
Ocular Tumors
Tumors may arise form extraocular structures such as the lacrimal gland,
optic nerve, lids and conjunctiva, or from intraocular structures such as the
choroid and the retina.
The more common extraocular tumors include mixed lacrimal gland
tumors, basal cell carcinoma of the eyelids, squamous cell carcinoma of the
conjunctiva and glioma of the optic nerve.
Benign intraocular tumors are very rare; the more common malignant
tumors may arise from the choroid (malignant melanoma) and the retina
(retinoblastoma).
Tumors of the eye and orbit are classified as following:
I. Epibulbar tumors (cornea, conjunctiva and sclera)
A. Benign as: conjunctival nevus.
- Limbal dermoid cyst
B. malignant as: squamous cell carcinoma
(epithelioma)
- Basal cell carcinoma
C. Secondary malignancy from bronchogenic carcinoma and cancer of the
breast.
II.Optic nerve tumors:
1. Glioma of the optic nerve: it arises from the glial tissue of the optic nerve.
It occurs before the age of 10 years causing unilateral axial proptosis, drop
of vision and optic atrophy.
2. Meningioma of the optic nerve: it arises from the meninges of the optic
nerve. It occurs in middle age, it is a locally malignant tumor. It causes
unilateral axial proptosis, drop of vision and optic atrophy.

INTRAOCULAR TUMORS
MALIGNANT MELANOMA OF THE CHOROID (MM)
It is the most common primary intraocular tumor in adults. The average
age of patients with choroidal melanoma is 50 years.
Clinical features:
The tumor appears as a unilateral, elevated, brown oval shaped mass. It
may be mottled with dark brown or black pigments
Early it may be asymptomatic unless it arises near the macular area.
Later the patient may complain of diminution of vision
Management:
• Observation: should be considered for
1. Eyes with small lesions since differentiation from nevus may be
difficult.
2. Single eye with slowly growing tumors.
• Local resection for small peripheral lesions.
• Radioactive plaques fixed to the globe may be suitable for small or
medium sized tumors near the posterior pole.
• Enucleation is indicated for large melanomas.
• External beam irradiation prior to enucleation to lessen the risk of
melonostic lesions.
• Transpupillary thermotherapy (TTT).

RETINOBLASTOMA
It is the most common primary intraocular malignancy in childhood.
It occurs in about 1 in 20,000 live births. It almost always presents prior
to the age of 3 years.
A positive family history is present in only 6% of cases; while sporadic
cases account for the remaining 94% of cases. The tumor may be bilateral in
20% of the cases.
Clinical features:
Retinoblastoma may present as:
1.
Leukocoria (from latin leukos = white and coria = pupil): white
pupillary reflex, accounts for 65% of cases.
2.
Strabismus (squint): if the tumor affects the macular area.
1.
Secondary glaucoma.
2.
Proptosis due to extraocular extension.
3.
Accidental discovery on routine examination.
Diagnosis: is based on
1.
Ophthalmoscopy: with maximal pupillary dilitation, preferably under
general anesthesia. It is of extreme importance to examine both eyes due to
the relatively high incidence of bilaterality.
2.
X- ray may show tumor calcification
3.
Ultrasonography
4.
CT scans.

Treatment:
1.
Photocoagulation for small central tumors.
2.
Transscleral cryotherapy or diode laser for small peripheral tumors.
3.
Radiotherapy or Trans- pupillary thermotherapy for medium tumors.
4.
Systemic chemotherapy.
5. Enucleation with excision of a long stump of the optic nerve is the
treatment of choice for large tumors affecting one eye.
Differential diagnosis of leukocoria in infants:
1.
Retinoblastoma.
2.
Congenital cataract.
3.
Retinopathy of prematurity: failure of vascularization of the peripheral
retina in prematures exposed to high oxygen tension in incubators with
subsequent fibrosis and tractional retinal detachment.
4.
Persistent Hyperplastic primary vitreous: congenital anomaly with
fibrosis of the anterior vitreous.
5.
Coats disease: unilateral extensive leakage from the retinal vessels
resulting in large masses of subretinal lipids.
6.
Retinal dysplasia: chromosomal defect resulting in development of a
disorganized retina.

EXTRAOCULAR TUMORS
MIXED LACRIMAL GLAND TUMOR
A locally malignant tumor of the lacrimal gland usually affecting middle –
aged patients. It is more common in females with a ratio of 3:2. It invades
the surrounding structures and extends along the nerve sheaths causing
severe pain.
PHAKOMATOSIS
This is a group of diseases characterized by the appearance of multiple
small benign proliferations. These proliferations affect the eyelids,
intraocular structures, face as well as other organs of the body. The most
common diseases are:
-
Neurofibromatosis: multiple neurofibromata of the skin subcutaneous
nerves and patches of skin pigmentation (café – au- lait – patches)
-
Sturge – weber syndrome: hemangioma affecting the eyelid and half
the face with severe secondary glaucoma and buphthalmos.
-
Tuberus sclerosis: multiple adenoma sebaceum of the face and
multiple retinal astrocytomas.
-
Von hippel – Lindau disease: solitary retinal angioma with multiple
cysts affecting the cerebellum pancreas and other organ.