TUMORS OF THE

EYE
By
Dr. Amr Mounir
Lecturer of ophthalmology
What are ocular tumors ????
Ocular tumors can appear on the eyelids, in the eye
(conjunctiva, choroid or retina) and in the orbit (the cavity
that houses the eyeball).
 Early diagnosis and treatment is necessary. Time •
is of the essence to save vision, the eye and even
the life of the patient in the most serious cases.
There are several types of benign and malignant tumors •
that affect the eye and its different structures

Include :
1- Tumors of the Orbit
2- Tumors of the Eyelid
3- Tumors of the Conjunctiva
4- Tumors of the Uveal tract
5- Tumors of the Retina
Orbital Tumors
A lesion in the orbit? •

Decide whether it is an ocular lesion OR •

a non-ocular lesion, i.e. is it involving the globe or •

involving the structures outside the globe.

If it is a non-ocular lesion, see the lesion is involving •

which space.
Intraconal space tumors
 Venous vascular malformation
 Dermoid
 Metastases
 Capillary and cavernous hemangioma
 Lymphoma
 Rhabdomyosarcoma
 Optic nerve lesions
◦ Optic neuritis
◦ Optic nerve glioma
◦ Optic nerve meningioma
 Schwannoma of 3rd, 4th and 6th cranial nerve
Extraconal space tumors :

 Dermoid
 Lacrimal gland tumors
 Metastases
 Schwannoma of the trigeminal nerve
DERMOID CYSTS

usually occur in children and make up 4% to 6% 

of orbital tumors.

Painless mass, free from the skin, with variable 

ocular displacement.
Mostly located near the lacrimal fossa or nasal bone. •

Grow slowly, remodeling adjacent bones or sutures. •

RADIOLOGIC FEATURES

The intensity of dermoid cyst is similar to that of fat on 

MRI and CT, making diagnosis easy.
On CT

appear as well defined low attenuating (fat 

density) lobulated masses. Calcifications may
be present in the wall. Enhancement is
uncommon.

The central cavity may appear heterogeneous 

as a result of keratin and other cystic debris.
This is a coronal CT scan demonstrating a dumbbell dermoid that straddles the
right lateral orbital wall. A bony channel in the lateral orbital wall connects the two
lobes. Note that the deep lobe displaces many of the lateral intraorbital structures.
This coronal CT image without contrast demonstrates a lateral dermoid cyst with
the characteristic hyperdense cyst wall and hypodense cyst cavity.
RHABDOMYOSARCOMA

Most common primary malignant tumor of the 

orbit in children.

A highly malignant tumor. 

Av age of presentation:7 yrs. 

M>F 
presents with rapidly progressive exophthalmos. •

Originates from extra-ocular muscles, •

nasopharynx, or paranasal sinuses.

Usually present in the superomedial orbit and •

may produce bone destruction.
On CT,
A bulky aggressive-looking mass. •

isodense or slightly hyperdense. •

shows uniform enhancement. •

Contrast-enhanced axial CT image through orbits demonstrates
right proptosis due to large, lobular, intraorbital mass.
Image at lower level demonstrates invasion of right maxillary sinus
as well as extension through lateral orbital wall,consistent with the
aggressive nature of this tumour.
(A) Axial and (B) coronal CT images with contrast medium.
There is a large mass in the superior right orbit which is
difficult to separate from the extra-ocular muscles. There is
deformity of the posterior wall of the globe and marked
proptosis. The mass shows uniform contrast enhancement.
ORBITAL METASTASIS

6% of orbital tumors. 

Most retrobulbar metastases are extraconal in 

location,

subsequently encroach on the intraconal 

compartment as they increase in size.
when large,produce infiltrating poorly marginated •
masses.

originate mostly from the greater wing of the •

sphenoid, resulting in bone destruction.
In children, the primary lesions are most •
commonly Ewing's sarcoma and neuroblastoma.

In Ewing's sarcoma, proptosis is usually unilateral •

with sudden onset & accompanying hemorrhage.
The presentation in neuroblastoma is similar; •
however, it is bilateral in 50% of cases.

Other pediatric malignancies that metastasize to •

the orbit are testicular tumors and leukemias.
In adults, the primary tumor is usually breast or •
lung carcinoma.

Tumor metastasizes more frequently to eye than •

the orbit (8:1 ratio).

The orbital metastases may be the initial •

manifestation of the lung, GIT, thyroid, or renal
Cancer.
 In adults, an infiltrative retrobulbar mass and •
enophthalmos is characteristic of scirrhous
carcinoma of the breast.
RADIOLOGIC FINDINGS
Metastases often are diffusely infiltrating and 
have indistinct margins. Less frequently, they
are well circumscribed.

