Block 20 | Module 5 | Lesson 5

OCULAR AND ORBITAL TUMORS

Jeremy Saquian, MD
MODULE 5
May 5, 2017; 8:00-10:00 AM

OUTLINE Most hordeola are caused by staphylococcal

I. Eyelid Tumors infections, usually Staphylococcus aureus.
A. Hordeolum Hordeolum result of an acute bacterial infection of
B. Chalazion one or more eyelid glands
C. Dermoid Cyst Hordeolosis - several glands affected at the same time
D. Syringoma Staphylococcus aureus is a common cause
E. Hidrocystoma
External Hordeolum appears on the margin of the
F. Xanthelasma
eyelid where the sweat glands are located
G. Nevus
H. Papilloma Internal Hordeolum is usually only revealed by everting
I. Inverted Follicular Keratosis the eyelid and is usually accompanied by a more
J. Squamous Cell CA severe reaction such as conjunctivitis or chemosis
K. Basal Cell CA A meibomian gland infection, along the tarsal plate,
II. Conjunctival Tumors sometimes you cant see a nodule or pustule in those
A. Common benign
located in the posterior lamella of the eyelid.
a) Dermolipoma
b) Pyogenic Granuloma
B. Squamous cell CA
III. Orbital Tumors
A. Hemangioma
B. Rhabdomyosarcoma
C. Optic Nerve Glioma
IV. Non Tumor Lesions
D. Orbital Pseudotumor
E. Graves Ophthalmopathy
BOOK |UPPER CLASS NOTES | LECTURERS AUDIO
Patient presented with axial proptosis left eye,
relative pupillary defect was seen. On fundoscopy, it Example of an external hordeolum. Yellowish in color
showed optic atrophy. Oblique skull x-ray showed with pus inside. Pain even on blinking. Tenderness
enlargement of the optic canal. Optic Nerve glioma upon palpation.
Base the diagnosis on what is the most common in TREATMENT
this age, sex, location, presentation and signs Warm compress - three or four times a day for 1015
Histopathology diagnosis - most definitive diagnosis minutes.
EYELID TUMORS Incision and drainage- hypodermic needle (if internal:
Hordeolum and chalazion are the most common apply anesthetic evert eyelid and look for the softest
inflammatory processes which present with a mass in portion of the pus then puncture and press).
the eyelid. Sometimes there are cases that you will
HORDEOLUM (STYE) puncture and the content is not liquid but more
Acute inflammatory reaction or infection of a solid type so you cannot express the content
Causative agents are Gram Positive organisms easily. Determine always that it contains pus.
Abscess of the gland of Zeiss If small: Topical Antibiotic Ointment (may or may not
Acute infection of the gland of Zeiss, Moll (external) - give, but if you want a faster resolution and to
in front of tarsal plate minimize inflammatory reaction, give this with
Meibomian gland (internal hordeolum) - within tarsal dexamethasone)
plate If really big: Oral Antibiotics: gram (+) coverage.
Painful and tender on palpation cloxacillin, penicillin, cephalosporin, other broad
spectrum antibiotics.
Usually there is the presence of a pustule
In cases that it cant be resolved by antibiotic, do
Pain, redness, and swelling are the principal
Incision and Debridement. If you know that it
symptoms.
contains pus
Conjunctival hyperemia and purulent discharge

