Skull Base Tumors Involving the Orbit

Skull Base Tumors Involving the Orbit

Donald J. Annino, Jr, MD, DMD

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Skull Base TumorsSkull Base Tumors

Challenging to treatChallenging to treatRareRareMultiple Multiple histologieshistologiesComplex anatomyComplex anatomy

Orbit Orbit –– Bony AnatomyBony Anatomy

Key structure middle 1/3 Key structure middle 1/3 faceface7 bones7 bonesCommunicates with Communicates with anterior, middle cranial anterior, middle cranial fossae, infratemporal and fossae, infratemporal and pterygopalatine fossaepterygopalatine fossaeSuperior and inferior Superior and inferior orbital fissuresorbital fissures

Skull Base Tumors Involving Skull Base Tumors Involving the Orbitthe Orbit

Primary Primary Secondary Secondary MetastaticMetastatic

Primary Orbital TumorsPrimary Orbital Tumors

Benign Benign --InflammatoryInflammatoryVascular Vascular ––Cavernous Cavernous hemangiomashemangiomasNerve sheathNerve sheathBonyBonyLymphangiomaLymphangioma

Primary MalignantPrimary Malignant

Lacrimal glandLacrimal gland50 % malignant50 % malignantACC, malignant ACC, malignant mixedmixed

Lacrimal sacLacrimal sacOsteosarcoma Osteosarcoma –– after after retinoblastomaretinoblastomaRhabdomyosarcomaRhabdomyosarcoma

Secondary Orbital TumorsSecondary Orbital Tumors

Paranasal sinusesParanasal sinusesIntracranialIntracranial

MeningiomasMeningiomas

SkinSkin

Paranasal SinusParanasal Sinus LesionsLesions

BenignBenignOsteomasOsteomasMucoceleMucocelePolypsPolyps

MalignantMalignantCarcinomaCarcinomaSarcomaSarcomaMucosal melanomaMucosal melanoma

Paranasal Sinus TumorParanasal Sinus Tumor

~50 % orbital symptoms~50 % orbital symptomsInvade by preformed Invade by preformed pathways or extension pathways or extension through bonethrough boneUp to 82 % ethmoid ca Up to 82 % ethmoid ca invade laminainvade laminaUp to 50 % invade Up to 50 % invade periorbitumperiorbitumUp to 80 % orbit Up to 80 % orbit involvement with maxillaryinvolvement with maxillary

Ganly et al. Head and Neck 2005;27:575Ganly et al. Head and Neck 2005;27:575--584584Suarez et al. Head and Neck 2004;26:136Suarez et al. Head and Neck 2004;26:136--144144

Metastatic Orbital TumorsMetastatic Orbital Tumors

25 % initial presentation25 % initial presentationBreast CancerBreast CancerLungLungProstateProstateMelanomaMelanomaGI tractGI tractRenal CellRenal CellNeuroblastomaNeuroblastoma

WorkWork--upup

CTCTBone detailBone detail

MRIMRISoft tissueSoft tissueIntracranialIntracranial

Ultrasound Ultrasound Ant, middle orbitAnt, middle orbit

MRAMRA

WorkWork--upup

BiopsyBiopsyFNAFNAOpenOpen

Skull Base Tumors TreatmentSkull Base Tumors Treatment

SinonasalSinonasal outcomes improving over 4 outcomes improving over 4 decadesdecadesImproved surgical techniquesImproved surgical techniquesConformal radiation Conformal radiation

Skull Base Tumors TreatmentSkull Base Tumors Treatment

Multimodality therapyMultimodality therapySurgerySurgeryRadiation Radiation ChemotherapyChemotherapy

Minimize morbidity and maximize quality Minimize morbidity and maximize quality of life of life

Orbital TumorsOrbital Tumors

OphthalmologistOphthalmologistHead and Neck SurgeonsHead and Neck SurgeonsNeurosurgeonsNeurosurgeons

Skull Base TumorSkull Base Tumor

Contraindications for surgeryContraindications for surgeryBrain involvementBrain involvementCavernous sinus extensionCavernous sinus extensionInternal carotid involvementInternal carotid involvement

Secondary Orbital TumorsSecondary Orbital Tumors

Survival not changed with invasion limited Survival not changed with invasion limited to periorbitato periorbitaSurvival impacted withSurvival impacted with

Brain Involvement Brain Involvement –– duraldural invasioninvasionHistologyHistologyOrbital soft tissue involvementOrbital soft tissue involvement

Suarez et al. Head Neck 2004:26:136Suarez et al. Head Neck 2004:26:136--144144Ganly et al. Head Neck 2005:27:575Ganly et al. Head Neck 2005:27:575--584584Howard et al. Head Neck 2006:28:867Howard et al. Head Neck 2006:28:867--873873

