Primary lymphoma of appendix presenting as acute appendicitis: A case report

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InternationalJournalofSurgeryCaseReports48(2018)30–33

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International

Journal

of

Surgery

Case

Reports

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

Primary

lymphoma

of

appendix

presenting

as

acute

appendicitis:

A

case

report

Giuseppe

Caristo

_,

_Guido

_Griseri

_,

_Rosario

_Fornaro

_,

_Antonio

_Langone

_,

Angelo

Franceschi

_,

_Veronica

_Errigo

_,

_Cecilia

_Ferrari

_,

_Marco

_Casaccia

_,

_Marco

_Frascio

_,

Angelo

Schirru

a_{SanPaoloHospital,ViaGenova30,Savona,17100,Italy}

b_{UniversityofstudyofGenoa(Italy),PoliclinicoSanMartinoLargoBenzi10,16132Genova,Italy}

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c

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f

o

Articlehistory:

Received18February2018

Receivedinrevisedform17April2018 Accepted29April2018

Availableonline7May2018 Keywords: Lymphoma Appendix Acuteappendicitis Casereport

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INTRODUCTION:Primarylymphomasofappendixareextremelyraretumors.Theincidenceis0.015%of allgastrointestinallymphomas.

PRESENTATIONOFCASE:Wepresentacaseofa75year-oldmalepatientwhopresentedwithacute abdominalpaininthelowerrightquadrantandfever.

DISCUSSION:Thepatientreceivedlaparotomicappendectomy.Thedefinitivehistopathological examina-tionrevealedthepresenceofdiffuselargecellB-lymphomaoftheappendix.Theneoplasmsofappendix usuallymanifestclinicallywithsignandsymptomsofacuteappendicitisfromluminalobstruction (30–50%).Preoperativediagnosisisdifficultandoftenoccursthroughhistopathologicalexamination. CONCLUSION: Primary appendiceal lymphoma is rare and there are no clear guidelines for ther-apy.Primarysurgicalresectionfollowedbypost-operativechemotherapyshowedhighefficacy.The histopathologicalexaminationofallappendectomyisessential.

©2018TheAuthors.PublishedbyElsevierLtdonbehalfofIJSPublishingGroupLtd.Thisisanopen accessarticleundertheCCBY-NC-NDlicense(http://creativecommons.org/licenses/by-nc-nd/4.0/).

1. Introduction

Primitivegastrointestinallymphomasare rareand represent only 1–4% of all tumors of the gastrointestinal tract [1]. Gas-trointestinaltract is themost common location for extranodal lymphomas[2]. The mostaffected organsare thestomach and thesmallintestine[3].Theprimarylymphomaoftheappendixis extremelyrare.WepresentacaseofprimarydiffuselargeB-cell lymphomaoftheappendixmanifestclinicallywithsignand symp-tomsofacuteappendicitis.Thiscaseisreportedinlinewiththe SCAREcriteria[4].

2. Casereport

A75-year-oldman,withoutmajormedicalhistory,wenttoour emergencyroomforabdominalpaininthelowerrightquadrant andfever (axillarytemperature38◦C)for about1day.Physical

∗ Correspondingauthor.

E-mailaddresses:caristogiuseppe@tiscali.it(G.Caristo),g.griseri@asl2.liguria.it

(G.Griseri),rfornaro@unige.it(R.Fornaro),a.langone@asl2.liguria.it(A.Langone),

a.franceschi@asl2.liguria.it(A.Franceschi),v.errigo@asl2.liguria.it(V.Errigo),

ce.ferrari@asl2.liguria.it(C.Ferrari),Marco.Casaccia@unige.it(M.Casaccia),

mfrascio@unige.it(M.Frascio),a.schirru@asl2.liguria.it(A.Schirru).

examinationwassignificantonlyforlowerabdominaltenderness withmore onthe rightiliac fossa withoutclear signs of peri-tonealirritation.Theperistalsiswaspoor.Laboratoryexamination revealed white cell count of 15610/mm3 with 12,720 neu-trophils/mm3andelevatedinflammationindex(C-reactiveProtein 12.65mg/dl).Theultrasoundrevealedthepresenceofasmallbowel loopoftheiliacorcecalregionwiththickenedwalls,notperistaltic, withsurroundingfreeliquidandanimportanthyperemiaofthe adjacentmesentericadiposetissue.NofreeliquidintheDouglas (Figs.1and2).Theradiographoftheabdomenrevealedisolated air-fluidlevelswithoutpathologicalsignificance(Fig.3).Painwas resistanttoantalgicdrugs.Thepatientthenreceivedlaparotomic appendectomy under clinicaldiagnosis ofacute appendicitis. A laparoscopicapproachwasinterruptedduetoexcessiveintestinal distension.Theappendixappearedenlarged,folded,phlogosated with gangrene at the tip. The postoperative period was regu-lar.Antibiotictherapy(piperacillin-tazobactam),intravenousfluid, analgesics(paracetamol),anti-emeticsandantithromboticswere administered.The discharge wasonthe5th postoperative day. Thedefinitivehistopathologicalexaminationrevealed,inthe con-textofperforatedappendicitiswithacuteinflammation,presence in the wall of habitus blastic lymphoid elements (Fig. 4), also in small aggregates in vascular-like spaces (Fig. 5), with an unusualimmunophenotypeduetoincompleteexpressionofCD20.

