J. Coloproctol. (Rio J.) vol.36 número2

jcoloproctol(rioj).2016;36(2):115–118

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

Journal

of

Coloproctology

Case

Report

Granular

cell

tumor

of

anal

border

Angelo

Fontes

Araújo

_,

_Igor

_Blohem

_Vasconcelos

_,

_Jadeilton

_Jipso

_de

_Souza

_Dias

_,

Luciano

Santana

de

Miranda

Ferreira

_,

_Rodrigo

_Guimarães

_Andrade

_,

Paulo

César

Boente

Santos

a_{CentrodeOncologiaeHematologiadaBahia(CEHON),Salvador,BA,Brazil} b_{LaboratórioStudart&Studart(LSS),Salvador,BA,Brazil}

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Articlehistory:

Received3March2015 Accepted3March2015 Availableonline22March2016

Keywords:

Analcanal Granularcelltumor Neoplasms Casereports

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b

s

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t

Theobjectiveofthisreportistodescribeacaseofgranularcelltumoroftheanalborderand toreviewthemostrelevanttopicsoftheliteratureonthesubject.Oursisafemalepatient,57 yearsold,withanasymptomaticnoduleintheanalborderfor2years.Surgicalexcisionwas performed,withahistopathologicaldiagnosisofgranularcelltumor.Thefirstdescriptionof thistumorwascarriedoutin1926byAbrikossoff.Thetechniquesofimmunohistochemistry andelectronmicroscopyallowedustodetermineitsorigininSchwanncells.Thesearerare tumors,mostoftendiagnosedbetweenthe4thand6thdecadeoflifeand,ingeneral,are benignformations–only2%ofthemaremalignant.Thesetumorscanoccurinanypartof thebody,althoughtheyaremorecommonintheoralmucosa,dermisandsubcutaneous tissue.Thetreatmentsolelybysurgeryhasacurativeeffect,anditsrecurrenceisunusual. Thelocationintheanal/perianalareaoccursevenmorerarely,andwefoundonly48cases previouslydescribedintheliterature.

©2016SociedadeBrasileiradeColoproctologia.PublishedbyElsevierEditoraLtda.This isanopenaccessarticleundertheCCBY-NC-NDlicense

(http://creativecommons.org/licenses/by-nc-nd/4.0/).

Tumor

de

células

granulares

de

borda

anal

Palavras-chave:

Canalanal

Tumordecélulasgranulares Neoplasias

Relatosdecasos

r

e

s

u

m

o

O objetivodeste relato é descreverum caso de tumor de células granulares de borda analerevisarostópicosmaisrelevantesdaliteraturaacercadotema. Trata-sedeuma pacientedosexofeminino,57anos,comhistóricodenódulonabordaanalassintomático há2anos.Foirealizadaressecc¸ãocirúrgicadalesão,comdiagnósticohistopatológicode tumordecélulasgranulares.Aprimeiradescric¸ãodestetumorfoiem1926por Abrikos-soff.Astécnicasdeimunohistoquímicaedemicroscopiaeletrônicapermitiramdeterminar asuaorigemnascélulasdeSchwann.Sãotumoresraros,maisfrequentesentrea4a

e6a décadadevidae,nogeral,benignos,apenas2%sãomalignos.Podemocorrerem qual-querpartedocorpo,emborasejammaiscomunsnamucosabucal,dermeetecidocelular

∗ _{Correspondingauthor.}

E-mail:angelofamed@hotmail.com(A.F.Araújo).

http://dx.doi.org/10.1016/j.jcol.2015.03.004

116

subcutâneo. O tratamento cirúrgico isolado é curativo e a recorrência incomum. A localizac¸ãonoânus/canalanal/perianaléaindamaisrara,sendoencontradosapenas48 casospreviamentedescritosnaliteratura.

©2016SociedadeBrasileiradeColoproctologia.PublicadoporElsevierEditoraLtda.Este éumartigoOpenAccesssobalicençadeCCBY-NC-ND

(http://creativecommons.org/licenses/by-nc-nd/4.0/).

