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Adenoid cystic carcinoma presenting as an ulcer on the floor of the mouth: a rare case report

Saba Khan1, Khalid Agwani2, Puneet Bhargava1, Sreeja P. Kumar3

1Department of Oral Medicine and Radiology, NIMS Dental College and Hospital, NIMS University, Jaipur, 2Department of Oral and Maxillofacial Surgery, Darshan Dental College, Udaipur,

3Department of Oral Medicine and Radiology, Amrita School of Dentistry, Kochi, India

Abstract (J Korean Assoc Oral Maxillofac Surg 2014;40:253-257)

Adenoid cystic carcinoma is a rare epithelial tumour, and comprises about 1% of all malignant tumours of the oral and maxillofacial region. It is a malignant tumour which may develop in the trachea, bronchus, lungs or mammary glands, in addition to the head and neck region. Occurrences in the head and neck are mostly detected in the major salivary gland, oral cavity, pharynx and paranasal sinus where it presents as a slow growing firm nodu-lar swelling. The aim of the article is to highlight the unique presentation of adenoid cystic carcinoma as a solitary ulcer on the floor of the mouth.

Key words: Carcinoma, Adenoid cystic carcinoma, Oral cavity, Salivary gland neoplasms[paper submitted 2014. 4. 25 / revised 1st 2014. 6. 6, 2nd 2014. 7. 26 / accepted 2014. 7. 28]

bular,cribriformandsolidpatterns.Ithasarelentlessclinical

courseandusuallyafataloutcome”3.Thepresentcasereport

isuniqueasitshowspresentationofadenoidcysticcarci-

nomaasasolitaryulceronthefloorofthemouthratherthan

theclassicalnodularswelling.

II. Case Report

A56-year-oldmalepatientpresentedwithachiefcom-

plaintofanulcerintheleftfloorofthemouthforoneweek.

Hegaveahistoryofpainofrespectontheleftsideofthe

jawwhichwascontinuous,dull,throbbing,andradiatingto

theearofthesameside.Thepatienthadundergoneextrac-

tionfortooth#18butthepainstillpersisted.Thereafter,he

noticedanulcerontheleftfloorof themouthwhichwas

initiallysmallandincreasedtothepresentsize.Therewasno

relevanthistoryofinjury,traumaticsurgeryorbiopsy.There

wasnohistoryofbleedingordischarge,buthedidhavedif-

ficultyeatingandspeaking.Therewasnohistoryofsmoking,

quidchewingoralcoholconsumption.

Intraoralexaminationofthesofttissues,thebuccalmucosa,

labialmucosa,tongue,andpalateshowednoabnormalities,

buttherewasasolitaryulcerontheleftfloorofthemouth.

Examinationofgingivalstatusrevealedhisoralhygienesta-

tustobepoor,withseverestainsandcalculusdeposits.On

hardtissueexamination,healingsocketwaspresentinrespect

I. Introduction

AdenoidcysticcarcinomawasfirstdescribedbyBillroth

in1859andcalled“cylindroma”due to itscharacteristic

histologicappearance1.In1953,FooteandFrazell2renamed

thelesionasadenoidcysticcarcinoma.Adenoidcysticcar-

cinomaisamalignantsalivaryglandtumourcharacterized

byadeceptivehistologicpattern,indolent,locallyinvasive

growthwithhighpropensityforperineuralinvasion,localre-

currenceanddistantmetastasis.Theseuncommonneoplasms

accountforfewerthan1%ofallheadandneckmalignancies

andfewerthan10%ofallsalivaryneoplasms.Theymake

up15%-30%ofsubmandibularglandtumours,30%ofmi-

norsalivaryglandtumours,and2%-15%ofparotidgland

tumors1.It isdefinedbytheWorldHealthOrganizationas“abasaloidtumourconsistingofepithelialandmyoepithelial

cellsinvariousmorphologicalconfigurations,includingtu-

CASE REPORT

Saba KhanDepartment of Oral Medicine and Radiology, NIMS Dental College and Hospital, NIMS University, Shobha Nagar, Jaipur-Delhi highway, Jaipur 303121, Rajasthan, IndiaTEL: +91-1426-513102 FAX: +91-141-2605050E-mail: dr.sabakhan23@gmail.com

This is an open-access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/), which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

CC

Copyright Ⓒ 2014 The Korean Association of Oral and Maxillofacial Surgeons. All rights reserved.

http://dx.doi.org/10.5125/jkaoms.2014.40.5.253pISSN 2234-7550·eISSN 2234-5930

J Korean Assoc Oral Maxillofac Surg 2014;40:253-257

254

mostlysolitary,deep,andpainful,withsmoothmarginsanda

reddishhaloandmaypersistformonthswithhistoryofreoc-

currences.

