- •Preface
- •Contents
- •Contributors
- •1 Primary Orbital Cancers in Adults
- •1.1 Lymphoproliferative Disorders
- •1.1.1 Presenting Signs and Symptoms, Histopathologic and Molecular Genetic Characteristics, and Diagnosis
- •1.1.2 Treatment
- •1.1.3 Follow-up
- •1.2 Mesenchymal Tumors
- •1.2.1 Fibrous Histiocytoma
- •1.2.2 Solitary Fibrous Tumor
- •1.2.3 Hemangiopericytoma
- •1.2.4 Other Mesenchymal Tumors
- •1.3 Lacrimal Gland Tumors
- •References
- •2 Nonmalignant Tumors of the Orbit
- •2.1 Presentation
- •2.2 Cystic Lesions
- •2.3 Vascular Tumors
- •2.4 Lymphoproliferative Masses
- •2.6 Mesenchymal Tumors
- •2.7 Neurogenic Tumors
- •2.8 Lacrimal Gland Tumors
- •References
- •3 Pediatric Orbital Tumors
- •3.1 Introduction
- •3.2 Cystic Lesions
- •3.2.1 Dermoid Cyst
- •3.2.1.1 Clinical Presentation
- •3.2.1.2 Imaging
- •3.2.1.3 Histopathology
- •3.2.1.4 Treatment
- •3.2.1.5 Prognosis
- •3.2.2 Teratoma
- •3.2.2.1 Clinical Presentation
- •3.2.2.2 Imaging
- •3.2.2.3 Histopathology
- •3.2.2.4 Treatment
- •3.2.2.5 Prognosis
- •3.3 Vascular Tumors
- •3.3.1 Capillary Hemangioma
- •3.3.1.1 Clinical Presentation
- •3.3.1.2 Imaging
- •3.3.1.3 Histopathology
- •3.3.1.4 Treatment
- •3.3.1.5 Prognosis
- •3.3.2 Lymphangioma
- •3.3.2.1 Clinical Presentation
- •3.3.2.2 Imaging
- •3.3.2.3 Histopathology
- •3.3.2.4 Treatment
- •3.3.2.5 Prognosis
- •3.4 Histiocytic Lesions
- •3.4.1 Eosinophilic Granuloma
- •3.4.1.1 Clinical Presentation
- •3.4.1.2 Imaging
- •3.4.1.3 Histopathology
- •3.4.1.4 Treatment
- •3.4.1.5 Prognosis
- •3.5 Neural Tumors
- •3.5.1 Optic Nerve Glioma
- •3.5.1.1 Clinical Presentation
- •3.5.1.2 Imaging
- •3.5.1.3 Histopathology
- •3.5.1.4 Treatment
- •3.5.1.5 Prognosis
- •3.5.2.1 Clinical Presentation
- •3.5.2.2 Imaging
- •3.5.2.3 Histopathology
- •3.5.2.4 Treatment
- •3.5.2.5 Prognosis
- •3.6 Malignant Lesions
- •3.6.1 Ewing Sarcoma
- •3.6.1.1 Clinical Presentation
- •3.6.1.2 Imaging
- •3.6.1.3 Histopathology
- •3.6.1.4 Treatment
- •3.6.1.5 Prognosis
- •3.6.2 Neuroblastoma
- •3.6.2.1 Clinical Presentation
- •3.6.2.2 Imaging
- •3.6.2.3 Histopathology
- •3.6.2.4 Treatment
- •3.6.2.5 Prognosis
- •3.6.3 Retinoblastoma
- •3.6.3.1 Clinical Presentation
- •3.6.3.2 Imaging
- •3.6.3.3 Histopathology
- •3.6.3.4 Treatment
- •3.6.3.5 Prognosis
- •3.6.4 Granulocytic Sarcoma
- •3.6.4.1 Clinical Presentation
- •3.6.4.2 Imaging
- •3.6.4.3 Histopathology
- •3.6.4.4 Treatment
- •3.6.4.5 Prognosis
- •3.6.5 Rhabdomyosarcoma
- •References
- •4.1 Introduction
- •4.2 Clinical and Radiological Presentation
- •4.3 Staging
- •4.4 Surgery
- •4.5 Chemotherapy
- •4.6 Radiation Therapy
- •4.7 Conclusions and Future Directions
- •References
- •5 Metastatic Orbital Tumors
- •5.1 Introduction
- •5.2 Incidence
- •5.3 Anatomical Considerations
- •5.4 Presentation and Clinical Features
- •5.5 Diagnosis
- •5.6 Treatment
- •5.7 Types of Cancer Metastatic to the Orbit
- •5.7.1 Breast Carcinoma
- •5.7.2 Lung Carcinoma
- •5.7.3 Prostate Carcinoma
- •5.7.4 Melanoma
- •5.7.5 Carcinoid Tumors
- •5.7.6 Other Cancers
- •5.8 Conclusion
- •References
- •6.1 Tumors of Intraocular and Ocular Adnexal Origin
