Base Tumors Involving the Orbit

Donald J. Annino, Jr, MD, DMD

a a SkullSkull BaseBase TumorsTumors

„„ ChallengingChallenging toto treattreat „„RareRare „„MultipleMultiple histologieshistologies „„ComplexComplex anatomyanatomy OrbitOrbit –– BonyBony AnatomyAnatomy

„ KeyKey structurestructure middlemiddle 1/31/3 faceface „ 77 bonesbones „ CommunicatesCommunicates withwith anterior,anterior, middlemiddle cranialcranial fossae,fossae, infratemporalinfratemporal andand pterygopalatinepterygopalatine fossaefossae „ SuperiorSuperior andand inferiorinferior orbitalorbital fissuresfissures SkullSkull BaseBase TumorsTumors InvolvingInvolving thethe OrbitOrbit

„„ PrimaryPrimary „„ SecondarySecondary „„ MetastaticMetastatic PrimaryPrimary OrbitalOrbital TumorsTumors

BenignBenign -- „ InflammatoryInflammatory „ VascularVascular –– CavernousCavernous hemangiomashemangiomas „ NerveNerve sheathsheath „ BonyBony „ LymphangiomaLymphangioma PrimaryPrimary MalignantMalignant

„ LacrimalLacrimal glandgland „ 5050 %% malignantmalignant „ ACC,ACC, malignantmalignant mixedmixed „ LacrimalLacrimal sacsac „ OsteosarcomaOsteosarcoma –– afterafter retinoblastomaretinoblastoma „ RhabdomyosarcomaRhabdomyosarcoma SecondarySecondary OrbitalOrbital TumorsTumors

„ ParanasalParanasal sinusessinuses „ IntracranialIntracranial „ MeningiomasMeningiomas „ SkinSkin ParanasalParanasal SinusSinus LesionsLesions

BenignBenign „ OsteomasOsteomas „ MucoceleMucocele „ PolypsPolyps MalignantMalignant „ CarcinomaCarcinoma „ SarcomaSarcoma „ MucosalMucosal melanomamelanoma ParanasalParanasal SinusSinus TumorTumor

„ ~50 % orbital symptoms „ Invade by preformed pathways or extension through „ Up to 82 % ethmoid ca invade lamina „ Up to 50 % invade periorbitum „ Up to 80 % orbit involvement with maxillary

Ganly et al. Head and Neck 2005;27:575-584 Suarez et al. Head and Neck 2004;26:136-144 MetastaticMetastatic OrbitalOrbital TumorsTumors

2525 %% initialinitial presentationpresentation „ BreastBreast CancerCancer „ LungLung „ ProstateProstate „ MelanomaMelanoma „ GIGI tracttract „ RenalRenal CellCell „ NeuroblastomaNeuroblastoma WorkWork--upup

„„ CTCT „ BoneBone detaildetail „„ MRIMRI „ SoftSoft tissuetissue „ IntracranialIntracranial „„ UltrasoundUltrasound „ Ant,Ant, middlemiddle orbitorbit „„ MRAMRA WorkWork--upup BiopsyBiopsy „„FNAFNA „„OpenOpen SkullSkull BaseBase TumorsTumors TreatmentTreatment

„„ SinonasalSinonasal outcomesoutcomes improvingimproving overover 44 decadesdecades „„ ImprovedImproved surgicalsurgical techniquestechniques „„ ConformalConformal radiationradiation SkullSkull BaseBase TumorsTumors TreatmentTreatment

„„ MultimodalityMultimodality therapytherapy „„SurgerySurgery „„RadiationRadiation „„ChemotherapyChemotherapy „„ MinimizeMinimize morbiditymorbidity andand maximizemaximize qualityquality ofof lifelife OrbitalOrbital TumorsTumors

„„ OphthalmologistOphthalmologist „„ HeadHead andand NeckNeck SurgeonsSurgeons „„ NeurosurgeonsNeurosurgeons SkullSkull BaseBase TumorTumor

ContraindicationsContraindications forfor surgerysurgery „„ BrainBrain involvementinvolvement „„ CavernousCavernous sinussinus extensionextension „„ InternalInternal carotidcarotid involvementinvolvement SecondarySecondary OrbitalOrbital TumorsTumors

„„ SurvivalSurvival notnot changedchanged withwith invasioninvasion limitedlimited toto periorbitaperiorbita „„ SurvivalSurvival impactedimpacted withwith „„BrainBrain InvolvementInvolvement –– duraldural invasioninvasion „„HistologyHistology „„OrbitalOrbital softsoft tissuetissue involvementinvolvement

Suarez et al. Head Neck 2004:26:136-144 Ganly et al. Head Neck 2005:27:575-584 Howard et al. Head Neck 2006:28:867-873 PeriorbitumPeriorbitum InvolvementInvolvement

