2. Classification
• Uveal tumors can be classified according to their
location, etiopathology, histopathology,
histogenesis, genotype, and various other
ontological methods.
• Etiopathogenic classification;- This system
categorizes uveal tumors as congenital, traumatic,
inflammatory, neoplastic, degenerative and
idiopathic. This classification is far from perfect.
• The anatomical listing is only approximate
7. Epithelial tumours
• Epithelial tumors of iris & ciliary body are
relatively rare.
• As anterior layer of epithelium gives rise to
smooth muscles, as all gradations of tumour
may exists from epithelioma to leiomyoma.
8. EPITHELIAL HYPERPLASIA
• Hyperplasia frequently takes the form of
proliferation into the cells of same type as
those of parent epithelium.
• The process may often be diffuse, it is
frequently localised so that a discrete plaque
or head up mass is formed having clinical
charact of true neoplasm.
• Hyperplasia of pigment epithelium may be
congenital in origin.
9. EPITHELIAL HYPERPLASIA
• It usually appears as discrete, flat & deeply
pigmented lesion showing no tendency to
grow, mainly seen on pupillary margin.
• EPITHELIAL hyperplasia of IRIS: simple
hyperplasia of pigmented layer of posterior
surface is particularly after prolonged
iridocyclitis, trauma (including surgery),
glaucoma or degenerated eyes.
10. EPITHELIAL HYPERPLASIA
• In primary glaucoma: The epithelial cells may
migrate through the stroma of iris to apper as
velvety black spot on anterior surface of iris,
mainly at collarette.
• EPITHELIAL hyperplasia of ciliary body: Fuch
emphasized difference between simple
reactive hyperplasia & epithelioma. Both this
conditions occurs in blind degenrative eyes.
11. EPITHELIAL HYPERPLASIA
• Hyperlasia is direct continuation of ciliary
epithelium forming tumour like masses
projecting inwards but showing no distinct
pattern of cells.
• In case of old RD the ciliary epithelium may
show hyperplasia near ora forming plaque
may develope into metaplasic changes.
12. IRIS CYST Epithelial cyst
• Iris cysts are
lesions
arising from
the iris
epithelium
mainly or
from stroma
rarely.
STROMAL
13. IRIS CYST
• Primary epithelial cysts: u/l or b/l. Solitary or
multiple may be brown or transparent.
location may be at pupillary border or iris root,
occasionally gets dislodged & floats in AC or
vitreous.
Majority of cysts are asymptomatic, rarely
cysts may obstruct vision & requires
treatment with argon laser photocoagulation.
14. IRIS CYST
• primary stromal cysts:presents in first year of
life.
solitary, unilateral with smooth translucent
anterior wall.
Cysts may suddenly enlarge to cause secondary
glaucoma & corneal decompensation.
neddle aspiration or surgical excision, injection
of ethanol into the cyst & removal can also
done.
15. Secondary cysts
• Implantation: m/c type originate after
deposition of surface epithelial cells from
conjunctiva or cornea on the iris after trauma.
• Pearls: white, solid lesion with opaque walls
located on stroma, not connected to wound.
• Serous: translucent cysts filled with fluid,
connected to wound, frequently enlarge
leading to corneal edema, antr uveitis &
glaucoma.
16. Secondary cysts
• Prolonged use of miotics: b/l small, multiple
cysts located along the pupillary border. It can
be prevented by concomitant use of topical
phenylephrine 2.5%.
WORM
• Parasitic: very rarely cysts may develop in
parasitic infection.
17. JUVENILE XANTHOGRANULOMA
• Rare idiopathic granulomatous d/s of
childhood.
• Involves skin,muscle,stomach, salivary glands.
• Iris involvement cause localised or diffuse
yellow lesion with spontaneous hyphema,
anterior uveitis or glaucoma
• TOC: topical steroids.
18. BENIGN EPITHELIOMA
• m/c site is pupillary border, appears as black/
brown masses with knobby or convulated
surface, periphery of iris near root is 2nd m/c
site.
• Histo: madeup of masses of cells typical of
pigment epithelium with normal nuclei
without mitotic activity, stromal elements
may be entirely absent, tumour may be
infiltrated by blood vessels.
19. BENIGN EPITHELIOMA
• Difference between epithelioma & melanoma
is dense blackness of former & its usual sharp
differentiation from surrounding.
