Skull 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 bone 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 Meningioma - 3 Thyroid Pseudotumor - 2 Renal cell Schwannoma - 1 Cutaneous - 4 Lacrimal sac - 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