UnitTwentyFiveADENOIDCYSTICCARCINOMAOFTHESA;IVARYGLANDSustainedCompleteResponsetoChemotherapy.G.THOMASBUDD.MD.AND.C.W.GROPPE.MDApatientdevelopedwidelymetastaticadenoidcysticcarcinomaoftheparotidelandafterseverallocalrecurrences.Acompleteregressionofpleural,pulmonaryparenchymal,cutaneous,andbonemetastaseswasseenfollowingtherapywith5-fluorouracil,Adriainycin,andmitomycinC.Thisisthesecondcompleteresponsetochemotherapyreportedforapatientwithmetastaticadenoidcysticcarcinomaofsalivaiyglandorigin.BothcompleteresponsesandseveralpartialresponseshavefollowedireatmeniwithAdriamycin,suggesting(hatthisdrugisactiveinthedisease.Cancer51:589-590,1983ADENOIDCYSTICCARCINOMAhasbeenanuncommon,slowgrowingtumorcharacterizedbymultiplerecurrences.Althoughthishistologicentityisoccasionallyrepresentedintumorsoftheskin,breastandcervix,aswellastumorsarisingfromlacrimalglandsand(heceruminousglandsoftheexternalauditorycanal,adenoidcysticcarcinomamostfrequentlyoriginatesinamajororminorsalivarygland.Thistumorwasfoundin10%ofpatientspresentingwithsalivaryneoplasmstoMemorialSloan-KetteringCancerCenterandin25%ofpatientspresentingtoM.D.AndersonHospitalwithneoplasmsofthemajorsalivaryglands(parotid,submaxillary,andsublingual),accountingfor5.2%ofallheadandneckcancers.Biologically,adenoidcysticcarcinomaischaracterizedbyslowgrowthwithinsidiousinvolvementoflocalstructures.Perineuralinvasionistypicalwhenthetumorislocatedinproximitytonervoustissue,andinfiltrativegrowthistherule.Clinically,thesecharacteristicsarcmanifestasatendencyforlocalrecurrenceinapproximatelytwothirdsofpatients.Althoughdirectextensioncaninvolvelymphnodes,lymphaticspreadtoregionalnodesisnotcommon,occurringinonly15%ofthepatients.Distantmetastasesontheotherhand,occurin42-58%ofthepatients.Thelungsarefavoredsitesformetastases,althoughbone,liver,centralnervoussystem,andotherorgansmaybecomeinvolved.Whilerelativelylongsurvivalcanoccurinthefaceofmetastaticdisease,effectivesystemictherapywouldbedesirableinviewofthetendencyforpatientswithadenoidcysticcarcinomatodevelopdistantmetastases.Theauthorsreportacompleteremissionfollowingtreatmentwith5-fluorouracil.Adriamycin(doxorubicin),andmitomycinC,inapatientwithwidelymetastaticadenoidcysticcarcinomaoftheparotidgland.CaseReportInOctober1980,a29-year-oldwhitewomanwasreferredtotheClevelandClinicformetastaticadenoidcysticcarcinomaoriginatingintheleftparotidgland.ThepatienthadundergoneresectionofaleftparotidtumorinSeptember1967foranadenoidcysticcarcinomashowinghistologicevidenceofearlyperineuralinvasion.AlocalrecurrenceinJanuary1972wastreatedbysurgicalresection.SubsequentlocalrecurrencesweretreatedbysubtotalparotidectomyinJanuary1976,andexcisioninJanuary1977,witharesectionofrecurrent(umorandaninvolvednodeinJanuary1978necessitatingsacrificeoftheleftfacialnerve.AlocalrecurrenceinOctober1978wastreatedwith300()radofmegavoltageradiationdeliveredbylinearacceleratoraswellas2000radoforthovoltageradiation.ThepatientfirstnotedchestpaininJuly1980,whichwasfollowedbythedevelopmentofdyspneaonexertionandadrycoughinSeptember1980.Aright-sidedpleuraleffusionwasnotedandadecubituschestx-rayrevealedtwonodulardensitiesintherightmid-lungfields.Thoracentesiswasperformedandthefluidwasfoundtocontainmalignantcellscylologicallyconsistentwithmetastaticadenoidcysticcarcinoma.Abonescanshowedincreaseduptakeoverthepubis.LI,T5andtherightribs;aliverspleenscanwasnormal.Serumchemistriesrevealedanelevationofthealkalinephosphataseto339U/L.Abiopsyspecimenofacutaneousnodulelocatedonthepatient'sabdomenrevealedmetastaticcarcinoma.OnOctober17,1980,thepatientreceivedherfirstcourseofcombinationchemotherapywithFAM.Treatmentwaswith5-fluorouracil500mg/ni2andAdriamycin30mg/m2intravenouslyeveryfourweeks.MitomycinCwasgivenonthefirstdayandeveryeightweeksatadosageof10nig/m2.ByFebruary26,1981,examinationofthepatient'schestx-rayshowedimprovementandtheserumalkalinephosphatasehadfallento118U/L.AbonescanperformedJune25,1981wasnormal.OnJuly2,1981,thepatient'schestXJraywasclearherserumalkalinephosphatasewasnormalat52U/L,andphysicalexaminationrevealednoevidenceofdisease.SheremainedincompleteremissionwhenseenonOctober30,1981.WhilestillreceivingtherapywithFAM,thepatientleadsaphysicallyactivelifeandremainsdiseasefreeafter12monthsoftreatment.DiscussionThisisthesecondreportedcaseofacompleteremissionofmetastaticadenoidcysticcarcinomaofthesalivaryglandinducedbychemotherapy.Theotherreportedcompleteresponseoccurredfollowingtherapywithvincristine,intravenouscyclophosphamide,andAdriamycin.inthatcase,disease13rogressionhadoccurredduringtreatmentwithvincristineandoralcyclophosphamide,suggestingthatAdriamycinwastheagentresponsiblefortheinductionofremission.PartialresponseshavebeenreportedfollowingtherapywithAdriamycinandtherelatedanthracyclinedaunomycin.OffivepatientswithadvancedcarcinomaoftheparotidglandtreatedwithAdtiamycin,cisplatinandcyclophosphamide,twocompleteandthreepartialresponseswerereported,althoughnoneofthesepatienttumorsdemonstratedadenoidcystichistology.Asinglepartialresponsetohexamethylmelaniinetherapyhasbeenreported,althoughclassicalalkylatingagentshavenotbeeneffective.5-Fluorouracilhasbeenshowntohavesomeactivityagainstadenoidcysticcarcinomawhengiveneigberintra-arteriallyorintravenously.Althoughthedifficultiesinherenlintheretrospectiveassessmentofthebenefitsoftherapyinthisuncommonandslowgrowingtumorsystemhavebeenpointedout,objectiveresponsescanbedocumented.Inourpatient,aclear-cutimprovementinperformancestatusandlifestylehasaccompaniedamarkedimprovementinobjectivemeasuresofdisease,andaprolongationofsurvivalmightbepresumed.Adriamycinwouldseemtobethemosteffectiveagentforthetreatmentofmetastaticadenoidcysticcarcinomaofthesalivaryglands,although5-fluorouracilhasalsoshownsomeactivity.Becausethetumorisuncommon,anyprospectivestudywouldbebatdonebyacooperativegroup,andshouldrecognizetheefficacyofAdriamycin,andperhaps5-fluorouracilinthetreatmentofthedisease.VOCABULARY1. histologicentitycervix2. perineuralinvasionlymphaticspread3. 5-flaorouraciladriamycin4. mitomycinmegavoltage5. linearacceleratordyspnea6. thoracentesisvincristine7. daunomycincisplatin8. clear-cutperformancestate9. metastaticadenoidcysticcarcinoma10. lacrimalglandsceruminousglands11. parotidectomydecubitus12. anthracyclinecyclophosphamide組織學(xué)本質(zhì)子宮頸神經(jīng)周擴(kuò)散淋巴道擴(kuò)散5一氟尿嗜啜阿霉素絲裂霉素巨電壓直線加速器呼吸困難胸腔穿刺術(shù)長春新堿正定霉素順鉗明確的生活自理能力轉(zhuǎn)移的，遷移的腺樣囊性癌淚腺盯耳寧腺腮'腺切除臥位芯環(huán)類抗生素環(huán)磷酰胺六甲蜜胺13. hexamethylmelarnine