A multidisciplinary approach to desmoid tumors. When intra-abdominal fibromatosis degenerates into an abscess, which is the right treatment?

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ContentslistsavailableatSciVerseScienceDirect

International

Journal

of

Surgery

Case

Reports

j o u r n al ho me p a g e : w w w . e l s e v i e r . c o m / l o c a t e / i j s c r

A

multidisciplinary

approach

to

desmoid

tumors.

When

intra-abdominal

fibromatosis

degenerates

into

an

abscess,

which

is

the

right

treatment?

夽

Giovanni

Alemanno

,

Daniela

Zambonin,

Alessandro

Sturiale,

Tiziana

Cavalli,

Francesco

Bellucci,

Benedetta

Pesi,

Carmela

Di

Martino,

Francesco

Giudici,

Francesco

Tonelli

DigestiveSurgeryUnit,DepartmentofClinicalPhysiopathology,UniversityofFlorenceMedicalSchool,CareggiUniversityHospital,Florence,Italy

a

r

t

i

c

l

e

i

n

f

o

Articlehistory:

Received17January2013

Receivedinrevisedform20April2013 Accepted9May2013

Available online 15 June 2013

Keywords: Desmoidtumor

familialadenomatouspolyposis

a

b

s

t

r

a

c

t

INTRODUCTION:Desmoidtumorsarerarebenigntumorsthatoriginatesinthefibroussheathor musculo-aponeuroticstructure.Histologicallybenign,theytendstoinvadelocallyandtoberecurrent.

PRESENTATIONOFCASE:Wereportararecaseofanintra-abdominaldesmoidtumorinapatientaffected byfamilialadenomatouspolyposis,whichdegeneratedintoabscess.Male,38years,washospitalizedfor abdominalpain,bowelobstructionandfever.Thecomputedtomographyshowedabigdishomogeneous massoccupyingthewholemesenterywithinternalmassiveliquefaction.Themassextendedfromthe epigastriumfor13cmuptoL3.Ontherightmesogastricsideasolid,thickmassofabout2cm,witha lengthof4.5cm,wasidentified;itwasnotcleavablefromthewallandfromsomeoftheloops.Wedecided toperformacomputedtomography-guidedpercutaneousdrainage.Twohundredmlofpurulentnecrotic materialwasaspirated,andwashingwithantibioticsolutionwascarriedout.Cytologicalexamination offluiddrainageshowedhistiocytesandneutrophils.Atfollow-up,thepatient’sclinicalconditionhad improved.Anabdominalultrasoundshowedasubstantialreductioninthediameterofthemass. DISCUSSION:Diagnosisandtreatmentofdesmoidstumorinpatientswithfamilialadenomatouspolyposis maybedifficult,especiallywhendesmoidsarelocatedintra-abdominallyandinthemesentery.Seldom willdesmoidtumorsbecomplicatedbyabscessformation.

CONCLUSION:Themanagementofdesmoidstumorsisnoteasyandthechoiceofthebesttreatmentmay bedifficultduetothedifferentpossibleanatomicalpresentations.

©2013 Surgical Associates Ltd. Published by Elsevier Ltd.

1. Introduction

Desmoidtumors(DTs)arerarebenigntumorsoriginatingfrom theproliferationofwelldifferentiatedfibroblast.Theyarebenign withnometastaticcapacity,yetlocallyaggressive.Theytendto invadelocally andtohave recurrencesfollowing localexcision; hence,may beconsidered asmalignant. Sporadicor associated withFamilialAdenomatousPolyposis(FAP),DTshavea multifac-torialetiology.InFAPpatients,theyareassociatedwithgermline

Abbreviations:DT,desmoidtumor;FAP,FamilialAdenomatousPolyposis;APC, AdenomatousPolyposisColi;CT,computedtomography;MRI,Magneticresonance imaging.

夽 Authorsdeclarethatonlytheabstractofthisclinicalcasewaspresentedtothe 35thSICO(ItalianSocietyofSurgicalOncology)NationalCongress(14June2012) andthereforepublishedintheAbstractssectionoftheEuropeanJournalofSurgical Oncology.

∗ Correspondingauthorat:CareggiHospital,DigestiveSurgeryUnit,Largo Bram-billa3,50134Florence,Italy.Tel.:+390557947449;fax:+390557947449.

E-mailaddress:[email protected](G.Alemanno).

mutationsbeyondcodon1444oftheAPC(AdenomatousPolyposis Coli)gene and theirappearance is more frequent after laparo-tomy.

Desmoid tumors can grow intra-abdominally, extra-abdominally or within the abdominal wall. Desmoid tumors localizedin theabdomenhavea worseprognosis,as theymay causeintestinalobstruction,ureterobstructionwithhydronefrosis andmayalsoinvademajorvessels.1_{Degenerationintoanabscess} isararecondition.

