Skull Base Tumors Involving the Orbit

Donald J. Annino, Jr, MD, DMD

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Skull Base Tumors „ Challenging to treat „ Rare „ Multiple histologies „ Complex anatomy

Orbit – Bony Anatomy „

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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 „

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Lacrimal sac Osteosarcoma – after retinoblastoma Rhabdomyosarcoma

Secondary Orbital Tumors „ „

Paranasal sinuses Intracranial „

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Meningiomas

Skin

Paranasal Sinus Lesions Benign „ „ „

Osteomas Mucocele Polyps

Malignant „ „ „

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 – „ „

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 „ „ „

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 „ „ „ „

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 „ „ „ „ „ „

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 „ „ „ „

Benign - 16 Mucoceles Cavernous hemangioma Bony „ „ „

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„ -4 -3 -3

Osteoma Fibrous dysplasia Cherubism

Meningioma Pseudotumor Schwannoma

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Malignant – 33 Carcinoma sinuses Sarcoma Esthesioneuroblastoma Mucosal melanoma Metastasis „

-3 -2 -1

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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 „ „

Transorbital Subcranial w/ exenteration

2

Frontotemporal Maxillectomy

8 11

w/ exenteration

4

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6 16

Endoscopy

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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 „ „

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 „ „

Improved 6 post-op 2 post-op, 1 short term, 1 persists in upward gaze

Outcomes „

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 „ „ „ „ „ „

19 yo M 2 years sx Snoring, nasal obstruction, proptosis Dx – allergies OS – 20/25 OD – able to count fingers

Case „ „

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 „ „ „

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