w w w . j c o l . o r g . b r
Journal
of
Coloproctology
Case
Report
Giant
appendiceal
mucinous
cystadenoma
treated
by
laparoscopy:
a
case
report
and
review
of
the
literature
Felipe
Ramos
Nogueira
a,∗,
Francisco
Wendel
de
Sousa
Arruda
a,
Carla
Camila
Rocha
Bezerra
a,
Benjamin
Ramos
de
Andrade
Neto
a,
Manoel
Italo
Pimentel
Santos
Lopes
a,
Edson
de
Macedo
Sousa
a,
Alessandra
Marques
dos
Santos
b,
Adryano
Gonc¸alves
Marques
c,
Lusmar
Veras
Rodrigues
c,d,e,f,gaUniversidadeFederaldoCeará(UFC),HospitalUniversitárioWalterCantídio(HUWC),Fortaleza,CE,Brazil
bUniversidadeFederaldoCeará(UFC),HospitalUniversitárioWalterCantídio(HUWC),ProgramadeResidênciaMédicaemPatologia,
Fortaleza,CE,Brazil
cUniversidadeFederaldoCeará(UFC),HospitalUniversitárioWalterCantídio(HUWC),ProgramadeResidênciaMédicaem
Coloproctologia,Fortaleza,CE,Brazil
dUniversidadeFederaldeSãoPaulo(UNIFESP),SãoPaulo,SP,Brazil
eUniversidadeFederaldoCeará(UFC),DepartamentodeCirurgia,Fortaleza,CE,Brazil
fUniversidadeFederaldoCeará(UFC),HospitalUniversitárioWalterCantídio(HUWC),Servic¸odeColoproctologia,Fortaleza,CE,Brazil
gUniversidadeFederaldoCeará(UFC),ProgramadePós-Graduac¸ãoemCirurgia,Fortaleza,CE,Brazil
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r
t
i
c
l
e
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n
f
o
Articlehistory:
Received11February2017 Accepted2April2017 Availableonline5May2017
Keywords:
Cystadenoma,mucinous Appendicealneoplasms Mucocele
Pseudomyxomaperitonei
a
b
s
t
r
a
c
t
Appendicealmucinouscystadenomaisarareentity,whichcausesappendicularmucocele. Itismorefrequentinwomenover50yearsold.Inhalfofthecasesitisasymptomatic. Tomographyoftheabdomenisthegoldstandardinitspreoperativediagnosis.The treat-mentissurgical,withgoodprognosis,thecompleteresectionevolveswithoutappendicular ruptureandextravasation.Wereportacaseofa64-year-oldmanwithappendiceal muci-nouscystadenoma.Alaparoscopicrighthemicolectomywasperformed.Thistherapythat canbesafelyusedtotreatappendicealmucocele,aslongasitiscautious.
©2017SociedadeBrasileiradeColoproctologia.PublishedbyElsevierEditoraLtda.This isanopenaccessarticleundertheCCBY-NC-NDlicense(http://creativecommons.org/ licenses/by-nc-nd/4.0/).
∗ Correspondingauthor.
E-mail:felipernogueira@gmail.com(F.R.Nogueira). http://dx.doi.org/10.1016/j.jcol.2017.04.002
Cistadenoma
mucinoso
apendicular
gigante
tratado
por
laparoscopia:
relato
de
caso
e
revisão
de
literatura
Palavras-chave:
Cistadenomamucinoso Neoplasiasapendiculares Mucocele
Pseudomixomaperitoneal
r
e
s
u
m
o
Ocistadenomamucinosoapendicularéentidaderaraquecausamucoceleapendicular, sendomaisfrequenteemmulheresacimados50anos.Emmetadedoscasos,o cistade-nomamucinosoapendicularéassintomático.Atomografiadoabdomeéopadrão-ouro paraum diagnósticopré-operatório. Otratamentoécirúrgicoetembomprognóstico;a ressecc¸ãocompletaevoluisemrupturaapendicularesemextravasamento.Relatamosum casodepacientehomemde64anoscomcistadenomamucinosoapendicular.Foirealizada hemicolectomialaparoscópicadireita.Esseéumprocedimentoquepodeserusadocom seguranc¸anotratamentodemucoceleapendicular,desdequesejaexecutadocomcautela. ©2017SociedadeBrasileiradeColoproctologia.PublicadoporElsevierEditoraLtda.Este ´eumartigoOpenAccesssobumalicenc¸aCCBY-NC-ND(http://creativecommons.org/ licenses/by-nc-nd/4.0/).
