IleocaecalrecurrenceofMerkelcellcarcinomaof theskinacasereport MichelleCheung HenryLeeSanjayPurkayasthaRobertGoldinPaulZiprin Abstract Introduction Merkelcellcarcinomaisanuncommonskinmalignanc ID: 939091
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CASEREPORTOpenAccess IleocaecalrecurrenceofMerkelcellcarcinomaof theskin:acasereport MichelleCheung * ,HenryLee,SanjayPurkayastha,RobertGoldin,PaulZiprin Abstract Introduction: Merkelcellcarcinomaisanuncommonskinmalignancythathasahighpropensityformetastatic spread.Asystematicliteraturesearchidentified17casesdescribingmetastasistothegastrointestinaltract,with7 casesinvolvingthesmallorlargebowel.Tothebestofourknowledge,thisistheonlycasedescribedofMerkel cellcarcinomametastasisingtotheileocaecalvalve. Casepresentation: Wepresenta74-year-oldFilipinowomandiagnosedwithMerkelcellcarcinomaoftheskin withregionalnodeinvolvement.Followingexcisionandradiotherapy,thetumourrecurredwithmetastasistothe ileocaecalvalve.Thepatientdied28monthsaftertheinitialdiagnosis. Conclusion: TheprognosisofmetastaticMerkelcellcarcinomaispoor.Currentlytheoptimalmanagementfor metastaticdiseaseisunclearandlacksafirmevidencebaseduetothesmallnumberofcasesreported. Introduction Merkelcellcarcinoma(MCC)isanuncommonand highlyaggressiveskinmalignancy.ItarisesfromMerkel cellsatthedermo-epidermaljunction,whichareofneu- roendocrineorigin.Sinceits firstdescriptionbyToker in1972[1],morethan2000caseshavebeenreportedin theliterature[2].Itsaetiologyisnotentirelyknown,but thereisconvincingevidencefortheroleofultraviolet radiation.MCChasapredilectionforsun-exposedareas ofthebodyandisassociatedwithothersun-relatedskin cancerssuchasbasalcellcarcinomaandsquamouscell carcinoma.TheoccurrenceofMCCinareasthatare notexposedtothesunsuggestsadditionalcauses. ReportsofMCCinorgantransplant,humanimmuno- deficiencyvirus(HIV)infectionandlymphohemopoietic malignancies,suchaschroniclymphocyticleukemia, implicatearoleforimmunosuppression[2,3]. TheincidenceofMCCis0.23per100,000inCauca- sians[2],whichisabout20timestheincidencecom- paredtotheAfro-Caribbeanpopulation.MCCisalso morecommoninoldermenwithameanageofdiagno- sisat69yearsold[2].Inareviewof1024patients,the primarytumourwasfoundintheheadandneckin 40%,inthee
xtremitiesin33%,andinthetrunkin23% ofpatients[4].Atpresen tationtheregionallymph nodesareinvolvedinaround25%ofcases,anddistant metastasesarefoundin4%[4].Metastasisusually occursintheskin(28%),lymphnodes(27%),liver (13%),lung(10%),bone(10%),andthebrain(6%)[2,4]. Metastasiscanalsoinvolvethegastrointestinal(GI) tractinveryrarecases. Asystematicsearchoftheliterature(seeAppendix) found17casesinvolvingGImetastasesmostcommonly involvingthestomach.Seve nofthesecasesdescribed bowelmetastasis(Table1).Shalhub etal .describedthe caseofa62-year-oldmanwithaxillarylymphadenopa- thyandmetastasistothestomachandthedescending colon.Thepatienthadaskinlesionexcisionwhichwas initiallydiagnosedasbasalcellcarcinoma[5].Thereare twocasereportsofstomachandsmallbowelMCCpre- sentingwithupperGIbleedfromKrasagakis etal .[6] andCanales etal .