• Management: observation over sometime,
preferably by photography. If doubt
iridectomy is done,many eyes involved have
been excised owing to the fear of malignancy.
20. EPITHELIOMA OF CILIARY BODY
• First description of benign epithelioma was
given by Fuch in 1883 in the eye of 70 year
female pt having absolute glaucoma known as
Fuch’s epithelioma.
• Occurs in wide age group 10 to 90 years of
age, no sex prediction.
• Prolonged irritations such as inflammation,
trauma or other intraocular tumour
predisposes their formation.
21. EPITHELIOMA OF CILIARY BODY
• Tumour is usually small around 1mm but
occasionally reaches to 5mm in diameter.
• Gonioscopy in dilated pupils shows brown
pigmented mass oval or round in shape lying
on the ridge of ciliary process or in intervening
valley.
• Histo: high degree of differentiation,
seperated from stroma, may lose its pigment
or become atrophied.
22. EPITHELIOMA OF CILIARY BODY
• In transitional cases which is between typical
simple growth & malignant
medulloepithelioma have been reported, this
tumour has cellular growth.
23. MEDULLO EPITHELIOMA
• Fuch classically divided medullo epithelioma
to two groups
A)Resesmbling embryonic retina DICTYOMATA.
B)Ciliary epithelium (malignant epithelioma of
ciliary body).
24. DICTYOMA
• A.k.a Embryonal medullo-epithelioma.
• It is a tumour arising from non pigmented layer
of ciliary epithelium having structure of
embryonic retina.
• Never b/l, no multicentric in origin & no
hereditary tendency, young children, present as
u/l buphthalmos/glaucoma/ cataract.
• Histo: made up of bands of cells arranged in one/
several rows forming intricate convolutions.
25. DICTYOMA
• Dictyoma should be suspected in all cases of
u/l buphthalmos, glaucoma in child ,
staphyloma, u/l catarctous or dislocated lens
or nay cyst formation in the Ac
• Management: only treatment of choice is
enucleation.
26. MALIGNANT CILIARY EPITHELIOMA
• Unlike dictyoma this type is formed by mature
cells.
• Involves eyes which have severe
inflammation.
• Small dark non translucent nodule bulging
from behind & eventually appearing over
pupillary margin.
• Course of tumour is slow & benign eye
becomes atrophic & degenerated.
27. MALIGNANT CILIARY EPITHELIOMA
Histo:
• a single layer of cell, tube formation may
occur giving the tumour in section a lace like
effect.
• Rosette like structures, primitive nerve fibres
formations resembles primitive optic vesicle.
28. MUSCULAR TUMOUR
• Leimyoma: Reported only in caucasians, F>M,
commonly on lower half of iris , temporally &
on sphincteric region.
• Generally forms a sessile mass on pupillary
margin, pink/ greyish white in colour.
• Difficult to diagnose from a sparsely
pigmented malignant melanoma.
29. VASCULAR TUMOUR
• Haemangiomas are more common in choroid
as compare to iris & ciliary body.
• Iris: vascular tumour is localised on the iris
surface, which may give rise to periodic
bleeding.
• Choroidal haemangioma is divided into
circumscribed.
diffuse.
30. CIRCUMSCRIBED
Not associated with any
systemic d/s shows mass within
the choroid, composed of
vascular channels.
Presents in 2nd-4th decades,
u/l blurring of vision, visual field
defect & metamorphopsia.
Hypermetropia+
An oval mass @ posterior pole.
Lesion is 6mm in dia & 3mm
thick.
Can cause RD, RPE degenration
& subretinal fibrosis.
31. CIRCUMSCRIBED
• FA shows spotty
hyperfluorescence
in early arterial
phase.
• ICGA also shows
hyperflourescence
in early frames.
32. CIRCUMSCRIBED
• US shows acoustically solid lesionwith sharp
anterior surface.
• Treatment: photodynamic therapy- may be
repeated after few months.
• TTT:if lesions are not involving macula.
• Radiotherapy.
• Intravitreal anti-VEGF therapy.
33. DIFFUSE
• Affects over half of choroid
• Enlarges very slowly.
• Exclusively seen in patients
with sturge webber syndm.
• Presents in 2nd decade of
life despite its formation @
birth.
