This document summarizes various types of intraocular and ocular adnexal tumors. It describes benign tumors of the conjunctiva including dermoids, dermolipomas, papillomas, and granulomas. Malignant conjunctival tumors discussed include squamous cell carcinoma and malignant melanoma. Uveal tract tumors covered are iris melanoma, ciliary body melanoma, choroidal melanoma, and retinal tumors such as retinoblastoma. Other topics summarized are tumors of the optic nerve, lid, lacrimal sac, and orbit. For each tumor type, characteristics, diagnosis and treatment are concisely outlined.

1. INTRAOCULAR TUMOR
NUR FARRA NAJWA BINTI ABDUL
AZIM
082015100035

2. CONJUNCTIVA
CONGENITAL i. Dermoid—
Dermoids are choristomas.
It is yellow-grey in colour.
• smooth, solid round lesions
• at corneal margin on the outer side of limbus.
• Epibulbar dermoid may be associated with other
congenital anomalies of the body.
• It consists of epidermoid, epithelium, sebaceous glands a
• stationary in growth.
• large size cause corneal astigmatism.
• It is dissected off and replaced by lamellar corneal graft for
cosmetic region.

4. Cont.
• Dermolipoma
– situated at the outer canthus usually.
– consists of fibrous tissue and fat.
– removed surgically.

5. Cont.
2. Papilloma
• at inner canthus, fornices and the limbus.
• It should be removed as it may turn malignant.

6. Cont.
3. Simple Granuloma
• consists of exuberant granulation tissue.
• polypoid
• at the chalazion site when chalazion is
insufficiently scraped.
• It should be completely removed by scissors.

7. Cont.
4. Squamous Cell Carcinoma
• It occurs at the limbus or
lid margin (transitional zone).
• It spreads over the surface
and into the fornices.
• penetrate the eyeball.
• It is removed and the base
is cauterized by diathermy.
• If it recurs, or in extensive
lesions the eye is enucleated.

8. Cont.
5. Pigmented Tumours
i. Naevi or congenital mole is rarely malignant.
ii. Precancerous melanosis is a diffusely spreading
pigmentation of the conjunctiva seen in elderly persons.
iii. Malignant melanoma occurs typically at the limbus in
old people.
– It spreads over the surface of the eyeball.
– Recurrences and metastases occur elsewhere in the body
commonly.
– It is treated by enucleation of the globe or exenteration
of the orbit in cases of extraocular extension

9. UVEAL TRACT
MALIGNANT MELANOMA
• The common primary malignant tumour
• derived from the sheaths of Schwann of
sensory nerves

10. Cont.
1. IRIS- Malignant melanoma of iris .
• occurs as an isolated nodule which grows rapidly.
• penetrate through the limbus.
• It consists of pigmented or unppigmented
spindle-shaped cells or round cells.
• Treatment
• Wide iridectomy is performed which should
include the tumour mass.
• Excise the eye if there is recurrence.

12. Cont.
2. Ciliary Body
• Clinical features and
treatment are same
as that for malignant
melanoma of
choroid.

13. Cont.
3. Choroid
• It is commonly seen in 40-60 years age group
• Unilateral and single usually.
• Types
– Pedunculated melanoma
– Flat melanoma.

14. Cont.
Stages
i. Quiescent stage—It is symptomless usually.
ii. Glaucomatous stage—The clinical features of
raised tension are present.
iii. Extraocular extension—There is local spread
into the orbital tissue. iv. Metastasis—Blood-
borne metastasis to central nervous system and
liver occur commonly.

16. cont.
Diagnosis
1. Defective vision—There is no perception of light
probably.
2. Retinal detachment—The retinal detachment site
is rounded and fixed at the summit of the tumour
with patches of black uveal pigment over it.
3. A peculiar pattern of dilated blood vessels is seen
over the tumour mass.
4. Transillumination indicates the tumour mass.

17. Cont.
Investigations
1. Ultrasonography (B-
scan)—It clearly
denotes the tumour
mass.
2. Fluorescein
angiography—It outlines
the abnormal blood
vessels.
3. Radioactive tracer—
Neoplastic tissue has an
increased rate of
phosphate (P32) uptake.

18. Cont.
Treatment
1. Small tumours (less than 10 mm in diameter)
1. plaque brachytherapy external beam radiation,
2. cryotherapy,
3. laser ablation
4. transpupillary thermotherapy.
2. Medium-sized tumours (10-15 mm in diameter)
1. plaque or external beam radiation.
3. Enucleation
4. Orbital exenteration—It is advised in cases of
orbital spread and extraocular extension.

19. Cont.
Prognosis
• If untreated, it is fatal within 5 years.
• The prognosis is fair if the tumour is small (less
than 10 mm in size) and intraocular.

20. RETINA
TUMOURS OF THE RETINA RETINOBLASTOMA (GLIOMA RETINAE)
• common congenital malignant tumour
• early childhood.
• proliferation of neural cells which have failed to evolve normally.
INCIDENCE
1. It is common in infants and young children (2-4 years).
2. Remain quiescent and manifest in the 5th or 6th year or even later.
3. Heredity—autosomal dominant inheritance (chromosome-13 and
trisomy-21).
4. It is unilateral usually but about 25% cases, the second eye is
affected.

21. Cont.
PATHOLOGY
• from neurosensory retina.
• two different types of cellular characteristics.
1. Poorly differentiated-small to medium sized round cells
with large hyperchromatic nuclei and scanty cytoplasm
along with necrosis.
– It resembles the nuclear layer of the retina.
2. Well-differentiated tumour cells may be arranged in two
special forms:
• a. Rosettes
• b. Fleurettes.

