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Skull Base Tumors Involving the
Orbit
Donald J. Annino, Jr, MD, DMD
a
a
Skull Base Tumors
„ Challenging to treat
„ Rare
„ Multiple histologies
„ Complex anatomy
Orbit – Bony Anatomy
„
„
„
„
Key structure middle 1/3
face
7 bones
Communicates with
anterior, middle cranial
fossae, infratemporal and
pterygopalatine fossae
Superior and inferior
orbital fissures
Skull Base Tumors Involving
the Orbit
„ Primary
„ Secondary
„ Metastatic
Primary Orbital Tumors
Benign „
„
„
„
„
Inflammatory
Vascular –
Cavernous
hemangiomas
Nerve sheath
Bony
Lymphangioma
Primary Malignant
„
Lacrimal gland
50 % malignant
„ ACC, malignant
mixed
„
„
„
„
Lacrimal sac
Osteosarcoma – after
retinoblastoma
Rhabdomyosarcoma
Secondary Orbital Tumors
„
„
Paranasal sinuses
Intracranial
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„
Meningiomas
Skin
Paranasal Sinus Lesions
Benign
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Osteomas
Mucocele
Polyps
Malignant
„
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„
Carcinoma
Sarcoma
Mucosal melanoma
Paranasal Sinus Tumor
„
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~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
Metastatic Orbital Tumors
25 % initial presentation
„ Breast Cancer
„ Lung
„ Prostate
„ Melanoma
„ GI tract
„ Renal Cell
„ Neuroblastoma
Work-up
„ CT
„ Bone
detail
„ MRI
„ Soft
tissue
„ Intracranial
„ Ultrasound
„ Ant,
„ MRA
middle orbit
Work-up
Biopsy
„ FNA
„ Open
Skull Base Tumors Treatment
„ Sinonasal
outcomes improving over 4
decades
„ Improved surgical techniques
„ Conformal radiation
Skull Base Tumors Treatment
„ Multimodality therapy
„ Surgery
„ Radiation
„ Chemotherapy
„ Minimize
of life
morbidity and maximize quality
Orbital Tumors
„ Ophthalmologist
„ Head and Neck Surgeons
„ Neurosurgeons
Skull Base Tumor
Contraindications for surgery
„ Brain involvement
„ Cavernous sinus extension
„ Internal carotid involvement
Secondary Orbital Tumors
„ Survival not changed with invasion limited
to periorbita
„ Survival impacted with
„ Brain Involvement – dural invasion
„ Histology
„ Orbital soft tissue involvement
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
Periorbitum Involvement
„ CT
& MRI not accurate
„ Direct observation in OR and frozen
section
Imola, Schramm. Laryngoscope 2002;112:1357-1365
Indications for Exenteration
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Extension through the
periorbitum
Periorbitum not violated
but involved then orbit
contents preserved
Violation of bone alone
not indication for
exenteration
Survival not improved
with orbital
exenteration/clearance
Suarez et al. Head and Neck 2008;30:242-250
Imola, Schramm. Laryngoscope 2002;112:13571365
Surgical Approaches
„
„
„
Location
Size
Goal –
Biopsy
„ Debulking
„ Total excision
„
Surgical Approaches
Transorbital –
„
Orbitotomy
Extraorbital –
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Endoscopic
Anterior Approaches –
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Lateral Approaches –
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Subcranial, frontal
Frontotemporal, frontoorbitotemporal craniotomy
Combined
Transorbital Approaches
„ Anterior lesions
„ No
extension to
the orbital apex
„ Can be combined
with extraorbital
approaches
Transorbital Approaches
„ Anterior
with or
with out
craniotomy
„ Medial
„ Lateral
Extraorbital Endoscopic Anterior
Skull Base Surgery
Approaches
„ Transnasal direct
„ Transseptal
„ Transethmoidal
„ Transsphenoidal
„ Transsphenoidal transclival
Extraorbital Approaches
Subcranial
„ Raveh
„ Minimizes frontal
lobe retraction
„ Excellent exposure to
midline and paranasal
sinuses
„ Cranialize frontal
sinus
Extraorbital Approaches
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Frontotemporal, frontoorbitotemporal
Access to orbital apex, superior orbital fissure lesions
Enter paranasal sinus pack with fat
Intraorbital Dissection
„
„
„
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Malleable retractors
Microscope
Microsurgical
dissectors
Cotton-tipped