34. DIFFUSE
• Fundus appears as deep red tomato ketchup
colour, most marked at posterior pole.
• Localised area of thickening is present,
simulating a circumscribed haemangioma.
• USG: shows diffuse thickening.
• Complictn: RD, neovascular glaucoma, retinal
cystoid degenratn.
• Treatmnt: radiotherapy/ PDT.
35. CHOROIDAL OSTEOMA
• Very rare benign,slow growing ossifying
tumour.
• b/L in 25% cases, F>M.
• Mature cancellous bone which causes
overlying RPE atrophy.
• Presents in 2nd- 3rd decades , yellowish-white
flat, minimally elevated lesion near disc or
posterior pole present.
36. CHOROIDAL OSTEOMA
• FA shows mottled
hyperfluoresecnce & late
staining.
• ICGA: early
hypofluorescence.
• USG: highly reflective antr
surface & orbital shadowing.
• No malignant potential,
managemant is directed @
preservation of vision
mainly.
37. NEUROFIBROMA
• Congenital condition representing a diffuse
proliferation schwann cells of the peripheral
nerves.
• HISTO: increase in thickness of choroid &
ciliary body, pear shaped deformity of the
pupil resembling colobomata may occur.
• Rx: Excision------ enucleation.
38. NEURILEMMOMA
• Benign encapsulated tumour of nerve sheath.
• Tumour composed of schwann cells arranged
in interlacing cords with palisading of the
nuclei.
39. METASTATIC TUMOUR
• Choroid is m/c site accounting for about 90%
followed by iris & ciliary body.
• Most frequent primary site is breast &
bronchus.
• A fast growing creamy white lesion with
indistinct margins most frequently at posterior
pole may be present.
• Deposits are multifocal, may cause RD.
41. RETICULOSIS
• Any part or whole of uveal tract may be
involved & ocular lesion may be B/L.
• In anterior segment: severe iridocyclitis,
nodular mass in AC.
• In posterior segment: pan uveitis, RD, vision
fails rapidly.
42. RETICULOSIS
HISTO: lymphocytic cells arranged either
diffusly, in reticular cells large round oval
nuclei & small cytoplasmic process resembling
the cells of germinal centre of lymph follicles
are present lying within the network of
reticulum.
MANAGEMENT: palliative, in some cases
enucleation is required.
43. RETICULOSIS
• MULTIPLE MYELOMA: abnormal plasma cells
in bone marrow.
deposition of plasma cells in the tissue
particularly in retina / optic nerve & bony
orbit.
44. UVEAL NEVUS
• The uveal nevus is a benign tumour that arises
from melanocytic cells derived from the
neural crest.
• m/c in whites, aprox 20% develop at least one
choroidal nevus >50yrs of age, M=F.
• 1 in 4000-5000 undergoes malignant changes.
• Asymptomatic may cause visual loss in
macular choroidal nevi.
46. IRIS NEVUS
• IRIS NEVUS :< 3mm in diameter, < 0.5mm
thick,pupillary peaking, focal ectropion iridis
or both, abnormal iris vasculature.
• Choroidal nevus:small grey to brown
choroidal tumour, < 5mm in diameter, < 1mm
in thickness, causes blurred/distorted vision
serous subretinal fluid, cystic degenration of
retina involving macula or choroidal
neovascularization.
47. MELANOCYTOMA
• Special type of nevus “melanocytoma”of optic
disc.
• Composed entirely of maximally pigmented
polyhedral nevus cells (magnocellular nevus
cells).
• Lesion appears striated because of insinuation of
pigment cells between axons in the nerve
fibrelayer which may inturn causes visual field
defect.
• Magnocellular nevus can occur in choroid, ciliary
body & iris.
49. DIAGNOSIS
• B-scan
• Fluoresceine angiogrphy & indocyanine green
angiography.
• Documented photogrphy shortly after
detection.
• FNAC can be used to determine larger
tumours >1.5mm thick using trans vitreal bent
neddle technique.
50. DIAGNOSIS
• Neurofibromatosis 1 patients comes with
multiple uveal nevi in both eyes, become
evident @ 10-15 yrs of age.
• Histo: spindle nevus cells are fusiform
melanocytes that typically have a relatively
small nucleaus.
• Magnocellular nevus: plump polyhedral
melanocytes containing numerous large intra
cytoplasmic melanin granules.
