Braz. j. . vol.82 número5

BrazJOtorhinolaryngol.2016;82(5):614---617

www.bjorl.org

Brazilian

Journal

of

OTORHINOLARYNGOLOGY

CASE

REPORT

Orbital

metastasis

as

the

primary

presentation

of

nasopharyngeal

carcinoma

夽

Metástase

orbital

como

apresentac

¸ão

primária

de

carcinoma

de

nasofaringe

Sung-Chan

Shin

_,

_Sung-Lyong

_Hong

_,

_Chang-Hoon

_Lee

_,

_Kyu-Sup

_Cho

a_{PusanNationalUniversityHospital,PusanNationalUniversitySchoolofMedicine,DepartmentofOtorhinolaryngologyand}

BiomedicalResearchInstitute,Busan,SouthKorea

b_{PusanNationalUniversityHospital,PusanNationalUniversitySchoolofMedicine,DepartmentofPathology,Busan,SouthKorea}

Received17March2015;accepted27April2015 Availableonline9September2015

Introduction

Metastasis to the orbit, which is uncommon due to the character of the orbital volume withrelative stenosis, is estimated to account for 1---13% of all orbital tumors.1

Orbital metastasis is believed to occur in approximately

2---3% of patients with systemic cancer.2 _{The incidence}

of metastatic orbital tumors varies widely, according to

geographicalareaandrace;themostcommonprimary

can-cers that metastasize to the orbit are breast, prostate,

liver,andlungcancer.1,2_{Althoughnasopharyngealcarcinoma}

(NPC) involves the orbits through direct extension tothe

orbitalapex,metastasisofNPCtotheorbithasrarelybeen

reported.This reportdescribes twocases of intraorbital,

extrabulbarmetastasesfromNPC.

夽 _{Pleasecitethisarticleas:ShinS-C,HongS-L,LeeC-H,ChoK-S.}

Orbitalmetastasisastheprimarypresentationofnasopharyngeal carcinoma.BrazJOtorhinolaryngol.2016;82:614---7.

∗_{Correspondingauthor.}

E-mail:choks@pusan.ac.kr(K.-S.Cho).

Case

reports

Case1

A52-year-oldmalewithabrupt-onsethoarsenessvisitedthe

authors’clinic.Hismedicalhistorywasotherwise

unremark-able.A1×1cmhard,fixedlymphnodewaspalpableinthe

rightlevelIIarea.Flexiblefiberopticlaryngoscopyrevealed

right vocal cord paralysis. An ulcerative nasopharyngeal

masswasobservedbynasalendoscopy.Computed

tomogra-phy(CT)oftheneckrevealedanenormousnasopharyngeal

mass extending into the right oropharyngeal, masticator,

carotid,prevertebral, andparavertebral space,with

mul-tiplebilateralcervicallymphnodesmetastases.Transnasal

endoscopic biopsy under localanesthesia and fine needle

aspiration on the right level II lymph node were

per-formed.Histopathologicexaminationrevealedkeratinizing

squamouscellcarcinoma(SCC)inboththenasopharyngeal

massandrightcervicallymphnode(Fig.1A).

Thepatientreceiveddocetaxelandcisplatin

chemother-apy. However, chemotherapy was stopped after the first

cycle due to neutropenic septic shock. Therefore, early

http://dx.doi.org/10.1016/j.bjorl.2015.04.006

Orbitalmetastasisofnasopharyngealcarcinoma 615

Figure1 Histopathologicfindingsofcase1.(A)Infiltratingkeratinizingcarcinomaofthenasopharynxisnotedbeneathnormal ciliatedcolumnarpseudostratifiedepithelium(H&E,×200).(B)Nasopharyngealkeratinizingcarcinomainfiltratingorbitalsofttissue

exhibitsdistinctcytoplasmickeratinformationwithpearlformation( )(H&E,×400).

radiotherapyofthenasopharynxandcervicallymphnodes

(totaldose=70Gy)wascompleted.Afteronemonth,mild

swellingintherightmedialcanthalareawasnoted.Visual

acuity,intraocularpressure,andocularmotilitywerewithin

normal limits and there was no exophthalmos. Orbit CT

revealed a newly developed 1.0×0.9cmsoft tissue mass

withindistinctmarginsontheinferomedialsideoftheright

orbit(Fig. 2Aand B). Magnetic resonance (MR) imagesof

thenasopharynxrevealedan oval-shapedorbitalmass

dis-playinglowsignalintensityonT1-weightedimages(T1WIs),

616 ShinS-Cetal.

intermediatesignalintensity onT2WIs, andmild

enhance-mentongadolinium-T1WIs(Fig.2CandD).Thiswasfollowed

by excisional biopsy that confirmed the diagnosis of

ker-atinizing SCC(Fig. 1B). On positron emission tomography

(PET)/CTscan,multipleareasofincreased

fluorodeoxyglu-cose(FDG)uptakeweredetectedintheliver,diagnosedas

distantlivermetastases.Therefore,thepatientreceivedsix

cyclesofpalliative chemotherapywithTS-1andcisplatin.

AlthoughprimaryNPCandorbitalmetastasisdidnotrecur,

thepatientdied17monthsafterdiagnosisoforbital

metas-tasisduetoliverfailure.

