J. Coloproctol. (Rio J.) vol.35 número2

jcoloproctol(rioj).2015;35(2):124–127

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

Journal

of

Coloproctology

Case

Report

Granular

cell

tumor

of

rectal

submucosa:

case

report

Suheyla

Pollyana

Pereira

Ribeiro

,

Stephanie

Fonseca

Levy

,

Flávia

Motta

Corrêa

,

Aryanna

Musme

de

Araujo

e

Sousa

_,

_Felipe

_da

_Costa

_Silveira

_,

Marcos

Rodrigo

Carvalho

_,

_Fang

_Chia

_Bin

a_{FaculdadedeCiênciasMédicasdaSantaCasadeMisericórdiadeSãoPaulo(FCMSCSP),SãoPaulo,SP,Brazil}

b_{UniversityHospital,FaculdadedeCiênciasMédicasdaSantaCasadeMisericórdiadeSãoPaulo(FCMSCSP),SãoPaulo,SP,Brazil}

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t

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Articlehistory:

Received2October2013 Accepted11August2014 Availableonline7April2015

Keywords:

Granularcelltumor Granularcellmyoblastoma Colonoscopy

Rectum

Immunohistochemistry

a

b

s

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Thisisacasereportofgranularcelltumorofrectalsubmucosainafemale,35-years-old patientcomplainingofhematochezia.Wedescribethehistologicaland immunohistochem-icalfeaturesofthelesionresponsiblebythisclinicalfind.Followingthat,wepresenta discussionofthecasebasedontheliteraturereview,whichallowedtoprovingthe infre-quencyofthetumorintherectalareaandconfirmsthebenignnatureofthetumorinthis case.

©2015SociedadeBrasileiradeColoproctologia.PublishedbyElsevierEditoraLtda.All rightsreserved.

Tumor

de

células

granulares

da

submucosa

rectal:

relato

de

caso

Palavraschave:

Tumordecélulasgranulares Mioblastomadecélulasgranulares Colonoscopia

Reto

Imunohistoquímica

r

e

s

u

m

o

Esteartigorelataocasodeumtumordecélulasgranularesdasubmocosaretal,empaciente de35anos,comqueixadehematoquezia.Fazemosadescric¸ãodosachadoshistológicose imuno-histoquímicosdalesão.Alémdisso,apresenta-seumadiscussãodocasocombase narevisãodaliteratura,quepermitiucomprovarainfrequênciadotumornaregiãoretale corroborarabenignidadedotumornopresentecaso.

©2015SociedadeBrasileiradeColoproctologia.PublicadoporElsevierEditoraLtda. Todososdireitosreservados.

∗ _{Correspondingauthor.}

E-mail:suheylaribeiro@hotmail.com(S.P.P.Ribeiro).

http://dx.doi.org/10.1016/j.jcol.2014.08.015

jcoloproctol(rioj).2015;35(2):124–127

125

Introduction

Thegranularcelltumor,formerlycalledgranularcelltumor myoblastomaorAbrikossoftumor,isarareneoplasticprocess predominantlybenign.Itaffectsdifferentregionsofthebody, butthelesionsinthegastrointestinaltractrepresentonly10% ofthecases.1–4

Thepatientwiththistumorisusuallyasymptomaticand diagnosedasanincidentalfinding duringtheinvestigation ofotherpathologies. Thestudy withpathologicalassays is thegoldstandardforconfirmingthepresenceofthiskindof tumor.5–7

Althoughmostpartofthelesionsarebenign,asmall num-ber shows malignant behavior and about 2%of them can metastasize,especiallythosewithatypicalhistologyorlarge diameter.Inthesecases,it isnecessarytoconduct further investigationsandapplymoreaggressivesurgeryresections asplan oftreatment.8–10 _{Wereporta caseof granularcell}

tumor, in a young female patient complaining of hema-tocheziaforoneyear,attheColoproctologyDepartmentofthe UniversityHospitalSantaCasadeSãoPauloin2013.

Case

report

L.V.A., female, 35 years old, born and raised in São Paulo –Brazil,complainedofanone-yearhistoryofhaemochezia associatedwithcolicabdominalpaininthelowerquadrants, withnobowelhabitschange,duringamedicalattendingof theColoproctologyClinicsatUniversityHospitalSantaCasa deSãoPaulo.Regardingherpastmedicalhistory,shehadbeen smokingfor10yearsandhadahistoryofhemorrhoidal dis-easefor17years.Withregardtothefamilyhistory,hermother hadintestinalpolypsanddiverticulardisease.

Physicalexamination

Onphysicalexamination,thepatientwasinpainonpalpation oftherightiliacfossa,withnoreboundtenderness.On procto-logicalexamination,skintagscompatiblewithhemorrhoids wereobservedintherightlateral,posteriorleft lateraland anterior regions. The anuscopy showed hemorrhoidal pro-trudingnipplesintherightlateral,leftlateralandposterior left.

Complementaryexams

The upper endoscopy identified an erosive esophagitis classifiedasLosAngelesA,aflatandmoderateerosive bul-boduodenitisandanesophagealpolypoidlesion.Pathologic examinationrevealedamildchronicesophagitis.

