A novel surgical approach with peritonectomy to extranodal multisystemic histiocytic sarcoma: A case report and literature review

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InternationalJournalofSurgeryCaseReports59(2019)213–216

Contents lists available atScienceDirect

International

Journal

of

Surgery

Case

Reports

j o u r n a l h o m e p a g e :w w w . c a s e r e p o r t s . c o m

A

novel

surgical

approach

with

peritonectomy

to

extranodal

multisystemic

histiocytic

sarcoma:

A

case

report

and

literature

review

夽

Giuseppe

Barbato,

Alessandro

Tarantini,

Francesco

Serra,

Francesca

Cabry,

Alberto

Farinetti,

Lorena

Sorrentino,

Anna

Vittoria

Mattioli,

Roberta

Gelmini

∗

DepartmentofSurgery,UniversityofModenaandReggioEmilia–PoliclinicoofModena,ViadelPozzo,7141100,Modena,Italy

a

r

t

i

c

l

e

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n

f

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Articlehistory:

Received30January2019

Receivedinrevisedform22April2019 Accepted12May2019

Availableonline22May2019 Keywords:

Histiocyticsarcoma Peritonectomy Peritonealmetastases

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INTRODUCTION:Histiocyticsarcoma(HS)isaveryraremalignanthistiocyticderivationcancer.The extra-nodalmultisystemicHShasanaggressiveclinicalcourseandpoorDisease-FreeSurvival(DFS)andOverall Survival(OS).Therearenosharedandeffectivetherapeuticprotocols;ourapproachaimstoimprovethe prognosisforadvanceddiseases.

PRESENTATIONOFTHECASE:53-year-oldfemalepatientadmittedtohospitalforintestinalobstructionin April2016withevidenceofanilealmassintherightiliacfossaandperitonealmetastases,undergoing urgentsurgeryofilealresection,ileostomyandnodularperitonealmassbiopsy.Histologicalexamination diagnosedHS.Stagingexamsexcludedtheinvolvementofothergastro-intestinaldistrictsintheabsence ofconcomitantlymphoproliferativedisorders,andPET-CTrevealedmultipleabdominalandmediastinal nodeshyperplasia.Thepatientperformedchemotherapyand,seeingthepartialabdominalextranodal response,wasperformedcytoreductivesurgery(CRS).Histologicalexaminationconﬁrmedthediagnosis ofHSwithmultipleperitoneal,ileal,colicandomentallocalisation.Adjuvantsecond-line chemother-apytreatment(4DHAPcycles)wasperformed.DiseaseFreeSurvivalattenmonthsfromcytoreductive surgery,OverallSurvivalat21monthsfromdiagnosis.

DISCUSSION:Despiteamultimodaltherapywithsurgeryandchemotherapy,extranodalmultisystemic HShasapoorprognosis.Uptonowtheroleofsurgeryislimitedtobiopsiesorcomplicationstreatment. OurresultsofDFSandOSshowthatcytoreductivesurgerymaybeavalidtherapeuticchoice.

CONCLUSION:Thesurgicalapproachwithmajorcytoreductivepurposescouldimprovetheprognosisin caseswithprevalentabdominalextranodallocalisation.

1. Introduction

Histiocyticsarcoma (HS) isa very rare malignant neoplastic

diseasewithonlyafewhundredcasesreportedintheliterature.

AccordingtodatafromtheSEERdatabaseof U.S.National

Can-cerInstitute,HSismorecommoninadultswithamedianageof

63yearsdespitehavinganextremelywiderange(from18to96

years)withaslightincidenceinfavourofmales(1.5:1)[1,2].The

pathogenesisis unclear,nopredisposinghereditaryor

environ-夽 TheworkwaswritteninlinewiththeSCAREcriteria[1].

∗ Correspondingauthorat:Dept.ofSurgery–PoliclinicoofModena,Universityof ModenaandReggioEmilia,ViadelPozzo,7141124,Modena,Italy.

