Clinical Medical & Case Reports

Open Journal of

ISSN2379-1039

Volume2(2016)Issue24

HochwaldS

OpenJClinMedCaseRep:Volume2(2016)

Gastritiscysticaprofunda:AchallengingdiseasediagnosedusinganovelapproachDanishShahab,MD;EmmanuelGabriel,MD;MoshimKukar,MD;AndrewBain,MD;StevenHochwald,MD*

*StevenHochwald,MD

DepartmentofSurgicalOncology,RoswellParkCancerInstitute,Buffalo,NY14263USA

Tel:(716)845-8244.

Abstract

Gastritiscysticaprofunda(GCP)isararehyperplasticlesionofunclearetiology.Clinicalsymptomsof

GCParevariableandrangefromnonspeci�icabdominalpaintogastricoutletobstruction.Oftenthe

diagnosisofGCPhasbeendif�iculttomakeassimpleesophagogastroduodenoscopy(EGD)guidedbiopsy

canfailtoyieldthediagnosis.Here,wereportacaseofGCPthatpresentedinachallenginganatomic

location,whichrequiredalaparoscopicintragastricsurgicaltechniquetomakethediagnosis.

Introduction

Gastritiscysticaprofunda(GCP)isarare,benigndiseasecharacterizedbypolypoidhyperplasia

andcysticdilatationofthegastricglandsthatextendintothesubmucosaofthestomach.Firstdescribed

in1947byScottandPayne,itwasn'tuntil1972thatLittlerandGilbermannsuggestedthatthepresence

ofcysticallydilatedgastricglandsinthesubmucosawasareactive,postsurgicalconditionforwhichthey

coinedtheterm“gastritiscysticapolyposa”[1].Thiswouldeventuallychangetothenowpreferredterm

“gastritiscysticaprofunda”becauseitresembledthesimilarlynamedconditioninthecolon[1].Clinically,

patientscanpresentwithupperabdominalpain,acidre�lux,nausea,anorexia,orbleeding;although

somepatientsmayexperiencenosymptoms[2].Inseverecases,patientscanexperiencemassiveupper

gastrointestinalhemorrhageandgastricoutletobstruction[2].GCPisoftenseenasgiantgastricfolds,

submucosal tumors,or isolatedpolyps [2].The lesionhasbeendescribedprimarily in theoperated

stomach, leading to thehypothesisofpriorgastricwall injuryas thepredominantcauseofGCP, [2]

although ithasbeendescribed in theunoperatedstomachaswell.Although theexactpathogenesis

mechanismremainslargelyunknown,GCPhasbeenfoundtobeassociatedwithischemiaandchronic

in�lammation[3].Itisalsothoughttobeapossibleprecancerouslesionsinceafewearlygastriccancers

havebeenassociatedwithit[4].Interestingly,thereisalsoanincreasedassociationincancerswithGCP

toEpstein-Barrvirus(EBV)positivepatients[5]andMenetrierdisease(MD)[6].Here,wedescribeacase

ofadif�iculttodiagnosegastricmassthatwasfoundtobeGCP.

CasePresentation

Ourpatientwasa72yearoldmalewithapastmedicalhistoryofanon-smallcelllungcancer

Keywordsgastritiscysticaprofunda;gastricglands;submucosaltumors

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treated with chemotherapy and radiation, coronary artery disease, and cardiomyopathy. Previous

esophagogastroduodenoscopy(EGD)performedatanoutsidefacilityinFebruaryof2014forsymptoms

ofre�luxshowedcircumferentialBarrett'sesophagusandasmalltomoderatehiatalhernia.Atthistime,

hewasreferredtoourinstitutionforcontinuedsurveillanceoftheBarrett'sesophagus.

InOctoberof2015,asurveillanceEGDshowedanewmassinthegastriccardiameasuring2.6x1.4

cm.Fineneedleaspiration(FNA)performedatthistimewasnondiagnostic.Themasswaslocatednear

the hiatal hernia pinch and was thought to represent a gastrointestinal stromal tumor (GIST) or

leiomyoma.Approximately6monthslater,anintervalEGDalsoanincreaseinthesizeofthegastriccardia

mass to approximately 4 cm (Figure 1). On endoscopic ultrasound (EUS), a heterogeneous mass

measuring 3.9 by 3.1 cm in the gastric cardia with small internal cystic spaces was described. He

underwent a second FNA of themass,which showed only in�lammation and again failed to yield a

diagnosis.ACTscanshowedasmoothwellmarginatedmassintheregionofthegastriccardiaandhiatal

hernia,suggestiveofasubmucosallocation(Figure2).

Giventheconcernforneoplasmbasedonitsincreaseinsize,surgicalresectionwasrecommended.

