J. Coloproctol. (Rio J.) vol.36 número1

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

Journal

of

Coloproctology

Case

Report

Gastrointestinal

stromal

tumor

of

rectum

diagnosed

by

three-dimensional

anorectal

ultrasound

夽

Raphael

Ferreira

Ratin

,

Suelen

Melão

,

Doryane

Maria

dos

Reis

Lima

,

Univaldo

Etsuo

Sagae

_,

_Gustavo

_Kurachi

a_{HospitalSãoLucas,FaculdadeAssisGurgacz(FAG),Cascavel,PR,Brazil}

b_{AnorectalPhysiologySector,GastroclínicaCascavel,Cascavel,PR,Brazil}

a

r

t

i

c

l

e

i

n

f

o

Articlehistory:

Received14February2014 Accepted3March2015

Availableonline20December2015

Keywords:

Gastrointestinalstromaltumor CD34

CD117

a

b

s

t

r

a

c

t

Gastrointestinalstromaltumors(GIST)arerelativelyrarelesionsofmesenchymalorigin, beingmorefrequentinthestomachandsmallintestine.Theseareclinicallyasymptomatic lesions,andinadvancedstagesmaypresentwithnausea,vomiting,bleeding,abdominal pain,apalpablemass,andevenintestinalobstruction.Theonlyeffectivetreatmentconsists ofacompletetumorresection.WereporttwocasesofGISTlocatedinthedistalrectumand evaluatedwiththree-dimensionalanorectalultrasonography,aprocedureofgreatvaluein identifyingthesizeofthelesion,itsinvolvementtowardnearbystructuresandlymphnode invasion.

©2015SociedadeBrasileiradeColoproctologia.PublishedbyElsevierEditoraLtda.All rightsreserved.

Tumor

estromal

gastrointestinal

de

reto

diagnosticado

por

ultra-sonografia

anorretal

tridimensional

Palavras-chave:

Tumorestromalgastrointestinal CD34

CD117

r

e

s

u

m

o

Ostumoresestromaisdotratogastrointestinal(GIST)sãolesõesrelativamenterarasde origemmesenquimal,sendomaisfrequentesnoestômagoeintestinodelgado. Clinica-mente, são lesõesassintomáticas eem estados avanc¸adospodem cursarcomnáusea, vômito,sangramento,dorabdominal,massapalpáveleatéobstruc¸ãointestinal.Oúnico tratamento efetivo é a ressecc¸ão completa do tumor. Relatamos dois casos de GIST

夽

StudylinkedtotheDepartmentofGeneralSurgery,HospitalSãoLucas,FaculdadeAssisGurgacz(FAG),Cascavel,PR,Brazil.

∗ _{Correspondingauthor.}

E-mail:raphaelratin@hotmail.com(R.F.Ratin).

http://dx.doi.org/10.1016/j.jcol.2015.03.003

(25–35%),colonandrectum(5–10%),andesophagus(<5%). With an incidence estimated at 10–20 cases/10 million inhabitants,4 _{GIST represents about 0.6% of all malignant}

rectaltumors,5_{comprisingonly1–3%ofallcancersofthe}

gas-trointestinaltract.Itsrelevancestemsfromthefactthatthey represent80%ofallprimarytumorsofthegastrointestinal tract.6,7 _{Thelargestnumberofcasesoccursinolderadults,}

especiallybetweenthefourthtosixthdecadeoflife.8_Thereis

noprevalenceingender.8

Clinical manifestations of GIST are related to its loca-tion,whichcanbemultiple,anditsdimensions.9 _Clinically,

theseare asymptomaticlesions,and inanadvanced state, there may be nausea, vomiting, bleeding, abdominal pain, apalpable mass, and even intestinalobstruction.9–11 _Tests

suchasabdominalultrasound,uppergastrointestinal(UGI) endoscopy,colonoscopy,echoendoscopy,computed tomogra-phy(CT)andnuclearmagneticresonance(NMR)areimaging methodsimportantfortheinvestigationofinjury.9_Theonly

effective treatment is surgical resection.10 _Anorectal

ultra-sonography (USG-A) is indicated in benign and malignant anorectal diseases, including anal incontinence, infectious and inflammatory diseases (abscesses, anorectal fistulae), chronicanorectalpain(endometriosis),andinthestagingand follow-upofanorectalneoplasms.12–14_{Thus,USG-Aallowsfor}

theevaluation ofanatomical structuresthatformthe anal canal,rectumandperi-anorectaltissueswithlowcostandin asafemanner,becausethisisaverylittleinvasivetest,being welltoleratedandwhichdoesnotexposepatientsto radia-tion.Thus,USG-Ahasbecomeacriticalimagingmodalityfor clinicaldiagnosis ofGISTanditsdifferentiation fromother submucosalneoplasms.

Fig.1–USG-A3Dshowingahypoechoiclesionintheupperregion.(A)Axialcut;(B)diagonalcut.