E-mailaddresses:gpp.barbato@gmail.com(G.Barbato),

alessandrotarantini@hotmail.it(A.Tarantini),serrafrancescomd@gmail.com

(F.Serra),francesca.cabry@unimore.it(F.Cabry),farinetti@unimore.it(A.Farinetti),

lorena.sorrentino@live.it(L.Sorrentino),Annavittoria.mattioli@unimore.it

(A.V.Mattioli),roberta.gelmini@unimore.it(R.Gelmini).

mentalfactorsareknown.Unliketheothersarcomas,theoriginis

frommicrocytic-macrophagesystemcellsexpressing

immunophe-notypical andmorphologicalcharacters ofhistiocyticderivation

[3–5].

The clinicopathological manifestation could be as primitive

extranodal neoplastic disease[6] or associated with malignant

haematological disorders suchas follicular lymphoma or acute

lymphoblasticleukaemia[5,7].Thediagnosisisbasedon

histolog-icalexaminationandimmunohistochemicalcharacterisation[3,8].

Theclinicalpresentationisfrequentlyasymptomatic,with

inciden-taldiagnosisduringradiologicalinvestigations;itmayoccurwith

astheniaorwithsymptomatologycorrelatedtoinvolved

surround-ingorgans,themostfrequentlocalisationsaresmallintestine,skin

andsofttissues.Themostcommonsymptomsonsetisthe

appear-anceofapalpablemasswithassociatedcompressivesymptomsor

systemiccomplaintssuchasweightlossorfever[3].TheHShas

anaggressiveclinicalcoursemainlyinmultisystemicdisease[6].

BecauseofHSlow incidenceandprevalence,in literature,there

https://doi.org/10.1016/j.ijscr.2019.05.027

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214 G.Barbatoetal./InternationalJournalofSurgeryCaseReports59(2019)213–216

Fig.1. CT-PETpreadjuvantchemotherapy.

arenoprospectivestudies.Theavailabledatacamefromsingle casereportsorsmallcaseseriesthatdonotprovidetoelaborate onawidelysharedmanagement.Cytoreductivesurgeryhasshown promisingresultsinthetreatmentofadvancedmultifocal malig-nancyandperitonealmetastasis[9–12].

Uptonow,theroleofsurgeryisalmostconﬁnedtobiopsies

orcomplicationstreatment.OurresultsofDFSandOSshowthat

cytoreductivesurgerymaybeavalidtherapeuticchoicefor

improv-ingtheprognosisofadvancedextranodalabdominalHS.

2. Presentationofthecase

53-year-oldfemalepatient witha history oflatent

tubercu-losisinfectioninprophylactictreatmentwithisoniazid,previous

surgery of tonsillectomy, appendectomy and cholecystectomy.

Familyhistory negative for neoplastic diseases. Hospitalised at

anotherinstituteinApril2016withclinicalmanifestationof

intesti-nalobstruction with evidence for CT scan of a solid occluding

massofadistalileusof5cmmaximumdiameterlocalisedinthe

rightiliacfossa.Multiplenodularneoformationsattheperitoneal

level,thelargestof2cmmaximumdiameterwithassociatedfree

ﬂuidintheabdomenandmultiplelymphadenopathiesoftheileal

mesenteryandsomeenlargedlymphnodesoftherightanterior

heart-phrenicangle.Thelatestwastheuniqueﬁndingof

extra-abdominaldiseasespread.Sothepatientunderwentsurgeryfor

ilealresectionandileostomy,withaperitonealnodulebiopsy.

Thedeﬁnitivehistologicalexaminationoftheilealmass(free

surgicalresectionmargins)characterizationshowedpositive

reac-tionsforhistiocyticmarkers(CD163andCD68);negativereactions

forlymphoidmarkers(CD45/LCA,CD20/L26,CD79a,CD3,CD2,

CD5,CD7,CD8,CD56,CD30/ BERH2and ALK1)and

myeloper-oxidase.The reaction for cNPM is negative. Negativereactions

for cytokeratins (MoAB MNF116, CK8,18, AE1+AE3), desmin,

caldesmon,MLactin,CD34,CD117,DOG1,S100protein,melanA,

CD21,CD1a.Cytoproliferativeactivity(MIB1-LI)in15–20%ofcells.

