifeitheroftheseinitialinvestigationsisnegative,theresponsibleclinicianshouldconsiderfurtherinvestigationpriortodischargebacktotheoriginalreferrer.

Anabdominalexaminationandappropriatebloodtests(forexample,testingforanaemia,thrombocytosis,renalfunction,LFTsandHbA1c)shouldbeperformed.Thesecanfacilitatetriageinsecondarycare.Notethatthrombocytosiscanbeanon-specificriskmarkerforunderlyingmalignancy.

Allpeoplewithhighriskfeaturesshouldbereferredtoateamspecialisinginthemanagementofoesophago-gastric,hepatobiliaryorpancreaticcancers,dependingonlocalarrangements.

11 CancerIncidenceReportInScotland(2016)https://www.isdscotland.org/Health-Topics/Cancer/Publications/2018-04-24/2018-04-24-Cancer-Incidence-Report.pdf?51368349791

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Oesophago-gastriccancerUrgentsuspicionofcancerreferral

• Dysphagia(interferenceoftheswallowingmechanismthatoccurswithinfivesecondsoftheswallowingprocess)orunexplainedodynophagia(painonswallowing)atanyage

• Unexplainedweightloss,particularly>55years,combinedwithoneormoreofthefollowingfeatures:

• neworworseningupperabdominalpainordiscomfort

• unexplainedirondeficiencyanaemia

• refluxsymptoms

• dyspepsiaresistanttotreatment

• vomiting

• Newvomitingpersistingformorethantwoweeks

Goodpracticepoints

Considerroutinereferralforpeoplepresentingwithnewuppergastrointestinalpainordiscomfortcombinedwithanyofthefollowingriskfactors:

• familyhistoryofoesophago-gastriccancerinafirst-degreerelative

• Barrett’soesophagus

• perniciousanaemia

• previousgastricsurgery

• achalasia(dysfunctionoftheoesophagealmuscle)

• knowndysplasia,atrophicgastritisorintestinalmetaplasia

Primarycaremanagement

Dyspepsiawithoutaccompanyingsymptomsorriskfactorsshouldbemanagedaccordingtolocalornationalguidelines

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HepatobiliaryandpancreaticcancerUrgentsuspicionofcancerreferral

• Painlessobstructivejaundice

• Unexplainedweightloss,particularly>55years,combinedwithoneormoreofthefollowingfeatures:

• upperabdominalorepigastricmass

• newonsetdiabetes

• anysuspiciousabnormality,inthehepatobiliarytract,foundonimaging(suchasbiliarydilatationorpancreatic/liverlesion)

• newonset,unexplainedbackpain(considerothercancercausesincludingmyelomaormalignantspinalcordcompression)

• ongoingGIsymptomsdespitenegativeendoscopicinvestigations

Goodpracticepoints

• ConsiderseekingadviceinpeoplepresentingwithnewonsetGIsymptomswithknownchronicliverdisease

Symptomsandsignsofoesophago-gastricandhepatobiliaryandpancreaticcancersoverlaptoalargeextent.Thistablesummarisesexamplesofsymptomsandsignsthatcanbeassociatedwiththedifferentcancers–theyareNOTbythemselvesnecessarilyreasonstorefer.

Associatedsymptoms/signs Pancreas,liverandgallbladdercancer

Oesophago-gastriccancer

Dysphagia 4

Irondeficiencyanaemia 4

Haematemesis 4

Refluxsymptoms 4

Vomiting(>twoweeks) 4 4

Upperabdominalpain 4 4

Unexplainedweightloss 4 4

Upperabdominalmass 4 4

Post-prandialpain 4 4

Earlysatiety(feelingfullupafterasmallamountoffood)

4 4

Unexplainedobstructivejaundice 4

Unexplainedbackpain 4

Lateonsetdiabetes 4

Newonsetirritablebowelsyndromeoverage40 4

Steatorrhoeaormalabsorption 4

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3.5 UROLOGICALCANCERS

ProstatecancerProstatecanceristhemostcommoncancerinmalesinScotland,withapproximately3,100newcasesdiagnosedeveryyear.Riskincreaseswithageandapproximately99%ofcasesarediagnosedinmenagedover50years.RiskofprostatecancerisincreasedifafirstdegreerelativehashadprostatecancerorifthereisafamilyhistoryofBRCAassociatedbreastorovariancancer.ThepresenceofaBRCAmutationincreasesrisksubstantially,inparticularforearlyoraggressivediseaseinBRCA2mutation.IntheUK,thelifetimeriskofprostatecancerinblackmen(1in4)isdoublethatofallmencombined(1in8).

Menpresentingwithunexplainedpossiblesymptomsandsignssuggestiveofprostatecancersuchaschangestourinarypatterns,erectiledysfunction,unexplainedvisiblehaematuria,lowerbackpain,bonepainorweightlossshouldhaveadigitalrectalexaminationandaprostatespecificantigen(PSA)testwithcounselling.12APSAtestmayberaisedwithinthreedaysofejaculationorsixweeksofaprovenUTI,catheterisationorotherinvasiveprocedure,suchasprostatebiopsy.5alphareductaseinhibitorssuchasfinasteridemayreducethePSAlevel.Itshouldbenotedthatthemajorityofmenwithprostatecancerhavenosymptomsatall.

BladderandkidneycancerVisiblehaematuriaisthemostcommonpresentingsymptomforbothbladderandkidneycancer.Otherpresentingfeaturesincludeloinpain,renalmasses,non-visiblehaematuria,anaemia,weightlossandpyrexia.Bothcancersareuncommon,witharound800newbladderand950newkidneycancerseachyear.Thereisevidenceofanassociationbetweenbladdercancerandaraisedwhitecellcountonabloodtestinpeopleagedover60.

TesticularandpenilecancerAlthoughscrotalswellingsareacommonpresentationinprimarycare,testicularcancerisrelativelyrare,witharound200newcasesperannum,ofwhichapproximately72%areinmenbetween15and45years.Solidswellingsaffectingthebodyofthetestishaveahighprobability(>50%)ofbeingduetocancer.Becauseofthepotentialforrapidprogressionoftesticularcancers,specialistservicesshouldconsidertriagingreferralsinordertoexpeditesuchcases.Cancerofthepenisisrare,witharound60newcaseseachyearinScotland,butitsincidenceisrising.Sexuallytransmitteddiseaseshouldbeexcludedandreferredonlyifalesionpersistsaftertreatment.

Allpeoplepresentingwithsymptomsorsignssuggestiveofurologicalcancershouldbereferredtoateamspecialisinginthemanagementofurologicalcancer,dependingonlocalarrangements.