Introduction

The first histopathological description of the granular cell tumorwasperformedin1926byAbrikossof.Thesearerare tumors,predominantlybenign.Theoccurrenceofmalignant granularcelltumorsbarelyreaches2%,requiringobservation ofhistologicalfeaturesofmalignancyandevidenceof metas-tasesforitsdiagnosis.1,2

Thesetumorscanshowawidedistributionthroughoutthe body,althoughthemostaffectedareasarethebuccalmucosa, dermis and subcutaneous tissue. Granular cell tumors are morefrequentbetweenthe 4th and 6th decade oflife.3 _In mostcaseseries, thetumorprevailsamongmales,ranging from64.5%to68%.2,4 _{Isolatedsurgicaltreatmentiscurative} anditsrecurrenceisunusualandasageneralruleoccursin thesamelocal,beingassociatedwithanincompleteresection oftheprimarylesion.2–4

Herewereportthecaseofagranularcelltumoroftheanal marginanddiscussthemostrelevantaspectsofliteratureand thosepresentedbythiscase.

Case

report

Femalepatient,57yearsold,Africandescent,teacher, mar-ried.Apreviouslyhealthypatientwithoutclinicalandsurgical comorbiditiesandnorelevantfamilyhistory;complainedof anasymptomaticnodulelocatedintheanalmarginforabout twoyears.She deniedchanges in herbowel habits and in characteristicsofevacuations.On examination,ahardened perianalformationmeasuringabout2cm,inthe5.00o’clock position,wasobserved.

Surgical excision was indicated. Preoperative evaluation with colonoscopy, chest X-ray and laboratory tests found no abnormalities.Thus, the entiremacroscopiclesion was resectedatsurgery.

In the macroscopic pathology, two surgical frag-ments of mucosa, with 3.2cm×1.3cm×1.6cm and 1.3cm×0.7cm×0.4cm,respectively,wereevaluatedtogether. Themicroscopyrevealedananalmucosalinedbystratified squamous epithelium without atypia, displaying typical irregular acanthosis featuresin the epithelium adjacent to thetumor.Inthecorium,wefoundcellproliferationwitha largeand granular cytoplasm,with regular nuclei, forming small groups amid intense fibrosis (Figs. 1 and 2). There wasno necrosisor significantmitotic activity.Theprocess wascompromisingtheadjacentadipose tissueandskeletal muscle,inadditiontolateralanddeepsurgicalmargins.The immunohistochemistrywaspositiveintheneoplasticcellsto S100,CD68(Figs.3and4),neuralspecificenolase,inhibinand calretinin;andnegativeforpan-cytokeratin(AE1andAE3).

Fig.1–Tumorcells,lowmagnification,hematoxylin-eosin staining(40×).

Imaging studies (abdominal computed tomography and magneticresonanceimagingofthepelvis)carriedoutafter surgeryshowedonlypostoperativechangesintheanalarea.

Discussion

Virtually,granularcelltumorscanoccur inanyanatomical location,andalthoughmorecasesarelocatedintheskinand subcutaneoustissue,thetongueissinglythemostcommon site.In5–8.5%ofthecases,thetumorsaremultiple.Rarely theseformationsarediagnosedbeforethesurgicalexcision orwhenobtainingabiopsyofsuspiciouslesions.2,4,5

Thepresence ofthis tumorinthe gastrointestinal tract variesfrom5%to19%.2,4 _{Amongthetumorsoftheperianal}

jcoloproctol(rioj).2016;36(2):115–118

117

Fig.3–Immunohistochemistry:groupsofpositivetumor cellsforS100protein(100×).

region,theremayberectalbleeding;butmostofthempresent aspainfulnodulesthatmaybeconfusedwithabscess.Many oftheseformationsarediagnosedincidentallyduringan eval-uationofotherchanges,suchashemorrhoidsandperianal fissures.6,7

Amongthedescribedhistopathologicalfeatures,onecan highlightthe positivityinimmunohistochemistryforS-100 proteinand CD-68.TheS-100 proteinisexpressed in vari-oustumors(neural tumors,melanomas, sarcomas ofclear cells,histiocytosis,andothers),andthepositivity,inthecase herepresented,supportsthe neuraloriginofthelesion,as it is currently accepted in the literature. Despite this ori-gin,thetumorcellssharemanymorphologicalandstructural characteristicsofmacrophages;thus,thesecellsarepositive forCD-68,amacrophage/histiocyticmarkerpar excellence. Anotherimportantaspectisthefrequentassociationofthese tumorswithsomedegreeofpseudoepitheliomatous hyper-plasiaoftheoverlyingepithelium,whichcanbeaconfounding factorwithsquamouscellcarcinoma.4,6

