2. Capillary haemangioma.
the most common tumor of orbit and
periorbital area in childhood
girls are affected more
maybe small or big.
Histology; Varying-sized small vascular channels
without true encapsulation
Signs;
Superficial lesions are bright red.
Presebtal tumor are more dark, large tumors
may enlarge and change in color during
crying and straining.
Deep tumors may present with proptosis.
5. Cavernous haemangioma;
Is the most common benign orbital tumor in adults.
Histology;
Endothelial-lined vascular channels of varying size
separated by fibrous septa.
Presentation;
Is in the 4th-5th decade with slowly progressive
unilateral proptosis.
Signs
-Axial proptosis.
-Transient blurring of vision.
Diagnosis;
CT
Treatment;
Surgical excision.
6. Pleomorphic lacrimal gland adenoma;
benign mixed-cell tumor , is the most common epithelial
tumor of the lacrimal gland.
Histology;
glandular tissue with squamous differentiation and keratin
production,and myxoid tissue
Presentation ;
In the 2nd-5th decade with painless progressive proptosis or
swelling in the supero-lateral part of orbit.
Signs;
Smooth firm non-tender mass in the lacrimal gland fossa.
Posterior extension may cause proptosis, ophthalmoplegia.
With the palpepral lobe tumor there is upper lid swelling.
Treatment;
Surgical excision.
7. Lacrimal gland carcinoma;
It is a rare tumor which carry high morbidity and
mortality.
Histological types.
a-Adenoid –cystic type.
b-Pleomorphic adenocarcinom..
c-Mucoepidermoid.
d-Squamus cell.
Presentation;
In the 4rth-6th decade with
Pain.
H/O excision of benign adenoma.
Long standing proptosis.
8. -Signs;
A mass in the lacrimal area.
Posterior extension may give to conjunctival
congestion and ophthalmoplegia
Hypo aesthesia in the area supplied by Lacrimal
nerve.
Optic disc swelling and choroidal folds.
Investigation;
CT , Biopsy,
Neuro;ogical assessment
Treatment;
Radical surgery.
Radiotherapy.
9. Optic nerve glioma;
It is slow-growing pilocytic astrocytoma
, which affect young girls and
occasionally young adults.
Presentation;
In the end of first decade with
progressive visual loss followed later by
proptosis;
10. Signs;
Optic nerve dysfunction.
optic nerve head first swollen then becomes
atrophic.
Investigation;
CT , MRI.
Management;
1. Observation in patients with no evidence of growth.
2. Surgical excision.
3. Radiotherapy combined with chemotherapy in cases
of itracranial extension.
11. Arise from meningiothelial cells of
arachnoid villi.
More common in females.
13. Lymphoma
Lymphoma of ocular adenexa constitute for about 8% of
extra nodal lymphoma.
Presentation;
In the 6th to8th decades.
Signs;
The involvement is usually bilateral.
Anterior lesions may be palpated.
Lymphoma may be confined to conjunctiva or lacrimal
gland.
Treatment;
Radiotherapy or localized lesions.
Chemotherapy for disseminated disease.
14. Rhabdomyosarcoma
It is the most common childhood
primary orbital malignancy.
Presentation;
Is in the first decade (7 years) with
rapidly progressive proptosis.
15. Signs;
In most cases the tumour is retro bulbar.
A palpable mass and ptosis in about 1/3rd of cases.
Swelling and injection of overlying skin.
Parameningial tumour show bony destruction, lymph node
involvement.
Investigation;
CT.
Chest X ray
LFT
Bone marrow biopsy
Lumbar puncture,and skeletal survey.
Treatment;
Radiotherapy followed by chemotherapy.
Surgical excision for recurrent or radio resistant tumor.
16.
17. Basal cell carcinoma
BCC is the commonest human malignancy and it affect
elderly patients and is the most prevalent lid tumour. More
common at lower lid then medial canthus then upper lid
then lateral canthus, it is slow growing and locally invasive
Histology;--The tumour arise from basal cell of the epidermis
which proliferate deeply. with palisading of cells at the
periphery of a tumour lobule.
Clinical types;--The main clinical features of epidermal
malignancy are ulceration, lack of tenderness, induration ,
irregular border and destruction of lid margin.
Nodular.
Noduloulcerative.
Sclerosing.
Treatment;
Surgical excission.
Radiotherapy.
18.
19.
20.
21. Squamous cell carcinoma
It is less common but more aggressive than BCC.
It may spread to regional lymph nodes,
Intracranial cavity.
Clinical types;
Nodular.