12 ProstateCancerRiskManagementProgramme–SummaryCardhttp://www.gov.scot/Topics/Health/Services/Cancer/Risk-Management/Prostate-Cancer-Summary-Card

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Urgentsuspicionofcancerreferral

ProstateCancer• Evidencefromdigitalrectalexaminationofahard,irregularprostate

• Elevatedorrisingage-specificProstateSpecificAntigen(PSA).RoughguidetonormalPSAlevels(ng/ml):

• Lessthan60years <3

• Aged60-69years <4

• Aged70-79years <5

Thesefiguresareapragmaticaidbasedonclinicalconsensus.TheprinciplesofRealisticMedicineshouldbeappliedwhenconsideringreferraland,inoldermen,routineornoreferralmaybeappropriateforPSAlevelsof:

• Aged80-85years >10

• Aged86yearsandover >20

Bladderandkidneycancer• Aged45andoverandhave:

• unexplainedvisiblehaematuriawithouturinarytractinfection,or

• visiblehaematuriathatpersistsorrecursaftersuccessfultreatmentofurinarytractinfection

• Age60andoverandhaveunexplainednon-visiblehaematuriaandeitherdysuriaoraraisedwhitecellcountonabloodtest

• Abdominalmassidentifiedclinicallyoronimagingthatisthoughttoarisefromtheurinarytract

Testicularandpenilecancer• Nonpainfulenlargementorchangeinshapeortextureofthebodyofthetestis

• Suspiciousscrotalmassfoundonimaging

• Menconsideredtohaveepididymo-orchitisororchitiswhichisnotrespondingtotreatment

• Anynon-healinglesiononthepenisorpainfulphimosis

Routinereferral

• Elevatedage-specificPSAwhereurgentreferralwillnotaffectoutcomeduetoageorcomorbidity

• Asymptomaticpersistentnon-visiblehaematuriawithoutobviouscause

• Unexplainedvisiblehaematuria<45yearsofage

• Patientsover40whopresentwithrecurrentUTIassociatedwithanyhaematuria

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3.6 SKINCANCERS

Approximately12,000peoplearediagnosedeveryyearwithnon-melanomaskincancerinScotland,ofwhicharound3,000aresquamouscellcarcinomas(SCC).Inadditionaround1,200malignantmelanomaareregisteredperannum.Theincidenceofbothmelanomaandnonmelanomaskincancerisrising.

Riskfactorsforallskincancertypesincludeexcessivesunlightexposure,sunbeduse,fairskinandsusceptibilitytosunburn.Formelanoma,alargenumberofbenignmelanocyticnaeviandfamilyhistoryareriskfactors.ForSCC,multiplesmallactinickeratoses,highlevelsofpreviousUV-Aphotochemotherapyandimmuno-suppressionarealsoriskfactors.Peoplewithmultipleatypicalnaeviandastrongfamilyhistorymayhaveanincreasedriskofdevelopingskincancer.Skincancersareveryinfrequentinpeoplewithdarkskinandinchildrenunder15years.

Guidesforassessmentincludethe7-pointchecklistandtheABCD(Asymmetry,Borderirregular,Colourirregular,Diameterincreasing)checklist.Somemelanomaswillhavenomajorfeatures.

Thedermatoscopeisausefultoolfortrainedcliniciansscreeningpigmentedlesionsasitcanincreasediagnosticaccuracy.

Peoplepresentingwithaskinlesionsuggestiveofcancershouldnormallybereferredtoadermatologist,dependingonlocalarrangements.

Urgentsuspicionofcancerreferral

Lesionsonanypartofthebodywhichhaveoneormoreofthefollowingfeatures:

• Changeincolour,sizeorshapeinanexistingmole

• MoleswithAsymmetry,Borderirregularity,Colourirregularity,Diameterincreasing or>6mm

• Newgrowingnodulewithorwithoutpigment

• Persistent(morethanfourweeks)ulceration,bleedingoroozing

• Persistent(morethanfourweeks)surroundinginflammationoralteredsensation

• Neworchangingpigmentedlineinanailorunexplainedlesioninanail

• Slowgrowing,non-healingorkeratinisinglesionswithinduration(thickenedbase)

• AnymelanomaorinvasiveSCCorhighriskBCCdiagnosedfrombiopsy

• Anyunexplainedskinlesioninanimmuno-suppressedpatient

• BCCinvadingpotentiallydangerousareas,forexampleperi-ocular,auditorymeatusoranymajorvesselornerve

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Goodpracticepoints

• Lesionswhicharesuspiciousformelanomashouldnotberemovedinprimarycare.Allexcisedskinspecimensshouldbesentforpathologicalexamination

• Lesionssuspiciousofbasalcellcarcinomas(BCC)maynotrequireurgentreferral,exceptthoseinvadingpotentiallydangerousareas

• Referralsshouldbeaccompaniedbyanaccuratedescriptionofthelesion(includingsize,painandtenderness)andphotosifpossible,subjecttoclinicalgovernancearrangements,topermitappropriatetriage

3.7 GYNAECOLOGICALCANCERS

OvariancancerOver90%ofwomenwithovariancancerareovertheageof40yearsondiagnosis.AmongwomeninScotlandwithnofamilyhistorythelifetimeriskofdevelopingovariancancerisestimatedtobe1in59.Approximately610newcasesofovariancancerarediagnosedinScotlandeveryyear.Ovariancancersareusuallydiagnosedlateandapproximately30%ofcaseshaveapalpablepelvicmass.Symptomsareoftennon-specificabdominalsymptomsbutarecharacterisedbytheirpersistencyandfrequency.

Familyhistory(bothmaternalandpaternal)ofbreastorovariancancercanbeusedtoidentifywomenwhohaveahigherriskofdevelopingovariancancer.Guidanceforreferraltoregionalgeneticcentres(Appendix5)forthosewithafamilyhistoryisavailablewithinSIGNguideline135Managementofepithelialovariancancer13.

EndometrialcancerMostpeople(95%)withendometrialcancerpresentwithpostmenopausalbleeding.Thiscancerisuncommoninpremenopausalwomen(<5%).Approximately690newcasesarediagnosedinScotlandeachyear.Riskfactorsforendometrialcancerinclude:tamoxifen,obesity,ageover45years,nulliparity,familyhistoryofcolonorendometrialcancerandexposuretounopposedoestrogens.Ahighersuspicionofriskshouldbeusedinthesewomen.Notethatthrombocytosisisariskmarkerforunderlyingmalignancyincludingendometrialcancer.

CervicalcancerCervicalcanceraffectsalladultagegroups,with50%ofcasesoccurringbetweentheagesof30and50years.TheincidenceofcervicalcancerinScotlandisaround12.3per100,000populationanditsestimatedlifetimeriskaround1in106.

Themajorityofcases(80%)arediagnosedonspeculumexaminationandupto40%arescreendetected.Typicalsymptomsincludevaginaldischarge,postmenopausalbleeding,postcoitalbleedingandpersistentintermenstrualbleeding.Acytologytestisnotrequiredbeforereferral,andapreviousnegativeresultisnotareasontodelayreferral.

13 SIGN135Managementofepithelialovariancancerhttps://www.sign.ac.uk/sign-135-management-of-epithelial-ovarian-cancer.html

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VulvalcancerMostcasesofvulvalcanceroccurinwomenover65yearsand90%ofpatientshaveavisibletumouronclinicalexamination.Patientsusuallypresentwithbleeding,discomfort,itchoraburningsensation.Thereareabout106newcasesofvulvalcancerdiagnosedeveryyearinScotland.

VaginalcancerVaginalcancerisrareandcompriseslessthan1%ofgynaecologicalcancers.Itismostcommonlydiagnosedinwomenabove60yearsandisrareinwomenlessthan40years.Approximately25newcasesofvaginalcancerarediagnosedinScotlandeveryyear.