Periorbitum InvolvementPeriorbitum Involvement

CT & MRI not accurateCT & MRI not accurateDirect observation in OR and frozen Direct observation in OR and frozen sectionsection

Imola, Schramm. Laryngoscope 2002;112:1357Imola, Schramm. Laryngoscope 2002;112:1357--13651365

Indications for ExenterationIndications for Exenteration

Extension through the Extension through the periorbitumperiorbitumPeriorbitum not violated Periorbitum not violated but involved then orbit but involved then orbit contents preservedcontents preservedViolation of bone alone Violation of bone alone not indication for not indication for exenterationexenterationSurvival not improved Survival not improved with orbital with orbital exenterationexenteration/clearance/clearance

Suarez et al. Head and Neck 2008;30:242Suarez et al. Head and Neck 2008;30:242--250250Imola, Schramm. Laryngoscope 2002;112:1357Imola, Schramm. Laryngoscope 2002;112:1357--

13651365

Surgical ApproachesSurgical Approaches

LocationLocationSizeSizeGoal Goal ––

BiopsyBiopsyDebulkingDebulkingTotal excisionTotal excision


Transorbital Transorbital ––OrbitotomyOrbitotomy

Extraorbital Extraorbital ––EndoscopicEndoscopicAnterior Approaches Anterior Approaches ––

Subcranial, frontalSubcranial, frontalLateral Approaches Lateral Approaches ––

Frontotemporal, frontoorbitotemporal craniotomyFrontotemporal, frontoorbitotemporal craniotomyCombinedCombined

Transorbital ApproachesTransorbital Approaches

Anterior lesionsAnterior lesionsNo extension to No extension to the orbital apexthe orbital apexCan be combined Can be combined with extraorbital with extraorbital approachesapproaches

Transorbital ApproachesTransorbital Approaches

Anterior with or Anterior with or with out with out craniotomycraniotomyMedial Medial LateralLateral

ExtraorbitalExtraorbital Endoscopic Anterior Endoscopic Anterior Skull Base SurgerySkull Base Surgery

ApproachesApproachesTransnasal directTransnasal directTransseptalTransseptalTransethmoidalTransethmoidalTranssphenoidalTranssphenoidalTranssphenoidalTranssphenoidal --transclivaltransclival

Extraorbital ApproachesExtraorbital Approaches

SubcranialSubcranialRavehRavehMinimizes frontal Minimizes frontal lobe retractionlobe retractionExcellent exposure to Excellent exposure to midline and paranasal midline and paranasal sinusessinusesCranialize frontal Cranialize frontal sinussinus


Frontotemporal, frontoorbitotemporalFrontotemporal, frontoorbitotemporalAccess to orbital apex, superior orbital fissure lesionsAccess to orbital apex, superior orbital fissure lesionsEnter paranasal sinus pack with fatEnter paranasal sinus pack with fat

Intraorbital DissectionIntraorbital Dissection

Malleable retractorsMalleable retractorsMicroscopeMicroscopeMicrosurgical Microsurgical dissectorsdissectorsCottonCotton--tipped tipped applicatorsapplicators


Stereotactic Stereotactic navigationnavigation

ReconstructionReconstruction

Loss of multiple walls Loss of multiple walls –– rigid reconstructionrigid reconstructionLoss floor of floor greater 80%Loss floor of floor greater 80%Periorbita reconstructed with fascia, allodermPeriorbita reconstructed with fascia, allodermOrbital roof no reconstruction if aloneOrbital roof no reconstruction if alone

Pulsations short termPulsations short term

Watertight closure of duraWatertight closure of duraPericranial flapPericranial flap


Epiphora Epiphora -- 36 %36 %Silastic stenting Silastic stenting ––13%13%

Anderson et al. Otolaryngol Head and Anderson et al. Otolaryngol Head and Neck Surg 1996;122:1305Neck Surg 1996;122:1305--13071307

Imola, Schramm. Laryngoscope Imola, Schramm. Laryngoscope 2002;112:13572002;112:1357--13651365

Orbital Tumors DFCIOrbital Tumors DFCI2006 2006 -- 20092009

49 patients, 200649 patients, 2006-- 2009200923 F / 22 M 23 F / 22 M 16 Benign 16 Benign 33 Malignant33 MalignantOrbital Orbital 14% (7/49)14% (7/49)SecondarySecondary 79% (39/49)79% (39/49)

Paranasal sinus Paranasal sinus 65%65% (32/49)(32/49)

MetastaticMetastatic 6% (3/49)6% (3/49)