51. MANAGEMENT
• Photographic documentation/ usg
documentation & periodic evaluation.
• For every small tumours 1-2 yearly followup,
slightly larger 6-12 months followup, mal changes
1-3 months.
• Focal laser therapy can be given in nevi causing
blurred visison.
• In choroidal neovascularisation focal obliterative
laser therapy/ photodynamic therapy can be
given.
52. Course / outcome
In IRIS LESION consider benign if:
1)Small size <3mm in dia, 0.5mm thick
2)cohesive surface
3)Absence of pig dispersion
4)lack of pupillary peaking.
5)ab of ectropion iridis.
6) Ab of cataract, vascularity.
Lack of symptoms & enlargement.
53. Course / outcome
• In choroidal lesion consider benign if:
1)Small size <5mm dia, 1mm thick.
2)Homogenous gray brown surface.
3) Feathered margins blends into surrounding
normal choroid.
4)Drusen/retinal pigment clumping & migration.
5) Ab of lipofuscin pig on surface lesion/ subretinal
fluid.
6) No sudden increase in size.
54. MALIGNANT MELANOMA
• Malignant neoplasm arising from
neuroectodermal melanocytes. m/c
intraocular tumour in whites.
• Key features are metastasize hematogenously,
m/c involve liver, choroid is commonly
involved.
• Incidence is 1 in 2000-5000 white individuals,
15 to 50 times less common in blacks, m/c in
men.
55. MALIGNANT MELANOMA
• Uveal tumours confined to iris appears to be
substantially less malignant as compare to
choroidal melanomas.
• Extent of tumour & management is different
for iris melanoma & ciliary body/ choroidal
melanomas.
• For this reason these two general forms are
discussed seperately.
56. IRIS MELANOMA
• Presents as an spot on the iris with no
symptoms.
• >1mm thick is main concern for its
malignancy.
• Dispersion of tumour cells, pigments, may
cause increase in IOP, pupillary peaking
ectropion iridis , iris splinting may present.
58. IRIS MELANOMA
• Histo: composed of atypical melanocytic cells,
larger nuclear to cytoplasmic ratio.
• Tumour cells have a fusiform shape & mild
atypical spindle melanoma cells.
• Those that have more spherical & more
pronounced anaplasia are called as epithelioid
melanoma cells.
59. MANAGEMENT
• Small melanomas should be observed without
intervention.
• Excision: iridectomy/ irideocyclectomy is
done.
• Plaque radiotherapy/ photon beam irridiation
performed in small number of cases but
appears to be effective in short term followup.
• Enucleation: for large iris melanomas,
extensive seeding, transcleral spread, blind
painful eye.
60. course
• Iris tumour grows relatively slowly.
• Replaces porportion of iris & ciliary body.
• Can cause secondary glaucoma.
• Post excision patient must be followed for
every 6 months for first 2-3 years & yearly
there after.
62. CHOROIDAL/CILIARY MELANOMAS
• 20% of choroidal melanomas breaks through
bruch’s membrane & RP to form a nodular
eruption.
• They exhibits prominenet clumps of orange
lipofuscin pigment on their surface.
• May be associated with non rhegmatogenous
RD clear, serous, shifting subretinal fliud.
• VH may be present.
63. CHOROIDAL/CILIARY MELANOMAS
• In ciliary body melanomas appears elevated,
nodular, dark brown mass in peripheral
fundus.
The surface of the tumor may
contain yellow drusen or
orange lipofuscin deposits
64. • Clinical features
• asymptomatic
• incidentally during ophthalmoscopy.
• In general, the more anterior their origin, the longer the
delay of any symptoms.
• Blurred visual acuity
• growth of the melanoma into the subfoveal retina
• cystoid macular edema disruption of choroidal circulation
→ ischemia → degeneration of retinal photoreceptors→
The retina overlying the tumor separates into cystoid
spaces and larger schisis cavities.
• Retinal detachment - Exudation of fluid into the subretinal
space → retinal detachment → enlarge the field loss→
can lead to total retinal detachment
65. • vitreous hemorrhage - Erosion of the melanoma into
blood vessels in adjacent tissues, or areas of necrosis
within the tumor
• cataract – involvement of lens
• Paracentral scotoma
• if the tumor affects the perifoveal retina.