Urgent suspicion of cancer referral

Ovariancancer • Abnormalultrasoundscanand/orCA125level

• Ascitesand/orultrasound-confirmedpelvicorabdominalmass(thatisnotobviouslyuterinefibroids,gastrointestinalorurologicalinorigin)

Endometrialcancer

• Anywomanonhormonereplacementtherapy(HRT),presentingwithpersistentorunexplainedpostmenopausalbleeding,aftercessationofHRTforfourweeks

• Unscheduledvaginalbleedinginapatienttakingtamoxifen

• Postmenopausalbleeding

• Persistentintermenstrualbleeding,especiallywithotherriskfactorsdespiteanormalpelvicexamination

• Awomanpresentingwithapalpableabdominalorpelvicmassonexaminationthatisnotobviouslyuterinefibroids,gastrointestinalorurologicalinoriginshouldbereferredurgentlyforultrasoundscanand,ifsignificantconcern,simultaneouslytoaspecialist.Awaitingresultsoftheultrasoundscanshouldnotdelayreferral

Cervicalcancer • Anywomanwithclinicalfeatures(vaginaldischarge,postmenopausal,postcoitalorpersistentintermenstrualbleeding)andabnormalitysuggestiveofcervicalcanceronexaminationofthecervix

Vulvalcancer • Anyunexplainedvulvallumpfoundonexamination

• VulvalbleedingduetoulcerationVaginalcancer • Anysuspiciousabnormalityofthevaginaonspeculum

examination

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Goodpracticepoints

Anabdominalpalpationshouldbeundertaken,CA125bloodserumlevelmeasuredandurgentpelvicultrasoundscancarriedoutin:

• anywomanover50yearswhohasexperiencednewsymptomswithinthelast12monthsthatsuggestirritablebowelsyndrome,or

• women(especiallythoseover50years)withoneormoreunexplainedandrecurrentsymptoms(mostdays)of:

• abdominaldistensionorpersistentbloating

• feelingfullquicklyordifficultyeating

• lossofappetite

• pelvicorabdominalpain

• increasedurinaryurgencyand/orfrequency

• changeinbowelhabit

Afullpelvicexamination,includingspeculumexaminationofthecervix,shouldbecarriedoutinwomenpresentingwith:

• significantalterationsintheirmenstrualcycle

• intermenstrualbleeding

• postcoitalbleeding

• postmenopausalbleeding

• vaginaldischarge,or

• pelvicpain

Avulvalexaminationshouldbecarriedoutforanywomanpresentingwithanyvulvalsymptom.

Ifthereissignificantconcern,awaitingtheresultsofanyinvestigationshouldnotdelayreferral.

Primarycaremanagement

• Symptoms(asabove)persistingorworseningforanywomanwhohasanormalCA125withnormalultrasound,assessforotherclinicalcausesandinvestigateasappropriateorrefertoappropriatesecondarycareservices,dependingonlocalarrangements

• Womenpresentingwithvulvalsymptomsofpruritusorpainshouldbeexaminedpriortoinitiationofanytreatmentandfollowupshouldalsoincludeexaminationuntilsymptomsareresolvedoradiagnosisisconfirmed

• Referurgentlyorroutinely,ifsymptomspersist,dependingonthesymptomsandthedegreeofconcernaboutcancer

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3.8 HAEMATOLOGICALCANCERS

Haematologicalcancerscanpresentwithavarietyofsymptoms.Acombinationofsymptomsandsigns,oftennon-specific,maysuggesthaematologicalcancerandwarrantfurtherexamination,investigationandpossiblereferral.

Leukaemia(acuteandchronic)Approximately690peoplearediagnosedwithleukaemiainScotlandeachyear.Althoughallagescanbeaffected,around70%ofcasesoccurinpeopleagedover60years.Symptomsand/orsignsofbonemarrowfailuresuchasfatigue,pallor,bruising,bleedingandinfectionscanoccur.Fatigueandvulnerabilitytoinfectioncanresultfrommosttypesofhaematologicalcancerbutareparticularlysevereinacuteleukaemia.Someleukaemiasmaypresentwithlymphadenopathyand/orhepatosplenomegaly.Themostcommonformofleukaemiainadultsischroniclymphocyticleukaemia(CLL),whichisoftenanindolentdiseaseandanincidentalfinding.

Non-Hodgkin’slymphomaApproximately1,000newcasesofnon-Hodgkin’slymphomaarediagnosedinScotlandeachyear.Althoughallagescanbeaffected,around75%ofcasesoccurinpeopleagedover60years.Commonsymptomsorsignsatpresentationincludefatigue,weightloss,nightsweats,lymphadenopathyandhepatosplenomegaly.

Hodgkin’slymphomaApproximately160newcasesofHodgkin’slymphomaarediagnosedinScotlandeachyear,with40%ofcasesoccurringinpeopleundertheageof40years.Clinicalfeaturesatpresentationaresimilartothosefornon-Hodgkin’slymphoma,but95%ofpeoplepresentwithlymphglandinvolvement.

MyelomaApproximately430newmyelomacasesarediagnosedinScotlandeachyear.About84%ofcasesoccurinpeopleagedover60years.Clinicalfeaturesatpresentationincludebonepain,symptomsofanaemia,renalimpairment,andsymptomsofhypercalcaemia(suchaspolyuriaandpolydipsia).

Thepresenceofanisolatedparaproteinormonoclonalgammopathyofunknownsignificance(MGUS)isnotacancer,andisacommonincidentalfindingintheelderly(10%over85years).12%ofpeoplewithMGUS,however,willdevelopmyelomaorrelateddiseasewithin10years.AllpatientswithMGUSshouldthereforebemonitoredtodetectprogressioninparaproteinlevel.Somepeoplewithaparaproteinareatmoreriskofdevelopingmyelomathanothers,andthiscanoftenbepredictedfromresults.Discussionwithahaematologististhereforeencouragedifinanydoubt.

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Forpeoplepresentingwiththesenon-specificsymptoms,theclinicianshouldalwaysconsidercheckinghumanimmunodeficiencyvirus(HIV)statusalongwithotherroutineinvestigations.Routinetestsandinvestigationsshouldberepeatedatleastonceifaperson’sconditionremainsunexplained.Ifmyelomaissuspected,urineaswellasserumelectrophoresisshouldbeperformed.

Arrangementsforbiopsyofpersistentabnormallymphglandsvarylocally.

Notethatthrombocytosisisanon-specificriskmarkerforunderlyingmalignancy,especiallylung,endometrial,gastric,oesophagealandcolorectalcancers(LEGO-C).

Allpeoplepresentingwithsymptomsorsignssuggestinghaematologicalcancershouldbereferredtoateamspecialisinginthemanagementofhaematologicalcancer,dependingonlocalarrangements.

Urgentsuspicionofcancerreferral

• Bloodcount/filmreportedassuggestiveofacuteleukaemiaorchronicmyeloidleukaemia*

• Lymphadenopathy(>2cm)persistingforsixweeksorincreasinginsizeorgeneralised(HIVstatusshouldalwaysbecheckedifgeneralised)

• Hepatosplenomegalyintheabsenceofknownliverdisease

• Bonepainassociatedwithaparaproteinand/oranaemia

• BoneX-raysreportedasbeingsuggestiveofmyeloma

• Thefollowingclinicalfeaturesmayalsomeriturgentreferral:

• fatigue

• nightsweats

• weightloss

• itching

• bruising

• recurrentinfections

• bonepain

• polyuriaandpolydipsia(hypercalcaemia)

*willnormallybeidentifiedinthelaboratoryandcommunicatedtotheclinicianformanagementtobeagreed.