„„ CTCT && MRIMRI notnot accurateaccurate „„ DirectDirect observationobservation inin OROR andand frozenfrozen sectionsection

Imola, Schramm. Laryngoscope 2002;112:1357-1365 IndicationsIndications forfor ExenterationExenteration

„ ExtensionExtension throughthrough thethe periorbitumperiorbitum „ PeriorbitumPeriorbitum notnot violatedviolated butbut involvedinvolved thenthen orbitorbit contentscontents preservedpreserved „ ViolationViolation ofof bonebone alonealone notnot indicationindication forfor exenterationexenteration „ SurvivalSurvival notnot improvedimproved withwith orbitalorbital exenterationexenteration/clearance/clearance Suarez et al. Head and Neck 2008;30:242-250 Imola, Schramm. Laryngoscope 2002;112:1357- 1365 SurgicalSurgical ApproachesApproaches

„ LocationLocation „ SizeSize „ GoalGoal –– „ BiopsyBiopsy „ DebulkingDebulking „ TotalTotal excisionexcision SurgicalSurgical ApproachesApproaches

TransorbitalTransorbital –– „ OrbitotomyOrbitotomy ExtraorbitalExtraorbital –– „ EndoscopicEndoscopic „ AnteriorAnterior ApproachesApproaches –– „ Subcranial, frontal „ LateralLateral ApproachesApproaches –– „ Frontotemporal, frontoorbitotemporal craniotomy „ CombinedCombined TransorbitalTransorbital ApproachesApproaches

„„ AnteriorAnterior lesionslesions „„ NoNo extensionextension toto thethe orbitalorbital apexapex „„ CanCan bebe combinedcombined withwith extraorbitalextraorbital approachesapproaches TransorbitalTransorbital ApproachesApproaches

„„ AnteriorAnterior withwith oror withwith outout craniotomycraniotomy „„ MedialMedial „„ LateralLateral ExtraorbitalExtraorbital EndoscopicEndoscopic AnteriorAnterior SkullSkull BaseBase SurgerySurgery ApproachesApproaches „ TransnasalTransnasal directdirect „ TransseptalTransseptal „ TransethmoidalTransethmoidal „ TranssphenoidalTranssphenoidal „ TranssphenoidalTranssphenoidal -- transclivaltransclival ExtraorbitalExtraorbital ApproachesApproaches

SubcranialSubcranial „ RavehRaveh „ MinimizesMinimizes frontalfrontal lobelobe retractionretraction „ ExcellentExcellent exposureexposure toto midlinemidline andand paranasalparanasal sinusessinuses „ CranializeCranialize frontalfrontal sinussinus ExtraorbitalExtraorbital ApproachesApproaches

„ Frontotemporal,Frontotemporal, frontoorbitotemporalfrontoorbitotemporal „ AccessAccess toto orbitalorbital apex,apex, superiorsuperior orbitalorbital fissurefissure lesionslesions „ EnterEnter paranasalparanasal sinussinus packpack withwith fatfat IntraorbitalIntraorbital DissectionDissection

„ MalleableMalleable retractorsretractors „ MicroscopeMicroscope „ MicrosurgicalMicrosurgical dissectorsdissectors „ CottonCotton--tippedtipped applicatorsapplicators ExtraorbitalExtraorbital ApproachesApproaches

„„ StereotacticStereotactic navigationnavigation ReconstructionReconstruction

„ LossLoss ofof multiplemultiple wallswalls –– rigidrigid reconstructionreconstruction „ LossLoss floorfloor ofof floorfloor greatergreater 80%80% „ PeriorbitaPeriorbita reconstructedreconstructed withwith fascia,fascia, allodermalloderm „ OrbitalOrbital roofroof nono reconstructionreconstruction ifif alonealone „ PulsationsPulsations shortshort termterm „ WatertightWatertight closureclosure ofof duradura „ PericranialPericranial flapflap ReconstructionReconstruction

„„ EpiphoraEpiphora -- 3636 %% „„ SilasticSilastic stentingstenting –– 13%13%

Anderson et al. Otolaryngol Head and Neck Surg 1996;122:1305-1307 Imola, Schramm. Laryngoscope 2002;112:1357-1365 OrbitalOrbital TumorsTumors DFCIDFCI 20062006 -- 20092009

