1 / 56

OCULAR TUMORS

OCULAR TUMORS. Vildan Öztürk, MD Assist. Prof. Of Ophthalmology. OCULAR TUMOURS. Conjunctiva Uvea Retina. Conjunctival Tumours. 1) Non-pigmented tumours Conjunctival papilloma Conjunctival intraepithelial hyperplasia Conjunctival squamous cell carcinoma Epibulber choristoma

tabithat
Download Presentation

OCULAR TUMORS

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript

  1. OCULAR TUMORS Vildan Öztürk, MD Assist. Prof. Of Ophthalmology

  2. OCULAR TUMOURS • Conjunctiva • Uvea • Retina

  3. Conjunctival Tumours 1) Non-pigmented tumours • Conjunctival papilloma • Conjunctival intraepithelial hyperplasia • Conjunctival squamous cell carcinoma • Epibulber choristoma • Conjunctival Kaposi Sarcoma • Conjunctival lymphoma

  4. Conjunctival Tumours 2) Pigmented tumours • Congenital ocular melanocytosis • Primary acquired melanosis • Conjunctival naevus • Conjunctival melanoma

  5. Tumours of the uvea • Iris melanoma • Iris naevi • Iris cysts • Ciliary body melanoma • Choroidal melanoma • Circumscribed choroidal haemangioma • Diffuse choroidal haemangioma • Metastatic carcinoma • Choroidal osseous choristoma • Intraocular lymphoma • Melanocytoma

  6. Tumours of the retina • Retinoblastoma • Astrocytoma • Capillary haemangioma • Cavernous haemangioma • Racemose haemangioma • Congenital hypertrophy of the retinal pigment epithelium • Combined hamartoma of the retinal pigment epithelium and retina

  7. 1) Pedunculated papilloma -Human papilloma virus types 6 and 11 -In childhood or early adult life -Signs: may be multiple, ocassionally bilateral, mostly palpebral conj., fornix and caruncle -Treatment: -High rate of spontaneous resolution of small lesions -Large lesions→ cryotherapy, excision Conjunctival Tumours / Non-pigmented tumoursConjunctival papilloma

  8. 2) Sessile papilloma -Nonviral -In midlle age -Signs: single, unilateral, mostly bulbar conj., or juxtalimbal -Treatment: -Complete excision, may need supplementary cryotherapy (malignant potential ) Conjunctival Tumours / Non-pigmented tumoursConjunctival papilloma

  9. -Synonyms: Carcinoma in situ, conjunctival dysplasia, intraepithelial epithelioma -Late adult life, rare, unilateral -Limbus → fornices and cornea -May evolve into invasive squamous cell carsinoma -Signs: slightly elevated, fleshy mass with blood vessels or gelatinous leukoplakic avascular lesion -Treatment: complete exicisional biopsy, cryotherapy or application of mytomycin C Conjunctival Tumours / Non-pigmented tumours Conjunctival intraepithelial hyperplasia

  10. Rare, slowly growing, may invade the slera, cornea even penetrate the globe, rarely metastizes Late adult life, from pre-existing intraepithelial hyperplasia Signs: gelatinous mass with feeder vessels, located at the limbus, may involve cornea Treatment: -mytomycin C for early cases -exicion and cryotherapy for large tumors -enucleation for advanced cases Conjunctival Tumours / Non-pigmented tumours Conjunctival squamous cell carcinoma

  11. 1) Dermoids: -the most common epider mal tumors of childhood -frequency with Goldenhar syndrome -soft, white, usually located at inferotemporale quadrant of the limbus, mostly unilateral Conjunctival Tumours / Non-pigmented tumours Epibulber choristoma

  12. 1) Lİpodermoids (dermolipomas) -congenital benign tm, bulbar conj., mostly temporal -yellow-white, solid tm, -surgical removal avoided because of frequent extension into the orbit Conjunctival Tumours / Non-pigmented tumours Epibulber choristoma

  13. Vascular, slow growing, low malignancy In patients with AIDS Bright red mass, mostly inferior fornix Focal radiotherapy is very effective, for small lesions not required Conjunctival Tumours / Non-pigmented tumoursConjunctival Kaposi Sarcoma

  14. Great variety of benign and malignant lymphoid lesions Salmon–coloured, subconjunctival infiltrate Diagnostic histologically Treatment by exicional biopsy, radiotherapy Refer to an internist for systemic evaluation Systemic lymphoma may not develop Conjunctival Tumours / Non-pigmented tumours Conjunctival lymphoma

  15. Congenital, episcleral, unilateral, blue-gray Ocular, dermal or oculodermal ( Naevus of Ota, most common) Associated with; ipsilateral iris hyperchromia, melanomas, glaucoma, iris mamillations Conjunctival Tumours / Pigmented tumours Congenital ocular melanocytosis

