1. Vildan Öztürk, MD Assist. Prof. Of Ophthalmology
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. Conjunctival Tumours / Non-pigmented tumours Conjunctival papilloma
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

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

9. Conjunctival Tumours / Non-pigmented tumours Conjunctival intraepithelial hyperplasia
-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

10. Late adult life, from pre-existing intraepithelial hyperplasia
Conjunctival Tumours / Non-pigmented tumours Conjunctival squamous cell carcinoma
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

11. Conjunctival Tumours / Non-pigmented tumours Epibulber choristoma
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

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

13. Conjunctival Tumours / Non-pigmented tumours Conjunctival Kaposi Sarcoma
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

14. Conjunctival Tumours / Non-pigmented tumours Conjunctival lymphoma
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

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

16. Conjunctival Tumours / Pigmented tumours Primary acquired melanosis (PAM)
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

17. Conjunctival Tumours / Pigmented tumours Conjunctival naevus
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

18. Conjunctival Tumours / Pigmented tumours Conjunctival melanoma (1)
%2 of all eye malignancies
% 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

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 uvea Iris 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 uvea Iris melanoma
Differential diagnosis:
iris naevus
ciliary body melanoma
metastases
primary iris cyst
adenoma of the iris pigment epithelium
leiomyoma

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

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

25. Tumours of the uvea Iris cysts
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

26. Tumours of the uvea Ciliary 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 uvea Ciliary body melanoma
Treatment:
Enucleation for large melanomas
Iridocyclectomy for restricted cases
Radiotherapy in selected cases

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

31. Tumours of the uvea Choroidal 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 uvea Choroidal 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 uvea Choroidal melanoma
Prognostic factors: 5-year mortality
Small tms (<10mm ) % 16
Medium tms ( 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. Tumours of the uvea Diffuse choroidal haemangioma
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

36. Tumours of the uvea Metastatic 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. Tumours of the uvea Choroidal osseous choristoma
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

38. Tumours of the uvea Intraocular lymphoma
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

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

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. Tumours of the retina Capillary haemangioma
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

51. Tumours of the retina Capillary haemangioma
Exophytic haemangioma: less common, arise from outer retina in the peripapillery region, presents central visual loss
Treatment
Argon laser photocoagulation ( small, peripheral tms)
Cryotherapy for larger peripheral tms
External beam radiotherapy or local excision for tms that do not respond to cryotherapy
Vitreoretinal surgery for iv haemorrhage or epiretinal fibrosis

52. Tumours of the retina Cavernous haemangioma
Rare, congenital, unilateral vascular hamartoma
Few have similar skin and CNS lesions
During 2nd and 3rd decade
Appearance is collection of aneurysm
Haemorrhage is uncommon
Vitrectomy in case of non absorbing vitreous haemorrhage

53. Tumours of the retina Racemose haemangioma
Rare, unilateral, congenital arteriovenous malformation
Direct communication of arteries and veins without capillary bed
Wyburn-Mason syndrome: ipsilateral involvement of midbrain, baso-frontal region
Young patients with poor vision
Blood vessels are enlarged, tortuous and more numerous
No fluoresceine leakage
No need to treat

54. Tumours of the retina Congenital hypertrophy of the retinal pigment epithelium
Common, benign
Typical form:
Typical solitary CHRPE: usually unilateral, solitary, flat, dark-gray or black, well demarcated, round lesion, depigmented central lacunae, or ring of hypopigmentation
Typical Multiple: variably sized, multiple, confined to one sector, organized in pattern simulating animal footprints

55. Atypical multiple CHRPE
Tumours of the retina Congenital hypertrophy of the retinal pigment epithelium
Atypical multiple CHRPE
Multiple, bilateral, widely separated, oval lesions with tail of hypopigmentation at one margin
Systemic implications:
Familial adenomatous polyposis ( colon)
Gardner syndrome is a variant of FAP; develop colorectal carcinoma by the age of 50 years.

56. Tumours of the retina Combined hamartoma of the retinal pigment epithelium and retina
Rare, unilateral, peripapillary or peripheral hamartomatous malformation
Predominantly affects males and with
increased frequency in patients with
neurofibromatosis-2
Presentation early with strabismus,
Slightly elevated, intraretinal gliosis, epiretinal membranes, dilated capillaries, vascular tortuosity
Contraction of glial elements may distort the adjacent retina
Treatment by vitreoretinal surgery occasionally beneficial