On CT, these lesions are isodense or 

hyperdense, and enhance.
Metastatic prostate carcinoma. Axial CT image (A) through
orbits demonstrates small lytic lesion of left lateral orbital wall in a patient
with prostate carcinoma. Soft-tissue windows (B) demonstrate contiguous
extension of soft tissue into lateral extraconal compartment with
medial displacement of the lateral rectus muscle.
Metastasis diffusely involving medial rectus muscle.
Coronal T1-weighted, fat-saturated MRI shows infiltration of the retrobulbar fat on right and
infiltration of the superior orbit on left. B,C,Axial T1-and T2 MRI show swelling and infiltration by
metastasis of the left orbit and eyelid.
Lacrimal gland tumors

- Very rare tumors

- Histopathologically classified into 2 types
1- Epithelial
2- Non Epithelial
- Most common benign type Mixed benign
tumor,
- Most common Malignant type adenoid
cystic adenocarcinoma
TUMOURS OF EYELIDS•
Benign tumours:
Epithelial tumours •
Melanocytic tumours •
Adnexal cystic lesions •
Sweat gland origin •
Hair follicle origin •
Miscellaneous lesions •
Epithelial:
Squamous papilloma: polyp, •
skin tag
Appearance: they can be
pedunculated & sessile.
Histopathology: fibrovascular •
core & hyperkeratosis of
overlying epidermis.
t/t : simple excision. •
KERATOCANTHOMA : •

a solitary,rapidly •
growing nodule on sun
exposed areas center
crater filled keratin &
rolled out margins

They gradual resolves •

on their own with
minimal scarring.
MELANOCYTIC TUMORS
CONGENITAL

derived from nevocytes •

PRESENT AT BIRTH & •
PRESENTS WITH HAIR
KISSING NEVUS- cause is •
nevocyte migration before
seperation of lids
Only 5% changes to malignancy… •
Acquired:-
Junctional nevus:arise in childhood & •
typically begin as a lightly pigmented,
nevocytes present in at the lid margin or
elsewhere.

Cells migrate to dermis—

thickness+pigmentation=compound nevus

EPIDERMIS:--1)LENTIGO SIMPLEX: small,

brown macules.
may be solitary/multiple-
associated with perioral lesions

.
SOLAR LENTIGO:-brownish macules found over sun •
exposed area
Slowly increases in size •

Freckles: a brown macule “increased melanin in the •

epidermal basal layer”.
MALIGNANT TUMORS
SIGNS OF MALIGNANCY:
SLOW,PAINLESS GROWING LESION •

ULCERATION,BLEEDING & CRUSTING •

PIGMENTARY CHANGES •

DESTRUCTION OF NORMAL EYELID MARGIN •

CENTRAL ULCERATION •

LOSS OF VELLUS HAIR. •

BASAL CELL CARCINOMA
It is a malignant cutaneous tumor. •
BCC: these does not metastasize. •
Rodent ulcers:- it invades tissue extensively. •

RISK FACTORS:-UV radiation,fair skin,unable to tan,exposure to •

arsenic.
C/F:- avg age 60 yrs •
tumor often arises in the lower lid & medial canthus
Morphological forms: nodular:shiny,firm,pearly nodule with small dilated •
vessels
it grows 0.5 cm in 1-2 yrs
nodulo-ulcerative:central ulceration,pearly raised
rolled edges dilated & irreguar vessels “it erodes”.
sclerosing :it infiltrates laterally beneath the
epidermis as an indurated plaque,the margins are difficult to delineate.
 HISTO:-cells proliferate downwards
Exhibits palisading at the periphery of a
tumour lobule of cells.
SQUAMOUS CELL CARCINOMA:
SCC arises in prickle layer. •
Second most common eyelid tumour •
Risk factors: •
UV rays, exposure to sunlight, immunosuppression, albinism, •
chronic skin lesions
C/F:-Nodular or plaque like lesions, ulceration,rolled,out •
edges, greyish white keratinisation.
Order of frequency: medial canthus—upper lid—lateral •
canthus.
 HISTO:arises from epidermis
Atypical epithelial cells with
prominent nuclei
Well differentiated tumours show
“keratin pearls”
SEBACEOUS CARCINOMA:
Arises from the sebaceous glands & is more common than •
BCC & SCC.
C/F:-nodule on a eyelid, yellowish,loss of lashes •
Shows intraepithelial spread—’’pategoid spread” •
Mimic a lot like chalazia •
Shows lymphatic & hematogenous spread. •

histology: -cells with pale foamy vacuolated lipid containing •

cytoplasm with hyperchromatic nuclei.
Malignant melanoma:
Common in fair skinned •
ulcerates& C/F: eyelid masses which show pigmentation, •
bleeds.
superficial spreading or maligna. May be nodular, •

atypical melanocytes within the dermis. histology: •

Conjunctival tumors
-Primary corneal tumors are exceedingly rare, and tumors
affecting the cornea are usually extensions of Conjunctival
tumors.
-Any Conjunctival cell type can potentially lead to one or
more particular Conjunctival tumor(s). The majority of
Conjunctival tumors are benign. Malignant tumors of the
conjunctiva are relatively rare.
-Conjunctival epithelial (including melanocytic) tumors are
more common than Conjunctival stromal tumors.
Some Nomenclature

