Primary extraperitoneal rectum lymphoma in AIDSpatient Coloproctology Journal of

jcoloproctol(rioj).2020;40(2):175–178

w w w . j c o l . o r g . b r

Journal of

Coloproctology

Case Report

Primary extraperitoneal rectum lymphoma in AIDS patient

Ana Maria Stapasolla Vargas Garcia

, Marlei Sangali

,

Antoninho Jose Tonatto Filho

, Caroline Lara

, Cibele Corbellini Rosa da Silva

, Marcos Paulo Barreto Saturnino

, Luciano Pinto de Carvalho

aHospitalNossaSenhoradaConceic¸ão,Servic¸odeCirurgiaGeral,PortoAlegre,RS,Brazil

bHospitalNossaSenhoradaConceic¸ão,Servic¸odeColoproctologia,PortoAlegre,RS,Brazil

a r t i c l e i n f o

Articlehistory:

Received20September2019 Accepted18November2019 Availableonline9December2019

Keywords:

Lymphoma Rectum

Gastrointestinallymphoma Coloniclymphoma

a bs t r a c t

Introduction:Thegastrointestinallymphomacanbeclassifiedinprimaryorsecondary,and thisisimportantregardingdiagnosisandsubsequenttreatment.Primarygastrointestinal lymphomaoftherectumisrareandthereforelacksdatainmedicalliterature.Itsincidence hasbeenincreasingandthatfactmayberelatedtoahigherincidenceinimmunosuppres- sivetherapyandimmunosuppressivediseases(suchasAIDS).

Metodology: 19 articleshave beenreviewed, searched onlineon theScielo and PubMed databases.Thegoalwastoincreasedataavailableregardingthispathologyandimprove itstherapy.

Discussion: Primary GIlymphomaofthe rectumpresentsas hematochezia,rectalpain, changeinbowelhabits.PET/CTisthefirstchoiceexamtopursueinvestigation;however abdominalCTandMRIrevealsufficientinformationandaremuchmoreavailableindaily practice.Plasmablastyclymphomaisanaggressivesubtypeandisusuallyassociatedwith AIDSpatients.Therearenoavailabletreatmentprotocolsforthisspecifictypeoflymphoma andcoloniclymphoma’stherapyisusuallyusedforthispatient(suchasECHOPandCHOP).

Conclusion: Asrareasthispathologyis,thisarticleaimstoimprovetheavailabledataand provideusefulinformationregardingdiagnosisandtherapy.

©2019SociedadeBrasileiradeColoproctologia.PublishedbyElsevierEditoraLtda.This isanopenaccessarticleundertheCCBY-NC-NDlicense(http://creativecommons.org/

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

∗ Correspondingauthor.

E-mail:[email protected](A.M.Garcia).

https://doi.org/10.1016/j.jcol.2019.11.488

2237-9363/©2019SociedadeBrasileiradeColoproctologia.PublishedbyElsevierEditoraLtda.ThisisanopenaccessarticleundertheCC BY-NC-NDlicense(http://creativecommons.org/licenses/by-nc-nd/4.0/).

176

LinfomaprimárioderetoextraperitonealempacienteSIDA

Palavras-chave:

Linfoma Reto

Linfomagastrointestinal Linfomacolón

r e s u m o

Introduc¸ão:OlinfomadoTGIpodeserdivididoentreprimárioesecundário,comimportância diagnósticaeterapêutica.Olinfomaprimárioderetoépatologiarara,poucorelatadaem literaturamédica.Suaincidênciatemaumentadoepossivelmenteessefenômenoesteja associadoaoaumentononumerodepacientescomimunossupressão(sejaporSIDAou drogasimunossupressoras).

Metodologia: Foramrevisados19artigosnasbasesdedadosScieloePubMed,comoobjetivo deaumentaronúmeroderelatosdessapatologiaeconsequentementeexpandiroconhec- imentodisponível,visandomelhoraraterapêuticae,principalmente,odiagnósticodesse tipodelinfoma.

Discussão: Quandoolinfomatemseusítioprimárionoreto,asprincipaismanifestac¸ões são sangramento, dor retal, tenesmo e mudanc¸a nos hábitos intestinais (diarreia ou constipac¸ão).Oexamedeinvestigac¸ãodeescolhaéoPET/CT,porémaTCeRNMfornecem asinformac¸õesnecessáriasesãomaisdisponíveisnapráticaclínica.Olinfomaplasmablás- ticoéumsubtipobastanteagressivoeassociadoaospacientescomSIDA.Nãoexistemainda protocolosdefinidosparaotratamentodolinfomaprimáriodereto,sendooptadoporseguir amesmaterapêuticadoslinfomasdecóloncomesquemasEPOCHeCHOP.