Malignantgranularcelltumorsare rare (2% ofcases).1,2 Some histological characteristics favoring the diagnosis of malignancyare:thepresenceofnecrosis,avesicularnucleus with anobvious nucleolus (in benign tumors, the nucleus

Fig.4–Groupsofpositivetumorcellsin immunohistochemistryforCD-68(100×).

ispyknotic),atrendfortheformationofspindlecells,and some mitoticactivity. Someauthors suggest the classifica-tionoftumorsinthosewithuncertainmalignantpotential, if these formations have any of the above characteristics, besidesfavoringamorerigorousclinical follow-upand the surveyforoccultmetastases.1

Malignanttumorsaffect(morethanbenigntumors)deep softtissues,esophagus,larynx,peritonealcavityand periph-eralnerves.Themajormetastaticsitesarelymphnodes(70%) andlung(50%).Malignantvariantshaveanaggressive behav-ior,witharateoflocalrecurrenceof70%,andresultindeath in65%ofcasesafteranaverageof2.5yearsafterdiagnosis. Amongthedifferentialdiagnosesofmalignantgranularcell tumors,alveolarsofttissuesarcomaandrhabdomyosarcoma canbeparticularlycitedand,lesscommonly, dermatofibrosar-coma,malignantfibroushistiocytomaandmelanoma.1

Literature

series

Lebranchuetal.publishedthelargestseriesofgranularcell tumors, with263 cases,of which15 were multiple and 12 wereassociatedwithmalignanciesofotherhistological fea-tures,butwithnocasesofmalignantgranularcell.Therewas onlyonecaseofrecurrenceafteranincompleteresection.Men wereaffectedin68%ofthetimeandthemainsiteswere:skin (38%),esophagus(19%)andtongue(10%).Ninecasesoccurred inthedigestivetract(21.03%oftotal)andonly1intherectum

and1intheanus2_(Table1).

Lacketal.reviewed118tumorsin110patientsandfound ahigherprevalenceofAfricandescent(29%)patients,64.5% inmen,44%locatedintheskinandsubcutaneoustissue.The predominanceofmenwasattributedtoapossibleselection biasofpatientswhocamefrommilitaryhospitals.Onlysix cases were locatedin the rectum/anus, which isthe most commonsiteinthegastrointestinaltract,followedby esoph-agus(2cases) andstomach(1case) (Table1).Inthis series, recurrencewas8%;andinallcases,thesurgicalmarginwas compromisedinthefirstresection.However,theabsenceof recurrencewasnumericallyhigher,evenincaseswitha pos-itivesurgicalmargin.4

Thelargestnumberoftumorsintheperianalregionwas reportedinaseriesonlyofthegastrointestinaltract:16cases inatotalof74.Intotal,only48casesofgranularcelltumors intheperianalregionhavebeendescribedintheliterature, highlightingthescarcityofthetumorinthissite.Amongthese cases,63.3%(19/30)occurredinmalesubjects,andthemean andmedianageswere40.9yearsand39years,respectively6

(Table1).

118

Table1–Descriptionofcasesofgranularcelltumors locatedintherectum/analcanalintheliterature.

Authorandyear ofpublication

N◦_ofcasesand

location

Gender/age

Crane,19458 _{1,perianal F/26}

Simon,19479 _{1,perianal F/50}

Rothchild,195310 _{1,perianal M/36} Rosenberg,

196011

1,perianal M/38

Winne,196112 _{1,perianal M/26}

Vance,196913 _{6,perianal (–)} Strong,19705 _{2,perianal (–)} Rickert,197814 _{3,perianal M/64}

M/39 F/57 Lack,19804 _3,rectal

3,anal

(–)

Johnston,19816 _{16,anal M/25}

M/39 M/23 M/44 M/31 M/41 F/50 M/26 M/24 F/22 F/32 M/51 M/25 F/52 F/44 (–)

Pruglo,198515 _{1,rectal M/33}

Lisato,199516 _{1,rectal (–)}

Lebranchu,19992 _1,rectal 1,anal

(–)

Nakachi,200017 _{1,rectal F/47}

Cohen,200018 _{1,anal F/75}

AlBouzidi,200319 _{1,anal M/56}

Kim,200320 _{1,rectal M/49}

Santoni,200621 _{1,anal F/28}

Mourra,20117 _{1,anal M/50}

(–),informationnotavailable;M,male;F,female.

Conflicts

of

interest

Theauthorsdeclarenoconflictsofinterest.

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