Habitassociatedlesionsseeninquidchewersmayalso

formulcerationsattheregionofquidplacement.Odontogen-

icinfectionsmaybeassociatedwithanulcerthatmayserve

asacloacalopeningofasinusdrainingachronicalveolar

abscess.Theyaremostlyseeninthepalateandsublingualor

vestibularareas.Pusexudationwithregionallyinvolvedteeth

wouldhelpindifferentiation.Otherconditionssuchasacute

necroticulcerativegingivostomatitisorgangrenousstoma-

titisusuallypresentasnecroticsloughingulcerativelesions

diffuselyinvolvingthegingiva.Solitaryulcersarealsoseen

innonodontogenicsystemicdiseasessuchasuncontrolled

diabetesmellitus,blooddyscrasias(leukemia,sicklecellane-

mia),gastrointestinalandimmunocompromisedindividuals

andautoimmuneconditions(pemphigus,pemphigoid,ery-

themamultiforme,epidermolysisbullosa).Theulcersinsuch

conditionsarewelldemarcated,painful,andshallowwithan

erythematoushaloandagreynecroticfloor,usuallyinthe

marginalandinterdentalgingiva.Salivaryglanddisorders

suchasadenoidcysticcarcinoma,mucoepidermoidcarcino-

ma,mucousadenocarcinoma,Warthintumorandnecrotising

sialometaplasiaareseenassolitarypalatalulcers.

Anorthopantomogramandamandibularcrossectional

occlusalradiographweretakenandshowednoabnormality

intheregionofinterest.(Fig.2)Amagneticresonanceim-

agewastaken,andtheaxialsectionsshowedaT1-weighted

imageofamassofmediumintensityextendingfromthe

midlineofthemandibletothepremolarregionontheleft

side.(Fig.3)Anincisionalbiopsywasperformedandthehis-

topathologicreportshowedthepresenceofuniformbasaloid

cellsarrangedintheformofsolidislands,alongwithacrib-

riformpatternatsomeplaces.Thetumourcellswerefound

tobeinfiltratingtheadjacenttissues.Normalsalivarygland

totooth#19.Onlocalexaminationofthelesion,inspectory

findingsrevealedasolitaryulcerpresentingasamucosaltear

involvingthemucosaandconnectivetissueextendingmesi-

allyfromleftsideofthelingualfrenumanddistallytoan

areacorrespondingtotheextractedtooth#19region.Theul-

cerwasseenincloseapproximationtotheleftalveolarridge.

Itwasovalinshape,3×2cmatitswidestpoint,and3cm

deep.Themarginswereeverted,theedgesoftheulcerwere

sloping,andthefloorwasnotevident.Thecolourwasthe

sameasthatoftheadjacentmucosa,buterythematousatthe

peripheryoftheulcer.Nobleedingordischargewasevident.

Photographsweretakenwithdueconsentfromthepatient.

(Fig.1)Onpalpationofthesite,thesizeandtheextentofthe

ulcerwereconfirmed.Theulcerwasmildlytenderonpalpa-

tion,withslopingedges.Themarginsandthebaseof the

ulcerweremildlyinduratedinaposterioraspect.Theulcer

restedonamuscularbase.

Basedonthehistorygivenbythepatientandtheclinical

features,aprovisionaldiagnosisofcarcinomaofthefloor

ofthemouthT4aN0M0,chronicgeneralizedperiodontitis,

partiallyedentulousinrespecttotooth#7,#5,#3,#13,#14,

#15,#24,#19,#25,and#31wasmade.Lesionspresenting

assolitaryulcersonthefloorofthemouthwereconsidered

inthedifferentialdiagnosis.Traumaticulcersaremostcom-

mononthetongue,lips,mucobuccalfold,gingivaandpalate.

Theypresentassolitaryulcerswithraisedreddishborders

andwhitenecroticfloorswithassociatedhistoryoftrauma.