  16. Hystologically; PAM without atypia: benign proliferation of melanocytes PAM with atypia: %5 risk of malignancy in 5 years Diagnostic by biopsy Treatment: no treatment for PAM without atypia, excisional biopsy with cryotherapy, radiotherapy or mitomycin C for PAM with atypia Conjunctival Tumours / Pigmented tumours Primary acquired melanosis (PAM)

  17. Benign, unilateral First decades of life Solitary, sharply demarcated, flat or slightly elevated, %30 non-pigmented At puberty, may enlarge Mostly juxtalimbal, plical and at caruncle Treatment by surgical excision, bare sclera technique Conjunctival Tumours / Pigmented tumours Conjunctival naevus

  18. %2 of all eye malignancies % 50-75 arises from PAM with atypia %20 arises from naevus Least common is novo Usually in 6th decade Usually limbal, solitary, black or gray nodule, may be non-pigmented, fixed to sclera Conjunctival Tumours / Pigmented tumours Conjunctival melanoma (1)

  19. Conjunctival Tumours / Pigmented tumours Conjunctival melanoma (2) • Differential diagnosis: -large naevus at puberty -ciliary body melanoma with extraocular extension -melanocytoma; congenital, black, can not be moved over the globe -pyogenic granuloma

  20. Conjunctival Tumours / Pigmented tumours Conjunctival melanoma (3) • Treatment: -Exicional biopsy often with supplemental cryotherapy unless intraocular or orbital involvement is present -Exenteration for extensive and aggressive disease, does not improve the survival

  21. Tumours of the uveaIris melanoma • Three times commoner in light blue – gray than brown eyes • %8 of all uveal melanomas • Slow growing, low malignancy • Localized or diffuse, >3mm in diameter, inferior half of the iris • May cause glaucoma

  22. Tumours of the uveaIris melanoma • Differential diagnosis: • iris naevus • ciliary body melanoma • metastases • primary iris cyst • adenoma of the iris pigment epithelium • leiomyoma

  23. Tumours of the uveaIris melanoma • Treatment: • Broad iridectomy for small tms • Brachytherapy for small tms • Iridocyclectomy for tms invading the angle • Radiotherapy • Enucleation

  24. Pigmented, < 3 mm in diameter, superficial Cogan-Reese syndrome; diffuse naevus, aspect of heterochromia Lisch nodules: small melanocytic hamartomas, in neurofibromatosis Tumours of the uveaIris naevi

  25. Primary cysts: Epithelial: dark brown Stromal: in children, clear anterior wall Secondary cysts: Following intraocular surgery, ocular trauma or prolonged use of long acting miotics Tumours of the uveaIris cysts

  26. Tumours of the uveaCiliary body melanoma • %12 of uveal melanomas • during the 6th decade • Signs: • dilated scleral veins • pressure on the lens • retinal detachment • anterior uveitis • incidental finding

  27. Tumours of the uveaCiliary body melanoma • Treatment: • Enucleation for large melanomas • Iridocyclectomy for restricted cases • Radiotherapy in selected cases

  28. Tumours of the uveaChoroidal melanoma • Most common primary, malignant, intraocular tm • %80 of all uveal melanomas • Usually 6th decade, • Symptoms: decreased vision, vision field defect, floaters, light flashes, pain, may be asymptomatic

  29. Tumours of the uveaChoroidal melanoma • Signs: • Elevated, subretinal, oval-shaped mass • Usually brown • Orange pigment (lipofucsin) in the RPE on the surface • May grow to mushroom shape • Secondary exudative detachment

  30. Tumours of the uveaChoroidal melanoma • Differential diagnosis: • Large choroidal naevus • Localized choroidal haemangioma • Metastatic tm • Retinal detachment • Choroidal detachment • Posterior scleritis • Choroidal granulomas • AMD

  31. Tumours of the uveaChoroidal melanoma • Investigations: • Binocular indirect ophthalmoscopy • Slitlamp biomicroscopy • Ultrasonography: most accurate, choroidal excavation and orbital shadowing • FFA: of limited value • ICG: superior to FFA • CT: for extraocular extensions • MRI: not pathognomic • Colour coded Doppler: for differentiating pigmented tm • Fine needle aspiration • A general medical examination to exclude metastase to choroid and to detect metastatic spread

  32. Tumours of the uveaChoroidal melanoma • Treatment: is complex, may be combined • Plaque radiotherapy ( brachytherapy ) is the first choice; less than 10 x 20mm • Charged particle irradiation: for which are not suitable for brachytherapy; within the 4mm of the disc or fovea • Transpupillary thermotherapy ( TTT, diode laser) for small tms • Laser photocoagulation • Trans-scleral local resection: < 16mm in dia. • Enucleation: if all useful vision has been irreversibly lost • Exenteration: for extraocular extension • Palliation with chemotherapy and immunotherapy

  33. Tumours of the uveaChoroidal melanoma • Prognostic factors: 5-year mortality • Small tms (<10mm ) % 16 • Medium tms ( 10-15 mm) % 32 • Larger tms % 53