[7].An85-year-oldJapanesewoman wasalsodiagnosedwithwidespreadupperGItract MCCmetastasisonautopsyfollowingintestinalobstruc- tion[8].NauntonMorganandHendersonreporteda manwithanenlargingnoduleonhisshin,whopre- sentedamonthlaterwithmelaenaandwhereameta- staticMCClesioninthepr oximaljejunumwasfound onsurgicalexploration[9].Meanwhile,Foster etal .also describedacaseofMerkelcellmetastasizingtothe smallbowelafteraprotractedtimecourse[10].In *Correspondence:michelle.cm.cheung@gmail.com AcademicSurgicalUnit,StMary sHospital,ImperialCollegeHealthcare, PraedStreet,LondonW21NY,UK Cheung etal . JournalofMedicalCaseReports 2010, 4 :43 http://www.jmedicalcasereports.com/content/4/1/43 JOURNAL OF MEDICAL CASE REPORTS ©2010Cheungetal;licenseeBioMedCentralLtd.ThisisanOpenAccessarticledistributedunderthetermsoftheCreativeCommons AttributionLicense(http://creativecommons.org/licenses/by/2.0),whichpermitsunrestricteduse,distribution,andreproductionin anymedium,providedtheoriginalworkisproperlycited. addition,therearecasesreportedofmetastasistothe rectum,theanalcanal,andthepancreas[11-21]. Casepresentation A74-year-oldFilipinowomanpresentedwithaskin lesio
ninherrightantecubitalfossa.Itwasa2cmsoft, mobile,well-circumscribedmasswhichappearedover threeweeks.Suspectingliposarcoma,hergeneralpracti- tionerreferredhertothehospital ssurgicalteam. Urgentexcisionbiopsyreve aledmetastaticcarcinoma expressingneuroendocrinemarkers.Immunohistochem- icalstainingshowedthatthepatienthadastrongposi- tivityforCK20,whichwasasensitiveandspecific markerforMerkelcellcarcinoma[22].Shewasalso highlypositiveforchromogranin,synaptophysinand CD117,thusconfirmingthediagnosisofMCC.She showednostainingforTTF-1,whichexcludedlungpri- marysmallcellcarcinoma. Subsequentcomputedtomography(CT)examinations ofherchest,abdomenandpelvisidentifiedsignificant right-sidedaxillarylympha denopathy,withthelargest nodemeasuring6cmindiameter.TheCTwascon- ductedtwomonthsafterourpatient sinitialclinicalpre- sentation,andtheaxillaryadenopathywasnowclinically apparent.Therewasnohilarormediastinallymphade- nopathyobserved.Herlungs,bonesandintra-abdominal organswereallclearofmetastasis.Apositronemission tomography(PET)bodyscanshowedhighuptakeinher rightaxilla,withmoderate heterogeneousuptakein somebowelloops.Thiswasthoughttorepresent inflammatorychangeinthepresenceofdiverticular disease.Nodefiniteprimarysource,however,was identified. Ataxillarylymphnodedissection,eightof26lymph nodes,aswellastheaxillaryvein,werefoundtocontain tumour.Adjuvantradiotherapywasadministeredtoour patient saxillaryandsubclavicularregions(42Gy,21 fractions,31days),butitwaspostponeduntilsix monthspostoperativelydue tothepresenceofseroma andlymphoedema.Surgicalandoncologicalfollow-upat intervalsoftwo,threeandsixmonthsfoundourpatient asymptomatic. At18monthsfromtheinitialdiagnosis,ourpatient wasreferredbyhergeneralpractitionerforurgent reviewwithsymptomsofobst ruction,specificallyearly satiety,bloating,colickypainsandoccasionalvomiting. Onexamination,afirmmassinherrightupperquad- rantwasclinicallydetectable.AwholebodyCTscan foundatumourintheileocaecalvalvethatextended i
ntothecaecallumen(Figure1andFigure2).The tumourwasassociatedwithmesentericlymphadenopa- thyandlocalinfiltrationintothepericolicfatand vessels. Atcolonoscopyatumourwasseenat70cmfromour patient sanus.Themorphologyandimmunoprofileof thebiopsyconfirmeditasarecurrenceofMCC.Mar- kersforcolorectalcancer(CA19.9,CEA)werenegative. Ourpatientunderwentalaparoscopicrighthemico- lectomy.Atsurgerythetumourwasseentobefungat- ingandinvolvedthefullthicknessofherbowelwall. Histologicalexaminationshowedclearresectionmargins butvascularinvasionandmultiplelymphnodeinvolve- mentwerealsonoted.Ourpatient spostoperative Table1Reportedcasesofgastrointestinalmetastasesof Merkelcallcancer. Author(s)SiteofMetastasis LiMandLiuC[11]Stomach CubiellaJ, etal .