Primarycaremanagement

• CLLinanolderpersonshouldbediscussedwithalocalhaematologistbutmanycasesdonotrequiredetailedhaematologicalreview

• Asymptomaticmonoclonalgammopathymaybefollowedupinprimarycaredependingonlocalarrangements–considerdiscussionwithahaematologistifanyconcern

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3.9 HEADANDNECKCANCERS

Theincidenceofheadandneckcancerisincreasing;around1,200peoplearediagnosedwithaheadandneckcancereachyearinScotlandandaround240withthyroidcancers.Theincidenceoforopharyngealcancerisincreasingintheyoungerpopulation,andappearstobeassociatedwithhumanpapillomavirus(HPV)infection.

Riskfactorsforheadandneckcancers(excludingthyroid)include:socialdeprivation;smoking;HPV;alcohol;drugs(especiallyopioidsandcannabis);poordiet;tobaccochewinghabits(includingbetel,gutkhaandpan);andolderage.TheriskofdevelopingnasopharyngealcancerishigherinpeopleofChineseoriginandahigherindexofsuspicionshouldbeusedinthesepeople.

Ifanyuncertaintyaboutthesignificanceofanabnormalityinthemouth,adentist’sopinionshouldbesoughtinthefirstinstance.Thereshouldbesystemsinplaceforurgentsuspicionofcancerreferralpathwaysfordentists.

Allpeoplewithfeaturessuspiciousofmalignancyshouldbereferredtoateamspecialisinginthemanagementofhead,neckorthyroidcancers,dependingonlocalarrangements.

Withthechangingpatternofdisease,age,non-smokingornon-drinkingstatusshouldnotbeabarriertoreferral.

Emergency(sameday)referral

• Stridor

Urgentsuspicionofcancerreferral

Headandneckcancer• Persistentunexplainedheadandnecklumpsfor>threeweeks

• Unexplainedulcerationorunexplainedswelling/indurationoftheoralmucosapersistingfor>threeweeks

• Allunexplainedredormixedredandwhitepatchesoftheoralmucosapersistingfor>threeweeks

• Persistent(notintermittent)hoarsenesslastingfor>threeweeks.Ifothersymptomsarepresenttosuggestsuspicionoflungcancer,refervialungcancerguideline

• Persistentpaininthethroatorpainonswallowinglastingfor>threeweeks

ThyroidCancer• Solitarynoduleincreasinginsize

• Thyroidswellingage16andunder

• Thyroidswellingwithoneormoreofthefollowingriskfactors:

• neckirradiation

• familyhistoryofendocrinetumour

• unexplainedhoarseness

• cervicallymphadenopathy

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3.10 BRAINANDCENTRALNERVOUSSYSTEMCANCERS

Approximately1,000peopleinScotlandarediagnosedwithprimarytumours(malignantandnon-malignant)ofthebrainandCNS,eachyear.Approximately41%ofthesecasesaremalignantbraincancer(excludingmeninges,cranialnerves,pituitarygland,craniopharyngealduct,andpinealgland),ofwhichmorethan80%occurinpeopleovertheageof40years.However,metastaticcancercommonlyinvolvesthebrainandpresentswithsimilarfeatures.

TheanatomicallocationofCNStumoursinfluencessymptomsthatincludephysical,cognitiveandpsychologicalcomponents.

Braintumoursarethecommonestcauseofcancerrelateddeathinchildrenandpeopleunder40years.Peoplewithbraintumourstypicallypresentwithprogressiveneurologicaldeficit(suchasprogressiveweakness,sensoryloss,dysphasia,ataxia),developingoverdaystoweeks.Othersignsandsymptomsinclude:seizuredisorder;headachewithevidenceofraisedintracranialpressure(suchasvomitingandpapilloedema);andcognitiveorbehaviouralchanges.Anadultpresentingwithnewonsetseizuredisorderofanytypehasaprobabilityof2-6%ofhavingabraintumour,whereasnewonsetstatusepilepticusisassociatedwithaprobabilityof10%ormore.Apersonpresentingwithchronicdailyheadachewithoutfeaturesofraisedintracranialpressurehasaverylowprobabilityofhavingabraintumour.

Spinalcordtumoursoftencauseneurologicalsymptomsincludingbackandneckpain,numbness,andtinglingandweaknessinthearmsorlegs.Tumoursinthelowerpartofthespinalcordmaycauselossofcontrolofthebladderandbowel.PleaserefertotheguidelineonMalignantSpinalCordCompressionforfurtherguidance.

Emergency(sameday)referral

Headache • Patientswithheadacheand/orvomitingwithpapilloedema

Urgentsuspicionofcancerreferral

Neurologicaldeficit

• Progressiveneurologicaldeficit(includingpersonality,cognitiveorbehaviouralchange)intheabsenceofpreviouslydiagnosedorsuspectedalternativedisorders(suchasmultiplesclerosisordementia)

Seizure • Anynewseizure

• Seizureswhichchangeincharactersuchaspost–ictaldeficit,headache,increasedfrequency,etc.

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Goodpracticepoints

• Considerurgentinvestigation/referralforpeoplewithnon-migrainousheadachesofrecentonset,whenaccompaniedby‘redflag’featuressuggestiveofraisedintracranialpressure(forexample:wokenbyheadache;vomiting;drowsiness),progressiveneurologicaldeficitornewseizuredisorder

• AllNHSBoardshavepathwaysforinvestigationofheadacheswhichshouldincludePrimaryCaredirectaccesstoimaging

• Ifanyuncertaintyaboutthepresenceofpapilloedema,thepersonshouldbeurgentlyreferredtoanoptometristforassessment.Iftherearered-flagssuspiciousofcancerasdetailedabove,asimultaneousurgentsuspicionofcancerreferraltosecondarycareshouldbemade.Ifpapilloedemaisconfirmed,theoptometristshouldreferdirectlytosecondarycare

• Anurgent,suspicionofcancerpathwayshouldexistinallNHSBoardsforoptometriststoreferdirectlytosecondarycareforpeoplewithopticdiscssuspiciousofpapilloedema

3.11 SARCOMAS AND BONE CANCERS

SofttissuesarcomasApproximately140peoplearediagnosedwithsofttissuesarcomasinScotlandeachyear.Around90%arediagnosedinpeopleaged40yearsorolderandalmost60%inpeopleovertheageof65.Thesetumoursarefrequentlymissedoronlyreferredafterrepeatpresentations.

Inadults,softtissuemassesthataresuperficial,painless,lessthan5cmandstaticinsizeareunlikelytobemalignant.

PrimarybonecancerApproximately50peopleinScotlandarediagnosedwithcanceroftheboneeveryyear.

OsteosarcomaOsteosarcomaisthemostcommontypeofprimarybonecancer.Itcanoccuratanyage,butismostcommonlyfoundinteenagersandyoungadults.Osteosarcomatypicallypresentswithpersistentlocalisedbonepain.Themostcommonsitesarearoundthekneejointandupperarm.

Ewing’ssarcomaTheincidenceofEwing’ssarcomapeaksat10–15yearsofage,andrarelyoccursundertheageoffive,orovertheageof30.Arisinginanybone,themostcommonlyaffectedsitesarethepelvis,lowerlimbbonesandchestwall.Thepredominantsymptomsarepersistentpainandswellingoftheaffectedarea.Ewing’ssarcomaisfrequentlymisdiagnosedasosteomyelitis.

