Ocular Oncology Bożena Romanowska-Dixon, Martine J. Jager, Sarah E. Coupland

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Abbreviations XVI 1. History of ocular tumor radiotherapy in Krakow - Bożena Romanowska-Dixon, Barbara Jakubowska 1 2. Genetic aspects of ocular tumours - Jakub Khzouz, Sarah E. Coupland 11 Introduction 11 Eyelid cancers 11 Basal cell carcinoma 11 Sebaceous carcinoma 13 Merkel cell carcinoma 14 Conjunctival cancers 15 Conjunctival squamous carcinoma and pre-invasive lesions 15 Conjunctival melanoma 17 Conjunctival lymphomas 18 Intraocular cancers 19 Medulloepithelioma 19 Retinoblastoma 20 Uveal melanoma 22 Vitreoretinal Lymphoma 23 Summary 24 3. Immunology of ocular tumors - Martine J. Jager, Jinfeng Cao 27 Introduction 27 Immune checkpoint inhibitors 27 Uveal melanoma: effect of checkpoint inhibitors 28 Other therapies 29 Presence of inflammation in UM 29 Phenotype-genotype comparisons 29 Comparison to other tumors 30 Conjunctival melanoma 31 Side effects of treatment 32 Conclusions 32 4. Eyelid tumors - Joshua Ford, Bita Esmaeli 35 Introduction 35 Overview of eyelid malignancies 36 Basal cell carcinoma (BCC) 36 Squamous cell carcinoma 37 Sebaceous carcinoma (SebC) 37 Periocular sweat gland carcinoma 38 Merkel cell carcinoma 38 Malignant hair follicle carcinoma 39 Eyelid melanoma 39 American Joint Committee on Cancer Staging (TNM) of eyelid malignancies 40 Surgical management 40 Basal cell carcinoma 40 Squamous cell and sebaceous cell carcinoma 40 Eyelid melanoma 41 Merkel cell 41 Sentinel lymph node biopsy (SLNB) 41 Adjuvant therapies 42 Topical therapies - Imiquimod (Aldara) 42 Targeted therapies 42 BRAF targeted therapy (vemurafenib, sorafenib, debrafenib) 43 Immune checkpoint inhibitors (pembrolizumab, ipilimumab, nivolumab, 43 Other therapies 44 Summary 44 5. Conjunctival tumors - Bożena Romanowska-Dixon, Barbara Jakubowska 49 Benign conjunctival tumors 50 Vascular lesions of the 60 Pigmented 69 Congenital and acquired melanosis 70 Malignant conjunctival tumors 76 Conjunctival squamous intraepithelial neoplasia (CIN; also termed C-SIN) 76 Invasive conjunctival squamous cell carcinoma (SCC) 76 Conjunctival melanoma 80 Lymphoid tumors 90 Metastatic tumors 94 Secondary conjunctival tumors 95 Differential diagnosis of conjunctival tumors 95 Conclusion 102 6. Uveal tumors 105 Iris cyst - Bożena Romanowska-Dixon, Barbara Jakubowska 105 Uveal nevi and melanoma - Bożena Romanowska-Dixon, Barbara Jakubowska 123 Iris nevus 124 Iris melanoma 140 Iris metastases 161 Ciliary body tumors - Bożena Romanowska-Dixon, Barbara Jakubowska 166 Ciliary body cyst 166 Ciliary body and peripheral choroidal melanoma 168 Ciliary body metastases 175 Ciliary body adenoma and ciliary body adenocarcinoma 176 Differential diagnosis of ciliary body tumors 176 Choroidal tumors 181 Choroidal hemangioma - Bożena Romanowska-Dixon, Barbara Jakubowska 181 Choroidal osteoma - Bożena Romanowska-Dixon, Barbara Jakubowska 201 Sclero-choroidal calcifications - Bożena Romanowska-Dixon, Barbara Jakubowska 211 Choroidal pigmented tumors - Bożena Romanowska-Dixon, Barbara Jakubowska, Izabella Karska-Basta 215 Management of ciliary body and/or choroidal melanoma 277 Surgical treatment - Bożena Romanowska-Dixon, Agnieszka Kubicka-Trząska 277 Endoresection of choroidal melanoma - Agnieszka Kubicka-Trząska 277 Exoresection - Bożena Romanowska-Dixon 282 Enucleation - Bożena Romanowska-Dixon, Barbara Jakubowska 285 Radiotherapy - Bożena Romanowska-Dixon, Barbara Jakubowska 295 Prediction of metastatic disease in patients with choroidal melanoma - Bertil 314 Intraocular metastases - Bożena Romanowska-Dixon, Barbara Jakubowska 317 Introduction 317 Diagnosis 318 Treatment 319 Examples of intraocular