Odd localisation for a gastric cancer histology

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Odd localisation for a gastric cancer histology

KUCZMA, Paulina, et al.

Abstract

More than 96% of signet-ring cell carcinomas occur in the stomach and the rest in other organs, including the gallbladder, pancreas, urinary bladder and breast. Primary signet-ring cell carcinoma of the colon and rectum is very rare, accounting for 0.1%-2.4% of all colorectal cancers.

KUCZMA, Paulina, et al . Odd localisation for a gastric cancer histology. International Journal of Surgery Case Reports , 2016, vol. 27, p. 51-54

DOI : 10.1016/j.ijscr.2016.07.028 PMID : 27543724

Available at:

http://archive-ouverte.unige.ch/unige:93333

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ContentslistsavailableatScienceDirect

International Journal of Surgery Case Reports

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

Odd localisation for a gastric cancer histology

Paulina Kuczma

^a,∗

, Jordi Vidal Fortuny

^a

, Saiji Essia

^b

, Ariane de Lassus

^b

, Philippe Morel

^a

, Frédéric Ris

^a

aDepartmentofVisceralSurgery,GenevaUniversityHospitals,RueGabrielle-Perret-Gentil4,1205Geneva,Switzerland

bDepartmentofPathology,GenevaUniversityHospitals,RueGabrielle-Perret-Gentil4,1205Geneva,Switzerland

a r t i c l e i n f o

Articlehistory:

Received23April2016

Receivedinrevisedform20July2016 Accepted23July2016

Availableonline27July2016

Keywords:

Signet-ringcellcarcinoma Colorectalcancer Bonymetastases Pathology Appendicitis Righthemicolectomy

a b s t r a c t

INTRODUCTION:Morethan96%ofsignet-ringcellcarcinomasoccurinthestomachandtherestin otherorgans,includingthegallbladder,pancreas,urinarybladderandbreast.Primarysignet-ringcell carcinomaofthecolonandrectumisveryrare,accountingfor0.1%–2.4%ofallcolorectalcancers.

PRESENTATIONOFCASE:Wereportacaseofa55-yearoldmanwhoisoperatedforacaecalmassevocative ofanappendicitisabscess.Intraoperatively,wediscoveralarge,ulceratedilio-caecalmasswithseveral lymphadenopathies.Thefurtherworkuprevealsaprimarysignet-ringcellcarcinomaofthecolonwith multiplelymphnodesandosteolyticbonymetastases.

DISCUSSION:Primarysignet-ringcellcarcinomaofthecolonandrectumpresentsusuallyasanadvanced stagediseasewithadismalprognosis.Itspreadsmainlytothelymphnodesandtotheperitoneumand veryrarelytotheliver.Themeanageofpatientsdiagnosedwithprimarysignet-ringcellcarcinomais signiﬁcantlyyoungerthanforordinaryadenocarcinoma.Theupperendoscopyistheinvestigationof choicetoexcludeaprimarygastricpathology.Thereareveryfewreportsaboutthistypeofcancerand noreportsaboutthistypeofcancerassociatedwithosteolyticbonymetastases.

CONCLUSION:Thecharacteristicsandpathophysiologyofaprimarysignet-ringcellcarcinomaofthe colonandrectumarenotwellunderstood.Usuallyonlypalliativetreatmentispossible.Theimportance ofanearlydiagnosisofthistumorismandatorytohaveacurativeapproach.

1. Introduction

Morethan96%ofsignet-ringcellcarcinomasoccurinthestom- achandtherestinotherorgans,includingthegallbladder,pancreas, urinarybladderandbreast[1].PrimarySRCcarcinomaofthecolon andrectumisveryrare,accountingfor0.1%–2.4%ofallcolorectal cancers[2].Comparedtothecolorectaladenocarcinoma,thistumor typeisdiagnosedatmoreadvancedstages,presentsatayounger ageandhasaworseprognosisthanthecommonadenocarcinoma [3,4].Itischaracterizedbyadismalprognosis.Thedissemination patternischieﬂyperitonealwithapredominantlylymphaticinva- sion.Livermetastasesareveryrare[3–6].Ithasbeendescribedthat itsradiographiccharacteristicscanmimicaninﬂammatoryprocess [7].

WereportacaseofaprimarySRCcarcinomaofthecaecumthat presentswithsymptomsandradiologicimagescompatiblewith

Abbreviations:SRC,signet-ringcell.

∗Correspondingauthor.

E-mailaddresses:pkuczma@gmail.com(P.Kuczma),

jordi.vidalfortuny@hcuge.ch(J.VidalFortuny),essia.saiji@hcuge.ch(S.Essia), ariane.delassus@hcuge.ch(A.deLassus),philippe.morel@hcuge.ch(P.Morel), frederic.ris@hcuge.ch(F.Ris).

anacuteappendicitisabscess.Itistreated bysurgicalresection.

Thefurtherworkuprevealsmultipleosteolyticbonymetastases (Fig.1).

Sofar,thereareveryfewreportsaboutthistumortypepre- sentingwithbonymetastasesandbecauseoftherarityoftheSRC carcinoma,its characteristicsand pathophysiologyare not well understood.