Ulcerating.
Cutaneous horn.
Treatment;
Surgical excision.
Radiotherapy.
22. Kaposi sarcoma
Is a vascular tumour which affect typically patients
with AIDS.
Histology;
Proliferation of spindle cells, vascular channels,
mitotic figures and inflammatory cells within the
dermis.
Signs;
The tumour is a pink , red-violent to brown lesion.
Treatment;
Radiotherapy.
Excision
23.
24. .
Conjunctival naevus
Benign, uncommon, usually unilateral.
Histology;
A-Junctional; Show nests of naevus cells at
epithelial-sub epithelial junction.
B-Compound; show naevus cells at epithelial
subepithelial junction and within the stroma.
Presentation;
During 1st tow decades of life with ocular
irritation or pigmented lesion.
25.
26. Signs;
Sharply demarcated flat or slightly elevated pigmented
intraepithelial bulbar lesion.
Common sites;
Juxtalimbal.
Plica and caruncle.
Signs of potential malignancy;
An unusual site.
Extension to the cornea.
Sudden increases in size or pigmentation.
Development of vascularity except in children.
Treatment;
Excision for cosmetic purposes or suspicious of
malignancy.
27. Malignant melanoma of conjunctiva
Histology;
Melanoma cells within the epithelium,
subepithelium, stroma.
Presentation;
Usually in the 6th decade.
Signs;
Black or gray nodule contaning dilated vessels.
Common site at the limbus.
There is also Amelanotic tumours.
Treatment;
Surgical excision with cryotherapy.
Radiotherapy for deep tumours.
29. Iris naevus
Histology;
Show proliferation of melanocytes in the
superficial iris stroma, predominantly
spindle cells.
30. Iris naevus
Typical Diffuse
• Diameter usually less than 3 mm
• Occasionally mild distortion of pupil and
ectropion uvea
• Obscures iris crypts
• May cause ipsilateral hyperchromic
heterochromia
• May be associated with Cogan-Reese
syndrome
• Pigmented, flat or slightly elevated
31. Iris melanoma
Histology;
The majority are composed of spindle cells and
are of low grade malignancy.
Presentation;
In the 5th-6th decade .
A pigmented or non-pigmented nodule at least 3
mm in diameter and 1mm thick
Usually located in the inferior iris with pupillary
distortion, ectropion uvea, and occasionally with
localized cataract.
32. Iris Melanoma
1. Very rare - 8% of uveal melanomas
2. Presentation - fifth to sixth decades
3. Very slow growth
4. Low malignancy
5. Excellent prognosis
33.
34. Iris melanoma
• Usually pigmented nodule at
least 3 mm in diameter
• Invariably in inferior half of iris
• Occasionally non-pigmented
• Surface vascularization
• Angle involvement may cause
glaucoma
• Pupillary distortion, ectropion
uveae and cataract
35. Differential diagnosis of iris melanoma
Large iris naevus distorting
pupil Leiomyoma
Adenoma of pigment
epithelium
Primary iris cyst Ciliary body melanoma
eroding iris root
Metastasis to iris
37. Treatment of iris melanoma
Small tumour
- broad iridectomy
Angle invasion by tumour
- iridocyclectomy
Non-resectable tumour
- radiotherapy or enucleation
38. Ciliary body melanoma
It comprise 5% of uveal melanoma.
Presentation;
Is in the 6th decade with visual symptoms but
occasionally it may be discovered incidentally.
Signs;
A tumor can be visualized by fundoscopy or gonioscopy.
Dilated episcleral blood vessels.
A dark epibulbar mass b extra ocular extension.
Exudative retinal detachment by posterior extension.
Investigation;
Triple-mirror contact lens.
Transillumination.
Ultrasonagraphy.
Biopsy.
42. Choroidal naevus
Histology;
The tumour is composed of proliferation of
spindle cell melanocytes.
Prresentation;
Mostly asymptomatic and detected by routine
examination.
Signs;
Usually post-equatorial oval or round slate-blue
or gray lesion.
Surface drusen may be present.
43.
44. Investigations.
Fluorescein angiography--Most naevi are a
vascular and pigmented giving rise to
hypofluoresence.
Indocyanine green.
Ultrasonography.
Treatment
Typical naevi do not require special follow up.
Suspicious naevi require baseline fundus
photography and U/S and special follow up.
45. Choroidal melanoma
It is the most common intraocular
malignancy in adults and account for 90%
of all uveal melanoma.
Histological Types;
Spindle cell.
Mixed cell melanoma.