- •6.1.1 Eyelid Tumors
- •6.1.2 Intraocular Tumors
- •6.2 Tumors of Sinus and Nasopharyngeal Origin
- •6.2.1 Squamous Cell Carcinoma
- •6.2.2 Other Tumors of Sinus and Nasopharyngeal Origin
- •6.3 Tumors of Brain Origin
- •6.3.1 Meningioma
- •6.3.2 Other Intracranial Tumors
- •References
- •7 Lacrimal Gland Tumors
- •7.1 Introduction
- •7.2 Lymphoproliferative Lesions of the Lacrimal Gland
- •7.3 Benign Epithelial Tumors of the Lacrimal Gland
- •7.3.1 Pleomorphic Adenoma
- •7.3.2 Other Benign Epithelial Tumors
- •7.4 Malignant Epithelial Tumors of the Lacrimal Gland
- •7.4.1 Adenoid Cystic Carcinoma
- •7.4.2 Other Malignant Epithelial Tumors
- •7.5 AJCC Staging for Lacrimal Gland Tumors
- •References
- •8.1 Introduction
- •8.2 Indications
- •8.3 Surgical Techniques
- •8.3.1 Medial Orbitotomy Approach
- •8.3.2 Medial Eyelid Crease Approach
- •8.3.3 Lateral Orbitotomy Approach
- •8.3.4 Lateral Canthotomy Approach
- •8.4 Possible Indications for ONSF in Cancer Patients
- •8.4.1 Metastatic Breast Cancer
- •8.4.2 Lymphomatous Optic Neuropathy Diagnosed by Optic Nerve Biopsy
- •8.4.3 Adjuvant Therapy in Optic Nerve Sheath Meningioma
- •8.4.4 Papilledema Associated with Brain Tumors
- •8.4.5 Radiation-Induced Optic Neuropathy
- •8.5 Complications of ONSF
- •8.6 Future Research
- •References
- •9 Management of Primary Eyelid Cancers
- •9.1 Introduction
- •9.2 Types of Eyelid Malignancies
- •9.2.1 Basal Cell Carcinoma
- •9.2.2 Squamous Cell Carcinoma
- •9.2.3 Melanoma
- •9.2.4 Sebaceous Gland Carcinoma
- •9.2.5 Other Primary Eyelid Malignancies
- •9.3 Management
- •9.3.1 Evaluation
- •9.3.2 Tumor Excision and Eyelid Reconstruction
- •9.3.3 Sentinel Lymph Node Biopsy
- •9.3.4 Nonsurgical Treatment
- •9.3.5 Follow-up
- •References
- •10 Management of Conjunctival Neoplasms
- •10.1 Introduction
- •10.2 Squamous Cell Neoplasms of the Conjunctiva
- •10.2.1 Conjunctival Intraepithelial Neoplasia
- •10.2.2 Invasive Squamous Cell Carcinoma
- •10.2.3 Management
- •10.2.3.1 Local Excision and Cryotherapy
- •10.2.3.2 Treatment of More Advanced Disease
- •10.2.4 Surveillance
- •10.3 Melanocytic Neoplasms
- •10.3.1 Nevus
- •10.3.2 Primary Acquired Melanosis
- •10.3.3 Conjunctival Melanoma
- •References
- •11 Surgical Specimen Handling for Conjunctival and Eyelid Tumors
- •11.1 Introduction
- •11.2 Communication with the Pathologist
- •11.3 Conjunctival Specimens
- •11.4 Eyelid Specimens
- •11.5 Mohs Micrographic Surgery
- •11.6 Summary
- •References
- •12 Neuroradiology of Ocular and Orbital Tumors
- •12.1 Introduction: Imaging and Protocol
- •12.2 Anatomy
- •12.3 Intraocular Lesions
- •12.3.1 Retinoblastoma
- •12.3.2 Uveal Melanoma
- •12.3.3 Uveal Metastases
- •12.4 Orbital Lesions
- •12.4.1 Lymphoma
- •12.4.2 Orbital Rhabdomyosarcoma
- •12.4.3 Orbital Nerve Sheath Tumors
- •12.4.4 Mesenchymal Tumors of the Orbit
- •12.4.5 Orbital Pseudotumor
- •12.4.6 Orbital Metastases
- •12.5 Optic Nerve Tumors
- •12.5.1 Optic Nerve Glioma
- •12.5.2 Optic Nerve Sheath Meningiomas
- •12.6 Lacrimal Gland Tumors
- •12.7 Secondary Tumor Spread to the Orbit