Biopsyhistological examination onthemajor of theperitoneal

nodularityconﬁrms thediagnosisof HS peritoneallocalisation.

Thepatientevaluationwasperformedwith

Esophagogastroduo-denoscopy and Colonoscopy that excluded the involvement of

other gastrointestinal tracts, bone marrow biopsy to conﬁrm

the absence of concomitant lymphoproliferative diseases.

PET-CT showed multiple uptakes at the ileal mesentery, right and

leftcolon, lymph nodeuptake alsoat thethoracic level at the

phrenicheartangle,aﬁndingalreadyknowninpreviousexams.

ThepatientthenperformedsixcyclesofCHOEPchemotherapy.

Afteradjuvanttherapy,thePET-CTdescribedapartialresponseto

treatmentwithregressionofuptakeareasatthoracicleveland

per-sistenceofabdominaluptakeofalreadyknowslocalisations(Fig.1).

Afteramultidisciplinaryevaluation,wasindicatedtheCRS:atthe

explorationoftheabdominalcavitywasestablishedthedisease

recurrencefrom15cm toileostomy,inthepelvisandalongthe

leftcolonicmesenteryandparietalperitoneumuntilhomolateral

hypochondrium,nearthespleen.Sothedecision itwasto

per-forman ileal en-blocresectionof theprevious ileostomy,total

colectomy, hysterectomy and bilateral oophorectomy, bilateral

obturator lymphadenectomy, omentectomy, splenectomy,

peri-tonectomy of all four abdominal quadrants removing multiple

peritonealnodulesoftheilealmesentery.Thespleenwasremoved

toguaranteetheradicalityofsurgeryconsideringthepresenceof

nodularlesionsonthediaphragmaticperitonealsurfacewherethe

spleenleans.PeritonealCancerindexwas13;attheendofthe

procedure,CytoreductionscorewasCC-0.

Post-operativecoursewascharacterisedbyclosemonitoringin

ICUuptothe3rdpostoperativedayandthentransferredtoour

ordinaryward,theonsetofinfectiouspneumoniaresolvedwith

antibiotictherapy.Dischargeofthepatientathomeonthe20th

postoperativeday.Histologicalexaminationconﬁrmedthe

diag-nosis ofHS withilealrelapse, multipleperitoneal, smallbowel

mesentery,colicandomentallocalisation,negativeforHS

localisa-tiontheothertissuesremoved.ThepostoperativeCTscanshowed

acompleteresectionofabdominalHSlocalisationsuchasCT-scan

atthreeandsixmounths-follow-up.Duringthesameperiodthe

patientunderwent a second-linechemotherapy treatmentwith

fourcyclesaccordingtoDHAPscheme(Dexamethasone,Cisplatin,

Aracytin).ThesubsequentCT-scanperformedattenmonthsfrom

cytoreductivesurgery (17monthsfromdiagnosis)highlighteda

diseaserelapsewithmultipleabdominalandthoraciclocalisations.

Exitusat21monthsafterdiagnosisduetoprogressivedecayofthe

generalperformancestatus.

3. Discussion

The mainfeature of this case is the relatively long

disease-freesurvivalofthepatientobtainedwithaninnovativesurgical

approach. HS is an extremely aggressive neoplasticdisease for

which,asasecond-linetreatmentafterpartialresponsetoadjuvant

chemotherapy,itwaschosen,aftermultidisciplinarydiscussion,

tosurgicalcytoreductivetreatment.Thereisnostandardise

ther-apyforHS,therarityandconsequently,thesmallnessdatapresent

inliterature doesnot allowtheformulationof awidely shared

diagnosticandtherapeuticmanagement.Afterpathologic

evalua-tionwiththeexclusionofotherpathologiesofhistiocyticaetiology

byimmunohistochemistry,forthepatientwassuggested

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G.Barbatoetal./InternationalJournalofSurgeryCaseReports59(2019)213–216 215