metastases 320 Leukemia - Bożena Romanowska-Dixon, Barbara Jakubowska 339 7. Differential diagnosis of intraocular tumors - Barbara Jakubowska, Bożena Romanowska-Dixon, Izabella Karska-Basta 347 Use of optical coherence tomography in a differential diagnosis of uveal tumor 353 Lesions masquerading as uveal melanoma 361 Use of indocyanine angiography in a differential diagnosis of uveal tumors 365 8. Retinal tumors - Bożena Romanowska-Dixon, Barbara Jakubowska, Joanna Kobylarz 369 Retinal tumors - Bożena Romanowska-Dixon, Barbara Jakubowska 369 Retinal glioma - Bożena Romanowska-Dixon, Barbara Jakubowska 384 Retinoblastoma - Joanna Kobylarz, Bożena Romanowska-Dixon 408 Introduction 408 Pathogenesis 408 Symptoms 408 Diagnosis 410 Clinical manifestation 413 Classification 415 Treatment 417 Post-treatment management 421 Prophylaxis 422 Summary 423 Intra-arterial chemotherapy for retinoblastoma - Christina Stathopoulos, Carol L. Shields 423 Congenital hypertrophy of RPE (CHRPE) - Bożena Romanowska-Dixon, Barbara Jakubowska 430 9. Intraocular lymphomas - Agnieszka Kubicka-Trząska 455 Epidemiology, classification, and clinical characteristics 455 Diagnosis 458 Imaging in intraocular lymphoma 459 Treatment 462 Prognosis 464 10. Retrobulbar Orbital Tumors - Jacek Składzień 467 Orbital anatomy 467 Diagnosing orbital tumor 471 Diagnosing and treatment orbital tumors 473 Imaging 474 Medical history 474 Selected orbital malignancies in the retrobulbar region 475 Dermoid cyst, epidermoid cyst and developmental cyst of the orbit 475 Cavernous hemangioma of the orbit 475 Capillary hemangioma 476 Lymphatic hemangioma of the orbit (venous-lymphatic malformation, vascular hamartoma) 476 Idiopathic orbital inflammation (IOI)(orbital 'pseudotumorIgG-4') 477 Astrocytoma (glioma) 477 Optic nerve meningioma 478 Lacrimal apparatus disorders 479 Metastases of orbital tumors 479 Surgical treatment 480 Summary - basic techniques for accessing tumors 488 Orbital exenteration 488 Rehabilitation after surgery 489 11. Radiotherapy of ocular and orbital tumors - Beata Sas-Korczyńska 493 Radiotherapy - characteristics 493 Interaction of ionizing radiation with matter 493 Energy and dose 496 Biological effects 496 Dose fractionation 497 External Beam Radiation Therapy (EBRT) 497 Brachytherapy 500 Radiotherapy of ocular and orbital tumors 501 Radiotherapy purpose and techniques 501 Radiation dose 502 Side effects in the radiotherapy of ocular and orbital tumors 504 Eyelid dermatitis 505 Conjunctivitis 505 Dry eye syndrome 505 Side effects in the cornea (keratopathy) 506 Cataract 506 Side effects on the iris 506 Side effects in the retina (retinopathy) 506 Optic neuropathy 507 Side effects in the sclera 507 Conclusion 507 12. Preparation of orbit material for histopathological examination - Jolanta Orłowska-Heitzman 511 13. Pathology of orbital tumors - Dariusz 515 Introduction 515 Non-cancerous orbital lesions 517 Most common types of orbital tumors and tumor-like lesions 519 Tumors of the optic nerve and its sheaths 519 Peripheral nerve tumors and other neurogenic tumors 522 Other neurogenic tumors 523 Tumors arising from the muscular or adipose tissue, fibrohistiocytic tumors, and other mesenchymal tumors 524 Osseous and chondral tumors and tumor-like lesions 525 Tumors and other proliferative lesions of the lymphatic, hematopoietic and histiocytic systems 527 Malignant secondary and metastatic orbital tumors 532 14. Experimental models to study metastasis in uveal melanoma - Martyna Elas 535 Cell culture and tumoroids 535 Animal models 536 CAM 536 Zebrafish 537 Rat, mouse and hamster 539 Rabbit 540 Transgenic models of UM 541 Conclusions 541 Index

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