- •12.8 Periorbital Skin Cancer and Perineural Spread
- •12.9 Conclusion
- •References
- •13 Radiation Therapy for Orbital and Adnexal Tumors
- •13.1 Indications
- •13.2 Radiation Therapy Terminology
- •13.3 Radiation Therapy Techniques
- •13.4 Radiation Therapy for Squamous Cell Carcinoma of the Eyelid
- •13.5 Adjuvant Radiation Therapy for Ocular Adnexal Tumors
- •13.6 Radiation Therapy for Optic Nerve Meningiomas and Orbital Rhabdomyosarcomas
- •13.7 Toxic Effects of Radiation Therapy
- •13.8 Summary
- •References
- •14.1 Historical Perspective
- •14.2 Presentation and Workup
- •14.4 Genetics
- •14.5 Pathologic Features
- •14.6 Treatment Options
- •14.6.1 General Considerations
- •14.6.2 Enucleation
- •14.6.3 Chemoreduction
- •14.6.4 Subtenon (Subconjunctival) Chemotherapy
- •14.6.5 Unilateral Disease
- •14.6.6 Bilateral Disease
- •14.7 Focal Therapies
- •14.7.1 Cryotherapy
- •14.7.2 Laser Photocoagulation
- •14.7.3 Brachytherapy
- •14.7.4 Thermotherapy
- •14.7.5 Radiation Therapy
- •14.8 Multi-institutional Clinical Trials
- •14.9 Animal Models of Retinoblastoma
- •14.10 Gene Transfer Technology for Treatment of Retinoblastoma
- •14.11 Future Development
- •References
- •15 Management of Uveal Melanoma
- •15.1 Epidemiology
- •15.2 Clinical Features
- •15.3 Diagnosis
- •15.4 Staging and Prognostic Factors
- •15.5 Background Studies
- •15.6 Overview of Management
- •15.7 Brachytherapy
- •15.8 Charged-Particle Radiotherapy
- •15.9 Surgical Techniques
- •15.9.1 Uveal Resection
- •15.9.2 Enucleation
- •15.9.3 Transpupillary Thermotherapy
- •15.9.4 Pathologic Assessment
- •15.9.5 Histologic Examination
- •15.10 Conclusion
- •References
- •16 Uveal Metastases from Solid Tumors
- •16.1 Introduction
- •16.2 Patient Characteristics
- •16.3 Symptoms
- •16.4 Clinical Features
- •16.5 Diagnosis
- •16.6 Treatment
- •16.6.1 Observation
- •16.6.2 External-Beam Radiation Therapy
- •16.6.3 Chemotherapy
- •16.6.4 Plaque Brachytherapy
- •16.6.5 Transpupillary Thermotherapy
- •16.6.6 Enucleation
- •16.7 Prognosis
- •16.8 Conclusions
- •References
- •17 Vascular Tumors of the Posterior Pole
- •17.1 Introduction
- •17.3 Circumscribed Choroidal Hemangioma
- •17.4 Management of Posterior Choroidal Hemangiomas
- •17.5 Acquired Vasoproliferative Tumors of the Retina
- •17.6 Conclusions
- •References
- •18 Reconstructive Surgery for Eyelid Defects
- •18.1 Introduction
- •18.2 General Principles
- •18.3 Eyelid Defects Not Involving the Eyelid Margin
- •18.4 Small Defects Involving the Lower Eyelid Margin
- •18.5 Moderate Defects Involving the Lower Eyelid Margin
- •18.6 Large Defects Involving the Lower Eyelid Margin
- •18.7 Small Defects Involving the Upper Eyelid Margin
- •18.8 Moderate Defects Involving the Upper Eyelid Margin
- •18.9 Large Defects Involving the Upper Eyelid Margin
- •18.10 Lateral Canthal Defects
- •18.11 Medial Canthal Defects
- •References
- •19.1 Introduction
- •19.2 Anatomy
- •19.3 Causes of Obstruction
- •19.4 Evaluation
- •19.5 Treatment
- •References
- •20.1 Introduction
- •20.2 Ectropion
- •20.2.1 Ectropion Due to Facial Nerve Paralysis
- •20.2.2 Cicatricial Ectropion