Conclusão: Porsetratardepatologiararaepoucodescritanaliteratura,espera-sequeeste relatocontribuanaformac¸ãodeprotocolosdetratamentoespecíficos.

©2019SociedadeBrasileiradeColoproctologia.PublicadoporElsevierEditoraLtda.Este

´eumartigoOpenAccesssobumalicenc¸aCCBY-NC-ND(http://creativecommons.org/

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

Introduction

Primary gastrointestinal lymphoma is a rare condition, defined aslymphomas involving the Gastrointestinal Tract (GIT)orpresentingwithgastrointestinalsymptoms.Thelym- phomawithsecondaryinvolvementofTGIismorecommonly found.^1,2Thisdifferentiationguidesthetreatment.

Primarycoloniclymphomaisrareandaccountsforonly 0.2 %–0.4% ofall colon cancers, 10 %–5 % of all primary gastrointestinallymphomas and about30 %ofextra-nodal lymphomas.^3,4 The most commonly affected sites are the stomach, followed by the small intestine and ileocecal transition.⁵

Themostcommoncoloniclocationisthecaecum(70%), followedbytherectumandascendingcolon.⁶

Intestinal lymphomas can be classified into B-cell lym- phomas(85 %)andT-celllymphomas(15 %).AmongB-cell lymphomas, mantle cell lymphoma has a worse progno- sis, whereas Mucosa-Associated Lymphoid Tissue (MALT) lymphomas have a better prognosis than other B-cell tumors.⁶

Studies suggest that the incidenceof primary gastroin- testinallymphomahasincreased,⁷whichmaybeexplained bythepresenceofimmunosuppression,eitherduetomedi- ated Acquired Immunodeficiency Syndrome (AIDS), by an increase in the prescription of immunosuppressive drugs usedaftertransplantationorassociatedwithtreatmentsfor autoimmune diseases.^7,8 Drugs related to increased inci- denceoflymphomasincludethiopurines(azathioprineand 6-mercaptopurine),^9,10and,toalesserextent,theanti-Tumor NecrosisFactor(anti-TNF)therapy.¹¹

Methodology and objectives

Totally, 19 articles retrieved from the Scielo and PubMed databaseswerereviewedinordertoincreasethenumberof reportsregardingthispathologyandconsequentlyexpandthe availableknowledge,aiming atimprovingthe therapyand, particularly,thediagnosisofthistypeoflymphoma.

Case report

Male patient, 19-year-old, treated at the HNSC emergency department,withahistoryofanalbleeding(brightredblood) forayear,associatedwithpainandanaltumorforamonth, whohadundergoneatleasttwoattemptstodrainthelesion.

Prior to hospitalization, he had hypothyroidism, clinically treatedwithhormonereplacement,inadditiontodepression andirregularpsychiatrictreatment.Analinspectionrevealed rightposterolateral perianallesion,about6cm indiameter, hardened, withoutdrainageofsecretion,but withincisions inthe centralportion.Theoverlyingskinhad hemorrhagic suffusion(Figs.1and2).Rectalexaminationrevealedahypo- tonicsphincterina3cmanalcanalwithapparenthardened lesion in the right posterior and lateral anal canal, adja- centtothedescribedperianaltumorlesion.Nobloodorpus.

Mild anemiawasevidencedduringthe initialexamination.

ApelvicMRIscan(Fig.3)demonstrateda11.2×7.6×7.4cm tumor extensively invading the right levator ani muscles (mainlythepuborectalis).Intherightinterglutealsulcusthere was a small area withfluid inside (1.2×1.0), with aprob- able fistulous path to the skin. The prostate and seminal

jcoloproctol(rioj).2020;40(2):175–178

177

Fig.1–Perineallesion.

Fig.2–Perineallesion.

vesiclesweredisplacedanteriorly,withoutinvasionandpre- servingthecapsulecontour.Definingcleavageplanwiththe sacrum;prominentbilateralinguinallymphnodes,especially totheright(largest=1.1×0.9);absenceoflymphnodeenlarge- mentorfreefluidinthepelvis.Subsequently,anincisional biopsy of the lesion was performed under general anes- thesia, with anatomopathological results compatible with largecellnon-Hodgkin’slymphomaandimmunohistochemi- calprofilecompatiblewithimmunophenotypeBlymphoma, plasmablasticlymphoma,positiveforCD10,CD20,CD138,ki- 67,Bcl-2andnegativeforEBV.Alsoduringtheinvestigation,he

Fig.3–PelvicNMRshowingextraperitonealrectallesionof 11.2×7.6×7.4cm.

wasdiagnosedwithHIVinfection,withsignificantimmuno- suppression(CD437andCV541,213copies).