Onthefloorofthemouth,theyareusuallyduetocalculus

orsharpdenturemargins.Majorrecurrentapthousulcersare

Fig. 2. Orthopantomogram does not reveal any changes in the region of interest.Saba Khan et al: Adenoid cystic carcinoma presenting as an ulcer on the floor of the mouth: a rare case report. J Korean Assoc Oral Maxillofac Surg 2014

Fig. 1. Solitary ulcer on the floor of the mouth.Saba Khan et al: Adenoid cystic carcinoma presenting as an ulcer on the floor of the mouth: a rare case report. J Korean Assoc Oral Maxillofac Surg 2014

Adenoid cystic carcinoma

255

deceptivelybenignhistologicalappearance,characterized

byindolentlocallyinvasivegrowthwithahighpropensity

forlocalrecurrenceanddistantmetastasis.Accordingtothe

literature,adenoidcysticcarcinomamostlyoccursinthefifth

orsixthdecadeoflife.Genderpredilectionisaninconsistent

featureintheliterature;however,itisslightlymorecommon

inwomenthaninmen4,5.Althoughthepalate is themost

commonlyinvolvedintraoralsite,othercommonlyinvolved

areasare themajorandminorsalivaryglandregions, the

floorofthemouth,thetongueandthegingiva(indecreas-

tissuewasfoundadjacenttotheconnectivetissue.

Basedontheclinicalfeaturesandthehistopathologicre-

port,afinaldiagnosisofadenoidcysticcarcinomaT4aN0M0

wasmade.(Fig.4)Wideexcisionofthelesionwithhemiman-

dibulectomywascompleted.(Fig.5)Thepatientwasthen

referredforfurtherradiotherapy.

III. Discussion

Adenoidcysticcarcinomaisamalignant tumourwitha

Fig. 3. Axial sections of an magnetic resonance imaging show T1-weighted images of a mass of medium intensity.Saba Khan et al: Adenoid cystic carcinoma presenting as an ulcer on the floor of the mouth: a rare case report. J Korean Assoc Oral Maxillofac Surg 2014

Fig. 4. Presence of uniform basaloid cells arranged in the form of solid islands (H&E staining, ×40).Saba Khan et al: Adenoid cystic carcinoma presenting as an ulcer on the floor of the mouth: a rare case report. J Korean Assoc Oral Maxillofac Surg 2014

Fig. 5. Wide excision of the lesion with hemimandibulectomy was completed.Saba Khan et al: Adenoid cystic carcinoma presenting as an ulcer on the floor of the mouth: a rare case report. J Korean Assoc Oral Maxillofac Surg 2014

J Korean Assoc Oral Maxillofac Surg 2014;40:253-257

256

adenoma8.

Possibletreatmentsofadenoidcysticcarcinomainclude

fourdifferentmodalities: surgical therapy, radiotherapy,

chemotherapyandcombinedtherapy.Surgicalexcisionwith

widemarginsis thetreatmentofchoice10.However, there

isstillcontroversyregardingtheadjuvanttreatmentofthis

tumour.Averyetal.11 recommendpostoperativeradiation

sinceradiationoftenproducestumourregressionandrelieves

symptoms.Postoperativeradiotherapyisalsoindicatedwhen

thetumourlocationisclosetothebaseofthecraniumwith

thepresenceofnecklymphnodemetastasisandperineural

invasion,withasolidhistologicalsubtype,andforrecur-

rent tumours11.Adenoidcysticcarcinomashowsalimited

responsetochemotherapy,anditisbelievedthatthislackof

aresponseisduetoitsslowgrowthrate.However,astudy

byAlcedoetal.12openedupanewpossibilityinthephar-

macological treatmentofthistumourbydemonstratingits

responsetoimatinibmesylate,apotentinhibitorofKITgenetyrosinekinase,whichisanenzymeinvolvedinthepatho-

genesisofthetumour.However,morestudiesareneededto

confirmitseffectiveness.Distantmetastasiscanoccureven

decadesaftertheprimarytumourhasbeentreatedandafter

havingachievedadequatelocoregionalcontrol.Thisleadsto

lowlong-termsurvivalrates.Someauthorsevenconsiderthis

tumourtobeanincurabledisease12.

Furthermore in thesettingof inoperable, incompletely

resectedorrecurrenttumours,outcomesafterconventional

therapyaredismalandremainatherapeuticchallenge.Some

authorshaveappliedotherformsofradiotherapy,particularly

neutronirradiation,aimingto improvetreatmentresults13.

However,adenoidcysticcarcinomaremainsincurablebe-

causefurtherimprovementsinlocal-regionalcontrolarenotlikelytoimpactsurvivalduetothehighnumberofdistant

failures.

Thepresentcasediffersfromusualcasesofadenoidcystic

carcniomawithrespecttositeandpresentation,makingita

rareentity.Thus,theaimofthisarticleistoemphasisethat

thoughtheliteraturestatesthatadenoidcysticcarcinomaul-

ceratesthesuperficiallesionalmucosa,itcanalsopresentas

anulcerativelesion.