Orbital Tumors DFCIOrbital Tumors DFCI2006 2006 -- 20092009

Benign Benign -- 1616MucocelesMucoceles -- 4 4 Cavernous hemangioma Cavernous hemangioma -- 33BonyBony -- 33

OsteomaOsteomaFibrous dysplasiaFibrous dysplasiaCherubismCherubism

MeningiomaMeningioma -- 33PseudotumorPseudotumor -- 22SchwannomaSchwannoma -- 11

Malignant Malignant –– 33 33 Carcinoma sinusesCarcinoma sinuses -- 12 12 SarcomaSarcoma -- 55EsthesioneuroblastomaEsthesioneuroblastoma -- 44Mucosal melanomaMucosal melanoma -- 3 3 MetastasisMetastasis -- 33

BreastBreastThyroidThyroidRenal cellRenal cell

CutaneousCutaneous -- 44Lacrimal sacLacrimal sac -- 22LymphomaLymphoma -- 11

Presenting SymptomsPresenting Symptoms

DiplopiaDiplopia 99HeadacheHeadache 99Nasal Nasal ObstructionObstruction 99ProptosisProptosis 99

EpiphoraEpiphora 66Decreased Decreased visual acuityvisual acuity 55EpistaxisEpistaxis 44AnosmiaAnosmia 44

Initial DiagnosisInitial Diagnosis

47 % (23/49) previous procedures 47 % (23/49) previous procedures SinusitisSinusitis 1919MigraineMigraine 44


TransorbitalTransorbital 66SubcranialSubcranial 1616

w/ exenterationw/ exenteration 22FrontotemporalFrontotemporal 88Maxillectomy Maxillectomy 1111

w/ exenterationw/ exenteration 44EndoscopyEndoscopy 88


Facial incisions avoided except in Facial incisions avoided except in transorbital, maxillectomy w/o intracranial transorbital, maxillectomy w/o intracranial extension and exenterationextension and exenterationEndoscopic assisted Endoscopic assisted


Most tumors involved more Most tumors involved more than one anatomic areathan one anatomic areaRestore volumeRestore volumePeriPeri--orbitum reconstructed orbitum reconstructed with fascia or allodermwith fascia or allodermSilastic tubes with orbital Silastic tubes with orbital preservation and division preservation and division lacrimal drainage systemlacrimal drainage systemTemporalis muscleTemporalis muscleBone reconstruction Bone reconstruction

TitaniumTitaniumMethyl methacrylateMethyl methacrylate

OutcomesOutcomes

6 patients with intraconal dissection6 patients with intraconal dissection2 Patients decreased VA (excludes exenteration)2 Patients decreased VA (excludes exenteration)

Pseudotumor VA 20/100 to NLPPseudotumor VA 20/100 to NLPMeningioma VA hand motion no change but decreased visual Meningioma VA hand motion no change but decreased visual fieldfield

1 Patient improved VA1 Patient improved VASchwannoma 20/300 to 20/70Schwannoma 20/300 to 20/70

Diplopia Diplopia Improved 6 postImproved 6 post--opop2 post2 post--op, 1 short term, 1 persists in upward gazeop, 1 short term, 1 persists in upward gaze

OutcomesOutcomes

EnophthalmosEnophthalmos2 patients2 patients3 walls resected3 walls resected

PtosisPtosis3 patients3 patients

Epiphora Epiphora –– 00Lower lid ectropian Lower lid ectropian –– 00Orbital pulsations selfOrbital pulsations self--limitedlimitedCSF CSF –– 00Cerebritis Cerebritis -- 11

CaseCase

19 yo M19 yo M2 years sx2 years sxSnoring, nasal obstruction, proptosisSnoring, nasal obstruction, proptosisDx Dx –– allergiesallergiesOS OS –– 20/2520/25OD OD –– able to count fingersable to count fingers

CaseCase

MRI, CT MRI, CT ––Large destructive lesion involving paranasal Large destructive lesion involving paranasal sinuses, bilat orbits, anterior cranial fossa, sinuses, bilat orbits, anterior cranial fossa, middle cranial fossa, extends through clivusmiddle cranial fossa, extends through clivus

CaseCase

Endoscopic biopsyEndoscopic biopsyPath Path –– Intermediate Intermediate chondosarcomachondosarcoma

CaseCase

Subcranial approachSubcranial approachEndoscopic assisted Endoscopic assisted Complete gross removalComplete gross removal

CaseCase

No complicationsNo complicationsVision unchangedVision unchangedPostPost--op proton beamop proton beam

ConclusionConclusion

TumorsTumors involving the orbit require involving the orbit require multidisciplinary approachmultidisciplinary approachVaried histologyVaried histologySafe, good outcomes with proper planning Safe, good outcomes with proper planning and approachand approach

Skull Base Tumors Involving the Orbit

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