This presentation gives a brief idea about ocular tumors and its management

1. TUMORS OF THE
EYE
By
Dr. Amr Mounir
Lecturer of ophthalmology

2. 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).

3. •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.

4. •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

5. Orbital Tumors

6. •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.

9. 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

10. Extraconal space tumors :
 Dermoid
 Lacrimal gland tumors
 Metastases
 Schwannoma of the trigeminal nerve

11. 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.

12. •Mostly located near the lacrimal fossa or nasal bone.
•Grow slowly, remodeling adjacent bones or sutures.

14. RADIOLOGIC FEATURES
The intensity of dermoid cyst is similar to that of fat on
MRI and CT, making diagnosis easy.

15. 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.

16. This is a coronal CTscan demonstrating a dumbbell dermoid that straddles the
right lateral orbital wall.Abony channel in the lateral orbital wall connects the two
lobes. Note that the deep lobe displaces many of the lateral intraorbitalstructures.

17. This coronal CTimage without contrast demonstrates a lateral dermoid cyst with
the characteristichyperdense cyst wall and hypodense cyst cavity.

19. RHABDOMYOSARCOMA
Most common primary malignant tumor of the
orbit in children.
A highly malignant tumor.
Av age of presentation:7 yrs.
M>F

20. •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.

22. On CT,
•A bulky aggressive-looking mass.
•isodense or slightly hyperdense.
•shows uniform enhancement.

23. 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.

24. (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.

25. ORBITAL METASTASIS
6% of orbital tumors.
Most retrobulbar metastases are extraconal in
location,
subsequently encroach on the intraconal
compartment as they increase in size.

26. •when large,produce infiltrating poorly marginated
masses.
•originate mostly from the greater wing of the
sphenoid, resulting in bone destruction.

27. •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.

28. •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.

29. •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.

30. •In adults, an infiltrative retrobulbar mass and
enophthalmos is characteristic of scirrhous
carcinoma of the breast.

31. 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.

32. 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.

33. Metastasis diffusely involving medial rectus muscle.

34. CoronalT1-weighted,fat-saturated MRI showsinfiltrationof the retrobulbarfat on right and
infiltrationof the superiororbit on left. B,C,AxialT1-andT2 MRI show swellingand infiltrationby
metastasisof the left orbit and eyelid.

35. 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

37. •TUMOURS OF EYELIDS

39. Benign tumours:
•Epithelial tumours
•Melanocytic tumours
•Adnexal cystic lesions
•Sweat gland origin
•Hair follicle origin
•Miscellaneous lesions

40. Epithelial:
•Squamous papilloma: polyp,
skin tag
Appearance: they can be
pedunculated & sessile.
•Histopathology: fibrovascular
core & hyperkeratosis of
overlying epidermis.
•t/t : simple excision.

41. •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.

42. CONGENITAL
MELANOCYTIC TUMORS
•derived from nevocytes
•PRESENT AT BIRTH &
PRESENTS WITH HAIR
•KISSING NEVUS- cause is
nevocyte migration before
seperation of lids
•Only 5% changes to malignancy…

43. 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
.

44. •SOLAR LENTIGO:-brownish macules found over sun
exposed area
•Slowly increases in size
•Freckles: a brown macule “increased melanin in the
epidermal basal layer”.

45. MALIGNANT TUMORS

46. SIGNS OF MALIGNANCY:
•SLOW,PAINLESS GROWING LESION
•ULCERATION,BLEEDING & CRUSTING
•PIGMENTARY CHANGES
•DESTRUCTION OF NORMAL EYELID MARGIN
•CENTRAL ULCERATION
•LOSS OF VELLUS HAIR.

47. 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.

49. HISTO:-cells proliferate downwards
Exhibits palisading at the periphery of a
tumour lobule of cells.

50. 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.

52. HISTO:arises from epidermis
Atypical epithelial cells with
prominent nuclei
Well differentiated tumours show
“keratin pearls”

53. 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.

55. Malignant melanoma:
•Common in fair skinned
•C/F: eyelid masses which show pigmentation,ulcerates&
bleeds.
•May be nodular,superficial spreading or maligna.
•histology:atypical melanocytes within the dermis.

56. Conjunctival tumors

57. -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.

58. 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.