Introduction
Appendiceal mucinous cystadenoma is a rare disease, whichcausesappendicealmucocele,anenlargementofthe appendixbymucin.1Inalargenumberofcases,itis
asymp-tomatic, being diagnosed incidentally,2 and may simulate
acuteappendicitis.1Completesurgicalresectionwithout
rup-tureoftheorganhasexcellentprognosis.2 However,incase
ofrupture,itmayevolveintopseudomyxomaperitonei,with poorprognosis.1,2
There is great deal of controversy among surgeons regardingthe useoflaparoscopy asasurgicalapproachfor resectionofmucocele,especiallyofpossibleneoplasticorigin, duetothe riskofperforationand mucin extravasation.1,3,4
However,thereare reportsofsuccessfuluse oflaparoscopy intheresectionofappendicealmucocele,associatedwiththe benefitsinherenttothemethod.5,6
Thus,itisnecessarythatsurgeonshaveknowledgeofthis method,foranappropriateperformance,providinggood evo-lutionandpreventingpseudomyxomaperitoneioccurrence.
Asymptomaticcaseisreported,manifestedbyabdominal pain,explosive diarrheaand hematochezia,unusual symp-tomsforthepathologyinquestion.Subsequently,diagnostic, histopathological,therapeuticandprognosticaspectsare dis-cussed.
Case
report
A 64-year-old male, a farmer, with moderate nonspecific abdominalpain,explosive andwatery diarrhea,and tenes-musfor4months,associatedwithanepisodeofhematochezia throughoutthis period. Thereare nocomorbidities, family history of colorectal cancer, allergies or use of medica-tions.Physicalexaminationshowedgoodgeneralcondition, no signs of anemia. Asymmetric abdomen, bulging in the right quadrants, with a large painless palpable mass, dull soundon percussion,extendingfrom the iliacfossa tothe right hypochondrium. Noevidence ofperitoneal irritation. Colonoscopyrevealed a subepithelialtumor atthe level of
theappendicealostium,displacingit,measuring8cminits largestdiameter,whosehistopathologicalevaluationrevealed onlynonspecificcolitis.Thecomputedtomographyshoweda thin-walled, vermiformappendixfilledwiththickcontents, measuring15cmonitslargestaxis,withanterosuperior dis-placementofthececum,withoutsignsofperforation(Fig.1). Appendicealmucocelewasdiagnosed.Thecolorectalsurgery group atthe Hospital Universitário Walter Cantídiohas as oneofitsstrengthstheexperienceinvideolaparoscopy,being thismodalitythegoldstandardforthetreatmentof colorec-tal diseases.Therefore,alaparoscopicrighthemicolectomy wasperformedinthiscase.Surgerywasperformedfollowing exactlythe samestepsasaregular videolaparoscopicright colectomy,withaninitialmedialapproachfollowedbylateral dissection.Tumorsizedidnotchangetheoperativetactic.The onlyadditionalcareisthedelicacyofthemovements,inorder nottoleakthecystadenoma.Thesurgicalpiecewasremoved by transumbilical incision. The histopathologicaldiagnosis was appendiceal mucinous cystadenoma,measuring10cm initslargestdiameter,withtumor-freemargin(Fig.2).There wassuperficialinfectionofthesurgicalwoundonthethird postoperativeday,treatedwithpartialopeninganddrainage of the surgicalwound. Thepatient was discharged on the eighthpostoperativeday.Thisminorintercurrentpostponed thehospitaldischarge.However,thisfactdidnotnegateallthe
Fig.1–Voluminouscecalappendixrevealedby
Mucin
Columnar epithelium
Fig.2–Histologyofthelesion,showingmucincoveredbymucussecretingcolumnarepithelium.
advantagesofvideolaparoscopicsurgery.Sevenmonthslater, hewasasymptomatic.
Discussion
Appendicealmucinouscystadenomaisabenignappendiceal neoplasmofsimpleepithelium,mucus-secreting, with sev-eraldegreesofdysplasia,and accumulationofappendiceal luminalmucin,theappendicealmucocele.1,2 Thistermwas
created by Fere in 1877. However, the pathological entity was first recognized by Rokitansky in 1842 and described by Virchow in 1863.7,8 In association with appendiceal
epithelial hyperplasia, appendiceal mucinous cystadenoma corresponds to 31–34% of the mucoceles of the appendix, rareentitiesthatcontributewith0.2–0.7%oftheappendiceal pathologies.1 Thus, appendiceal mucinous cystadenoma is
notdiscussedindividually,butaspartofalargerentity,the appendicealmucocele.
Appendicealmucoceleismorefrequentinwomenover50 yearsold.3
Theclinical picture ofthe appendiceal mucocele is not specific.About50%ofthecasesareasymptomatic,with inci-dentaldiagnosisbyendoscopic,imagingorsurgicalfindings. Insymptomaticcases,abdominalpainisthemostcommon symptom(80%ofpatients),asinthecaseofthe patientin question.Furtherfindingsincludepalpablemass,whichwas foundonexaminationofthepatient;nausea,vomiting,weight loss, lower intestinal hemorrhage, peritonism or intestinal occlusivedisease.1,3,4
Preoperative diagnosis is difficult. However, it is of greatimportance forthe selection ofanadequate surgical approach.9
Thecomplementaryexamshelp inthediagnostic eluci-dationandtreatmentplanning.Ultrasoundoftheabdomen candifferentiateacuteappendicitisfrommucocele,in addi-tiontofacilitatingtheidentificationofappendicealmassand visualizingthe ovary inwomen. It shows anencapsulated cysticlesion,firmlyattachedtothececum,withliquid con-tentofvariableechogenicity,accordingtothedensityofthe mucus.Computedtomographyisthegoldstandard.Carried out in the investigation ofthe reported case,it shows the extentofthediseaseandmayidentifyassociated complica-tions.Itshowsacylindrical,encapsulated,thin-walledmass
communicating with the cecum,2,4,10 exactly as reported
(Fig.3).Itisnotusefulindifferentiatingbetweenbenignand malignant mucocele.11 Colonoscopymay show a soft,
ery-thematouselevation,containingtheappendicealostium,and theremay beayellowishmucoiddischargeknownas “vol-canosign”,whichispathognomonic.4,10Inthecasereported,
onlyasubepithelialelevationwasobservedatthelevelofthe appendicealostium,withoutdischarge.