https://doi.org/10.1016/j.ijscr.2018.04.031

2210-2612/©2018TheAuthors.PublishedbyElsevierLtdonbehalfofIJSPublishingGroupLtd.ThisisanopenaccessarticleundertheCCBY-NC-NDlicense(http://

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G.Caristoetal./InternationalJournalofSurgeryCaseReports48(2018)30–33 31

Figs.1and2. Ultrasound:smallbowelloopoftheiliacorcecalregionwiththickenedwalls,notperistaltic,withsurroundingfreeliquidandanimportanthyperemiaofthe

adjacentmesentericadiposetissue.

Fig.3.Radiographoftheabdomenrevealedisolatedair-fluidlevelswithout

patho-logicalsignificance.

Fig.4. EELargelymphoidelementswithablastichabitus.

Fig.5.CD20+inLargelymphoidelements.

Immunophenotipicfindings:CD45+,CD20+(insomeelements), Pax5+(insomeelements),MUM1+,OCT2-,BOB1+(insome ele-ments),CD3+(inrareelements),CD5+(inrareelements),CD10-, Bcl2-,Bcl6-, CD138-,CD68-,CD30+,(insomeelements),S100-, MPO-,ALK1-,lightchainsKandlambdanotstrains,CKAE1AE3-, EMA-,Chromogranin-,Ki67(Mib1)+inmostoftheelements(80%). Monoclonalrearrangementwasnotfound(Studymethodis per-formedonthe DNAextracted fromthe sample):lymphocyte B polyclonalpatternandlymphocyteToligoclonalpattern.PET-CT didnotdemonstratemetabolicallyactivesitesintheinvestigated bodysegments.Thepatientwasassignedtohematologyforthe continuationofthediagnosticandtherapeuticprocess.

3. Discussion

Malignantneoplasmofthegastrointestinaltractarecommon and the most diffuse type is adenocarcinoma. Malignant lym-phomaisrareandcomprises1–4%ofthemalignantgastrointestinal neoplasms[1].ThelymphomascanbedividedinHodgkinand non-Hodgkin,thelasttypebeingclassifiedinBcellorTcelllymphomas [2].Thelymphomasofthegastrointestinaltractmainlyaffectthe stomachfollowedbythesmallintestine,pharynx,colonand esoph-agus.Menaremoreaffectedandthemedianageatdiagnosisfor

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32 G.Caristoetal./InternationalJournalofSurgeryCaseReports48(2018)30–33

lymphomasnon-Hodgkinofgastrointestinaltractwas55years[3]. Primarylymphomasofappendixareextremelyraretumors.The incidenceofprimarylymphomaofappendixis0.015%ofall gas-trointestinallymphomas[5].

Theclinicalonsetofthediseaseisoftenconstitutedbyasurgical complicationthatrequiresurgentintervention[6].Theneoplasms of appendix usually manifest clinically with sign and symp-tomsof acute appendicitis from luminal obstruction (30–50%). Otherimportantclinicalmanifestationsmaybeanasymptomatic palpablemass,incidentalimagingfindings,intussusception, gas-trointestinalbleedingandureteralobstructionorhematuriaand increasingabdominalgirthfromruptureofamalignantmucocele, resultinginpseudomyxomaperitonei[7].

The surgical approach and obviate additional surgery may changewithdetectionoftheseneoplasmsatpreoperativeimaging [7].Inthepast,preoperativediagnosisofappendicealneoplasms wasrarebutisbecomingmorecommonwiththeincreaseduse ofcomputedtomography(CT).Apeculiardiagnosticelementfor lymphomaisdiffuseenlargementoftheappendixfrom lymphoma-tousinfiltrationonboth ultrasoundandCT.Diffuse appendiceal enlargement(diameterof6–7mm)isalsowellestablishedasthe diagnosticthresholdforacuteappendicitis,especiallyifitis asso-ciated withstranding of theperiappendiceal fat. The inflamed appendix withoutneoplasm willusually not exceed 15 mm in diameteronCT,enlargementbeyondthissizeshouldbeviewed withsuspicion [3]. Although the CT finding of enlargement of theappendixseemstobefairlycharacteristicof non-Hodgkin’s lymphoma,suchafindingisnotpathognomonic.Neuroendocrine tumorscanshowaninfiltrativepatternofgrowth.Specificityfor lymphoma will be increased in thesetting of abdominal lym-phadenopathyoraneurysmaldilatationoftheappendiceallumen [3].Oneapparenterrorintheultrasoundortomographicdiagnosis ofappendiceallymphomais theerroneousinterpretationofthe grosslyabnormal appendixas athickened small-bowelloop. In ourcase,theultrasoundinterpretedtheenlargedappendix asa smallbowelloopthickenedwithfreefluidaroundandhyperemia ofperiappendicealfat.PET/CTisimportantforstaginglymphoma. PET/CThasgreatersensitivity,comparedtoCTalone,in identify-inglymphnode,extranodalsitesandbonemarrowinvolvementby lymphoma.PET/CTcanindicatethemetabolicresponsetotherapy, earlierdetectionofdiseaserecurrenceandalsotheoveralllevel ofmetabolicactivityoflymphoma,whichcorrelateswithlevelof aggressivenessandrepresentaprognosticpredictor[8].Inourcase, PET/CT,afterappendectomy, didnotidentifyareasofincreased metabolicactivitywhichdidnotrequirefurthersurgicalmeasures. Primaryappendiceallymphomaisrareandtherearenoclear guidelinesfortherapy.Intheliteratureprimarysurgicalresection followed by post-operative CHOP (cyclophosphamide, hydrox-ydoxorubicin, vincristine, prednisolone) chemotherapy showed highefficacyinpatientswithlocalizedintestinaldiffuselargeB celllymphoma[9].Therewasnoevidenceofrecurrence6months afteroperation[10].However,closefollow-upiscertainly recom-mended.