Dysplasia: – is mitosis occurring in a disordered fashion

in suprabasalar epithelial cells. If the full thickness of
epithelial cell layers are dysplastic it is known as
“carcinoma in situ” or the closest thing to malignancy
without being malignant. A malignancy would include
breaking through the basement membrane and obtaining
access to the circulation and thereby potentially causing
metastasis.
Acanthotic: – means thickened epithelium •
Leukoplakia: – keratin formed on mucosal •
surfaces in white plaques
 CONJUNCTIVAL TUMOURS
1. Benign
• Naevus
• Papilloma
• Epibulbar dermoid
• Lipodermoid
2. Pre-malignant
• Primary acquired melanosis ( PAM )
• Intraepithelial neoplasia (carcinoma in situ
3. Malignant
• Melanoma
• Squamous cell carcinoma
• Kaposi sarcoma
• Lymphoma
Classification of Epidermal Tumors of the Conjunctiva
1- Non-melanocytic Benign:
- Squamous papilloma
Keratotic plaque (and actinic (solar) keratosis which is •
Believed to be due to prolonged ultraviolet exposure.
listed below under premalignant, •
Reactive Hyperplasia (pseudoepitheliomatous •
hyperplasia)
Non-melanocytic Premalignant and malignant •

-Actinic (solar) keratosis (See Keratotic Plaque above) •

-Conjunctival intraepithelial neoplasia (CIN) •
 Naevus

• Presents in first two decades • Most frequently juxtalimbal

• Sharply demarcated and slightly• 30% are almost non-pigmented
elevated
 Papilloma
Pedunculated Sessile

• Presents in childhood or early adulthood

• Presents in middle age
• Infection with papilloma virus • Not caused by infection

• May be multiple and bilateral • Single and unilateral

 Epibulbar dermoid
Signs Association

• Presents in childhood • Occasionally Goldenhar

• Smooth, soft mass syndrome
• Usually juxtalimbal
 Lipodermoid

• Presents in adulthood
• Soft, movable, subconjunctival mass
• Most frequently at outer canthus
 Intraepithelial neoplasia
(carcinoma in situ)
Signs Progression

• Presents in late adulthood • May become vascular and extend ont

cornea
• Juxtalimbal fleshy avascular mass
• Malignant transformation is uncommo
Squamous cell carcinoma
 Primary acquired melanosis (PAM)
Signs Types

• Presents in late adulthood • PAM without atypia is benign

• Unilateral, irregular areas of flat, • PAM with atypia is pre-malignant

brown pigmentation
• May involve any part of conjunctiva
 Conjunctival melanoma
From PAM with atypia From naevus Primary

• Most common type • Very rare • Solitary nodule

• Sudden appearance of • Sudden increase in size• Frequently juxtalimba

nodules in PAM or pigmentation but may be anywhere
 Treatment of Conjunctival melanoma
Localized tumour Diffuse tumour Orbital recurrence

• Excision • Excision of nodules • Excision and

radiotherapy
• Adjunctive cryotherapy • Adjunctive cryotherapy or
mitomycin C • Exenteration
 Squamous cell carcinoma
Signs Progression

• Arises from intraepithelial • Slow-growing

neoplasia or de novo
• May spread extensively
• Presents in late adulthood
• Rarely metastasizes
• Frequently juxtalimbal
 Kaposi sarcoma

• Affects patients with AIDS

• Vascular, slow-growing tumour of low malignancy
• Very sensitive to radiotherapy
• Most frequently in inferior fornix
 Lymphoma

• Usually presents in adulthood

• Benign or malignant
• Salmon-coloured, subconjunctival infiltrate
INTRAOCULAR
TUMOURS
Intraocular tumours
Uveal tract tumours – iris, ciliary body, and choiroidal
melanomas

Retinal tumours – Retinoblastoma

Metastatic tumours
Uveal tract tumours
IRIS
Neavi – Benign – flat to slightly elevated lesions . •

Melanoma – 5-10% of uveal melanomas – age 50-60 •

years, elevated and more pigmented
Treatment: local resection +/- radiotherapy – good
prognosis
Uveal tract tumours
Iris naevus Iris melanoma
Ciliary body melanomas
10% of uveal melanomas – only visualised when pupil •
is widely dilated.

Presentation depends on size and location – lens •

subluxation or localised lens opacities, sentinal
vessels, erosion into anterior chamber, posterior
extension  retinal detachment.