Afterwards, the patient was transferred to the hema- tology department for therapeutic planning and, dur- ing this period, he presented with compressive urinary tract symptoms (with doxazosin response) and psychi- atric decompensation, requiring antipsychotic drugs due to the high risk of suicide. After a bone marrow biopsy negative for lymphoma infiltration, chemotherapy with Etoposide+Doxorubicin+Vincristine (EPOCH) was started.

Antiretroviral Therapy (ART) was started with an alterna- tiveregimen(abacavir+lamivudina+dolutegravir),associated withprophylactictrimethoprim-sulfamethoxazole.

Afterthesecondcycleofchemotherapy,acontrolCTscan was performed, which showed complete regression of the rectal lesion. The chemotherapy regimen was maintained untilthefourthcycleandthenPET-CTwasperformed,which demonstratedabsenceofareasofabnormalmetabolicactiv- ity characteristic ofactive lymphoproliferativedisease and absenceofparietalthickeningorabnormalmetabolicincrease intherectum(Luganoscore1).

Discussion

The clinical, radiological, and endoscopic features of the primary gastrointestinal lymphoma are nonspecific, which mayhinderthediagnosis,makingtheclinicalpictureoften indistinguishablefromothercolondiseases,whetherneoplas- tic or inflammatory.When lymphomahas its primary site in the rectum, the mainmanifestations are bleeding, rec- talpain, tenesmusandchangeinbowelhabits(diarrheaor constipation).¹²

PET/CTistheimageofchoiceforanalysisofmass,area ofstenosis,andlymphnodeinvolvement,¹³butimagingtests suchaspelvicCTandMRIareoftensufficienttoidentifythe lesionandaremoreavailableinclinicalpractice.Colonoscopy canshowvariablemucosalinvolvement,whetheritisamass, ulcerationorinfiltration,¹⁴andallowsbiopsiesforhistopatho- logicaldiagnosis.

PlasmablasticLymphoma(PBL)isclassifiedbytheWorld Health Organization as a typeof matureB-cell lymphoma that expresses plasmaantigens (CD38, CD138,MUM1) and common B-cell antigens (CD20,CD19, PAX5) withnegative CD45.^15,16Whileitspathogenesisisnotyetfullyunderstood, it hasbeen shown thatEpstein BarrVirus(EBV)is present

178

in mostcases. In addition, an association with MYCgene rearrangement has been found in a small percentage of cases.⁴PBLlymphomawasinitiallyidentifiedintheoralcav- ityofpatientswithHumanImmunodeficiencyVirus(HIV)and approximately80%ofPBLcasesareassociatedwiththisHIV- positivepopulation.¹⁷PBLhasalsobeenfoundinareasoutside theoralcavity,favoringsitessuchastheGastrointestinal(GI) tract,lymphnodes,andskin.¹⁸TheGItractisoneofthemost common extranodal sites. PBL is considered an aggressive lymphomawithamedianoverallsurvivalof14months.^17,18 Regardingtreatment,therearenoexclusiveprotocolstoguide theextraperitonealrectallymphomamanagement,andcases aretreatedfollowingtheguidelinesofintraperitonealrectal lymphoma.AlthoughbothCHOPandEPOCHareconsidered commontherapeuticchoices,standardtherapyortreatment guidelineshavenotyetbeenestablished.Autologoustrans- plantationisconsideredoptionalandtendstohaveagood outcome,butthereislittleexperiencewiththistreatment.¹⁹ Surgicaltreatmentsarerarelyneededand areusuallyindi- catedincaseofcomplications.

Conclusion

Primary gastrointestinal lymphomas are themselves rare pathologies,withextraperitonealrectallymphomabeingan evenrarerform.Primarygastrointestinallymphomasarerare pathologiesinthemselves, withextraperitonealrectallym- phomabeinganevenrarerform.Itrepresentsadiagnostic challengeandlacksspecificprotocolstoguidetherapy.With thisreport,itisexpectedtoadddatatothecurrentlyavailable literature.

Conflicts of interest

Theauthorsdeclarenoconflictsofinterest.

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