Myofibroblastic sarcoma of the mandible: a case report sarcoma of the mandible: a case report 241...
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Myofibroblastic sarcoma of the mandible: a case report
Kyung-Ran Park1, Hyo Won Jang1, Ji-Hoon Won1, Hyun-Sil Kim2,3, In-Ho Cha1,3, Hyung Jun Kim1
Departments of 1Oral and Maxillofacial Surgery, 2Oral Pathology, 3Oral Cancer Research Institute, College of Dentistry, Yonsei University, Seoul, Korea
Abstract (J Korean Assoc Oral Maxillofac Surg 2012;38:240-4)
Myofibroblastic sarcoma is a rare tumor that mostly develops in the soft tissues of the head and neck. Within the oral cavity, a tongue lesion is the most common. A myofibroblastic sarcoma tends to recur locally instead of metastasizing. We encountered a myofibroblastic sarcoma of the mandible of a 9-year-old male and performed mass excision and additional marginal alveolectomy. So far, there is neither recurrence nor metastasis. We report this case because of the uncommon location of this tumor type and its surgical approach compared to other forms of sarcomas.
Key words: Myofibroblastic sarcoma, Mandible, Alveolectomy, Uncommon location[paper submitted 2011. 9. 22 / revised 2011. 10. 31 / accepted 2011. 11. 8]
approachcomparedtootherformsofsarcoma.
II. Case Report
InDecember2010,a9-year-oldmalepatientvisitedusdue
tothepainlesssofttissuelesionintherightmandible,which
hefeltstartingamonthearlierwhenhereceivedexcisional
biopsyof the lesion in themandiblebyadentistandgot
diagnosedwithpyogenicgranuloma.Twoweeksafter the
excisionalbiopsy,however,itrecurred;thus,thepatientwas
referredtousafterhereceivedexcisionbiopsyagainandwas
finallydiagnosedwithmyofibroblasticsarcoma.
Intheclinicalexaminationperformedonthedayof the
firstvisit,a3×2cmreddishlesionwithrelativelydistinct
boundaryevaginatedtotherightlingualsideofthemandible
wasidentified;thesurfacewascoveredwithinflammatory
granulationtissues,andithadeasybleedingtendency.An
ulcerwasidentifiedonsomepartof thesurfaceoflesion.
(Fig.1)#46,84, and85adjacent to the lesion showed
mediumlevelofmobilitylaterallyandverticallywhenthe
wire fixationplacedby thedentistwas removed. In the
examinationbytouch,thelesionwasfoundtoextendinto
themandible.Thoughthepatientexperiencedinconvenience,
therewerenospontaneouspainandsignificantparesthesia
in the lower teeth, lower lip,andchin. In thepanoramic
radiographs,onthe#46,85apicalarea,osteolyticlesionwith
relativelydistinctboundarywasobserved,includinginvasion
oflesionontopof#45toothgerms.(Fig.2)Thecomputed
I. Introduction
Intheheadandneck,epithelialmalignanttumorsaccount
forat least80%,andmesodermalmalignant tumorsare
relativelyinfrequent1.Amongmesodermalmalignanttumors,
myofibroblasticsarcomaisaveryrarediseasethatcanoccur
inmostorgans includingskin, limbs,abdominalcavity,
pelviccavity,thoraciccavity,anddiaphragmbutgenerally
afflictstheheadandnecksuchastongue,floorofthemouth,
pharynx,larynx,parapharyngealspace,salivarygland,and
maxillarybones2-6.Withintheoralcavity,tonguelesionisthe
mostcommon.Thoughthetreatmenthasyettobeestablished
clearlyduetothelowincidence,treatmentplansincluding
theextentofsurgicalexcisionaredeterminedconsideringthe
gradeofmalignancy,sizeandlocationoflesion,ageofthe
patient, invasionintoadjacenttissues,andmetastasisafter
biopsyduetovariousgradesofmalignancy7.Weencountered
acaseofmyofibroblasticsarcomaof themandiblewitha
9-year-oldmale,andwearereportingthiscasebecauseof
theuncommonlocationofthistumortypeanditssurgical
Hyung Jun KimDepartment of Oral and Maxillofacial Surgery, College of Dentistry, Yonsei University, 50, Yonsei-ro, Seodaemun-gu, Seoul 120-752, KoreaTEL: +82-2-2228-3132 FAX: +82-2-2227-8022E-mail: [email protected]
This is an open-access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/), which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
CC
CASE REPORThttp://dx.doi.org/10.5125/jkaoms.2012.38.4.240
pISSN 2234-7550·eISSN 2234-5930
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Myofibroblastic sarcoma of the mandible: a case report
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partofthelesion.Nonetheless,thetumorwasdeemedtohave
beenremovedcompletelybecauseadditionalalveolectomy
wasperformedsimultaneouslyonthelowerpartofthelesion.
Thoughthepatientexperiencedtemporaryparesthesiainthe
rightlowerlipandchinafterthesurgery,after3monthsthe
patientwasrestoredandcuredwithoutcomplicationssuchas
inflammationorinfection.