„ 4949 patients,patients, 20062006-- 20092009 „ 2323 FF // 2222 MM „ 1616 BenignBenign „ 3333 MalignantMalignant „ OrbitalOrbital 14%14% (7/49)(7/49) „ SecondarySecondary 79%79% (39/49)(39/49) „ Paranasal sinus 65% (32/49) „ MetastaticMetastatic 6%6% (3/49)(3/49) OrbitalOrbital TumorsTumors DFCIDFCI 20062006 -- 20092009 „ BenignBenign -- 1616 „ MalignantMalignant –– 3333 „ Mucoceles - 4 „ Carcinoma sinuses - 12 „ Cavernous hemangioma - 3 „ Sarcoma - 5 „ Bony - 3 „ Esthesioneuroblastoma - 4 „ Osteoma „ Mucosal melanoma - 3 „ Fibrous dysplasia „ Metastasis - 3 „ Cherubism „ Breast „ - 3 „ Thyroid „ Pseudotumor - 2 „ Renal cell „ Schwannoma - 1 „ Cutaneous - 4 „ - 2 „ Lymphoma - 1 PresentingPresenting SymptomsSymptoms

„ DiplopiaDiplopia 99 „ EpiphoraEpiphora 66 „ HeadacheHeadache 99 „ DecreasedDecreased „ NasalNasal visualvisual acuityacuity 55 ObstructionObstruction 99 „ EpistaxisEpistaxis 44 „ ProptosisProptosis 99 „ AnosmiaAnosmia 44 InitialInitial DiagnosisDiagnosis

„„ 4747 %% (23/49)(23/49) previousprevious proceduresprocedures „„ SinusitisSinusitis 1919 „„ MigraineMigraine 44 SurgicalSurgical ApproachesApproaches

„ TransorbitalTransorbital 66 „ SubcranialSubcranial 1616 „ w/w/ exenterationexenteration 22 „ FrontotemporalFrontotemporal 88 „ MaxillectomyMaxillectomy 1111 „ w/w/ exenterationexenteration 44 „ EndoscopyEndoscopy 88 SurgicalSurgical ApproachesApproaches

„„ FacialFacial incisionsincisions avoidedavoided exceptexcept inin transorbital,transorbital, maxillectomymaxillectomy w/ow/o intracranialintracranial extensionextension andand exenterationexenteration „„ EndoscopicEndoscopic assistedassisted ReconstructionReconstruction

„ Most tumors involved more than one anatomic area „ Restore volume „ Peri-orbitum reconstructed with fascia or alloderm „ Silastic tubes with orbital preservation and division lacrimal drainage system „ Temporalis muscle „ Bone reconstruction „ Titanium „ Methyl methacrylate OutcomesOutcomes

„ 66 patientspatients withwith intraconalintraconal dissectiondissection „ 22 PatientsPatients decreaseddecreased VAVA (excludes(excludes exenteration)exenteration) „ Pseudotumor VA 20/100 to NLP „ Meningioma VA hand motion no change but decreased visual field „ 11 PatientPatient improvedimproved VAVA „ Schwannoma 20/300 to 20/70 „ DiplopiaDiplopia „ Improved 6 post-op „ 2 post-op, 1 short term, 1 persists in upward gaze OutcomesOutcomes

„ EnophthalmosEnophthalmos „ 2 patients „ 3 walls resected „ PtosisPtosis „ 3 patients „ EpiphoraEpiphora –– 00 „ LowerLower lidlid ectropianectropian –– 00 „ OrbitalOrbital pulsationspulsations selfself--limitedlimited „ CSFCSF –– 00 „ CerebritisCerebritis -- 11 CaseCase

„ 1919 yoyo MM „ 22 yearsyears sxsx „ Snoring,Snoring, nasalnasal obstruction,obstruction, proptosisproptosis „ DxDx –– allergiesallergies „ OSOS –– 20/2520/25 „ ODOD –– ableable toto countcount fingersfingers

CaseCase

„ MRI,MRI, CTCT –– „ LargeLarge destructivedestructive lesionlesion involvinginvolving paranasalparanasal sinuses,sinuses, bilatbilat orbits,orbits, anterioranterior cranialcranial fossa,fossa, middlemiddle cranialcranial fossa,fossa, extendsextends throughthrough clivusclivus

CaseCase

„„ EndoscopicEndoscopic biopsybiopsy „„ PathPath –– IntermediateIntermediate chondosarcomachondosarcoma CaseCase

„ SubcranialSubcranial approachapproach „ EndoscopicEndoscopic assistedassisted „ CompleteComplete grossgross removalremoval

CaseCase

„„ NoNo complicationscomplications „„ VisionVision unchangedunchanged „„ PostPost--opop protonproton beambeam

ConclusionConclusion

„„ TumorsTumors involvinginvolving thethe orbitorbit requirerequire multidisciplinarymultidisciplinary approachapproach „„ VariedVaried histologyhistology „„ Safe,Safe, goodgood outcomesoutcomes withwith properproper planningplanning andand approachapproach