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ChondrosarcomaChondrosarcomaismostoftenfoundinadultsovertheageof40yearsandisrareundertheageof20.Themostcommonsitesinvolvedarethepelvis,femur,andshouldergirdle.Clinicalpresentationisusuallyabonymasswithpainoftenasalatefeature.

Urgentsuspicionofcancerreferral(softtissuesarcoma)

Asofttissuemasswithoneormoreofthefollowingcharacteristics:

• size>5cm

• increasinginsize

• deeptofascia,fixedorimmobile

• recurrenceafterpreviousexcision

• regionallymphnodeenlargement

Investigationforsuspectedbonecancer

AnX-rayoftheappropriateareashouldberequestedonpatientswhohave:

• unexplainedbonepainortenderness,whichis:

• persistent

• increasing

• non-mechanical

• nocturnaloratrest

IfX-rayissuggestiveofbonetumour,referasurgentsuspicionofcancertosarcomaservice.

Goodpracticepoints

• SarcomasofthelongbonesareusuallyexcludedbynormalX-raybutfurtherinvestigationmayberequiredforspine,pelvis,ribsorscapula

• IfsymptomspersistbutX-rayisnormal,repeatX-ray(followingdiscussionswithradiologist)andconsiderreferral

• Suspectedspontaneousorlowimpactfractureshouldraisesuspicionofunderlyingmalignancy

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3.12 CHILDREN,TEENAGERSANDYOUNGADULTCANCERS

Whilecancerinchildren,teenagersandyoungadults(CTYA)israre,itisasignificantcauseofmortalityandmorbidityandearlydetectionisimportant.Approximately120childrenagedlessthan15years,and180youngpersonsagedbetween15and24years,arediagnosedwithcancerinScotlandeachyear.Theyoungestagegroup(0-4years)accountsfor48%ofallchildhoodcancers.Thedistributionofcancersinteenagersandyoungadultsisdifferentfromthatinbothyoungchildrenandadults.Thetwomostcommonlyoccurringcancersinchildhoodareleukaemia(acutelymphoblasticleukaemia)andcentralnervoussystem(mostlybrain)tumours.Thetwomostcommonlyoccurringcancersinmaleteenagersandyoungadultsarelymphomaandtesticularcancer,andmalignantmelanomaandlymphomainfemalesinthisagegroup.

Noriskfactororfamilialsusceptibilitycanbeidentifiedinmostcases.However,geneticsusceptibilityisapparentinsomecaseswithassociatedconditions(suchasDownsyndrome,familialadenomatouspolyposiscoli,neurofibromatosis,aniridiaandLiFraumenisyndrome).Theabsenceofafamilyhistoryshouldnotdelayfurtherinvestigations.

Cancersinchildren,teenagersandyoungadultsarefrequentlydiagnosedlateduetothenon-specificnatureofmanyofthesymptoms.AusefulresourceforparentsistheawarenesscardidentifyingwarningsymptomsandsignsofchildhoodcancerproducedbytheGraceKellyLadybirdTrust14.

Manyofthecancerspecificguidelinesinthisdocumentarerelevanttoallagese.g.melanoma,brain&CNS,sarcoma,etc.Someconditionsspecifictothisagegroupneedspecialmention:

Lymphomas • Hodgkin’s lymphoma:approximately30newcasesarediagnosedinchildrenandyoungadultslessthan24years,inScotlandeveryyear.Approximately83%ofthesecasesareagedbetween15and24years.Hodgkin’slymphomararelyoccursundertheageoffive.Itsnaturalhistorymaybelong(months),andaboutathirdofpatientshavesystemicsymptoms.

• Non-Hodgkin’s lymphoma:approximately20newcasesarediagnosedinchildrenandyoungadultslessthan24years,inScotlandeveryyear.Approximately70%ofthesecasesareagedbetween15and24years.Non-Hodgkin’slymphomahasamorerapidprogressionofsymptomsthanHodgkin’slymphoma.

Leukaemia • Approximately55newcasesarediagnosedinchildrenandyoungadultslessthan24yearsinScotlandeveryyear(about70%areagedunder15andabout38%underfive).Thisaccountsforabout8%ofpeoplediagnosedwithleukaemia,everyyear.

14 GraceKellyLadybirdhttps://www.gracekellyladybird.co.uk/knowthesigns

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Neuroblastoma • Themajorityofchildrenpresentwithabdominaldistension(withorwithoutapalpablemass)andsymptomsofbonemarrowfailure.Infantsunderoneyearofagemayhavelocalisedabdominalorthoracicmasses;veryyounginfants,lessthansixmonthsold,mayhavemassivehepatomegalyandskinlesions.

Braintumours • Braintumoursremainoneofthemostcommoncausesofcancer-relateddeathinchildrenandpeopleunder40years.Approximatelytwofifthsofdeathsamongchildrenagelessthan15dyingfromcancerareduetoCNStumours.

• Headsmart15isanonlineresourcewhichpresentsevidencebaseddetailedguidanceindifferentagegroups.

Wilms’tumour(nephroblastoma)

• Commonpresentingfeaturesincludeunexplainedvisiblehaematuria,orunilateralabdominalmass,withorwithoutpain,inawell-child.

Bone tumours • Osteosarcoma:canoccuratanyagealthoughapproximately60%presentintheseconddecadeoflife.Mostcommonsitesarefemur,tibiaandhumerus.

• Ewing’ssarcoma:peakincidenceisbetween10and15years.Ewing’ssarcomararelyoccursundertheageoffiveyearsorovertheageof30years.Mostcommonlyaffectedsitesarethepelvis,femur,tibia,fibula,ribandhumerus.

• SarcomasofthelongbonesareusuallyexcludedbynormalX-raybutfurtherinvestigationmayberequiredforspine,pelvis,ribsorscapula.

Retinoblastoma • Commonsymptomsofretinoblastomaincludewhiteorabsentpupillaryredreflexandsquint.

• Thereisafamilyhistoryinapproximately15%ofcases.Gonadaltumours • Testicularcancerisoneofthemostcommoncancersinmale

teenagersandyoungadults.

• Testicularorotherscrotalmassescanbedifficulttodifferentiate–anynontransilluminablemassassociatedwiththetestisissignificant.

• Ovariantumourscanbeassociatedwithprecociouspuberty.

Generalrecommendations

• Considerreferralforanypatientwithrepeatpresentations(threeormoretimes)ofanysymptomswhichdonotappeartoberesolvingorfollowinganexpectedpattern,takingintoaccountparentalorcarerandpatientconcern

• Inachildwheresymptomsandsignsdonotclearlyfitwiththeseguidelines,butneverthelessleadtoconcernaboutexcludingcancer,thereferrershouldconsiderdiscussingthecasewithaseniorpaediatriccolleagueattheirearliestconvenience

15 Headsmarthttps://www.headsmart.org.uk/

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SpecificrecommendationsUrgentsuspicionofcancerreferral

• Unexplainedpetechiaeorpurpuraisalwaysanindicationforemergencyreferral

• Unexplainedfatigue,persistentpallor,failuretothriveorweightloss

• Anynewpersistentunexplainedpain,particularlybackpainornocturnalpain

• Unexplainedabdominalmassordistension

• Unexplainedvisiblehaematuria

Bonepain,especiallyif: • diffuseorinvolvestheback

• persistentlylocalisedatanysite

• nocturnalpain

• limping

• requiringanalgesia,or

• limitingactivity

Lymphadenopathy,if: • nontender,firm/hardandgreaterthan2cmsinmaximumdiameter

• progressivelyenlarging

• associatedwithothersignsofgeneralillhealth,feverorweightloss

• involvesaxillarynodes(nolocalinfectionordermatitis)oranysupraclavicularlymphadenopathy