Thecolonoscopy exam revealed two polypsin the rec-tum and sigmoid colon and ayellowish elevated lesion of 0.5cmindiameter,5cmawayfromtheanaledge,whichwas totallyresected.Thepathologyofthe earlylesionsshowed hyperplastic polypsofcolon and rectal mucosa.The latter injury, however, was a polypoid fragment coated byrectal mucosaconsistingofmatureneoplasmcharacterizedbya pro-liferationofcellswithabundantcytoplasm,micro-vacuoles,

eventually granular, and building blocks interspersed with bandsofcollagenfibers,asshowninFig.1.The immunohisto-chemistryrevealedtheexpressionofCD56,S100,CD34inthe normalvascularendothelium,CD68inthenormalhistiocytes andalow(1%)percentageofKi67cellproliferation(Fig.2).

Diagnosis

Throughtheproctologicalexam,internalhemorrhoidswere diagnosed. The upper endoscopy showed mild chronic esophagitis and the colonoscopy revealed a granular cells tumoroftherectalsubmucosa.

Medicalmanagement

Preoperative tests were ordered to perform hemorrhoidec-tomy.Giventhebenignnatureofgranularcelltumorinthis case,itwaschosentomonitorthepatientafterendoscopic resection.

Discussion

Thegranularcelltumorwasdescribedforthefirsttimeinthe oralcavitybyAbrikossof,in1926anditisarareneoplasm, pre-dominantlybenign1.Itcanaffectanyareaofthebody,being morefrequentintheoralcavityandsubcutaneoustissue.3_In

thegastrointestinaltract,theorganwiththehighest preva-lence ofthetumoristhe esophagus,followed bythe large intestine4.Theincidenceishigherinfemales(1.5:1),withwide variationintherangeofageinthediagnosis,butwithapeak ofincidencebetweenthefourthandsixthdecadesoflife.2

Thisneoplasmhasamesenchymalorigin,probablyderived fromSchwanncells,ahypothesisthathasbecomestrongdue tothe discoveryofahighimmunohistochemicalaffinityof this tumorforS-100protein,myelinandmyelin-associated glycoprotein.8

The granulosa cells have typically benign pattern, with polygonal or fusiform arranged shape in compact nests separatedbycollagenfibers.4_{Thesecellshaveabundant}

cyto-plasm,eosinophilicwithPAS-positivegranulesandsmalland uniformnuclei.11,12_{Thehistologicaland}

immunohistochem-icaldescriptionsfoundinthecaseareconsistentwiththose foundintheliterature.

The tumorusually presents as a non-ulcerated nodule, painless,withlessthan 3cm, withslowgrowth.5–7 _Inmost

of thecases, the tumoris asymptomatic,but the onset of symptoms is more common proportionally to the size or numberoftheneoplasticlesions.4_{About10%ofthepatients}

havemultipletumors.11_{Therateofrecurrenceaftersurgical}

resectionisalsolow:around5–10%.8_{Thefindingofthisinjury}

is usuallymade during the investigation ofother diseases throughthetestssuchasendoscopyandcolonoscopy.8_The

tumorusuallypresentsasasessilepolyp,yellow-grayish,with firmconsistency.4,8_{Thediagnosisismadethroughexcisional}

biopsyandpathologicalstudy.

Themalignantpotentialofthetumoranditscriteriaare stillquestionedintheliterature.About2%oftumors gener-atemetastasis.7_{Yamadaetal.suggestthatthemainfactorfor}

126

Fig.1–(A)Histologicoverviewofthelesion.(B)Detailofthehistology.

Fig.2–Immunohistochemistryofthelesion.(A)ImmunohistochemistryforS100marker.(B)Immunohistochemistryfor CD56marker.

in tumors larger than 4cm in diameter.8,9 _{Fanburg-Smith}

etal.suggesttheuseofhistologicalcriteriasuchascellular necrosis,pleomorphism,increasednuclear-cytoplasmicratio, enlargednucleoli,increasedmitoticactivityandcell elonga-tion.Malignancyissuspectedwhen3ormoreofthesefactors arepresent.10_{Besidesthose,localrecidivationorrapidgrowth}

tumorshouldalsobeconsideredascriteria.4 _Noneofthese

featureswerepresentinthelesionfoundinthepatient, sug-gestingbenignity.

Intheabsenceofmalignancycriteria,therecommended treatmentisendoscopic excision,and eventhe trackingof tumorssmallerthan1cmcanbemadewithoutresection.4,13

Incasesofmultipletumors,it isnecessarytomonitorthe patient,duetothehigherriskofmalignity.13_{Duetothebenign}

featuresofthetumorinourpatientwechosetofollowherwith annualcolonoscopies.

Thegranularcelltumorisarareneoplasm,predominantly benignthatcanaffectanypartofthebody,includingthe gas-trointestinaltract.Asmallpercentageoftheselesionshave malignantpotentialandgeneratemetastasis,beingimportant toanalyzethediameterand histologicalfeaturesbeforeits management.Inmajorityofthecases,wecansafelyperform theendoscopicresection.

Conflicts

of

interest

Theauthorsdeclarenoconflictsofinterest.

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quergestreifterwillknerilicherMuskulatur.VirchowsArchA PatholAnatHistopathol.1926;260:215–33.

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