[12]Stomach IdowuM, etal .[13]Stomach WolovK, etal .[14]Stomach KrasagakisK, etal .[6]Stomach,smallbowel CanalesL, etal .[7]Stomach,smallbowel ShalhubS, etal .[5]Stomach,descendingcolon HizawaK, etal .[8]Stomach,distalduodenum, pancreas OliveroG, etal .[15]Intestinal NauntonMandHendersonRG[9]Jejunum FosterR, etal .[10]Smallbowel HuangWS, etal .[16]Rectum PatersonC, etal .[17]Analcanal AdsayNV, etal .[18]Pancreas BachmannJ, etal .[19]Pancreas DimDC, etal .[20]Pancreas OuellettJR, etal .[21]Pancreas Figure1 Computedtomographyshowingarecurrenceof Merkelcellcarcinomainthecaecumat18monthsafterthe initialdiagnosis .Thereisa6×4.3cmsoft-tissuemassseeninthe regionoftheileocecalvalve(A)whichextendsintothelumenof thecaecum.Therearemultipleabnormallymphnodesmeasuring upto2cmwithintheileocolicmesentery.Thereisnodularityand irregularityseenaroundthetumourextendingintothepericolicfat suggestiveofalocaltumourinfiltration. Cheung etal . JournalofMedicalCaseReports 2010, 4 :43 http://www.jmedicalcasereports.com/content/4/1/43 Page2of5 recoverywasuneventful.Follow-upCTscan3months aftertheresectionrevealedperitonealdepositswithno recurrenceintherightax illa(Figure3).Shewasthen scheduledforpalliativechemotherapy. Priortochemotherapyourpat
ientbecameincreas- inglyfrailanddeterioratedrapidly.Shediedofbronch- opneumonia28monthsaftertheinitialdiagnosisof metastaticMerkelcellcarcinoma. Discussion Merkelcellcarcinomahasbeendescribedasonethe mostaggressiveoftheskinmalignances[4,22].InMed- ina-Franco sreviewof1024cases,thismetastaticdisease affects31%ofpatientseitheratpresentationoratlater stage[2].Excludingthepatientswithmetastasisatpre- sentation,31%developlocalrecurrence,andtheaverage disease-freeintervalisonly7.4months[2].Ofthefac- torsrelatedtoprognosisinMCC,lymphnodestatus hasbeenshowntobemostconsistentwithclinical outcome[22-25].Thisisreflectedinthestagingofthe disease,wherestageIdescribeslesionsconfinedtothe skin,stageIIdescribestheinvolvementofregional nodes,andstageIIIdescribesthepresenceofmetastasis. Five-yearsurvivalratesare64%,47%and11%,respec- tively[2,23,25,26].Thiscompareswitha7%to19%sur- vivalrateformalignantmelanomawithmetastasis[27]. Aretrospectivereviewof109patientsbyAllen etal . revealedthatinpatientswithstageIdisease,thetumour sizeatpresentationwasalsoanindependentpredictor ofsurvival.Inviewofthis,a4-tieredsystemisalso widelyusedtoreflectamoreaccurateprognosis.Itclas- sifiespatientswithlymphnode-negativecutaneousdis- easeofcmasstageI,andcutaneouslesionsof-36;.79;é¦è2 cmasstageII.Lymphnode-positivediseaseisclassified asstageIII,whilethepresenceofdistantmetastasesis stageIV[25]. ThetreatmentofchoiceforMCCissurgicalexcision withwidemargins.Thereisnoconsensusonthewidth Figure2 Microscopicappearanceoftheileocaecalvalvetumour .Themucosashowsinfiltrationbyma lignantcellswithglassynuclei, prominentnucleoliandscantcytoplasm.Thereisahighproliferationfraction.ImmunostainingwaspositiveforCK20,chromgraninA, synaptophysin,CD56,andMNF116.ItwasnegativeforCD3,CD30,CD10,CD20,CD23,CD5.Themorphologyandimmunoprofileconfirmsthisto beametastaticMerkelcellcarcinoma. Cheung etal . JournalofMedicalCaseReports 2010, 4 :43 http://www.jmedicalcasereports.com/content/4/1/43 Page3