23. Cont.
Rosettes Fleurettes
i. Flexner-Wintersteiner rosettes—
These rosettes are specific for
retinoblastoma. Single layer of
columnar cells are arranged around a
clear central lumen.
b. These are highly specific for
retinoblastoma. There is flower
bouquet type aggregation of tumour
cells. 3.
ii. Homer Wright rosettes—These are
not specific for retinoblastoma. The
tumour cells are arranged radially
around a central core of neural fibres.
This type of cell arrangement can also
be seen in neuroblastoma and
medulloepithelioma.
PSEUDORESSETE
Sometimes tumor cell clustered around blood vessel in necrotic
retinoblastoma

27. Cont.
SYMPTOMS
1. Leucocoria—“amaurotic cat’s eye” is usually noticed by
the parents. common presentation. It is due to reflection
of light from the yellow-white mass in the retrolental
area.
2. Squint usually convergent
3. Nystagmus is seen in bilateral cases.
4. Severe-pain may be present due to raised intraocular
pressure.
5. Enlargement of the globe with protrusion of the
eyeball is a common complaint.

29. Cont.
SIGNS
1. Multiple polypoid masses are seen in
the fundus. There may be
haemorrhages on the surface of the
tumour at times.
2. The tumour mass may spread into
the vitreous cavity.
3. Pseudohypopyon with esotropia
(convergent squint) may be the
presenting clinical feature.
4. The second eye may show a larger
retinal tumour mass surrounded by
numerous punctate satellites.

30. Cont.
TYPES
1. Glioma exophytum—It grows outwards
separating the retina from the choroid. It
resembles detachment of retina.
2. Glioma endophytum—It grows inwards
towards the vitreous

31. Cont.
Stage 1 The quiescent stage—
It lasts from six months to one year.
Stage 2 The glaucomatous stage—
There is enlargement of the globe,
proptosis and severe pain associated with
raised intraocular tension.
Stage 3 The stage of extraocular extension—
The tumour bursts through the limbus
followed by rapid growth
Stage 4 The stage of metastasis—
The tumour spreads by the:
i. Lymphatic
ii. Direct extension
iii. Bloodstream

33. Cont.
DIAGNOSIS
1.There is raised intraocular tension usually.
2. Lactic dehydrogenase and phosphoglucose
isomerase enzyme levels are raised in the aqueous
humor.
3. Plain X-ray orbit —Calcification occurs in 75%
cases of retinoblastomas.
4. It is a progressive condition.
5. Ultrasonography, computerised tomography (CT
scan) and MRI confirm the diagnosis.

34. Cont.
TREATMENT
1. Enucleation
2. Excision of the eye is done.
3. Exenteration of the orbit
4. Radiation and chemotherapy—
Retinoblastoma is a highly radiosensitive
tumour.
5. Cryotherapy

35. Cont.
Prognosis
1. Bad- if untreated.
2. Fair- if the eye is removed before the onset of
extraocular extension.
3. Poor- if the optic nerve is involved, tumour cells
are undifferentiated and in 3rd and 4th clinical
stages.
4. Spontaneous regression- with massive necrosis
and calcification may occur occasionally due to the
immunological mechanisms.

36. OPTIC NERVE
GLIOMA
• It is a congenital tumour occurring
• age group of 2-5 years.
• It is slow growing and self-limiting with good prognosis.
MENINGIOMA
• It occurs in middle-aged women usually.
• There is early visual loss and proptosis.
• The prognosis is good because of the slow growth and
peripheral situation of the tumour.

38. LID
TUMOURS OF THE LIDS
1. The xanthoma,
2. Naevus,
3. Haemangioma
4. Neurofibroma

39. -- Benign Tumour
XANTHOMA
• These are often bilateral, symmetrical, slightly raised yellow plaques situated
near the inner canthus.
• They are common in elderly women and diabetics with excess cholesterol
formation.
HAEMANGIOMA
• It occurs as a localized capillary angioma or cavernous angioma.
• The lid may be affected along with the facial angioma as in Sturge-Weber
syndrome.
MOLE (NAEVUS)
• It occurs at the lid margin, involving both skin and conjuncitva.
• It is removed by excision as it may turn malignant.
NEUROFIBROMA
• It is usually of plexiform type affecting the upper lid as in von
Recklinghausen’s disease or generalized neurofibromatosis.
• The thickened nerves can be felt through the skin as hard cords.

40. -- Malignant Tumour
SQUAMOUS CELL CARCINOMA
• Affects the elderly persons usually.
• It is seen at the edge of the lid
(transition zone) where the
characteristic of epithelium
changes.
• It starts as a nodule which
ulcerates.
• The preauricular lymph nodes are
often enlarged.
• Treatment—it is excised
completely.

41. Cont.
BASAL CELL CARCINOMA (RODENT ULCER)
• It is the most common malignant tumour
of the lid.
• It is seen in the lower lid near the inner
canthus usually.
• It is locally malignant.
• The epithelial growth spreads under the
skin in all directions.
• It invades and destroys the lids, orbit and
bone.
• Treatment—if small, it is excised
completely. If large, it can be treated with
radiation therapy.

42. LACRIMAL
BENIGN TUMOUR
• Pleomorphic adenocarcinoma (mixed tumour).
• Occurs in middle life.
• Slowly progressive painless swelling in the upper
lid.
• Result in mechanical ptosis.
• It should be excised.
MALIGNANT TUMOUR
• Short history and pain.
• Radical surgical removal is necessary.