2. Presentationofcase

A55-yearoldpatientpresentstoouremergencydepartment withrightlowerquadrantpainforthelast24haccompaniedby a subfebrile state.The patient is in excellentgeneral condition withoutprevioussurgicalhistory.Hehasamildhypertensionand diabetesonoralmedication.Thepainisaccompaniedbynausea withoutvomiting.Hedeniesanyweightloss,changeinstoolhabits, hematocheziaormelena.Hedoesnotreportanyprevioushistory ofabdominalpain.

Physicalexaminationrevealsarightlowerquadranttenderness.

Thebowelsoundsarenormal.

ThelabtestsshowaCRPof22mg/l,andnoleucocytosis.Alow doseCTscanisperformedbecauseofahighsuspicionofanappen- dicitisand showsaninﬂammatorythickeningoftheceacalwall

http://dx.doi.org/10.1016/j.ijscr.2016.07.028

creativecommons.org/licenses/by-nc-nd/4.0/).

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52 P.Kuczmaetal./InternationalJournalofSurgeryCaseReports27(2016)51–54

Fig.1. Intraoperativestatuswithanabscessedileo-caecalmass.

withacollectionoftheileo-caecalzonewhichcouldcorrespondto anabscessedappendix.Theappendixitselfisnotvisualized.There areretroperitoneallymphadenopathiestobefound(Fig.2).

Theclinicalpresentationand theimage onthelow-dose CT scanare highlyevocative ofan acuteappendicitiswitha small abscess.Thepatientisplannedforalaparoscopicappendectomy.

Theabdominalexplorationrevealsathickenedceacumpatched togetherwiththeterminalileumontheposteriorabdominalwall.

Aconversionintoalaparotomyisdecidedduetoveryinﬂamma- torytissuesandinabilitytocarryonsafelywiththelaparoscopic approach. We discover a largemass involvingthe ceacum and the terminal ileum. Frozen section reveals the presence of an adenocarcinoma.Anoncologicrighthemicolectomyis therefore permormed, which turns out to beextremely difﬁcultbecause themassispatchedontheiliacvesselsandontherightureter.

Anileo-transverseanastomosisisperformedwithuncomplicated postoperativerecovery.TheﬁnalpathologicanalysisrevealsaSRC carcinomaof10,5cm,inﬁltratingtheserosa,withanextensivelym- phaticinvasionand15metastaticganglions(15/15).Theresection marginsarewithouttumorandtheK-RASistestedpositive.

Inordertoexcludeaprimarygastricpathology,weperforma gastroscopy,asthishistologyhasmostofthetimeaprimarygas- triclocalization.ThegastroscopyisnormalandathoracicCT-scan showstheabsenceofsecondarylesions.ButthePETCTshowsmul- tipleosteolyticbonemetastases:totheribs,tothevertebralbodies, therighthumerusandadestructionofthetwoomoplates,allof themassociatedwithahighfracturerisk.

His post-operative tumor marker CEA level is elevated at 251␮g/landPSAandCA19-9levelsarenegative.Thealkalinephos- phataseisatanormallevelpreoperatively(115U/l)andrisesupto 144U/l5weekslater.

Folfox regimen with Irinotecan and Bevacizumab has been startedandthepatientisstillundergoingtreatment(Fig.3).

3. Discussion

SRCcancerischaracterizedbytheabundantintracytoplasmatic mucinthatpushesthenucleustotheperiphery,creatingamicro- scopicsignet-ringappearance.Thetumormustconsistofatleast

Fig.2.A.Histology.Thetumorcellsaresignetringshaped,withprominent intracytoplasmicmucin,whichpushesthenucleustothecellperiphery.According totheWHOclassiﬁcation,thedesignationsignetringcellcarcinomaisused ifatleast50%ofthetumorcellsaresignetring-shaped.B.Massiveregionallymph nodemetastasisfromthesignetringcellcarcinoma.

50%ofthesesignetcells[8]:Morethan96%oftheSRCcarcinomas occurinthestomach[1].TheincidenceoftheprimarySRCcarci- nomaofthecolonandrectumisreported0.6per100000persons intheyear2000.Theincidencerateisincreasingwithanannual percentchangeof4.8%incontrasttotheordinaryadenocarcinoma theincidenceofwhichisslightlydecreasing[6].

ThemeanageofpatientsdiagnosedwithprimarySRCcarcinoma is39.6–60−whichissigniﬁcantlyyoungerthanforanordinary adenocarcinoma(70years).[1,5,9].

Metastasisfromgastriccarcinomaisprobablythemostcom- monsecondarylesioninthecolonandrectum[10].Theyappear mostlyinthetransversecolon,whiletheprimarySRCcancerofthe colonappearspredominantlyintherectum(32.9%),followedby thesigmoidcolon(20.4%),thecaecum(11.2%)andtheascending colon(10.5%).ThiscanbeafeaturedistinguishingaprimarySRC carcinomaofthecolonfromthegastricSRCmetastasis.Theupper endoscopyistheinvestigationofchoicetoexcludeaprimarygastric pathology.