TheCEAandCA19-9tumormarkersareassociatedwith appendicealneoplasticprocessesandshouldbeincludedin thediagnosticinvestigation.1
Due to the difficulty in distinguishing from cystadeno-carcinoma,andthepotentialcomplicationofrupture(about 20%) and evolutioninto pseudomyxoma peritonei, the rec-ommended treatmentforcystadenomaissurgical,1,3,4 with
removal ofthe piece without curative rupture of mucoce-lesofbenignetiology.12,13Adequatepreoperativeevaluation
and careful intraoperative dissection are very important in preventing rupture and malignant transformation that can be associated with mucoceles of malignant etiology.3
Itis believed that the open procedure is the most recom-mendedbecauseitreducesthechanceofruptureandmucin extravasation.1–4However,theuseoflaparoscopymayallow
abetterevaluationoftheintraoperativelesionandassistin thesurgicalplanning.Sincesurgeonswithexperiencein min-imally invasive surgery are available, laparoscopic-assisted righthemicolectomycanbesafelyperformedformucinous cystadenomaorcystadenocarcinomaoftheappendixto min-imize the unnecessary complication of peritoneal mucin leakage,whichmayoccurduringtheconventionalor laparo-scopicmethod.5Inaddition,patientsbenefitfromaminimally
invasivesurgery,includingaminorwoundandshorterperiod ofconvalescence.5,6,12,14Furthermore,therearereportsofuse
ofasafetechniqueinthelaparoscopicsurgicaltreatmentof appendicealmucocelewithasingleaccesstotheperitoneal cavity.15,16Asasafetyrecommendationforlaparoscopic
resec-tions,thesurgicalpieceshouldberemovedinendobag.9,17–19
Analgorithmforchoosingthetypeofsurgeryhasbeen createdbyDhage-IvaturyandSugarbaker.20Itprovidesfor
sev-eralfactors:whetheramucoceleisperforatedornot;whether the base of the appendix (resection margins) is involved in the process; and if there are positive lymph nodes in themesoappendixand ileocolicchain.Asaresult,patients may require different surgeries: appendectomy to right colectomy, including cytoreductive surgery, intraoperative intraperitoneal hyperthermic chemotherapy or immediate postoperative intraperitoneal hyperthermic chemotherapy. Cecectomyand right hemicolectomyare recommended for appendicesinwhich adequatesurgicalmargins cannotbe obtained(broadbaseandprojectioninthececallumen).6,12
Thehistopathologyshowspureorglandularvilloustissue, withepitheliumshowingmild,moderateorseveredysplasia, identifyingitselfwithtubularortubulovillousadenomasof thecolon.Theproductionofmucinisprominentandcanlead toflatteningoftheepithelium.13
Theprognosisisexcellentwhenthereiscompleteresection withoutrupture(100%survivalin5years).2Withruptureand
extravasationofmucinincavity,itcanevolveto pseudomyx-omaperitoneiin2%ofthecasesofcystadenomas,1 witha
5-yearsurvivalofonly20–25%.11Althoughthereisthe
afore-mentionedstatistic,inapreviousstudyonhistopathologyof themucoceleassociatedwiththeappendicealcystadenoma, patientsin which therewere rupture and extravasationof periappendicealmucin,itdidnotshowepithelialcellsandthe patient’sfollowupdidnotidentifyevolutioninto pseudomyx-omaperitonei,suggestingabenignpathologicalcondition.13
Inabout20%ofthecases,appendicealmucinous cystade-nomamaybeassociatedwithsynchronousormetachronous coloncancer.3
Conclusion
Appendiceal mucinous cystadenoma is a rare entity, more frequentinwomenover50yearsold.Itisasymptomaticin
halfofthecases,opensurgicaltherapyisthemostaccepted approach.However,throughmeticulousdissectionandextra caretothemanipulationandremovalofthepiece,the laparo-scopicsurgerycanandhasbeenused,withalowerindexof postoperativemorbidity,earlyrecoveryandimprovementof abdominalestheticsassociatedwiththesurgicalincision.
Conflict
of
interest
Theauthorsdeclarenoconflictofinterest.
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