Ultrasound may be necessary •

Treatment – enucleation, local resection, radiotherapy •

Prognosis is poor as presentation is usually •
late
Ciliary body
melanoma
Picture on left showing
black mass in red reflex

Picture on right showing

tumour pushing on and
displacing the lens
Choroidal melanoma/ Malignant melanoma
85% of uveal melanomas, most common during •
sixth decade of life
Raised pigmented oval shaped •
mass(occasionally amelanotic)
Commonly asymptomatic – found on routine •
fundal examination – may cause decreased
visual acuity or defect in visual field – can cause
an exudative retinal detachment, secondary
glaucoma, cataract or uveitis
Choroidal melanoma MM
Peripheral MM MM at macula
Diagnosis of choroidal melanoma
Ocular ultrasound – gives a measurement of size of •
tumour particularly the height, also differentiates between
a normal retinal detachment (RD) and RD caused by
tumour
MRI of orbits and optic nerves to check for extra scleral •
spread
Fluorescein angiography, shows increased vascularity •
and leakage from tumour
Differential diagnosis of choroidal
melanoma
Retinal detachment •
Metastatic tumour •
Neovascular ARMD •
Large choroidal Naevus •
Medical evaluation of patient with
choroidal melanoma
Exclude a metastatic tumour – lung tumours in males and •
breast tumours in females are the commonest tumours
that spread to the eye
Detection of distant metastases – choroidal melanomas •
spread to the liver and lung
Chest x ray, abdominal ultrasound, MRI, •
mammography
Management of choroidal melanoma

Consider visual acuity of involved eye •

Size, location, extent and apparent •
activity of involved eye
State of fellow eye •
General health and age of patient •
Treatment of choroidal melanoma
Radioactive plaques •
Enucleation •
Cyclotron – generated charged particle radiation •
Photocoagulation •
Trans pupillary thermotherapy •
Localised resection •
Exenteration •
Palliation with chemotherapy •
Retinoblastoma
Tumours of primitive photoreceptor cells of •
eye.
Most common primary malignant •
intraocular tumour in childhood – one in
20,000 live births
Retinoblastoma
Average age at diagnosis 18 months – majority •
diagnosed by three years of age

Early treatment can save vision, and the life of •

the patient

Other primary tumours such as sarcomas may •

develop in about 10% of patients
There are two forms of the disease, •
a heritable form and non-heritable form
Retinoblastoma
Children present most commonly with

Leucocoria

and/or

Squint
Left convergent squint and leucoria
Differential diagnosis

1. Persistent hyperplastic primary vitreous (PHPV):

Congenital developmental anomaly of the eye resulting
from failure of the embryological, primary vitreous and
hyaloid vasculature to regress, where by the eye is shorter,
develops a cataract, and may present with whitening of the
pupil.
2. Coats disease: a typically unilateral disease
characterized by abnormal development of blood vessels
behind the retina, leading to blood vessel abnormalities in
the retina and retinal detachment to mimic retinoblastoma
3. Toxocara canis: •
an infectious disease of the eye associated with •
exposure to infected puppies, which causes a retinal
lesion leading to retinal detachment.
4. Retinopathy of prematurity (ROP): •
associated with low birth weight infants who receive •
supplemental oxygen in the period immediately after birth,
it involves damage to the retinal tissue and may lead to
retinal detachment.
Retinoblastoma
Fundal picture
Pinkish white raised lesions with blood vessels on
surface (may show calcification on U/S)
or
Retinal detachment
Microscopic picture:
Undifferentiated elements appear as collections of small, •
round cells with hyperchromatic nuclei; differentiated
elements include Flexner-Wintersteiner rosettes, Homer
Wright rosettes, and fleurettes from photoreceptor
differentiation.
Retinoblastoma
1/3 are bilateral –these present earlier than unilateral •
tumours.
Most bilateral tumours are familial, autosomal dominant. •
Only 6% of patients have a positive family history.
Patients with familial retinoblastoma have a 50% risk of
transmitting the disease to their children.

Sporadic cases usually uni-ocular but can be bilateral. •

Retinoblastoma
These tumours spread trans sclerally to orbits, via
the optic nerves to the brain and via blood to
bone marrow

Investigations – ultrasound, CT, MRI,

Retinoblastoma
Treatment –
Enucleation, •
radiotherapy (external beam, •
plaque),
thermotherapy, •
cryotherapy, •
chemotherapy •
Retinoblastoma
Very important

Any child under 5 years of age who has Leucocoria, a

squint or loss of vision must be examined to out rule
Retinoblastoma
Metastatic Tumours
More common than primary malignancies
Common primary site in women – breast
In men – bronchus
Less common sites kidney, testis, GIT.
May present with decreased visual acuity in one or
both eyes
Solitary or multiple creamy white placoid or oval •
lesions.
Treatment: Chemotherapy and/or radiotherapy •
Metastatic tumour from breast cancer
Thank you