GiventhechallenginglocationofthelesionclosetotheGEjunction,alaparoscopicintragastricapproach

withendoscopicassistancewasrecommended.Thisisanovelapproachwherebythelaparoscopicports

areinsertedthroughtheabdominalwallandthenintothestomachunderdirectendoscopicvisualization.

Duringtheprocedureuponmanipulationofthemass,purulentdrainagewasnotedfromtheareaofthe

mass,whichresultedincollapseofthemass.Sincethemassdecompressed,wedecidednottoresectit.

Therewasnoevidenceofmalignancyonpreviousbiopsies.Thus, the justi�icationforamoreradical

operationwasnotpresent.However,priortocompletionofsurgerymultiplecoreneedlebiopsiesofthe

masswereobtainedusingthelaparoscopicintragastricapproach.Thepatienttoleratedtheprocedure

well.Hewasdischargedonpostoperativeday3withoutcomplications.Surgicalpathologyshowedthat

themasswasconsistentwithgastritiscysticaprofunda(Figure3).

Discussion

Gastritiscysticaprofundaisaconditioncharacterizedbybenign,cysticgrowthofgastricglands

into the submucosa of the stomach [7]. Although GCPs are thought to be associatedwith previous

gastrectomy,thislesioncanalsooccurwithoutpreviousgastricoperations,[8]asobservedinourcase.

GCPisoftenlocatedintheposteriororanteriorwallofthegastricbodyandintheintermediatezone

betweenthefundicandpyloricglands[9].Ingeneral,laboratorytestsarenotusefulinmakingadiagnosis

ofGCP[3].ThepathogenesisofGCPislikelyduetochronicischemiaandin�lammation.Thedisruptionof

integrity ofmuscularismucosa causes themigration of epithelial content into the submucosawith

subsequentatrophicgastritis,intestinalmetaplasiaandcysticdilatationofgastricglands[7].Gastritis

cysticaprofunda isabenign lesion, althoughapossibleprecancerousnaturehasbeenhypothesized

[10].GCP has been shown to occur more frequently in the presence of gastric cancers [11]. In a

pathologicalstudyof10,728patientswithgastriccancer,GCPwasfoundin161patients[11].

ThereappearstobeapathogeneticroleofEpstein-Barrvirus(EBV)inthedevelopmentofGCPand

cancer[8].IthasbeendemonstratedthatthereisadelayofapoptosisinEBV-positivegastriccarcinomas

associatedwithupregulationofBCL-2andp53,andadecreaseincellulardifferentiationassociatedwith

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a decrease in E-cadherin expression [8]. EBV infection can alter the normal cell cycle, resulting in

disruptioninthecellularprocessesandcheckpointsresponsibleforregulatingcelldivision,whichthen

resultsinthecarcinogeneticeffectsofEBV[8].EBVtitershavebeenshowntobesigni�icantlyhigherin

dysplasticgastricmucosaversusnon-neoplasticgastricmucosa.InastudydonebyKimetal,theauthors

showed thatwithin the transitional areabetweenGCP and gastric carcinoma, thereweredysplastic

changespositiveforEBV[8].AnothergroupfoundthattheEBVpositiveratewassigni�icantlyhigherina

GCPgastriccancergroup(31.1%)thaninanon-GCPgastriccancergroup(5.8%),whichsuggeststhatGCP

wassigni�icantlyassociatedwithEBV-positivegastriccancersandthatEBVinfectionsmayplayarolein

thedysplasticchangesassociatedwithGCP[11].

Menetrierdisease(MD)isanotherpremalignantconditionwhichhasshownsomeassociation

withGCP[12].MDisanuncommon,idiopathic,hyperplasticgastropathycharacterizedbyhyperplasiaof

foveolarmucouscells,whichresultsinthickeningofgastricfoldsandhypoalbuminemia[6].Itmostly

involvesthegastricfundusandbody[6].Therehavebeenmanyreportsshowingcloseassociationwith

MDandGCPaspotentialprecancerouslesions[6].Furtherwork-upincludestestingforcytomegalovirus

and H. pylori. Treatment options include proton pump inhibitors (PPI) or H2 blockers, octreotide,

monoclonalantibodiestoepidermalgrowthfactorreceptor(EGFR),andgastrectomy[6].

RegardingthediagnosisofGCP,standarddiagnosisbyEGDisoftendif�icultbecausethestandard

FNAbiopsyspecimenisusuallylimitedtothemucosa,whichcannotprovidesuf�icientinformationabout

thedeepersubmucosa[13].Infact,inmanycasesthepreoperativediagnosisofGCPremainschallenging

despitethecurrentadvancesinendoscopictechniquesandthuspatientsmayhavetoundergosurgical

resectionfor�inalpathologicdiagnosistoguidetreatment[13].Oftentimesandinourcase,CTscanand

EUShavebeenusedasacomplementarytooltodelineateadditionalcharacteristicsoftheselesions[13].