Case2

A 67-year-old male presented with abrupt-onset ocular

pain and blurred vision, which he had first noticed one

month prior to presentation. He was seen by a

neuro-ophthalmologist, who observed visual loss to no light

perceptionontheright.The patient’smedicalhistorywas

otherwiseunremarkable.Nasalendoscopyrevealed amild

contourprotrusionwithoutmucosalulcerationornecrosisin

thenasopharynx.NeckCTrevealedasofttissuemassinthe

extraconalandintraconalspaceoftherightorbit.Moreover,

asymmetryof the left nasopharynx andmetastatic lymph

nodeswerepresentintheleftlevelII,III,IV,and

retropha-ryngealareas (Fig.3).On PET/CT scan, the mainmass in

therightorbitwasofahypermetabolicnaturewitha

maxi-mumstandardizeduptakevalue(maxSUV)of3.5.Abnormal

FDGuptakewaspresentinmultipleleftnecklymphnodes,

identical to CT findings. Transnasal endoscopic biopsy, of

the right orbital mass and left nasopharyngeal mucosa,

was performed undergeneral anesthesia. Histopathologic

examination of both specimens revealed undifferentiated

carcinoma(Fig.4).

Thepatient receivedtwocyclesof chemotherapywith

docetaxelandcisplatin.However,herefusedfurther

treat-mentforeconomicreasonsanddiedsixmonthsafterorbital

metastasisdiagnosis.

Discussion

NPC is a tumor that arises from epithelial cells covering

thesurfaceandliningofthenasopharynx.3_{Threesubtypes}

of NPC are recognizedby the World HealthOrganization:

keratinizingSCC, nonkeratinizingcarcinoma, and

undiffer-entiatedcarcinoma.3_{AlthoughNPCcommonlymetastasizes}

tocervical lymphnodes,orbital metastasesarerare.The

majority of orbital involvement cases involvedirect

inva-sion, typically via the pterygopalatine fossa and inferior

orbitalfissure,butoccasionally throughthe ethmoidsinus

and sphenoid sinus, into the apex, causing proptosis and

muscleparalysis.4

Orbitalmetastasisofnasopharyngealcarcinoma 617

Figure4 Histopathologicfindingsofcase2.(A)Infiltratingundifferentiatednon-keratinizingcarcinoma(*)ofthenasopharynxis notedbeneathnormalciliatedcolumnarpseudostratifiedepithelium(H&E,×200).(B)Nasopharyngealundifferentiatedcarcinoma

infiltratesorbitalsofttissueanddestroysextraocularstriatedmuscles( )(H&E,×400).

Orbital metastatictumors are characterized by rather

abruptonsetofdiplopia,blurredvision,andpain.Avisible

lumpmayalsobepresentbeneaththeeyelidandprogress

isrelativelyrapid.Examinationmaydiscloseproptosis,

dis-placement of the globe, blepharoptosis, and a visible or

palpablemass.1,2 _{Thefindings inthepresent casesaccord}

withprevious reports: orbital metastases becameevident

fivemonthsafter NPCdiagnosis inone patientand

repre-sentedthefirstsignofNPCintheother.

The diagnosisoforbitalmetastasisshouldbesuspected

whenapatientwithahistoryofcancerexhibitsthe

afore-mentionedsymptoms.Ifthepatienthasnohistoryofcancer,

suchfindingsshouldpromptasystemicsurveytodetect a

primaryneoplasm andother sites ofmetastasis. Although

theultimatediagnosisoforbitalmetastasis isrenderedby

biopsyorfine-needleaspiration,CTorMRIorbitalimaging

studiesshouldalsobepreformed.CTistypicallyemployed

firstbecauseitprovidesbetterevaluationofbone.However,

MRIusuallyprovidesthebestresolution fororbital

metas-tasisevaluationbecausethemajorityoforbitalmetastases

principallyaffect orbitalsofttissues.MRItypicallyreveals

aninhomogeneouslowsignalmassonT1images,increased

signalintensityonT2images,andadegreeofenhancement

withcontrastagents.5_{Inthepresentcases,orbital}

metasta-sisfromNPCwascharacterizedbyadiffuseorwell-defined

softtissuemass,withlowsignalintensityonT1WIsand

inter-mediatesignalintensityonT2WIswithmildenhancement.

The main aimduringtreatment oforbital metastasisis

toalleviate sufferingand maintainvisual function.

Radio-therapy is the mainstay treatment for orbital metastasis

fromNPC,due toitssensitivity,but chemotherapyis also

usedincertainpatients.4_{Ifthetumoriswell-circumscribed}

and amenableto complete removal, it should be treated

bycompleteexcisionalbiopsy.1_{Prognosisisgenerallypoor,}

becausepatientsaretypicallyatanadvancedstageof

dis-ease.Theoverallmeansurvivaltimeafterorbitaldiagnosis

is 15 months;1 _{oneof this report’s patients died after 17}

months,theotheraftersixmonths.

Conclusion

Although NPC with orbital metastasis is rare, NPC can

develop metastases in orbital and ocular regions. If NPC

patients complain of ophthalmological symptoms such as

localpain,impairedvision,eyelidswelling,ordiplopia,itis

importanttoconsiderorbitalorocularmetastaticdisease.

Conflicts

of

interest

Theauthorsdeclarenoconflictsofinterest.

References

1.Shields JA, Shields CL, Brotman HK, Carvalho C, Perez N, EagleRC Jr. Cancer metastatic tothe orbit: the2000 Robert M. Curts Lecture. Ophthal Plast Reconstr Surg. 2001;17: 346---54.

2.Amemiya T, Hayashida H, Dake Y. Metastatic orbital tumors in Japan: a review of the literature. Ophthal Epidemiol. 2002;9:35---47.

3.Brennan B. Nasopharyngeal carcinoma. Orphanet J Rare Dis. 2006;1:23.

4.ColacoRJ,BettsG,DonneA,SwindellR,YapBK,SykesAJ,etal. Nasopharyngeal carcinoma: a retrospective review of demo-graphics,treatmentandpatientoutcomeinasinglecentre.Clin Oncol(RCollRadiol).2013;25:171---7.