Case

report

1

Male patient, 57, industrial worker, previously healthy, attendedtheColoproctologyClinicofGastroclínicaCascavel toperformcolonoscopyaspartofamedicalcheck-up. Dur-ingsymptomatologyquestioning,thepatientdidnotreport complaintsorcomorbidities.Thedigitalrectalexamination revealedanelevated,hardened,well-defined,painlesslesion, fixed on the anterior rectal wall, without rectal mucosa changes.The lesionwas locatedabout 5cm from the anal marginandmeasured4cmindiameter.Thepatient under-wentcolonoscopyandUSG-A(Fig.1).Localtransanalexcision wasindicated.Thepatientwashospitalizedontheprocedure day,andarectalcleaningwithfleetenemawascarriedout2h beforetheprocedure.Thesurgicalprocedurewasuneventfully carried out,evolvingonthefirst postoperativedaywithout complaints;thepatientwasdischargedingoodcondition,and hasbeenfollowed-upatourcoloproctologyoutpatientclinic, beingasymptomaticfor3years.

Case

report

2

Fig.2–(A)Viewofaseptalbulging;(B)USG-3D,sagittalcut;(C)USG-3D,crosscut;(D)USG-3Daxialcut.

anorectal junction (rectovaginal septum) measuring about 2.0cm×2.0cm×2.0cm,withoutpenetrationofrectalmuscle

andwithnolymphnodeevidence(Fig.2B–D).Thenodulewas excisedtransvaginally,andthehistopathologyrevealed mes-enchymalneoplasm,withimmunohistochemistrypositivefor CD117(polyclonal)(Fig.3).

Discussion

Histologically,GIST is characterizedby the proliferation of spindle cells, forming small bundles arranged in multiple directions.15 _{Theimmunohistochemical study is}

character-izedbyCD34markerpositivity,rangingfrom46%to100%and withCD117(c-kit)positivityinallcases.16

It is difficult to predict the degree of malignancy for GIST. Hsu et al.3 _{identified histological features indicating}

malignancy,suchastumorsizeandmitoticactivity.Tumors greater than 5.0cm or withmorethan twomitoses byten

high-powerfields(HPF)indicateincreasedriskofmetastasis orrecurrence.17

Theonlyeffectivetreatmentisbysurgicalresectionofthe tumorwithtumor-free margins.Thelaparoscopicapproach isasatisfactoryoptionintheresectionofsmalltumors;in severalseriesofcasesofgastricGISTresection,thisapproach provedtobesafeandwithlowrecurrencerates.18

InpatientswithlargedistalrectalGIST,an abdominoper-inealamputationwillbeneeded.

Neoadjuvantoradjuvantradiotherapyandchemotherapy doesnotprovidegoodresults.19_{However,imatinibmesylate}

(STI571orGleevec),whichactsasatyrosinekinaseinhibitor, hasbeendescribedwithgoodresultsasmonotherapyforthe treatmentofmetastaticGIST.10,17,20_{Asaneoadjuvantagent,}

imatinibcanproducetumordownsizingandallowfor sphinc-terpreservation.

Tothisend,agooddiagnosticevaluationforchoosingthe appropriate surgical treatment should be obtained. USG-A hasbeenwidelyappliedtoclarifythediagnosisofanorectal

parallelimagingsequence(0.25mm),characterizedbythe for-mationofacube.25,26,30,32,52–54

Themobilizationmechanismmaybemanualorautomatic, dependingonthetypeoftransducerused.Theimageformed intoacubecan berecorded and widelydeployed,enabling theoperatortoacquirealltypesofcuts,eveninmultivision, whichconstitutesinsimultaneousviewingfourandsix spe-cializedimages,besidesthepossibilityofsubsequentrevision oftheseimagesasoftenasdesired.Thissignificantlyimproves theaccuracyofthetest,inadditiontoincreasingtheamount ofinformationobtained.52

IncasesofrectalGIST,thescarceliteraturesuggests per-formingnuclearmagneticresonance54_{sothatonecanattain}

accurate assessments. The ultrasound may be an alterna-tive in this area, but the authors used a low-frequency, two-dimensionalendoscopicprobe,55_{whichresultsina}

low-resolutionimagequality.

Inacomparativestudyofresonanceandtwo-dimensional anorectalultrasonographyintheevaluationofpatientswith endometriosis,thesetwotechniqueswereequivalentinterms ofimagequality.Butastudyrelatedtotheevaluationof peri-anorectal layers and tissues with three-dimensional mode versusMRIhasnotyetbeenpublished.56

DespitethegrowingnumberofpublicationsonGIST,there islittleinformationonitsrectalpresentationandonthe eval-uationwithUSG-A.Thistechnologyhastheabilitytoanalyze thesizeofthetumoranditslocation,extentanddepth,aswell asitsrelationshiptoadjacentstructures,besidesanalyzing lymphnodeinvolvement.

Thisstudy aimstotakeinto accountthe importanceof USG-3Dintheevaluationofanorectalregionand perianorec-taltissues,inordertopredictthebesttreatmenttobechosen in the case of suspicion of an anorectal injury, and more specifically,arectalGIST,becausethismethodallowsagood assessmentoftheaffectedrectallayers,besidesdiagnosingif thereisaninvasionofadjacentorgansandblood/lymphatic vessels.

Conclusion

TheuseofanorectalultrasonographyincasesofrectalGIST hasgreatvalueinidentifyingaffectedrectallayers,lesionsize, andinvolvementofadjacentorgansandofblood/lymphatic vessels,helpingintheguidanceforthebesttreatmenttobe proposed.

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