applicators
Extraorbital Approaches
„ Stereotactic
navigation
Reconstruction
„
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„
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Loss of multiple walls – rigid reconstruction
Loss floor of floor greater 80%
Periorbita reconstructed with fascia, alloderm
Orbital roof no reconstruction if alone
„
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Pulsations short term
Watertight closure of dura
Pericranial flap
Reconstruction
„ Epiphora
- 36 %
„ Silastic stenting –
13%
Anderson et al. Otolaryngol Head and
Neck Surg 1996;122:1305-1307
Imola, Schramm. Laryngoscope
2002;112:1357-1365
Orbital Tumors DFCI
2006 - 2009
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49 patients, 2006- 2009
23 F / 22 M
16 Benign
33 Malignant
Orbital
14% (7/49)
Secondary
79% (39/49)
„
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Paranasal sinus
Metastatic
65%
(32/49)
6% (3/49)
Orbital Tumors DFCI
2006 - 2009
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Benign - 16
Mucoceles
Cavernous hemangioma
Bony
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-4
-3
-3
Osteoma
Fibrous dysplasia
Cherubism
Meningioma
Pseudotumor
Schwannoma
„
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„
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Malignant – 33
Carcinoma sinuses
Sarcoma
Esthesioneuroblastoma
Mucosal melanoma
Metastasis
„
-3
-2
-1
„
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„
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„
- 12
-5
-4
-3
-3
Breast
Thyroid
Renal cell
Cutaneous
Lacrimal sac
Lymphoma
-4
-2
-1
Presenting Symptoms
„
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Diplopia
Headache
Nasal
Obstruction
Proptosis
9
9
9
9
„
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Epiphora
Decreased
visual acuity
Epistaxis
Anosmia
6
5
4
4
Initial Diagnosis
„ 47
% (23/49) previous procedures
19
„ Sinusitis
4
„ Migraine
Surgical Approaches
„
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Transorbital
Subcranial
w/ exenteration
2
Frontotemporal
Maxillectomy
8
11
w/ exenteration
4
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6
16
Endoscopy
8
Surgical Approaches
„ Facial incisions avoided except in
transorbital, maxillectomy w/o intracranial
extension and exenteration
„ Endoscopic assisted
Reconstruction
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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
Outcomes
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6 patients with intraconal dissection
2 Patients decreased VA (excludes exenteration)
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1 Patient improved VA
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Pseudotumor VA 20/100 to NLP
Meningioma VA hand motion no change but decreased visual
field
Schwannoma 20/300 to 20/70
Diplopia
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Improved 6 post-op
2 post-op, 1 short term, 1 persists in upward gaze
Outcomes
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Enophthalmos
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Ptosis
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2 patients
3 walls resected
3 patients
Epiphora – 0
Lower lid ectropian – 0
Orbital pulsations self-limited
CSF – 0
Cerebritis - 1
Case
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19 yo M
2 years sx
Snoring, nasal obstruction, proptosis
Dx – allergies
OS – 20/25
OD – able to count fingers
Case
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MRI, CT –
Large destructive lesion involving paranasal
sinuses, bilat orbits, anterior cranial fossa,
middle cranial fossa, extends through clivus
Case
„ Endoscopic biopsy
„ Path – Intermediate chondosarcoma
Case
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Subcranial approach
Endoscopic assisted
Complete gross removal
Case
„ No complications
„ Vision
unchanged
„ Post-op proton beam
Conclusion
„ Tumors involving
the orbit require
multidisciplinary approach
„ Varied histology
„ Safe, good outcomes with proper planning
and approach