  34. Tumours of the uvea Circumscribed choroidal haemangioma • In adults, unilateral • Smoothly elevated, red-orange choroidal mass • USG shows high internal reflectivity • FFA shows early hyperfluorescense and late leakage (typical) • Treatment by external irradiation if vision is threatened

  35. Usually affects over half of the choroid, enlarges very slowly Typically occurs in patients with Sturge-Weber syndrome Thickening of the choroid Deep red colour, may be missed if not compared with the fellow eye Tumours of the uveaDiffuse choroidal haemangioma

  36. Tumours of the uveaMetastatic carcinoma • More common than primary malignancies but undetected or overshadowed • Most frequent primary site is breast in women and the bronchus in both sexes • Less common: kidney, testis,gastrointestinal tract • May cause visual impairment • Fast growing, creamy white, placoid lesion • Infiltrates laterally, ill defined borders • Teratment : radiotherapy, systemic therapy for the primary tm, enucleation if painful

  37. Very rare, benign, ossifying tumour which affects healthy young women %25 both eyes are affected but not necessarily simultaneously Orange-yellow, well defined, oval or round, geographical borders Most commonly near the optic disc or at the posterior pole Secondary NV may develop FFA shows diffuse mottled hyperfluorescence USG shows very dense lesion Tumours of the uveaChoroidal osseous choristoma

  38. Two forms of non-Hodgkin B-cell lymphoma frequently involve the eye; Systemic lymphoma (lymph nodes, visceral organs, uveal tract) CNS lymphoma Chronic anterior uveitis, intermediate uveitis, subretinal multifocal, yellowish plaques Neurological evaluation important Treatment with radiotherapy and chemotherapy but long term prognosis is poor Tumours of the uveaIntraocular lymphoma

  39. Tumours of the uveaMelanocytoma • May arise anywhere in uveal tract • Most frequently affects optic nerve head and dark skinned individuals • Deep seated tumour causes optic nerve dysfunction • Black lesion with feathery edges occupying anterior part of optic nerve head • Elevated and occupies the entire disc • Few develop into melanomas

  40. Tumours of the retina Retinoblastoma

  41. Most common primary, malignant, intraocular tm in childhood In 1/20000 live births Becomes apparent before the age of 3 years No sexual predilection Tumours of the retina Retinoblastoma

  42. Tumours of the retina Retinoblastoma • Genetic aspect: malignant transformation of primitive retinal cells before final differentiation • RPE1 gene is located at region 14 on the long arm of chromosome 13 • Non-heritable: %60, single tm, at age of 2 years, no pass to offspring • Heritable: %40, autosomal dominant transmission with high penetrance, but only %6 have family history

  43. Tumours of the retina Retinoblastoma • Heritable: • % 85 develop bilateral, multiple tms at about 18 months of age • risk of transmission is % 50 Genetic counselling: DNA analysis can be performed in suitable cases

  44. Tumours of the retina Retinoblastoma • Presentation: • Leukocoria • Strabismus • Secondary glaucoma • Orbital inflammation • Proptosis • Metastases to regional lymph nodes and brain

  45. Tumours of the retina Retinoblastoma • Signs: • An early intraretinal tm: flat , round white lesion • An endophytic tm : projects from surface as a white mass ( cottage cheese) • An exophytic tm: grows as white mass with an overlaying retinal detachment

  46. Tumours of the retina Retinoblastoma • Investigations: • USG: detects calcification and tm dimensions • CT : detects calcification and tm extension • MRI: is superior to CT for optic nerve evaluation

  47. Tumours of the retina Retinoblastoma • Treatment: • Laserphotocoagulation, TTT, cryotherapy, brachytherapy, chemotherapy, external beam radiotherapy, • Enucleation if chemoreduction fails or good status of the fellow eye makes aggressive chemotherapy impossible or poor vision with high risk or reccurence

  48. Tumours of the retina Retinoblastoma • Prognostic factors: mortality %10 • Optic nerve involvement: mortality % 65 • Choroidal invasion is adverse factor • Tm size and location • Cellular differentiation • Older children have worse prognosis

  49. Tumours of the retina Astrocytoma • Rare, benign, non-vision-threatening tm ( no trt) • Mostly seen in patients with tuberous sclerosis (% 50 have astrocytomas, %15 bilateral) • Signs: • semitranslucent, well circumscribed nodular lesion, solitary or multifocal • Later a more dense white colour • Multiple calcification areas causes mulberry-like appearance

  50. Rare, benign but vision threatening With systemic lesions, is referred to as von Hippel-Lindau syndrome (life threatening, autosomal dominant trait) Presentation; 2nd and 3rd decade, mostly with ocular involvement Endophytic hamenagioma arise from the retinal surface or optic nerve head large peripheral tms cause dilatation and tortuosity of artery and veins ( not ON head haemangiomas) FFA shows leakage Tumours of the retinaCapillary haemangioma

More Related