Inthehistologicalexamination, thinandspindle-shaped
myofibroblastswereintersectedwitheachother, invading
thesurroundingtissueslocally.Mostmyofibroblastshada
spindle-shapedcore,butsomehadacircleorapleomorphic
coreandshowedpartialhyperchromatism.Thoughsome
pleomorphic cells showedhigh-gradedysplasia,most
myofibroblastsexhibitedlow-gradedysplasiawithouttissue
necrosis.Bloodcirculationinthesurroundingareawasmore
tomography(CT)andmagneticresonanceimaging(MRI)
revealeda lesionwithrelativelydistinctboundary in the
mandibledestroyingthelingualcorticalboneandextending
totheinsideofthemandiblewithoutinvadingadjacentareas
suchasfloorof themouth,sublingualspace,andtongue.
Therewasnolymphnodemetastasis.(Fig.3)
OnDecember31,2010,thepatientunderwentmassexcision
includingextractionof#84,85,44,45,46,and47and
alveolectomyonthelowerpartofthelesionundergeneral
anesthesia.(Fig.4)Theresectedsoftmassmeasured3×2.5cm
andwaseasilydividedfromthesurroundingtissues;inthe
finalbiopsy,however,tumorcellsweredetectedonthelower
Fig. 1. Intraoral view of soft tissue mass on the lingual side of the mandible showing granulomatous tissue overgrowth.Kyung-Ran Park et al: Myofibroblastic sarcoma of the mandible: a case report. J Korean Assoc Oral Maxillofac Surg 2012
Fig. 2. Initial panoramic view showing radiolucent lesion on #85, 46 apical area with loss of crypt wall of #45.Kyung-Ran Park et al: Myofibroblastic sarcoma of the mandible: a case report. J Korean Assoc Oral Maxillofac Surg 2012
Fig. 3. Radiographic findings of myofibroblastic sarcoma. A. Computed tomography showing osteolytic lesion in the right mandible with cortical destruction on the lingual side. B, C. Magnetic resonance imaging showing 2.5 cm enhancing mass arising from the right mandible with extra-mandibular extension; no gross invasion in the floor of the mouth, tongue, and right sublingual space, with no pathological lymph nodes.Kyung-Ran Park et al: Myofibroblastic sarcoma of the mandible: a case report. J Korean Assoc Oral Maxillofac Surg 2012
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Oncology,Pedodontics,andOralandMaxillofacialSurgery.
III. Discussion
Myofibroblastisacellintheintermediatestageoffibro-
blast’smyogenesisprocess.Itislocatedinthesofttissues
ofmostorgans6.Duringthetreatmentofwoundorreaction
of responsivecells, temporary fibroblastsareobserved,
but theydiebyapoptosiswhenthe treatment is finished.
If there isaproblemin thisprocess, theybecometumor
cells.Thereasonandphenomenological interpretationof
themyofibroblast’sdifferentiationintotumorcellhavenot
beendiscovereddespitethemanyresearchstudies,andthe
thansufficient,andmitotic indexwasrelatively lowat5
mitoticfiguresper10high-powerfields.(Fig.5)Moreover,
myofibroblast testedpositive for immunohistochemical
stainingwithCD34.Consideringthehistological,clinical,
andradiologicalopinions, thepatientwasdiagnosedwith
pT1N0M0, stage I, low-grademyofibroblastic sarcoma
(LGMS)inthemandible.
Thepatientdidnotreceiveadditionaltreatmentsincluding
radiationandanticancertreatments; threemonthsafterthe
surgery, removabledenturewasplaced in theedentulous
area.Eighteenmonthsafter thesurgery, therewasneither
recurrencenordistantmetastasis.(Fig.6)Thepatienthas
beenexaminedregularlybytheDepartmentofPediatrics,
Fig. 4. Resected specimen showing well-encapsulated mass. A. Buccal side view. B. Lower part side view.Kyung-Ran Park et al: Myofibroblastic sarcoma of the mandible: a case report. J Korean Assoc Oral Maxillofac Surg 2012
Fig. 5. Histological findings of myofibroblastic sarcoma. A. Microscopic appearance showing streaming fascicles of spindle-shaped and stellate myofibroblasts exhibiting pleomorphism (H&E staining, ×200). B. Immunohistochemical staining with CD34 (×100).Kyung-Ran Park et al: Myofibroblastic sarcoma of the mandible: a case report. J Korean Assoc Oral Maxillofac Surg 2012
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Myofibroblastic sarcoma of the mandible: a case report
243
powerfields),andpartialtissuenecrosis12.Ifthediagnosis
ishighgradeaftersurgicalexcision,radiationtreatmentis
generallyrecommended,andanticancer treatmentmaybe
usedincombination incaseofhighpossibilityofdistant
metastasis11.Fisherreported that,outof39LGMScases,
13patients (33%)experiencedpartial recurrenceand3
patients(8%)haddistantmetastasis,andthat,outof22cases
ofhigh-grademyofibroblasticsarcoma,7patients (32%)
experiencedpartial recurrenceand15patients(68%)had
distantmetastasis10.AccordingtoMentzeletal.7,outof18
LGMScases,2patientsexperiencedrecurrence,1patienthad
metastasis,14patientsreceivedsurgicalexcision,and4other
patientsreceivedsurgicalexcisionandadditionaltreatments
suchasradiationandanticancertreatments.