- •20.3 Entropion
- •20.4 Ptosis
- •20.5 Eyelid Retraction
- •20.6 Periorbital Edema Secondary to Imatinib Mesylate
- •References
- •21.1 Introduction
- •21.2 Anatomic Considerations
- •21.2.1 Orbital Margin
- •21.2.2 Nasal and Paranasal Sinuses
- •21.2.3 The Lacrimal System
- •21.2.4 Maxilla
- •21.3 Repair of Orbital Defects
- •21.3.1 Overview of Approaches
- •21.3.1.1 Maxillectomy with Orbital Exenteration
- •21.3.1.2 Maxillectomy Without Orbital Exenteration
- •21.3.2 Types of Maxillary Defects and Strategies for Their Repair
- •21.3.2.1 Type I Defect
- •21.3.2.2 Type II Defects
- •21.3.2.3 Type III Defects
- •21.3.2.4 Type IV Defects
- •21.3.3 Reconstruction After Orbital Exenteration
- •21.4 Conclusion
- •References
- •22.1 Introduction
- •22.2 Surgical Technique
- •22.2.2 Resection of Optic Nerve in Patients with Retinoblastoma
- •22.2.3 Maintenance of Globe Integrity
- •22.3 Choice of Implant
- •22.4 Management of the Anophthalmic Socket After Enucleation and Radiation Therapy
- •22.4.1 Patients with Retinoblastoma
- •22.4.2 Patients with Uveal Melanoma with Microscopic Extrascleral Extension
- •22.4.3 Patients with Head and Neck Cancer
- •22.5 Evisceration
- •References
- •23.2 Indications
- •23.3 Preoperative Evaluation
- •23.4 Surgical Techniques of Orbital Exenteration
- •23.5 Reconstructive Options
- •23.6 Surgical Complications
- •23.7 Rehabilitation After Orbital Exenteration
- •Suggested Readings
- •24.1 Introduction
- •24.2 Relevant Anatomy
- •24.3 Clinical Evaluation
- •24.3.1 Evaluation of Muscle Function
- •24.3.2 Evaluation of Lacrimal Gland and Lacrimal Drainage System Function
- •24.4 Medical Management
- •24.5 Surgical Management
- •24.5.1 Treatment of Lagophthalmos and Exposure Keratopathy
- •24.5.2 Treatment of Lower Eyelid Laxity and Ectropion
- •24.5.3 Reanimation of the Midface
- •24.5.3.1 Static Reanimation
- •24.5.3.2 Dynamic Reanimation
- •24.5.4 Options for Correction of Brow Ptosis
- •24.5.5 Additional Procedures for Management of Facial Droop
- •24.6 Special Circumstances in Cancer Patients with Facial Nerve Paralysis
- •24.7 Conclusion
- •References
- •25.1 Introduction
- •25.4 Conclusions and Recommendations
- •References
- •26 Lacrimal and Canalicular Toxicity
- •26.1 Introduction
- •26.2 5-Fluorouracil
- •26.4 Docetaxel
- •26.5 Epiphora Associated with Other Chemotherapeutic Drugs
- •26.6 Conclusions
- •References
- •27.1 Introduction
- •27.2 Orbital, Periorbital, and Orbital Teratogenic Side Effects by Individual Drug
- •27.2.1 Busulfan
- •27.2.2 Capecitabine
- •27.2.3 Carmustine
- •27.2.4 Cetuximab
- •27.2.5 Cisplatin
- •27.2.6 Cyclophosphamide
- •27.2.7 Cytarabine
- •27.2.8 Docetaxel
- •27.2.9 Doxorubicin
- •27.2.10 Erlotinib
- •27.2.11 Etoposide
- •27.2.12 Fluorouracil
- •27.2.13 Imatinib Mesylate
- •27.2.14 Interferons
- •27.2.15 Interleukin-2, Interleukin-3, and Interleukin-6
- •27.2.16 6-Mercaptopurine
- •27.2.17 Methotrexate
- •27.2.18 Mitomycin C
- •27.2.19 Mitoxantrone Dihydrochloride
- •27.2.20 Plicamycin
- •27.2.21 Thiotepa
- •27.2.22 Vincristine
- •27.3 Summary
- •References
- •28.1 Introduction