Conflict of Interest

Nopotentialconflictofinterestrelevanttothisarticlewas

reported.

ingorder).Adenoidcysticcarcinomausuallypresentsasa

slowlygrowing,firm,unilobularmassintheglands.How-

ever,inourcaseitpresentedasasolitaryulceronthefloorof

themouth.Theclinicalcourseischaracterizedbyaninitial

periodofslowandindolentgrowththatisusuallyasymptom-

atic,althoughboneinvasionorperineuralspreadcancause

painorhypoesthesia5.Thetumourusuallyspreadsthrougha

haematogenousroutewithdistantmetastasis.Cervicalnode

metastasisisrare6.

Adenoidcysticcarcinomaofthesalivaryglandsisama-

lignantepithelial tumourwithbidirectionaldifferentiation

towardsluminal(ductal)andabluminal(myoepithelialand

basal)cells.Thetumouriscomposedofbasaloidcellswith

small,angulated,andhyperchromaticnucleiandscantcyto-

plasmarrangedintothreeprognosticallysignificantpatterns:

cribriform,tubular,andsolid.Thecombinationofsurgery

andpostoperativeradiationtherapyhasimprovedlocoregion-

alcontrolofthedisease.Despitethisachievement,latelocal

recurrenceanddistantmetastasisratesremainhighandmay

occurdecadesafterinitialdiagnosis5.

Initsmostfrequentlyseenhistologicalpattern,themajority

ofthecellsaresmallanddarklystainedwithscantcytoplasm.

Thecellsarearrangedinnestsorsheetsthatarefenestrated

byroundorovalspaces,creatingthecharacteristic“cribri-

form”design.Occasionally, the tumourshaveapredomi-

nantlysolidcellulargrowthwithabasaloidoranaplasticap-

pearancethathasfew,ifany,fenestrations.Thesolidvariant

oftendemonstratessmallareasofnecrosis.Tubularstructures

withminimalstratificationoftheliningepitheliumareoften

mixedwiththeclassiccribriformandsolidareas6.Several

authorshavesuggestedthatasolidhistologicalpatternindi-

catesamoreseriousprognosisthanacysticpattern.Stewart7

firstnotedtheincreasedaggressionsuggestedbythesolid

variant,althoughhecreditstheinitialobservationtoPatey

andThakray8.

Littleinformationexistsoncytogeneticabnormalitiesin

salivaryglandneoplasms,butinadenoidcysticcarcinoma,

anomaliesintheterminalpartofthe6qand9pchromosomes

havebeenreported.Recentstudieshavedemonstratedahigh

incidenceoflossofheterozygosityatchromosome6q23-359.

Thedifferentialdiagnosisofadenoidcysticcarcinomaincludes

tumoursthatalsoexhibittubularandcribriformstructures,

suchaspolymorphouslow-gradeadenocarcinoma,tumours

withbasaloidcellularmorphology,suchasbasalcelladenoma,

andbasalcelladenocarcinomaandtumourswithadualpopu-

lationofductalandmyoepithelialcells,suchaspleomorphic

Adenoid cystic carcinoma

257

lightofapathologicalstudyofparotidectomymaterial.BrJSurg1958;45:477-87.

9. RapidisAD,GivalosN,GakiopoulouH,FaratzisG,StavrianosSD,VilosGA,etal.Adenoidcysticcarcinomaoftheheadandneck.Clinicopathologicalanalysisof23patientsandreviewoftheliterature.OralOncol2005;41:328-35.

10. Norberg-SpaakL,DardickI,LedinT.Adenoidcysticcarcinoma:useofcellproliferation,BCL-2expression,histologicgrade,andclinicalstageaspredictorsofclinicaloutcome.HeadNeck2000;22:489-97.

11. AveryCM,MoodyAB,McKinnaFE,TaylorJ,HenkJM,Lang-donJD.Combinedtreatmentofadenoidcysticcarcinomaofthesalivaryglands.IntJOralMaxillofacSurg2000;29:277-9.

12. AlcedoJC,Fábrega JM,ArosemenaJR,UrrutiaA. Imatinibmesylateas treatmentforadenoidcysticcarcinomaof thesali-varyglands:reportoftwosuccessfullytreatedcases.HeadNeck2004;26:829-31.

13. HuberPE,DebusJ,LatzD,ZierhutD,BischofM,WannenmacherM,etal.Radiotherapyforadvancedadenoidcysticcarcinoma:neutrons,photonsormixedbeam?RadiotherOncol2001;59:161-7.

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