46. Presentation;
Peak at around the age of 60 years.
An asymptomatic tumour is detected by routine
fundus examination.
A symptomatic tumour causing decreased visual
acuity , metamorphopsia,visual field loss.
Signs;
An elevated, subretinal, dome-shaped mass may
be pigmented or non pigmented.
clumps of orange pigment are frequently seen in
the RPE overlying the tumour.
49. Treatment;
Brachytherapy with ruthenium-106 or iodine-125.
( tumours <20 mm in basal diameter)
Charged particle irradiation with charged particles
as protons. (tumors unsuitable for brachytherapy
either because of size or posterior location)
Radiotherapy
Transpupillary thermotherapy. (small pigmented
tumour)
Trans-scleral choriodectomy.
Enucleation (in cases of large tumour, Optic disc
invasion, Extensive involvement of the ciliary body
or angle, irreversible loss of vision.
Exentration; Removal of the globe and orbital
contents when orbital disease can not be
controlled by radiotherapy.
50. Metastatic Choroidal tumour--
The choroid is the most common ocular
tissue as a site for secondary tumors.
Secondary tumors of the choroid are more
common than primary.
The most frequent primary site is the
breast in females and bronchus in males
Other less common primary sites are GIT,
kidney and skin melanoma.
51. Malignant intraocular tumour that originates in the
outer nuclear layer of retina. Most cases appear
before 3 years of life(average age of diagnosis 18
month), unilateral cases being diagnosed at around
24 months and bilateral cases before 12 months
Most common intraocular tumour of childhood .
30 – 25% of cases are bilateral .
Family history present in 6% cases(autosomal
dominant) and remaining 94% are sporadic
52. Chromosomal abnormality deletion of the long
arm of chromosome-13 and with trisomy-21
all bilateral cases are related to hereditary and 5
– 10% of unilateral are hereditary also.
Other cases are thought to arise by chromosomal
mutation .
Orbital sarcoma may occur in the irradiated orbits
after enucleation of retinoblastoma
Osteogenic sarcoma of femur and skull in patients who
have survived from bilateral retinoblastoma
Trilateral retinoblastoma is bilateral retinoblastoma
with pinealoblastoma
53. Amaurotic cat s eye(leukocoria)- white mass or
greyish red reflex in pupil (most common
presentation about 56- 60%)
red painful eye {tumor necrosis }.
Squint/strabismus {in 20% cases }.
Enlargement of the globe {2ry glaucoma }.
Proptosis due to orbital involvement
Endophthalmitis/anterior uveitis
Visual difficulties
Nystagmus in bilateral case
62. Mid dilated pupil non reactive .
White mass behind lens .
Ophthalmoscopically ---- white or pale pink mass with
newly formed blood vessels on its surface .
The tumor may grow outwards separating retina from
choroid , with/without total exudative RD & difficult to
see, {Glioma exophytum } and retinal vessels are seen
over the tumor.
grows inwards toward vitreous, seen as white or pink
colored mass with calcium deposits –{Glioma
endophytum }. The retinal vessels are not seen on the
tumor surface.
63. Growth of the tumor ---- enlargement of globe --- iris
pushed forward against trabecular meshwork ----
increase IOP --- 2ry glaucoma .
strabimus
Increase level of aqueous humor lactate dehydrogenase
.
Extraocular extension of the tumor to optic nerve ,
break through limbus as large fungating mass .
Metastasis ---- locally direct to intra ocular tissues and
extra ocular tissues , orbital bones & CNS via optic
nerve and regional pre-auricular & cervical lymph nodes
{ blood metastasis is rare to bone ,liver ,lungs }
64.
65.
66.
67.
68.
69.
70.
71.
72.
73.
74. 1-quiescent stage(6-12 months)
2-Glaucomatous stage
3-Stage of extra ocular extension
4-Stage of metastasis
76. Direct/indirect ophthalmoscopy
X-ray of orbit for calcification and erosion of optic foramen
Ultrasound(B-scan)
CT-scan
Aqueous humour paracentesis for cytology & LDH enzyme
assay
Carcino embryonic antigen
ELISA-for toxocara endophthalmitis
Lumber puncture and bone marrow aspiration for metastasis
Fine needle aspiration cytology(FNAC)when diagnosis is not
confirmed
Fluorescein angiography
77.
78.
79. Depends on size , location and number or tumors
Small tumor can be treated with radiotherapy(cobalt-
60) sometimes combined with chemotherapy .
Cryotherapy or photocoagulation(xenon arc)—for small
tumours .