- •28.2 Epidemiology
- •28.2.1 Bacterial
- •28.2.2 Viral
- •28.2.3 Fungal
- •28.3 Pathogenesis and Host Defense
- •28.4 Ocular and Orbital Manifestations of Infection
- •28.4.1 Bacterial
- •28.4.2 Viral
- •28.4.3 Fungal
- •28.4.3.1 Candida Species
- •28.4.3.2 Aspergillus Species
- •28.4.3.3 Other Fungal Species
- •28.5 Conclusion
- •References
- •29.1 Introduction
- •29.2 Ophthalmologic Findings with CN III, IV, and VI Palsies
- •29.3 CN III, IV, and VI Palsies due to Primary Cranial Nerve Neoplasms and Direct Extension from Primary Brain, Brain Stem, or Skull base Tumors
- •29.4 CN III, IV, and VI Palsies due to Metastasis to the Brain, Brain, Stem and Skull Base from Distant Sites
- •29.5 Cranial Nerve III, IV, and VI Palsies due to Head and Neck Cancers
- •29.6 Cranial Nerve III, IV, and VI Palsies due to Leptomeningeal Disease
- •29.7 Other Causes of CN III, IV, and VI Palsies in Cancer Patients
- •29.8 Conclusion
- •References
- •30 Skull Base Tumors
- •30.1 Introduction
- •30.2 Anatomy of the Skull Base
- •30.3 Imaging and Diagnosis of Skull Base Tumors
- •30.4 Skull Base Tumors and Neuro-ophthalmic Correlations
- •30.4.1 Esthesioneuroblastoma
- •30.4.2 Chordoma
- •30.4.3 Craniopharyngioma
- •30.4.4 Meningioma
- •30.4.5 Sinonasal and Nasopharyngeal Tumors
- •30.4.6 Schwannoma
- •30.4.7 Pituitary Tumors
- •30.4.8 Myeloma
- •30.4.9 Paraganglioma
- •30.4.10 Metastases
- •References
- •31.1 Optic Pathway Gliomas
- •31.1.1 Demographics and Presentation
- •31.1.2 Histopathology
- •31.1.3 Imaging and Lesion Location
- •31.1.4 Differential Diagnosis
- •31.1.5 Management
- •31.1.6 Prognosis
- •31.2 Optic Nerve Sheath Meningiomas
- •31.2.1 Incidence
- •31.2.2 Histology and Pathophysiology
- •31.2.3 Clinical Presentation
- •31.2.4 Imaging
- •31.2.5 Treatment
- •References
- •32 Leptomeningeal Disease
- •32.1 Introduction
- •32.2 Epidemiology
- •32.3 Clinical Presentation
- •32.3.1 LMD due to Solid Tumors
- •32.3.2 LMD due to Hematogenous Tumors
- •32.3.3 LMD due to Primary Brain Tumors
- •32.4 Diagnosis
- •32.4.1 Radiographic Imaging
- •32.4.2 Optic Neuropathies in LMD
- •32.5 Treatment
- •32.6 Prognosis
- •32.7 Conclusion
- •References
- •33 Paraneoplastic Visual Syndromes
- •33.1 Introduction
- •33.2 Pathogenesis
- •33.3 Carcinoma-Associated Retinopathy
- •33.4 Carcinoma-Associated Cone Dysfunction Syndrome
- •33.5 Melanoma-Associated Retinopathy
- •33.6 Autoimmune Retinopathy
- •33.7 Paraneoplastic Optic Neuropathy
- •33.8 Diagnostic Testing
- •33.9 Differential Diagnosis
- •33.10 Treatment and Prognosis
- •33.11 Conclusion
- •References
- •34.1 Introduction
- •34.2 NF1 and the Optic Pathway
- •34.3.1 Description and Clinical Issues
- •34.3.2 Evaluation and Management
- •34.4 Intraorbital Optic Nerve Glioma
- •34.4.1 Description and Clinical Issues
- •34.4.2 Evaluation and Management
- •34.5 Chiasmal and Hypothalamic Glioma
- •34.5.1 Description and Clinical Issues
- •34.5.2 Evaluation and Management
- •34.6 Intraparenchymal Astrocytoma
- •34.6.1 Description and Clinical Issues
- •34.6.2 Evaluation and Management
- •34.7 Conclusion
- •References