4. Summary

Lymphomaoftheappendixisrare.Usuallymanifestclinically withsignandsymptomsofacuteappendicitis.Thedetectionofthis neoplasmatpreoperativeimagingisverydifficultalthougha char-acteristicCTscanappearancemayleadtopreoperativediagnosis. PET/CTis important for staging lymphoma. The histopatholog-ical examination of all appendectomy is essential and should bemandatory.Intheliterature,thecombinationofsurgery and chemotherapyisthebesttreatmentforappendiceallymphomas.

Conflictsofinterest

Thereisnoconflictofinteresttodeclare.

Fundingsource

None.

Ethicalapproval

Thepaperisnotaresearchstudy.ForcasereportourInstitute exemptedtotakeethicalapproval.

Consent

Writteninformedconsentwasobtainedfromthepatientfor publicationofthiscasereport.

Authorcontribution

GiuseppeCaristoidealizesstudy,design,dataanalysis,critical reviewandwritesthearticle.

GuidoGriseriidealizesstudy,dataanalysisandcriticalreview ofimportantintellectualcontent.

RosarioFornaroreviewsitcriticallyforimportantintellectual contents.

AntonioLangonereviewsitcriticallyforimportantintellectual contentsandparticipatesindataacquisition.

AngeloFranceschireviewsitcriticallyforimportantintellectual contents.

VeronicaErrigoreviewscriticallytheanatomicalpathological evaluation.

CeciliaFerrariparticipatesindataacquisition.

MarcoCasacciareviewsitcriticallyforimportantintellectual contents.

MarcoFrascioreviewsitcriticallyforimportantintellectual con-tents.

Angelo Schirru idealizes study and reviews it critically for importantintellectualcontents.

Registrationofresearchstudies

researchregistry3715.

Guarantor

DrGiuseppeCaristo. DrGuidoGriseri.

References

[1]M.H.Shiwani,Primarymalignantlymphomaoftheappendixassociatedwith

acuteappendicitis,J.Coll.Phys.Surg.Pak.16(1)(2006)79–80.

[2]B.Mastalier,V.Deaconescu,W.Elaiah,Multipleintestinallymphoma,Rom.J.

Intern.Med.53(1)(2015)73–78.

[3]P.J.Pickhardt,A.D.Levy,C.A.RohrmannJr.,Non-Hodgkin’slymphomaofthe

appendix:clinicalandCTfindingswithpathologiccorrelation,AJRAm.J.

Roentgenol.178(5)(2002)1123–1127.

[4]R.A.Agha,A.J.Fowler,A.Saetta,I.Barai,S.Rajmohan,D.P.Orgill,fortheSCARE

Group,TheSCAREstatement:consensus-basedsurgicalcasereport

guidelines,Int.J.Surg.34(2016)180–186.

[5]S.Radha,T.Afroz,G.Satyanarayana,PrimarymarginalzoneB-celllymphoma

ofappendix,IndianJ.Pathol.Microbiol.51(3)(2008)392–394.

[6]R.Ferraris,M.Nahum,R.Fornaro,Intestinalinvaginationcausedbyprimary

lymphomaoftheileum,MinervaChir.43(7)(1988)619–623.

[7]P.J.Pickhardt,A.D.Levy,C.A.RohrmannJr.,Primaryneoplasmsofthe

appendix:radiologicspectrumofdiseasewithpathologiccorrelation,

Radiographics23(3)(2003)645–662.

[8]C.G.Cronin,R.Swords,M.T.Truong,ClinicalutilityofPET/CTinlymphoma,

CASE

REPORT

–

OPEN

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G.Caristoetal./InternationalJournalofSurgeryCaseReports48(2018)30–33 33

[9]J.Lee,W.S.Kim,K.Kim,Prospectiveclinicalstudyofsurgicalresection

followedbyCHOPinlocalizedintestinaldiffuselargeBcelllymphoma,Leuk.

Res.31(3)(2007)359–364.

[10]T.Y.Fu,J.S.Wang,H.H.Tseng,Primaryappendiceallymphomapresentingas

perforatedacuteappendicitis,J.Chin.Med.Assoc.67(12)(2004)629–632.

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