Thepeculiaraspectofthiscaseistheabscessformationonan intra-abdominaldesmoidtumor.Inliterature,fewcasesofDTs pre-sentedasanabscessarereported,andthetreatmentofchoicefor thesetumorsisnotyetclearlydefined.

2. Presentationofcase

Male,38.At31hewasdiagnosedFAPaftercolonoscopy(father diedat33ofcoloncancer).Thepatientunderwentsurgery:atotal colectomy,anileo-rectalanastomosisandatemporaryileostomy

2210-2612© 2013 Surgical Associates Ltd. Published by Elsevier Ltd.

http://dx.doi.org/10.1016/j.ijscr.2013.05.015

Open access under CC BY-NC-ND license.

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758 G.Alemannoetal./InternationalJournalofSurgeryCaseReports4 (2013) 757–760

Fig.1.Abdominalcomputedtomographyshowedalargemesentericmass,

extend-ing13cmfromtheepigastriumtoL3,withinternalmassiveliquefaction.

wereperformed.After3monthheunderwentsurgeryforthe

clo-sureofthestoma.Twelvemonthsaftersurgery,inatimeofgood

healthconditions,therewasanonsetofabdominalswellingnear

thesurgicalscarclosureofthestoma.Computedtomography(CT)

showedthickeningoftherootofthemesentery.A15-cm solid

anddishomogeneous massontherightwallofabdominal

cav-ityappeared,stretchinguntothepelvis,overthebladderandthe

rightparacolicsite.Exploratorylaparotomyshowedthemassto

becomposedby2 separateunits:thefirstoccupyingtheentire

wallfromtherighthip,undertheskin,totheperitoneum;the

secondextendedthroughoutthemesenteryfromTreitzligament

tothepelvis,andwassurroundedbysatellitenodules.Following

anatomicalconsideration,wedecidedtoperformaresectionon

themasswall(9cm×7cm×5.5cm),leavingthemesentericmass

inplace,asitwasimpossibletopreservethevascularityofthe

bowel,andbecauseoftheconsiderablenumberofnodules.

Histo-logicalexaminationwaspositiveforabdominalfibromatosis.After

surgeryamedicaltherapywasadministered,withRaloxifenefor

3months.TheabdominalcontrolCTshowednochangesinthe

diameteroftheabdominalmass;hence,thepatientwastreated

withchemotherapy: Dacarbazine 150mg/m2_{/day,7cyclesfor a}

totalof6months.AfterchemotherapythecontrolCTshoweda

reductioninthediameteroftheintra-abdominalmass(thepelvic

region:3cm versus5cm;thecranialpart:6cmversus 7.5cm),

withamoredishomogeneousaspectlikelytooverlapnecroticand

regressiveprocesses.ThepatientbegantherapywithTamoxifen

40mgandMeloxicam7.5mgandwasfollowed-upfor3years

per-formingregularexaminationsandcontrolX-rayseverysixmonths.

Throughoutthattime,thesizeofthemassremainedunchanged.

Threemonthago,hewashospitalizedforabdominalpain,bowel

obstructionandfever.AnabdominalX-rayshowedaslight

over-distensionoftheilealloopsandsmallair–fluidlevels.TheCTscan

showedabigdishomogeneousmassoccupingthewhole

mesen-terywithinternalmassiveliquefaction,andhyperemicandblurred

bowel walls (Fig.1). The mass extended fromthe epigastrium

for13cmup toL3.On therightmesogastricside a solid,thick massof about 2cm, with a lengthof 4.5cm, wasidentified; it

Fig. 2.Magnetic resonanceimaging performed after CT-guided percutaneous drainage.Theabdominalmasshadasmallerdiameter(2.5cm).

was not cleavable from the wall and fromsome of theloops. Consequently, the safest procedure was a CT- guided percuta-neousdrainage.Twohundredmlofpurulentnecroticmaterialwas aspirated,andwashingwithantibioticsolutionwascarriedout. Cytologicalexaminationoffluiddrainageshowedhistiocytesand neutrophils.Atfollow-up,thepatient’sclinicalconditionimproved, and obstruction symptoms and fever regressed. An abdominal ultrasoundshowedasubstantialreductioninthediameterofthe mass (2.5cm), subsequently confirmed by the MRI (Fig. 2).At dischargethe patientcontinuedtreatmentwith Tamoxifenand Meloxicam.