- •35 Other Optic Nerve Maladies in Cancer Patients
- •35.1 Introduction
- •35.2 Optic Neuropathies Related to Elevated ICP
- •35.2.1 Causes of Elevated ICP
- •35.2.2 Treatment of Elevated ICP
- •35.4 Optic Neuropathies Caused by Drugs
- •35.4.1 Optic Disc Edema Secondary to Drug-Induced Elevated ICP
- •35.4.1.1 Retinoids
- •35.4.1.2 Imatinib Mesylate
- •35.4.1.3 Cyclosporine A
- •35.4.1.4 Cytarabine
- •35.4.2 Elevated ICP Secondary to Cerebral Venous Thrombosis
- •35.4.2.1 Cisplatin
- •35.4.2.2 L-Asparaginase
- •35.4.3 Optic Disc Edema Usually Without Elevated ICP
- •35.4.3.1 Cisplatin
- •35.4.3.2 Carboplatin
- •35.4.3.3 Carmustine
- •35.4.3.4 Vincristine
- •35.4.3.5 5-Fluorouracil
- •35.4.3.6 Cyclosporine A
- •35.4.3.7 Tacrolimus
- •35.4.4 Optic Neuropathy Without Disc Edema
- •35.4.4.1 Fludarabine
- •35.4.4.2 Tacrolimus
- •35.4.4.3 Paclitaxel
- •35.4.4.4 Methotrexate
- •35.4.4.5 Cytarabine
- •35.5 Optic Neuropathies Caused by Radiation
- •References
- •36 Management of Endogenous Endophthalmitis
- •36.1 Introduction
- •36.2 Epidemiology
- •36.3 Microbiology
- •36.4 Clinical Manifestations and Diagnosis
- •36.5 Treatment
- •36.5.1 Bacterial Endophthalmitis
- •36.5.2 Fungal Endophthalmitis
- •36.5.2.1 Yeast Endophthalmitis
- •36.5.2.2 Mold Endophthalmitis
- •36.6 Prognosis
- •36.7 Summary
- •References
- •37 Viral Retinitis in the Cancer Patient
- •37.1 Introduction
- •37.2 Epidemiology
- •37.3 Clinical Features
- •37.3.1 CMV Retinitis
- •37.3.2 Acute Retinal Necrosis
- •37.3.3 Progressive Outer Retinal Necrosis
- •37.4 Treatment
- •37.4.1 CMV Retinitis
- •37.4.1.1 Intravitreal Injections
- •37.4.1.2 Ganciclovir Implant
- •37.4.2 Acute Retinal Necrosis
- •37.4.3 Progressive Outer Retinal Necrosis
- •37.5 Role of Vitreoretinal Surgery in Viral Retinitis
- •37.5.1 Argon Laser Photocoagulation
- •37.5.2 Retinal Detachment Repair
- •37.6 Prognosis
- •37.6.1 CMV Retinitis
- •37.6.2 Acute Retinal Necrosis
- •37.6.3 Progressive Outer Retinal Necrosis
- •37.7 Conclusion
- •References
- •38.1 Introduction
- •38.2 Indications for Diagnostic Vitrectomy
- •38.2.1 Vitreous Biopsy
- •38.2.2 Uveal Biopsy
- •38.3 Preoperative Considerations
- •38.3.1 Thrombocytopenia
- •38.3.2 Anesthesia
- •38.4 Vitreous Biopsy
- •38.4.1 Technique
- •38.4.2 Effect of Vitrector Gauge on Vitreous Sample
- •38.5 Uveal Biopsy
- •38.5.1 Technique
- •38.5.2 Complications
- •38.5.3 Collaboration with Pathology
- •38.6 Pathologic Processing
- •38.6.1 Cytology
- •38.6.2 Interleukin Measurement
- •38.6.3 Polymerase Chain Reaction
- •38.6.4 Genetic Analysis
- •38.6.5 Cytogenetic Uveal Melanoma Studies
- •38.7 Results of Diagnostic Vitrectomy
- •38.7.1 Common Diagnoses
- •38.7.2 Diagnostic Utility
- •38.8 Postoperative Considerations
- •38.9 Conclusion
- •References
- •39.1 Introduction and Epidemiology
- •39.2 Presentation and Diagnosis
- •39.3 Management
- •39.4 Future Considerations
- •39.5 Conclusions
- •References
- •Index
5 Metastatic Orbital Tumors |
73 |
tissue architecture and limited volume of tissue, both of which may limit ability to make a diagnosis in some cases [17–19].