• visual field loss Painless and progressive as peripheral
melanoma grows
• Floaters when areas of necrosis within the tumor or
adjacent structures produce vitreous hemorrhage or
hyphema.
• Severe ocular pain Occasionally when they impinge
into posterior ciliary nerves.
• can also cause pain secondary to high intraocular pressure
from acute angle-closure glaucoma.
66. • History of weight loss, marked fatigue, cough, or
change in bowel or bladder habits, should prompt
consideration of primary non-ocular malignancy
with choroidal metastasis
67. DIAGNOSIS
• The diagnostic error rate in the COMS was only
0.48%.
• Misdiagnoses occur in less than 4% of enucleated
eyes at tertiary referral centers.
• The diagnostic error rate in the COMS, however, was
measured under ideal conditions; all tumors were
well visualized and photographed. Patient were
excluded from the COMS if the ocular media was
opaque. In real-world situations, the misdiagnosis
rate for posterior melanoma may be greater than
that reported by the COMS
68. • A-scan ultrasound
• tumors more than 2-3 mm thick.
• characteristically shows an initial prominent spike,
followed by low-to-medium internal reflectivity. Vascular
pulsations can be seen as fine oscillations of the internal
spiking pattern within the tumor. Standardized
ultrasonography has a diagnostic accuracy of more than
95%.
• B-scan ultrasound to help establish the diagnosis, to
evaluate possible extraocular extension, and to estimate
tumor size for periodic observation and plan therapeutic
intervention
• Low-to-medium reflectivity
• Excavation of underlying uveal tissue
• Internal vascularity
• An acoustic quiet zone at the base of the tumor called
acoustic hollowing
69. • Fluorescein angiography do not show
pathognomonic signs of choroidal melanoma but can
help point to its diagnosis.
• Small choroidal melanomas may show fluorescein
angiographic changes similar to some choroidal nevi, as
follows: ranging from normal angiography to
hypofluorescence secondary to blockage of background
fluorescence.
• Larger melanomas may show a patchy pattern of early
hypofluorescence and hyperfluorescence followed by late
intense staining.
• intrinsic vascularization, visible throughout the
angiogram.
• The angiographic sign called "double circulation" pattern
refers to simultaneous fluorescence of retinal and
choroidal circulation within the tumor. When it occurs, it
is fairly distinctive of choroidal melanomas
70. • Early fluorescein
angiogram of
choroidal
melanoma
showing intrinsic
vascularity
71. • CT scan orbit
• is more expensive than ultrasound and
• currently is not as sensitive.
• It is useful to see extraocular extension and may help
differentiate between choroidal or retinal detachment and
a solid tumor.
• Choroidal melanoma shows enhancement with contrast,
where as exudation does not.
• CT scan also is very sensitive detecting calcium, a feature of
some tumors that are different than uveal melanoma
(characteristically choroidal osteoma).
72. • Fine needle and incisional biopsy
• usually are not required
• to distinguish amelanotic melanomas from metastatic
tumors, and if results from other ancillary tests are
equivocal.
• accuracy of more than 95% in tumors larger than 3 mm.
• Incisional biopsy is more invasive and may have more
associated complications, but it has less false-negative
and false-positive results.
• Risk of spread of cancerous cells with needle biopsy is
small for choroidal melanoma (unlike retinoblastoma).
Follow biopsy by prompt treatment to avoid extrascleral
extension.
73. • Histologic evaluation - after enucleation can confirm
the diagnosis and evaluate prognosis.
• Three distinct cell types are recognized:
• Spindle A - elongated nuclei and uncommonly have
mitotic figures
• Spindle B - prominent nucleolus
• found more commonly, have an elongated profile but are
slightly larger than spindle A cells
• Epithelioid – most aggressive behavior and carries a
poorer prognosis for the patient's long-term survival
• highly anaplastic, poorly cohesive, and have considerable
• frequent mitotic figures.
74. Modified callender classification
• Histologically can be divided into 3 types;
1.Spindle cell melanoma- good prognosis.
2.Mixed cell melanoma- intermediate.
3.Epitheloid cell melanoma- worst prognosis.