radicalaspossible,soitwasnecessarytomakethetotalcolectomy, theoophorectomyandthesplenectomy.Thespleenwasremoved toguaranteetheradicalityofsurgery.Inliterature,dataonOSand DFSforHSwithmultisystemicabdominallocalisationarefewand fragmented.Pilericonductedoneofthemajorreviewsin2002[5]

whichanalyses61cases.Selectingamongthecasesinthereview

wehavefoundfourpatients(6.5%ofanalysedHS)with

dissemi-natedextranodalHS(cfr:Casesnumber7-8-10-16withstage>III)

alltreatedwithchemotherapyalone;noneofthesereachesthe

ﬁrstfollow-upstepforDOD(deathduetothedisease).Another

importantstudy[13] analyseshistiocytesordendriticcells

sar-comatreatedatMemorialSloanKetteringCancerCenterbetween

1995and2014.Fromthestudyarisenonlyfourcaseswith

dissem-inatedextranodalHSandtherearenotstratiﬁedenoughdatatobe

abletoextrapolateusefulcasesforcomparison.Detailedliterature

researchdidnotidentifycasereportsorotherstudiesthatshow

correspondencewiththeillustratedcase.

HSisanextremelyaggressiveneoplasticdiseaseforwhichinour

centre,asasecond-linetreatmentafterpartialresponsetoadjuvant

chemotherapy,itwaschosen,aftermultidisciplinarydiscussion,

tosurgicalcytoreductivetreatmentanoticeableresultwhen

com-paredtothepoorprognosisofmultisystemextranodalcases.

ThelimitedknowledgeoftheHSnaturalhistorymakesevery

reportprecious.Developmentofclinicaltrialsthatallowthe

elab-orationof a widelyshared therapeutic,diagnostic procedureis

needed.In suchrare diseases,randomised or prospective

stud-iesarehardtoachieve,andcasereportswillcontinuetobethe

foundationmanagementofthesepatients.

4. Conclusion

Proper integration between oncologists and haematologists,

radiologistsandsurgeonsresultedinthecorrectmanagementofa

patientsufferingfromararedisease.Theauthorssuggest,in

well-selectedpatientsandafterthetumourboarddiscussion,toconsider

CRSasanothertherapeuticaloptioninthisaggressivedisease,ever

afteradequatechemotherapythat,todate,mustbeconsideredthe

ﬁrstlineoftherapyever.

Further studies are needed to validate the hypothesis and

broadentheknowledgeofthisraredisease.

Conﬂictsofinterest

Noconﬂictsofinterest.

Sourcesoffunding

Nonfundingwereused.

Ethicalapproval

Theethicalapprovalforthiscasereportisbeenexempt

Thesubmittedcasereportwasnotastudy,thereforenoethical

approvalorinformedwrittenconsentwasneeded

Consent

Writteninformedconsentwasobtainedfromthepatientfor

publicationofthiscasereportandaccompanyingimages.Acopy

ofthewrittenconsentisavailableforreviewbytheEditor-in-Chief

ofthisjournalonrequest

Authorcontribution

BarbatoGiuseppe,MD-Datacollectionandauthorofcasereport

anddiscussion.

TarantiniAlessandro,MD-Datacollectionandauthorofcase

reportanddiscussion.

SerraFrancesco,MD-Reviewofsurgicaltechniqueliteratureand

co-authorofdiscussion.

CabryFrancesca,MD-Reviewofsurgicaltechniqueliterature

andauthorofintroduction.

FarinettiAlberto,MD-Reviewofliteratureandco-author.

SorrentinoLorena,MD-Reviewofsurgicaltechniqueliterature

andauthorofintroduction.

Registrationofresearchstudies

Thesubmittedcasereportisnotaresearchstudy.

Guarantor

GelminiRoberta.

Disclosurestatement

Theauthorshavenothingtodisclose.

Provenanceandpeerreview

Notcommissioned,externallypeer-reviewed.

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