In the case of suspected but undiagnosed breast malignancy, estrogen receptor expression should be measured in the orbital biopsy specimen, not only to aid in diagnosis but also to determine the value of hormonal therapy.
5.6 Treatment
Treatment for orbital metastases is palliative in the sense that the presence of these lesions suggests a hematogenous spread of cancer even if no other metastases are identifiable. Various treatment modalities exist, including radiotherapy, chemotherapy, hormonal therapy, surgery, and combinations of these. The goals of treatment for orbital metastases are to maximize the patient’s quality of life and maximize the patient’s visual function. Each treatment modality should always be carefully considered in the context of the patient’s general state of health, life expectancy, and anticipated effects of treatment.
Radiotherapy is the mainstay of treatment for orbital metastases and is usually administered to control tumor growth, preserve vision, decrease proptosis and exposure keratopathy, and/or improve patient comfort. The recommended dose is 20–40 Gy delivered in fractions over 1–2 weeks [3–9, 20]. Radiotherapy alleviates symptoms in 80% of cases and in some cases restores vision [5]. Shielding of the globe may lessen the risk of radiation-induced side effects, such as radiation retinopathy and cataract formation, in some situations [21].
Systemic chemotherapy may be helpful in the treatment of orbital metastases, especially for chemosensitive tumors, such as small-cell lung cancer and neuroblastoma [22]. Hormonal therapy plays an important role in the treatment of metastases from hormone-sensitive tumors, such as breast cancer and prostate cancer.
Generally, orbital surgery to remove the tumor mass is not curative and may be associated with significant ocular morbidity [2–5, 7–9]. However, in selected cases, partial resection may improve the patient’s symptoms and help restore visual function. Orbital exenteration or other radical measures offer no advantage in terms of slowing disease progression or increasing survival time and should be used only in cases of intractable ocular pain or unmanageable local hygiene because of rapid tumor growth.
5.7 Types of Cancer Metastatic to the Orbit
5.7.1 Breast Carcinoma
Breast cancer is the most common primary source of orbital metastases, and the median age of patients with newly diagnosed breast cancer is in the fifth or sixth decade of life [23]. Various large studies show that a breast primary accounts for
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28.5–58.8% of cases of orbital metastasis [4, 8, 13]. Most patients, 89% in one series, have an established diagnosis prior to presentation [4]. The mean interval from the diagnosis of breast cancer to the detection of orbital metastasis has been reported to range from 4.5 to 6.5 years [4].
The characteristic presentation of orbital metastases from breast cancer consists of infiltration of the extraocular muscles and surrounding orbital fat, causing motility deficits, proptosis, globe dystopia, and enophthalmos. The association between an enophthalmic presentation and scirrhous adenocarcinoma is well known, but it is important to recognize that breast cancer metastasis to orbit may also produce proptosis rather than enophthalmos.
The histologic features of adenocarcinoma of the breast differ, and the histology of orbital metastases may vary from that of the primary tumor. Orbital metastatic cells are usually undifferentiated anaplastic cells showing single-file infiltration of fat or dense lakes of cellular lobules [4]. In a report by Garrity et al. [4], 94.6% of cases (35/37) were anaplastic grade 3 or grade 4 tumors.