Headache,ifincreasinginseverityorfrequencyand:

• worseinthemorningorcausingearlywakening,or

• associatedwithvomitingoranynewneurologicalsigns

Anynewneurologicalsigns,signs(suchasweakness,lossofbalance,etc.)especiallyif:

• associatedwithbehaviouralchangeordeteriorationinnormaldailyorschoolperformance

Otherpossiblesignsofbraintumours:

• increasingheadcircumference

• failureoffontanelleclosure

• abnormalheadpositionsuchaswryneck,headtiltorstiffneck

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Softtissuemass,if: • showsrapidorprogressivegrowth

• sizegreaterthan2cmmaximumdiameter

• deeptofascia,fixedorimmobile,regardlessofsize

• recurrenceafterpreviousexcisionofsarcoma

• associatedwithregionallymphnodeenlargement

Eyes: • anynewsquint,ifassociatedwithheadacheorotherneurologicalsigns(otherwiseconsideroptometristandophthalmologyassessment)

• changeinpupillaryredreflextoabsentorwhite

Primarycaremanagement

• X-rayifthereisunexplainedbonepainof:

• increasingseverity

• persistent

• tender

• non-mechanicalbonepainparticularlyifdisturbingrestorsleep

• IfsymptomspersistbutX-rayisnormal,repeatX-ray(afterdiscussionwitharadiologist)andconsiderreferral,especiallyifthepatientpresentsthreeormoretimes

• Spontaneousorminortraumafractureshouldraisesuspicionofbonecancer

3.13 MALIGNANT SPINAL CORD COMPRESSION

Thetrueincidenceofmalignantspinalcordcompression(MSCC)andepiduraldiseaseisunknown.Approximately5-10%ofpatientswithcancerdevelopmetastaticspinalcordcompression.ThemajorityofpatientsdiagnosedwithMSCChaveanestablisheddiagnosisofcancer,butforsome(10-20%),MSCCisthepresentingfeatureofmalignancy.ManypeoplewithcancerareatriskofMSCCbutparticularlythosewithlung,breast,prostatecancerormultiplemyeloma,whichaccountforapproximately60%ofcasesofMSCC.

About90%ofpatientsareover50yearsofageandnearlyallMSCCpatientshavepain,usuallyseverespinalnerverootpain(80%)withorwithoutlocalbackpain.ThesiteofpainandthesiteofcompressiondonotalwayscorrelateandX-raysandbonescansmaybemisleading.MSCCisusuallydiagnosedlate,bywhichtimelytreatmentmaywellbeineffective–onceparaplegiadevelopsitisusuallyirreversible.MSCCshouldbedealtwithasanoncologicalemergency.

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AnormalneurologicalexaminationdoesnotprecludeepiduraldiseaseorevolvingMSCC.ThedefinitivemethodofinvestigationisMRIofthewholespine.Allpatientswithbonemetastasis,orconsideredbytheircliniciantobeathighriskofdevelopingMSCC,shouldbegivenwrittenguidanceonearlysymptomswithadvicetocontactahealthcareprofessionalpromptly.Thisinformationshouldalsobesenttothe GP.WritteninformationonearlysymptomsshouldalsobegiventopatientsfollowingtreatmentforMSCC.AllScottishcancernetworkshavedevelopedlocallyagreedMSCCpathways.MoreinformationisavailableviatheScottishPalliativeCareGuidelineswebsite.16

Urgentsuspicionofcancerreferralforpatientswithknowncancer(particularlyprostate,breast,lungormultiplemyeloma)

Peoplewithahistoryofcancerandanyofthefollowingsymptoms:

• significantlocalisedbackpain,especiallythoracic

• severe,progressivepainorpoorresponsetomedication

• spinalpainaggravatedbystraining(forexample,atstool,orcoughingorsneezing)

• nocturnalspinalpain,especiallyifpreventingsleep

• radicularpain(forexample,roundchest,downfrontorbackofthighs)

• limbweaknessordifficultyinwalking

• sensoryloss(includingperinealorsaddleparaesthesia)

• bladderorboweldysfunction

16 ScottishPalliativeCareGuidelineshttps://www.palliativecareguidelines.scot.nhs.uk/guidelines/palliative-emergencies/malignant-spinal-cord-compression.aspx

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APPENDICES

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APPENDIX1:METHODOLOGYUSED–2014VERSION

Thesteeringgroupagreedthatthestartingpointfortherevisionprocesswouldbetheexistingreferralguidelines,enhancedbyareviewofevidence-basedrecommendationsforreferralfromacrosstheworld.Theserecommendationswereidentifiedfromasearchofthewebsitesforanumberofguideline-producingorganisationsinJune2012.ThiswassupplementedwithasearchforrelevantguidelinesinMedlineandEmbase.ThesearchwasupdatedinJanuary2013toensurethatallrelevantguidelineswereidentified.OnlyguidelinespublishedinEnglishwereconsidered.

AlltheguidelinesidentifiedbythesearchwereappraisedformethodologicalqualityusingtheAppraisalofGuidelinesforResearchandEvaluationII(AGREEII)instrument.AGREEIIisavalidatedtoolusedfortheassessmentofclinicalpracticeguidelines.Itconsistsof23itemsorganisedintosixqualitydomainsthatcoverseparatedimensionsofguidelinequality.Eachguidelineisassignedanoverallqualityratingandadecisionregardingwhethertheguidelinewouldberecommendedforpracticeisalsomade.Eachitemisratedona7-pointscale(1=stronglydisagreeto7=stronglyagree).Anoverallsummaryofrecommendationsandqualityratingforeachguidelinewascompiledintoevidencetableswhichareavailableatwww.healthcareimprovementscotland.org.

Thegroupsidentified,reviewedandsystematicallyconsidereddifferencesinrecommendationsemergingfromtheguidelines,inthelightoftheirclinicalandpracticalexperienceaswellastheirexpertknowledgeoftheliterature,whiletakingaccountoftheScottishcontext.WhereScottishIntercollegiateGuidelinesNetwork(SIGN)guidelinesareinplaceorarebeingrevised,effortwasmadetoensureconsistencybetweentheseguidelinesandtherelatedSIGNguideline.