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Fig.3. A.Imagingofthepatient.PETCT.Multipleosteolyticmetastasestothever- tebralbodiesandtotheleftomoplate,associatedwithafracture.B.AbdominalCT scan.Amasswithapossibleabscessisvisualizedintheileo-caecalzone.

Astothetumorgrade,theSRCcarcinomaofthecolonwasfound topresentwithhighgrade,poorlydifferentiatedtumors(73.5%of G3orG4)whichissigniﬁcantlydifferentfromtheordinaryadeno- carcinoma(only17.5%ofG3orG4)[6].

TheprimarySRCcarcinomaofthecolonismorelikelytopresent withlaterstagelesionsormetastases.Signiﬁcantlymorepatients sufferfromdistantmetastases.80.9%arediagnosedatstageIII/IV comparedwiththe49.5%oftheordinaryadenocarcinoma⁶.How- ever,thedisseminationpatternsarequitedifferent:SRCcancer disseminationischieﬂyperitonealwithlymphnodeandperitoneal involvement[1,3,5].Therate oflivermetastases hasbeenseen in2.9%–14.3%ofcasescomparedtothe32%–43%oftheordinary adenocarcinoma.Theincidenceofbonemetastasesfromcolorec- talcancerisbetween4.7%–10.9%[11–13].Thereareextremelyfew casereportsintheliteratureabouttheSRCcarcinomaofthecolon

withdiffusebonemetastases[14,15].Ourpatientdidn’tpresent anylivernorlungmetastasesbutmanylymphnodemetastasesand multipleosteolyticbonelesions.Becauseoftheextremelyrareevi- denceoftheosteolyticbonelesionssecondarytotheSRCcarcinoma ofthecolonintheliterature,weneedtoexcludeasynchronous prostatecarcinoma.ThePSAlevelisnormal.Theusualpathophys- iology ofthebonymetastases is thatthedetached cancercells reachtheliverthroughtheportalveinandthengotothelungs andtothearterialtreeorreachthelungsdirectlythroughtheinfe- riorvenacava.Inourpatient,theabsenceofsecondarylungand liverlesionssuggeststhatthetumorcells reachthevenoussys- temdirectlythroughlymphaticductsthatdrainsintothevenous system.

The radiographicfindings of theSRC carcinomainclude the thickening of the bowel wall with a “target appearance” and perirectal/pericolicinfiltration[16].Its radiographiccharacteris- ticscanmimicaninflammatoryprocess[7].Alsoinourpatient, theradiographicfeaturestogetherwiththeclinicalpresentation wereevocativeofanacuteappendicitis.

TheSRCcarcinomaofthecolonhasasigniﬁcantlypoorerprog- nosiswithﬁve-yearsurvivalof26.8%comparedtothe62.9%ofthe ordinaryadenocarcinoma[6].Themedianandmeansurvivaltime isrespectively20and45months[8,17,18].Inconsequence,theSRC carcinomacanberegardedasanindependentprognosticfactorfor pooreroutcomes[3,6,17,18].

Therearesomereportsthatsuggestanassociationbetweenthe SRCcarcinomaofthecolonwiththeinﬂammatoryboweldisease with12–14%ofpatientswithSRChavingahistoryofIBD[2,19].

4. Conclusion

In conclusion,theprimary colorectalSRCcarcinomaischar- acterizedbyyoungerage,moreadvancedstagesatpresentation, highertumorgrade,morefrequentdistantmetastasisthatinvolve mainlythelymphnodesandtheperitoneumandrarelytheliver, adismalprognosisandradiographicfeatures thatcanmimican inﬂammatoryprocess.Usuallyonlyapalliativesurgeryispossi- ble.Theimportanceofanearlydiagnosisofthistumorshouldbe stressed.TheroleofresectionforlatestageSRCshouldbecarefully evaluated.

Consent

Alltheauthorsconﬁrmthatthemanuscriptrepresentsourown work,isoriginal andhasnotbeencopyrighted,published, sub- mitted,oracceptedforpublicationelsewhere.Theauthorsfurther conﬁrmthattheyallhavefullyreadthemanuscriptandgivecon- senttobeco-authorsofthemanuscript.

Conﬂictofintereststatement Thereisnoconﬂictofinterests.

Sourcesoffunding

Thewasnoinvolvement ofanysort of fundingfor ourcase report.

Ethicalapproval

Our casereportis notaresearchstudyand thusit doesnot requireanethicalapproval.YoumaycontacttheEthicsCommittee of Geneva if you wish to obtain further information. Refer- enceNumber: Req-2016-00067. Contact:Prof. BernardHirschel

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54 P.Kuczmaetal./InternationalJournalofSurgeryCaseReports27(2016)51–54

Président,Commissioncantonaled’éthiquedelarecherche,1211 Genève14,Tél.+41795533491.

Authorcontribution

Allofthe authorscontributed equallytothepaper concept, design,datacollection,dataanalysisandinterpretationandwriting thepaper.

Guarantor

PaulinaKuczma,MD,GenevaUniversityHospital,Switzerland.

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