75. • Prognosis
• 30-50% of patients with choroidal melanoma will die
within 10 years from diagnosis and treatment
• tumor features found to correlate with increased
mortality,
• larger size,
• anterior location,
• transscleral extension,
• growth through Bruch's membrane,
• optic nerve extension,
• lack of pigmentation, and
• histologic characteristics such as mitotic activity and cell
type
76. • Surgical Care:
• Enucleation -large (basal diameter >15 mm and height
>10 mm) and complicated tumors, which compromise
visual function, and where other therapies tend to fail.
• Because of potential release of malignant cells into the
bloodstream, manipulation of the globe should be kept to
a minimum.
• Particular care has to be taken to avoid perforation of the
globe during surgery. If transcleral extension is found, the
tumor should be removed in one piece, pre enucleation
radiotherapy is given, followed by cryotherapy of the
involved orbital soft tissues.
77. • Plaque brachytherapy
• alternative to enucleation for medium size posterior uveal
melanomas (<10 mm in height and <15 mm in diameter).
• Iodine 125(USA), preferred because of its
• lower energy emission (lack of alpha and beta rays),
• good tissue penetration, and
• its commercial availability.
• Mechanism damage of DNA in cancerous cells and tumor
vessels, with consequent tumor necrosis and regression.
• A computerized calculation is used to determine the dose
and duration of plaque application for a radiation delivery
of approximately 400 Gy to the base and 80-100 Gy to the
apex of the tumor.
78. • A margin of 2 mm over the largest tumor basal dimension
is adequate.
• Postoperative imaging confirmation of correct plaque
localization is required. Radioactive plaques are left in
place for 3-7 days.
• The goal of successful treatment is to achieve arrest of
tumor growth or regression in size.
• Local recurrence, usually requiring enucleation, occurs at
a rate of about 12-16%.
• complications
– cataract,
– rubeosis,
– scleral necrosis,
– keratopathy,
– radiation retinopathy, and
– optic neuropathy but at a reduced rate compared with external
beam irradiation
79. • External beam irradiation using charged particles,
either protons or helium to treat medium size choroidal
melanomas (<10 mm in height and <15 mm in diameter),
although it has been used for larger tumor It has similar
indications and success rates to plaque brachytherapy.
• radiopaque tantalum rings usually are sutured to the
sclera to serve as reference markers for alignment of the
radiation beam. A collimated beam delivers about 70 Gy,
divided usually in 5 sessions.
• Vital ocular structures are avoided through careful
positioning of the head and eye. Irradiation causes
damage of DNA in cancerous cells and tumor vessels,
with consequent tumor necrosis and regression..
• survival rate comparable to those treated by
enucleation.
80. • Small choroidal melanomas, when they are located
away from the fovea and are less than 3 mm in thickness
• Laser photocoagulation - to seal off the blood supply to
the tumour and destroy it
• Transpupillary thermotherapy
• Photodynamic Treatment
– Animal study
– melanin precursors become extremely phototoxic in
melanoma tumor cells when activated with certain
light sources
– No additional phototoxic agents are administered
– differs from conventional photodynamic therapy in
that it uses pulsed, longer wavelength light
81. The Collaborative Ocular Melanoma Study
• initiated in 1986
• long-term, multicenter, randomized
controlled trials
• conducted in 43 clinical centers located in
major population areas of the United States
and Canada
82. SMALL MEDIUM LARGE
Type of study Nonrandomized, Prospective Prospective
prospective randomized randomized
follow-up clinical trial clinical trial
study
Number of 204 1317 1003
patients
Size of Apical height: Apical height: Apical height:
melanomas 1.0 to 2.5mm 2.5 to 10.0mm 10.0 mm or
included in Largest basal Largest basal larger
study diameter: 5mm diameter: 5 to Largest basal
16 mm diameter:
16 mm or
larger
83. • Findings of study
• baseline characteristics associated with growth of small
tumors
– 21% grew by 2 years
– 31% grew by 5 years
– Characteristics associated with growth: initial tumor, thickness
and diameter, presence of orange pigment, absence of drusen,
absence of RPE changes
• No clinically or statistically significant difference in
survival rates between I125 Brachytherapy vs
enucleation for treatment of medium tumors for up to 12
years after treatment
• No significant difference in survival rates between Pre-
enucleation radiation vs. enucleation alone for treatment of
large tumors . 5-year survival rates - 60 percent
• Age and largest basal diameter of the tumor are the only
factors that affect prognosis