Regardless of the primary tumor, the presence of orbital metastases portends a poor prognosis; the mean survival after diagnosis of such metastases is 31 months (range, 1–116 months) [4, 24]. The only appropriate surgical intervention for breast carcinoma metastatic to the orbit is biopsy to establish the diagnosis, after which external-beam radiotherapy is the mainstay therapy used to stabilize the disease. Chemotherapy or hormonal therapy may also be administered, depending on the overall disease burden.
5.7.2 Lung Carcinoma
Lung cancer remains the leading cause of cancer-related mortality in the Western world and is the second most common carcinoma to metastasize to the orbit. It accounts for 8–12% of cases of orbital metastasis. Patients have an aggressive presentation reflecting the degree of mass effect and extraocular muscle infiltration. Compared to breast carcinoma, lung carcinoma metastasizes to the orbit earlier after diagnosis and has a poorer prognosis, with a shorter median survival time (188 vs. 666 days) [4, 25].
Treatment is guided by cancer type and tumor histology. Though there are four principal variants, non-small-cell lung cancer accounts for 80% of thoracic malignancies. The types most likely to metastasize to the orbit are large-cell undifferentiated carcinoma and small-cell carcinoma; squamous cell carcinoma and adenocarcinoma have a low incidence of orbital metastasis [2, 4].
As the majority of patients with metastatic lung carcinoma succumb to disease within a relatively short period, palliative orbital radiotherapy is the only real therapeutic option for patients with orbital metastatic lesions. Debulking surgery or orbital exenteration is reserved for severe cases of intractable orbital pain and should be avoided if at all possible.
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5.7.3 Prostate Carcinoma
Despite being the second most common malignant neoplasm in men, prostate carcinoma accounts for only 3–10% of all orbital metastases reported [4, 26]. Several studies report that prostate cancer is the third most common tumor to metastasize to the orbit [3, 4, 7, 9]; however, in some other series it is reported to metastasize to the orbit less frequently than melanoma [3, 8, 15].
Common symptoms include proptosis, diplopia, eyelid swelling, decreased vision, ptosis, and red eye. Because bone metastasis is common in prostate cancer, pain is also a more common symptom in patients with prostatic orbital metastases. Ninety percent of metastatic lesions are predominantly or entirely osteoblastic [27]. When an osteoblastic lesion presents, one must keep the differential diagnosis of meningioma in mind, especially if the sphenoid bone is affected. The rapid development of osteoblastic orbital lesions in an elderly man is highly suggestive of metastatic prostate carcinoma [4].
If metastatic prostate cancer is suspected, the clinician must also inquire about nocturia, weight loss, and pain. Most prostate cancers are adenocarcinomas and range from well to very poorly differentiated. When such tumors are poorly differentiated, the primary site may remain unknown; in such cases, immunohistochemical stains can be used as a diagnostic tool. Specifically, immunoperoxidase stains should be used for prostatic-specific acid phosphatase as these levels are abnormal in more than 80% of patients with metastatic prostate cancer. The prostate-specific antigen level will often be high. Prostate cancer metastatic to the orbit can be managed safely and effectively because it is a radiosensitive malignancy; treatment for orbital disease usually consists of radiotherapy combined with hormonal therapies.
5.7.4 Melanoma
Orbital metastases from cutaneous melanoma represent 5.3–15% of all metastatic tumors of the orbit [3, 8, 15]. These are usually seen in patients with a preexisting diagnosis and disseminated end-stage disease. The primary site of origin is usually the dermis but can also be a mucosal site or the uveal tract [4, 28].
The clinical signs of orbital metastatic melanoma are similar to those of other orbital metastases; however, metastasis to extraocular muscles was seen in more than half of patients [3, 15]. This affinity for muscle would be consistent with the main presenting symptom of diplopia. Imaging studies show smooth enlargement of the muscle rather than a pattern of infiltration into the orbit (Fig. 5.3).
The survival of patients with melanoma metastatic to the orbit depends on the extent of metastatic disease and overall disease burden but generally does not exceed 12 months. However, the mean survival in one series was 19.7 months, much longer than the 5.75 and 8.4 months reported in two other published series [9, 15].
In certain cases, surgical resection to debulk the mass, even incomplete resection, may be appropriate as a palliative measure. In the case of an isolated eyelid or orbital