ThemostcommonendoscopicfeaturesofGCPbyconventionalwhite-lightendoscopyarenonspeci�ic[3].

Infact,manygastroenterologistsnowsuggestthatthediagnosticmodalityofchoiceisEUS[3].GCPon

EUSshowsprimarily3majorechoicpatterns:anechoic(35.3%),mixedheterogeneouswiththickened

overlyingmucosa(50%),andhypoechoicwithmicrocysts(14.7%)[3].The�inaldiagnosishoweverstill

hastobedeterminedbyhistologicalexam[3].

ThereiscurrentlynoconsensusontheoptimalGCPtreatment.Duetoaninsuf�icientamountof

informationonGCP,therehavebeenavarietyoftreatmentrecommendations,rangingfromobservation

toradicalresection[3].XuGetaldevelopedastandardprotocoltosystemicallyinvestigatetheselesions

withEUSbeforeendoscopicsubmucosalresection/endoscopicsubmucosaldissection(EMR/ESD)[3].

GiventhefactthatthediagnosisofGCPmainlyreliesonhistopathology,endoscopicresectionservesboth

adiagnosticandtherapeuticprocedure.Ifperformedsuccessfully,endoscopicresectionwithEMRorESD

islessinvasive,safer,andmoreeconomicalthanopensurgicalmethods[3].Moreimportantly,endoscopic

resectionofGCPbetterpreservesgastricfunctionwithminimalinjury[3].Itshouldbenotedtherewere

limitations to theirproposedprotocol includingsmall samplesize, single institutionexperience,and

retrospectivenatureofthestudy.

Inourcasereport,wepresentedapatientwhoselesionwasfoundtobeinachallengingproximal

anatomiclocationassociatedwithahiatalhernia.ItwasincloseproximitytotheGEJ,thusmakingit

unresectablethroughastandardlaparoscopicapproach.Thus,alaparoscopicintragastricapproachwith

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endoscopicassistancewasattempted.However,themassdecompressedandwasnolongeramenableto

resection. Two previous endoscopic-guided FNAs failed to establish the diagnosis. Therefore, we

performed core biopsies through the intragastric port, which successfullymade the diagnosis. This

approachwasfoundtobediagnosticallyeffectiveandhadtheadvantageofstayingminimallyinvasive.In

conclusion,GCP isa rareandoftendif�icultdiagnosis tomake,but incertaincasessuchasours the

diagnosis can be facilitated using a novel surgical method consisting of a laparoscopic intragastric

approachwithendoscopicassistance.

Figures

Figure1:(A)EGDshoweda4cmsubepithelialmassinthegastriccardiaatthehiatalherniapinchlocatedat40cm.

(B)OnlimitedEUS,therewastheheterogeneousmassmeasuring39mmby31mminthegastriccardiawithsmall

internalcysticspaces.

Figure2:(A)ThepatientCTscanshoweda3.5x1.7cmsmoothlymarginatedmassadjacenttothegastricwalljust

beyondtheGEjunctioninthegastriccardia(arrow).(B)Therewasalsoahiatalhernia(arrowhead).Differential

diagnosisincludedgastrointestinalstromaltumor(GIST)andleiomyoma.

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Figure3:(A)Lowpower40xand(B)Highpower100xmagni�ication.Thelaparoscopicintragastriccoreneedle

biopsy showed �ibrous core tissue with islands of benign glandular cells with variable degree of chronic

in�lammation. There was no neutrophilic in�iltration to suggest acute in�lammation. These �indings were

consistentwithgastritiscysticaprofunda.

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12.LimJK,JangYJ,JungMK,etal.Menetrierdiseasemanifestedbypolyposisinthegastricantrumandcoexisting

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ManuscriptInformation:Received:November10,2016;Accepted:December27,2016;Published:December29,2016

1 2 2 3AuthorsInformation:DanishShahab,MD ;EmmanuelGabrielMD ;MoshimKukarMD ;AndrewBainMD ;StevenHochwald2*MD

1DepartmentofMedicine,UniversityatBuffalo,Buffalo,NY14263USA2DepartmentofSurgicalOncology,RoswellParkCancerInstitute,Buffalo,NY14263USA3DepartmentofMedicine,RoswellParkCancerInstitute,Buffalo,NY14263USA

Citation:ShahabD,GabrielE,KukarM,Bain,Hochwald.Gastritiscysticaprofunda:achallengingdiseasediagnosedusinga

novelapproach.OpenJClinMedCaseRep.2016;1204

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