Large tumor ---- Enucleation with long piece of optic
nerve.
Orbital involvement—exentration
Advanced cases with distant metastasis—
chemotherapy(cyclophosphamide & vincristine)
Genetic counselling—if two or more cases in the family
If retinoblastoma is unilateral ---- annual exam of
fellow eye {after treatment of affected eye }.
If bilateral ---- Enucleation of more advanced eye and
medical therapy in other eye .
80.
81. Treatment;
Small tumours (not more than 3mm)
1. photocoagulation.
2. Cryotherapy.
3. Chemotherapy.
Medium-sized tumours (up to 12mm )
1. Brachytherapy.
2. Chemotherapy.
3. External beam radiotherapy.
Large tumours.
1. Chemotherapy.
2. Enucleation.
83. Ciliary body melanoma
• Rare - 12% of uveal melanomas
• Presentation - 6th decade
• May be discovered by chance
• Prognosis - guarded
84. Signs of ciliary body melanoma
• Sentinel vessels • Extraocular extension
• Erosion through iris root • Lens subluxation or cataract
• Retinal detachment
85. Treatment options of ciliary body melanoma
1. Iridocyclectomy
- small or medium tumours
2. Enucleation
- large tumours
3. Radiotherapy
- selected cases
86. Choroidal melanoma
• Most common primary intraocular
tumour in adults
• Most common uveal melanoma -
80% of cases
• Presentation - sixth decade
• Prognosis - usually good
88. Choroidal melanoma (2)
• Surface orange pigment (lipofuscin) is
common
• Mushroom-shaped if breaks through
Bruch’s membrane
• Ultrasound - acoustic hollowness,
choroidal excavation and orbital
shadowing
89. Differential diagnosis of choroidal melanoma
Large choroidal naevus Metastatic tumour
Localized choroidal
haemangioma
Choroidal detachment Choroidal granuloma
Dense sub-retinal or
sub-RPE haemorrhage
90. Treatment of choroidal melanoma
1. Brachytherapy
- less than 10 mm elevation and 20 mm diameter
2. Charged particle irradiation
- if unsuitable for brachytherapy
3. Transpupillary thermotherapy
- selected small tumours
4. Trans-scleral local resection
- carefully selected tumours less than 16 mm in diameter
5. Enucleation
- very large tumours, particularly if useful vision lost
6. Exenteration
- extraocular extension
91. Histological classification of uveal melanomas
Spindle cell (45%) Pure epithelioid cell (5%)
Mixed cell (45%) Necrotic (5%)
92. Poor Prognostic Factors of Uveal Melanomas
1. Histological
• Epithelioid cells
• Closed vascular loops
• Lymphocytic infiltration
2. Large size
3. Extrascleral extension
4. Anterior location
5. Age over 65 years
93. Typical choroidal naevus
• Common - 2% of population
• Round slate-grey with
indistinct margins
• Surface drusen
• Flat or slightly elevated
• Diameter less than 5 mm
• Location - anywhere
• Asymptomatic
94. Suspicious choroidal naevus
• Diameter more than 5 mm
• Elevation 2 mm or more
• Surface lipofuscin
• Posterior margin within
3 mm of disc
• May have symptoms due
to serous fluid
95. Circumscribed choroidal haemangioma
• Presentation - adult life
• Dome-shaped or placoid,
red-orange mass
• Commonly at posterior
pole
• Between 3 and 9 mm in
diameter
• May blanch with external
globe pressure
• Surface cystoid retinal
degeneration
• Exudative retinal
detachment
• Treatment - radiotherapy
if vision threatened
96. Diffuse choroidal haemangioma
Typically affects patients with Sturge-Weber syndrome
Diffuse thickening, most marked at
posterior pole
Can be missed unless compared with normal
fellow eye as shown here
97. Choroidal metastatic carcinoma
Most frequent primary site is breast in women and bronchus in both sexes
• Fast-growing, creamy-white,
placoid lesion
• Most frequently at posterior pole
• Deposits may be multiple
• Bilateral in 10-30%
98. Choroidal osseous choristoma
• Very rare, benign, slow-growing
ossifying tumour
• Typically affects young women
• Orange-yellow, oval lesion
• Well-defined, scalloped,
geographical borders
• Most commonly peripapillary
or at posterior pole
• Diffuse mottling of RPE
• Bilateral in 25%
99. Melanocytoma
• Affects dark skinned
individuals
• Usually asymptomatic
• Most frequently affects
optic nerve head
• Black lesion with feathery
edges