3. Discussion

Theetiology ofDTs isstill unclear.Extra-abdominalDTs are more frequent on the shoulders, the upper limbs and in the glutealregion;intra-abdominalDTsarelocated,in85%ofcases, in the mesentery.2,3 _{Consequently, they would be responsible} forincreasingmorbidityand mortalityratesinpatientsaffected byFAP.4,5 _{Mesentericdesmoids originateaswhite plaques and} progresstoformalobulatedmassofconsiderablesize.6_Theabscess formation in desmoid tumors is a rare finding. Different inter-pretationsofthisphenomenonhavebeenprovidedinliterature, andtheetiopathogeneticprocessisnotyetcompletelyclear.Some researchershave,however,hypothesizedthatsurgical manipula-tionmaycontributetotheformationofanabscessduetotransient bacteremia.Moreover,desmoidtumorscompressingthevascular structuresmayleadtointestinalischemia,withconsequent bac-terial transposition.Venous and lymphatic drainageof bacteria withinintra-mesenteryDTs,resultinginabscessformation,7_may beanotherpossibletriggeringfactor.

Only few reports of intra-abdominal DTs degenerated into abscesswerereportedinliterature,asshown inTable1.7–10 _In themajority of casesthefirst approach toDTs complicated by abscessformationconsistedofapercutaneousdrainageplus antibi-otictherapy.Asasecondapproach,duetopersistentsymptoms

Table1

SummaryofreportsofDTscomplicatedbyabscessformation.

Report Year No.ofpatients FAPrelated 1sttreatment(no.ofpatients) 2ndtreatment(no.ofpatients)

Maldjianetal.7 _{1995 3 3 Percutaneousdrainage(3)+antibiotics(3) Surgicalresection(2)}

Cholongitasetal.9 _{2006 1 None Percutaneousdrainage(1)+antibiotics(1) Surgicalresection(1)}

Ebrahimi-Daryanietal.10 _{2008 1 None Antibiotics(1) Surgicalresection(1)}

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Fig.3.Intraoperativepictureofadesmoidtumoroftheabdominalwall.

related to DTs, a surgical resection of the tumor was

per-formed.

Diagnosis and treatment of DTs might be difficult, in the

majorityofcases.Toperformadifferentialdiagnosiswithother

neoplasmssuchaslymphoma,pleomorphicsarcomaand

fibrosar-coma, an histopathological examination is mandatory before

treatment.Imaging examinationis important todeterminethe

extensionandtheanatomicalrelationshipofDTsandthepossible

resectability. From a clinical point of view DTs are

character-izedbydifferentphasesofgrowthandprogression,stabilization

and/or regression. The management of DTs is not easy and

the choice of the best treatment may be difficult due to the

different possibleanatomical presentations. Thereare different

treatmentoptionsdependingonthelocation,theinvasionof

adja-centstructuresandanatomicconsiderations.Surgicalexcisionis

recommended for DTs located in the abdominal wall (Fig. 3).

For symptomatic, small, well-defined, no-invasiveDTs, surgical resection may be considered as a first treatment.11 _{If DTs are} unresectable,non-surgicaltreatmentoptionssuchasradiotherapy, systemicchemotherapy,non-steroidalanti-inflammatoryagents, anti-estrogen hormonal therapy have proven to be effective.3,6 As suggested by NCCN guidelines, a post-treatment surveil-lance is mandatory, with a clinical and imaging examination every 3–6 months for 2–3 years and subsequently, once a year.12

4. Conclusion

The treatment of desmoid tumors is still controversial and there are nocontrolled trials in literature.A surgical approach could be performed if the DT is resectable; radiotherapy if the DT is localized and accessible. Radiation and surgery are impracticalforintra-mesenteryDTs;hence,firstlinetreatmentis non-steroidalanti-inflammatorydrugsand/oraselectiveestrogen receptor modulator.13 _{Systemic chemotherapy is to be} pre-ferred only when DTs are not responsive to less aggressive therapies.14

Conflictofinterest

Authorscertifythatthereisnoactualorpotentialconflictof interestinrelationtothisarticleandtheystatethatthereareno financialinterestsorconnections,directorindirect,orother situa-tionsthatmightraisethequestionofbiasintheworkreportedor theconclusions,implications,oropinionsstated–including per-tinentcommercialorothersourcesoffundingfortheindividual

author(s)orfortheassociateddepartment(s)ororganization(s), personalrelationships,ordirectacademiccompetition.

Funding

None.

Consent

Writteninformedconsentwasobtainedfromthepatientfor publicationofthiscasereportandaccompanyingimages.Acopy ofthewrittenconsentisavailableforreviewbytheEditor-in-Chief ofthisjournalonrequest.

Authorcontributions

Allauthorscontributedtothiswork:G.A.,D.Z.,T.C.collectedthe data,G.A.,T.C.,D.Z.,C.D.M.,A.S.,F.B,F.G,B.P.analyzeddata,G.A., D.Z.andF.T.wrotethemanuscript,F.T.obtainedinformedconsent andsupervisedallthemanuscript.

Acknowledgement

Prof.MariaRosariaBuri,ProfessionalTranslator/AiicConference Interpreter,UniversityofSalento,forEnglishlanguageediting.

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