Giventhevarietyofexistingtreatmentsandclinicalresults
ofmyofibroblasticsarcoma,histopathologicalevaluation
andclinical/radiographicdeterminationsareveryimportant
factorsofprognosis.Inparticular,forchildrenpatientssuch
asthepatientinthiscase,decidingthepropersurgicalmargin
iscriticalwhileminimizingproblemsinthemandibularbone.
Inthiscase,wewereabletoobtainasuccessfultreatment
resultbyperformingonlyalveolectomycoveringthelesion
whileminimizingproblemsinthegrowthanddevelopment
ofmandible,consideringthefactsthatthelesionhaddistinct
boundaries, therewasno invasion into the surrounding
tissuesexcept the lingual sideof themandibleand#45
permanenttoothgerms,thepatientwasyoungandhehadlow
pathologicalgrade,andthelesiontendedtoshowmorelocal
recurrencethanmetastasis.Nonetheless,themostimportant
thingistosecureacleansurgicalmargin,andthereisaneed
tocheckforrecurrencethroughlong-termobservation.
treatmentmethodformyofibroblasticsarcomahasyet to
beestablished7.This isbecausetherearenotenoughcase
reports,myofibroblasthasvariousandcomplexhistological
differentiationsandgradesofmalignancy,anditsprognosis
canchangeaccording to the locationandsizeof lesion8.
Inexistingresearch,high-grademyofibroblasticsarcoma
isaggressive,showingmoredistantmetastasis than local
recurrence.LGMS,however,showsmorelocalrecurrence
thandistantmetastasisandtendstoinvadeintosofttissues8-10.
LGMSoccurs2.5 timesmorefrequently inmales than
females,generallyafflictingsofttissue,fascia,andsubcutan-
eoustissuessuchasheadandneck,limbs,pelvis,lung,chest,
abdomen,genitals,diaphragm,thyroid,andskin.Withinthe
headandneck,tonguelesionisthemostcommonfollowed
bysalivarygland,floorofthemouth,parapharyngealspace,
andoccipitalarea2,3,5-7,9.Mandibularlesionisanextremely
rarelesionevenforLGMS.Generallyoccurringinthetongue,
itisfeltbythepatientafterthesizeofthelesionincreases
withoutclinicalsymptomorpain,showingdistinctboundaries
inradiologicalimagesandexhibitingosteoclasiaandinvasion
onsomeboundaries.Thiscasehassimilaraspects4.
Forgeneralsarcoma,extensive2-5cmexcisionisperfor-
med,buttherangeofsurgicalexcisionforLGMSisdeter-
minedconsidering theclinicaldecisionsandhistological
opinionsoftheoperator11.Therefore,therangeofexcision
maybedeterminedaccording to theageof thepatient,
sizeandlocationoflesion,destructionofadjacenttissues,
metastasis,andhistologicalgradeofmalignancy.Generally,
thediagnosis ishighgradewhencombiningsize larger
than10cm,deeplocation,highhistologicalgradeofmalig-
nancy(highmitoticindex>10mitoticfiguresper10high-
Fig. 6. Post-operative panoramic X-ray (A) and clinical photo (B) after 18 months.Kyung-Ran Park et al: Myofibroblastic sarcoma of the mandible: a case report. J Korean Assoc Oral Maxillofac Surg 2012
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7. MentzelT,DryS,KatenkampD,FletcherCD.Low-grademyofibroblasticsarcoma:analysisof18casesinthespectrumofmyofibroblastictumors.AmJSurgPathol1998;22:1228-38.
8. FisherC.Myofibrosarcoma.VirchowsArch2004;445:215-23.9. ArtopoulouII,LemonJC,ClaymanGL,ChambersMS.Stent
fabrication forgraft immobilization followingwide surgicalexcisionofmyofibroblastic sarcomaof thebuccalmucosa:aclinicalreport.JProsthetDent2006;95:280-5.
10. FisherC.Myofibroblasticmalignancies.AdvAnatPathol2004;11:190-201.
11. KawaguchiN,AhmedAR,MatsumotoS,ManabeJ,MatsushitaY.Theconceptofcurativemargininsurgeryforboneandsofttissuesarcoma.ClinOrthopRelatRes2004;(419):165-72.
12. KhouryJD,CoffinCM,SpuntSL,AndersonJR,MeyerWH,ParhamDM.Gradingof nonrhabdomyosarcoma soft tissuesarcomainchildrenandadolescents:acomparisonofparametersusedfortheFédérationNationaledesCentersdeLutteContreleCancerandPediatricOncologyGroupSystems.Cancer2010;116:2266-74.
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