59. •Acanthotic: – means thickened epithelium
•Leukoplakia: – keratin formed on mucosal
surfaces in white plaques

60. 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
CONJUNCTIVAL TUMOURS

61. Classification of Epidermal Tumors of the Conjunctiva
:1- Non-melanocytic Benign
Squamous papilloma-

62. •Keratotic plaque (and actinic (solar) keratosis which is
Believed to be due to prolonged ultraviolet exposure.
•listed below under premalignant,

63. •Reactive Hyperplasia (pseudoepitheliomatous
hyperplasia)

64. •Non-melanocytic Premalignant and malignant
•-Actinic (solar) keratosis (See Keratotic Plaque above)
•-Conjunctival intraepithelial neoplasia (CIN)

65. Naevus
• 30% are almost non-pigmented
• Most frequently juxtalimbal
• Sharply demarcated and slightly
elevated
• Presents in first two decades

66. Papilloma
Pedunculated Sessile
• Presents in middle age
• Not caused by infection
• Single and unilateral
• Presents in childhood or early adulthood
• Infection with papilloma virus
• May be multiple and bilateral

67. • Presents in childhood
• Smooth, soft mass
• Usually juxtalimbal
• Occasionally Goldenhar
syndrome
Epibulbar dermoid
Signs Association

68. Lipodermoid
• Presents in adulthood
• Soft, movable, subconjunctival mass
• Most frequently at outer canthus

69. Intraepithelial neoplasia
(carcinoma in situ)
• Juxtalimbal fleshy avascular mass
• May become vascular and extend ont
cornea
• Presents in late adulthood
• Malignant transformation is uncommo
Signs Progression

70. Squamous cell carcinoma

71. Primary acquired melanosis (PAM)
• PAM without atypia is benign
• PAM with atypia is pre-malignant• Unilateral, irregular areas of flat,
brown pigmentation
• May involve any part of conjunctiva
• Presents in late adulthood
Signs Types

72. Conjunctival melanoma
From PAM with atypia
• Sudden appearance of
nodules in PAM
From naevus
• Sudden increase in size
or pigmentation
Primary
• Solitary nodule
• Frequently juxtalimba
but may be anywhere
• Very rare• Most common type

73. Localized tumour
• Excision
Treatment of Conjunctival melanoma
Diffuse tumour
• Excision of nodules
Orbital recurrence
• Excision and
radiotherapy
• Adjunctive cryotherapy or
mitomycin C • Exenteration
• Adjunctive cryotherapy

74. Squamous cell carcinoma
• Rarely metastasizes
• Arises from intraepithelial
neoplasia or de novo
• Frequently juxtalimbal
• Slow-growing
• Presents in late adulthood
• May spread extensively
Signs Progression

75. Kaposi sarcoma
• Most frequently in inferior fornix
• Affects patients with AIDS
• Vascular, slow-growing tumour of low malignancy
• Very sensitive to radiotherapy

76. Lymphoma
• Salmon-coloured, subconjunctival infiltrate
• Usually presents in adulthood
• Benign or malignant

77. INTRAOCULAR
TUMOURS

78. Intraocular tumours
Uveal tract tumours – iris, ciliary body, and choiroidal
melanomas
Retinal tumours – Retinoblastoma
Metastatic tumours

79. 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

80. Uveal tract tumours
Iris naevus Iris melanoma

81. 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

82. Ciliary body
melanoma
Picture on left showing
black mass in red reflex
Picture on right showing
tumour pushing on and
displacing the lens

83. 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

84. Choroidal melanoma MM
Peripheral MM MM at macula

85. 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

86. Differential diagnosis of choroidal
melanoma
•Retinal detachment
•Metastatic tumour
•Neovascular ARMD
•Large choroidal Naevus

87. 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

88. 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

89. Treatment of choroidal melanoma
•Radioactive plaques
•Enucleation
•Cyclotron – generated charged particle radiation
•Photocoagulation
•Trans pupillary thermotherapy
•Localised resection
•Exenteration
•Palliation with chemotherapy

90. Retinoblastoma
•Tumours of primitive photoreceptor cells of
eye.
•Most common primary malignant
intraocular tumour in childhood – one in
20,000 live births

91. 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

92. Retinoblastoma
Children present most commonly with
Leucocoria
and/or
Squint

93. Left convergent squint and leucoria

95. 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

96. •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.

97. Retinoblastoma
Fundal picture
Pinkish white raised lesions with blood vessels on
surface (may show calcification on U/S)
or
Retinal detachment

101. 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.

103. 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.

104. 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,

105. Retinoblastoma
Treatment –
•Enucleation,
•radiotherapy (external beam,
plaque),
•thermotherapy,
•cryotherapy,
•chemotherapy

106. 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

107. 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

108. Metastatic tumour from breast cancer

109. Thank you