APPENDIX2:MEMBERSHIPOFGUIDELINESTEERINGGROUP2018

PeterHutchison Chair,retiredGeneralPractitioner,NHSDumfries&Galloway

HughBrown GeneralPractitioner,NHSAyrshire&Arran,ChairofTheScottishPrimaryCareCancerGroup

SaraTwaddle DirectorofEvidence,HealthcareImprovementScotland

NicolaBarnstaple ProgrammeDirector,CancerAccessTeam,ScottishGovernment

ValDoherty ClinicalAdvisor,CancerAccessTeam,ScottishGovernment

AndrewGrierson ProjectManager,CancerAccessTeam,ScottishGovernment,NHSNationalServicesScotland

LorraineSloan StrategicPartnershipManager,Scotland,MacmillanCancerSupport

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APPENDIX3:MEMBERSHIPOFTHEGUIDELINEDISSEMINATIONGROUP

PeterHutchison Chair,retiredGeneralPractitioner,NHSDumfries&Galloway

HughBrown GeneralPractitioner,NHSAyrshire&Arran.ChairofTheScottishPrimaryCareCancerGroup

SaraTwaddle DirectorofEvidence,HealthcareImprovementScotland

DianePrimrose ProgrammeManager,CancerAccessTeam,ScottishGovernment

AndrewGrierson ProjectManager,CancerAccessTeam,ScottishGovernment,NHSNationalServicesScotland

LorraineSloan StrategicPartnershipManager,Scotland,MacmillanCancerSupport

CaraTaylor MacmillanCancerNurseConsultant,NHSTayside

PaulBaughan GeneralPractitioner,NHSForthValley,HealthImprovementScotlandClinicalLeadPalliativeandEndofLifeCare,MacmillanGeneralPractitionerAdvisor(Scotland)

LornaPorteous GeneralPractitionerLeadforCancerandPalliativeCareinLothian,MacmillanGeneralPractitionerAdvisor(Scotland)

MarionO’Neill RegionalManager,ScotlandandNorthernIreland,CancerResearchUK

DouglasRigg GeneralPractitioner,NHSGreaterGlasgow&Clyde

AdamOsprey Policy&DevelopmentPharmacist,CommunityPharmacyScotland

MeilingDenney AssistantDirectorofPostgraduateGeneralPractitionerEducation,NHSEducationforScotland&SigiJoseph,GeneralPractitioner,NHSLothian

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APPENDIX4:MEMBERSHIPOFGUIDELINESUBGROUPS

UrologySubGroup2018

PeterHutchison Chair,retiredGeneralPractitioner,NHSDumfries&Galloway

HughBrown ViceChair,GeneralPractitioner,NHSAyrshire&Arran.ChairofScottishPrimaryCareCancerGroup

AndrewGrierson ProjectManager,CancerAccessTeam,ScottishGovernment,NHSNationalServicesScotland

LorraineSloan StrategicPartnershipManager,Scotland,MacmillanCancerSupport

AlexLaird ConsultantUrologist,NHSLothian

SeamusTeahan WoSCANLeadClinicianCancerandUrologicalSurgeon,NHSForthValley

AdamGaines Director,ProstateScotland

Children,TeenagersandYoungAdultsSubGroup2018

PeterHutchison Chair,retiredGeneralPractitioner,NHSDumfries&Galloway

HughBrown ViceChair,GeneralPractitioner,NHSAyrshire&Arran.ChairofScottishPrimaryCareCancerGroup

AndrewGrierson ProjectManager,CancerAccessTeam,ScottishGovernment,NHSNationalServicesScotland

LorraineSloan StrategicPartnershipManager,Scotland,MacmillanCancerSupport

PamNeilson FoundingMemberandCampaigner,GlowGoldChildhoodCancerCampaign

PatHayes Campaigner,GlowGoldChildhoodCancerCampaign

EmmaBarron ParentRep,GlowGoldChildhoodCancerCampaign

JulietteMurray ConsultantBreastSurgeon,NHSLanarkshire

KirstyKilpatrick FY2,NHSAyrshire&Arran

KatyMarshall FY2,NHSAyrshire&Arran

HamishWallace ProfessorofPaediatricOncology,UniversityofEdinburgh&RoyalHospitalforSickChildren

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HeadandNeckSubGroup2018

PeterHutchison Chair,retiredGeneralPractitioner,NHSDumfries&Galloway

HughBrown ViceChair,GeneralPractitioner,NHSAyrshire&Arran.ChairofScottishPrimaryCareCancerGroup

AndrewGrierson ProjectManager,CancerAccessTeam,ScottishGovernment,NHSNationalServicesScotland

CatrionaDouglas Ear,NoseandThroatSurgeon,NHSGreaterGlasgow&Clyde

CraigWales ConsultantOralMaxillofacialSurgeon,NHSGreaterGlasgow&Clyde

GuyVernham ConsultantHeadandNeckSurgeon,NHSLothian

JenniferMontgomery Ear,NoseandThroatSurgeon,NHSGreaterGlasgow&Clyde

BrainandCentralNervousSystemSubGroup2018

PeterHutchison Chair,retiredGeneralPractitioner,NHSDumfries&Galloway

HughBrown ViceChair,GeneralPractitioner,NHSAyrshire&Arran.ChairofScottishPrimaryCareCancerGroup

AndrewGrierson ProjectManager,CancerAccessTeam,ScottishGovernment,NHSNationalServicesScotland

LorraineSloan StrategicPartnershipManager,Scotland,MacmillanCancerSupport

LindsayCampbell ManagedClinicalNetwork(MCN)Manager,WestofScotlandCancerNetwork(WoSCAN)

ImranLiaquat ConsultantNeurosurgeon,NHSLothian

ValDoherty ClinicalAdvisor,CancerAccessTeam,ScottishGovernment

CameronMiller HeadofPolicy&PublicAffairs,BrainTumourCharity

SaraTwaddle HeadofEvidence&Technologies,DirectorofScottishIntercollegiateGuidelinesNetwork(SIGN),HealthcareImprovementScotland

JennyBennison GeneralPractitioner,NHSLothian,ViceChairofScottishIntercollegiateGuidelinesNetwork(SIGN)

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LungSubGroup2018

PeterHutchison Chair,retiredGeneralPractitioner,NHSDumfries&Galloway

HughBrown ViceChair,GeneralPractitioner,NHSAyrshire&Arran.ChairofScottishPrimaryCareCancerGroup

LorraineSloan StrategicPartnershipManager,Scotland,MacmillanCancerSupport

JohnMaclay ConsultantPhysician,RespiratoryMedicine,NHSGreaterGlasgow&Clyde

JorisvanderHorst ConsultantRespiratoryPhysician,NHSGreaterGlasgow&Clyde

DouglasRigg GeneralPractitioner,NHSGreaterGlasgow&Clyde

LorraineDallas DirectorofInformationandSupport,TheRoyCastleLungCancerFoundation

LukeDaines AcademicGeneralPractitioner

JulieMencnarowski ClinicalNurseSpecialist,NHSLothian

MohammedAsif ConsultantCardiothoracicSurgeon,NHSGreaterGlasgow&Clyde

DavanandSharma ConsultantRespiratoryPhysician,NHSGreaterGlasgow&Clyde

BreastSubGroup2018

PeterHutchison Chair,retiredGeneralPractitioner,NHSDumfries&Galloway

HughBrown ViceChair,GeneralPractitioner,NHSAyrshire&Arran.ChairofScottishPrimaryCareCancerGroup

LorraineSloan StrategicPartnershipManager,Scotland,MacmillanCancerSupport

ElizabethSmyth ConsultantBreastSurgeon,NHSGrampian

LawrenceCowan NationalManager(Scotland),BreastCancerNow

JulietteMurray ConsultantBreastSurgeon,NHSLanarkshire

LauraWilkinson ConsultantRadiologist,NHSGreaterGlasgow&Clyde

MikeMcKirdy ConsultantBreastSurgeon,NHSGreaterGlasgow&Clyde

JulieDoughty ConsultantBreastSurgeon,NHSGreaterGlasgow&Clyde

AlastairMcMurray FY2,NHSGreaterGlasgow&Clyde

KatyMarshall FY2,NHSAyrshire&Arran

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LowerGISubGroup2018

PeterHutchison Chair,retiredGeneralPractitioner,NHSDumfries&Galloway

HughBrown ViceChair,GeneralPractitioner,NHSAyrshire&Arran.ChairofScottishPrimaryCareCancerGroup

AndrewGrierson ProjectManager,CancerAccessTeam,ScottishGovernment,NHSNationalServicesScotland

LorraineSloan StrategicPartnershipManager,Scotland,MacmillanCancerSupport

GraemeWilson ConsultantColorectalSurgeon,NHSLothian

ClaireDonaghy HeadofScotland,BowelCancerUK

CraigMowat ConsultantGastroenterologist,NHSTayside

SandraMelville LeadPharmacist,RoyalPharmaceuticalSociety

AngusMacDonald ConsultantColorectalSurgeon,NHSLanarkshire

JackWinter ConsultantGastroenterologist,NHSGreaterGlasgow&Clyde

RobBoulton-Jones ConsultantGastroenterologist,NHSGreaterGlasgow&Clyde

LouiseGorman GeneralPractitioner,NHSForthValley

DavidLinden RetiredGeneralPractitionerandClinicalAdvisor,CancerAccessTeam,ScottishGovernment

UpperGISubGroup2018

PeterHutchison Chair,retiredGeneralPractitioner,NHSDumfries&Galloway

HughBrown ViceChair,GeneralPractitioner,NHSAyrshire&Arran.ChairofScottishPrimaryCareCancerGroup

AndrewGrierson ProjectManager,CancerAccessTeam,ScottishGovernment,NHSNationalServicesScotland

LorraineSloan StrategicPartnershipManager,Scotland,MacmillanCancerSupport

LindsayCampbell ManagedClinicalNetwork(MCN),WoSCAN

AlasdairMacmillan ConsultantGastrointestinalSurgeon,NHSLothian

RossCarter PancreaticSurgeon,NHSGreaterGlasgow&Clyde

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SamiShimi NOSCANClinicalLead,NHSTayside

ColinMckay ClinicalDirectorforSurgery,NHSGreaterGlasgow&Clyde

AndrewCrumley ConsultantGeneralandUpperGISurgeon,NHSForthValley

JackWinter ConsultantGastroenterologist,NHSGreaterGlasgow&Clyde

RobBoulton-Jones ConsultantGastroenterologist,NHSGreaterGlasgow&Clyde

CatherinePollock ConsultantGastroenterologist,NHSFife

JaneMoir NurseEndoscopist,NHSAyrshire&Arran

FionaBrown DevelopmentManager,PancreaticCancerScotland

SuzyMercer DevelopmentConsultant,PancreaticCancerScotland

MairiHandy DevelopmentOfficer,PancreaticCancerScotland

NikWhite Head,PancreaticCancerUK

NeilPryde MacmillanLeadCancerGP,andCancerStrategyLead,SpecialtyDoctor,PalliativeCare,NHSFife

HamishMcRitchie ConsultantRadiologistandClinicalLeadforNRTP,NHSBorders

LornaPorteous GeneralPractitionerLeadforCancerandPalliativeCareinLothian,MacmillanGeneralPractitionerAdvisor(Scotland)

JennyBennison GeneralPractitioner,NHSLothian,ViceChairofScottishIntercollegiateGuidelinesNetwork(SIGN)

BenHall GPVocationalTrainee

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APPENDIX5:REGIONALGENETICSCENTRES

AdviceaboutreferralpathwaystoclinicalgeneticsisavailablefromeachoftheRegionalGeneticsCentres:

• Glasgow:www.nhsggc.org.uk/about-us/professional-support-sites/west-of-scotland-genetic-services/clinical-genetics/

• Edinburgh:www.nhslothian.scot.nhs.uk/Services/A-Z/ClinicalGeneticsService/Pages/default.aspx

• Dundee:www.nhstayside.scot.nhs.uk/OurServicesA-Z/Genetics/index.htm

• Aberdeen:www.nhsgrampian.org/medicalgenetics/

APPENDIX6:EQUALITYANDDIVERSITY

TheScottishGovernmentandHealthcareImprovementScotlandarecommittedtoequalityanddiversityinrespectofthenineequalitygroupsdefinedbyage,disability,genderreassignment,marriageandcivilpartnership,pregnancyandmaternity,race,religion,sex,andsexualorientation.

Theguidelinesdevelopmentprocesshasbeenassessedandtheguidelinesareexpectedtohaveapositiveimpactoncertainagegroups(dependingonthetumourtype)andmoredeprivedpopulationsinScotland.Thecompletedequalityanddiversitychecklistisavailableonwww.healthcareimprovementscotland.org.

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APPENDIX7:KEYREFERENCES

• CancerIncidenceinScotland2016https://www.isdscotland.org/Health-Topics/Cancer/Publications/2018-04-24/2018-04-24-Cancer-Incidence-Report.pdf?51368349791

• ScottishCancerRegistry,InformationServicesDivision(ISD),April2018(basedontotalScottishpopulationof5,404,700asat30June2016)http://www.isdscotland.org/Health-Topics/General-Practice/Workforce-and-Practice-Populations/

• NationalRecordsofScotlandmid2016populationestimatesScotlandhttps://www.nrscotland.gov.uk/statistics-and-data/statistics/statistics-by-theme/population/population-estimates/mid-year-population-estimates/mid-2016

• BeatingCancer:AmbitionandActionhttps://www.gov.scot/publications/beating-cancer-ambition-action/

• PractisingRealisticMedicinehttps://www.gov.scot/publications/summary-practising-realistic-medicine/

• NICEGuideline[NG12]Suspectedcancer:recognitionandreferral(June2015lastupdatedJuly2017)https://www.nice.org.uk/guidance/ng12

• CancerResearchUK:YourUrgentReferralhttps://publications.cancerresearchuk.org/categories/your-urgent-referral

• FamilialBreastCancerReportwww.healthcareimprovementscotland.org/our_work/cancer_care_improvement/programme_resources/familial_breast_cancer_report.aspx

• DiagnosisandManagementofColorectalCancerhttp://www.sign.ac.uk/sign-126-diagnosis-and-management-of-colorectal-cancer.html

• ProstateCancerRiskManagementProgramme–SummaryCardhttp://www.gov.scot/Topics/Health/Services/Cancer/Risk-Management/Prostate-Cancer-Summary-Card

• Managementofepithelialovariancancerhttps://www.sign.ac.uk/sign-135-management-of-epithelial-ovarian-cancer.html

• Headsmart–TheBrainTumourCharity,Children’sBrainTumourResearchCentreandtheRoyalCollegeofPaediatricsandChildHealthhttps://www.headsmart.org.uk/

• GraceKellyLadybirdTrusthttps://www.gracekellyladybird.co.uk/ https://www.gracekellyladybird.co.uk/knowthesigns

• CancerWorkingGroupoftheScottishClinicalBiochemistryManagedDiagnosticNetworkhttp://www.mcns.scot.nhs.uk/scbmdn/wp-content/uploads/sites/10/2018/09/Tumour-Marker-bookmark-NHS-download.pdf

• Statisticsonpreventablecancershttps://www.cancerresearchuk.org/health-professional/cancer-statistics/risk/preventable-cancers

• Clinicalrelevanceofthrombocytosisinprimarycare:BrJGenPract2017;67(659):e405-e413.DOI:https://doi.org/10.3399/bjgp17X691109

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