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4B | Javier Juanitas Jumawan Labaclado
Block 20 | Module 05| Lesson 05
OCULAR AND ORBITAL TUMORS
CHALAZION
Subacute/chronic inflammatory process, thus
patient will not experience tenderness
Non painful because mass is confined in between
tarsal plate
Product of the non-healing / non-resolving type
of hordeolum
Non tender nodule as a result of an obstructed
meibomian gland, associated and preceded with
internal hordeolum that is not properly treated
(Usually if hordeolum is not treated for more than 4
weeks, it will turn into a chalazion)
On examination the nodule is more quiet, not
hyperemic and without pustule formation
compared to hordeolum
With extrusion of lipid into the soft tissues ->
stimulates lipogranulomatous inflammation and
fibrosis
Due to lipid reaction within the mass
Instead of pus as seen in hordeolum, chalazion
extrudes a meaty substance (granuloma)
Idiopathic sterile chronic or subacute granulomatous
inflammation of a meibomian gland.
It may begin with mild inflammation and tenderness
resembling hordeolum.
Differentiated from hordeolum by the absence of
acute inflammatory signs
Point toward the conjunctival surface, which may be
slightly reddened or elevated.
Large chalazion may press on the eyeball and cause
astigmatism.
Chalazion is a firm nodular bulb within the tarsus
Occur relatively frequently and are cause by a chronic
granulomatous inflammation due to buildup of
secretion from the meibomian gland
Firm painless nodule that develops very slowly
Usually asymptomatic
A nodular lesion without hyperemia. May move with
palpation.
TREATMENT
Warm compress (usually done to children)
Intralesional steroid injection: occasionally done
for lipogranulomatous reaction with the use of
triamcinolone; can cause hypopigmentation
Steroid injection will NOT decrease size
Most of the time because patient has been having
this condition for several months, might as well opt to
do incision and curettage: you first slash the eyelid,
slice tarsal plate, and then scrape the contents of the
chalazion
Heals faster with incision and curettage
Does not respond to antibiotics
DERMOID CYST
Dermoids are not true neoplasms but benign
choristomas arising from embryonic tissue not
usually found in the orbit.
Orbital dermoids arise from surface ectoderm and
often contain epithelial structures such as keratin,
hair, sebum, nail, and even teeth. Most are cystic
and filled with an oily fluid that can incite a severe
inflammatory reaction if liberated into the orbit.
Choristomas - arise from sequestration of epidermal
tissues in deeper layers and the mature normal tissue
growing in an abnormal or not the usual location
Hamartoma is growth of tissue in its normal location
Slow growing, painless
Superficial/deep orbital mass
Classically located superotemporally
Rare superonasally
Why is it that most of the time in ocular conditions,
they always mention this lesion is located
superonasally, superotemporally, etc? Because ocular
tumors when you open the orbit because they are like
Pandora's box. You don't know what's inside unless
you open it. In research and in practical experience,
they always record the site of tumor/ lesion.
Example: Patient has a superotemporal mass noted
since childhood growing slowly non painful and does
not involve any part of the bone. So more or less you
would have an idea that this is just a benign tumor or
probably a dermoid cyst because of the location in
contrast to the same nodule/mass located
superonasally which in practice or clinical
presentation is a rhabdomyosarcoma. Although there
are some instances wherein dermoid cysts can be
located superonasally. So one of the differentiating
characteristic in your differential diagnosis would be
the location of the mass. We always emphasize where
the location is.
Contains ectodermal tissues (hair, keratin, sebaceous
gland components, etc)
Most of the time sebaceous gland secretions and hair
Deep tumors may arise from any orbital sutures
CT scan is needed to define bony fossa formation (not
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OCULAR AND ORBITAL TUMORS
erosion), its full extent and location
Associated with a defect in the bone that may
communicate with the intracranial cavity.
Erosion of the bone is due to pressure effect and
not direct invasion (bony erosion not lytic lesion)
Do CT scan prior excision biopsy because it can
encroach the suture lines and erode the bone causing
thinning of bony parts
Can be present in infancy and childhood causing
pressure effect
Dermoid cysts, with adnexal structures are congenital
but may not become apparent until later in life
When they increase in size or rupture, they elicit a
granulomatous inflammatory response
Non metastatic
Superficial orbital cyst. Movable, non-tender, slow-
growing, classic example may be mobile or non-mobile
TREATMENT
Complete Excision without breaking the capsule to
avoid recurrence, chronic orbital inflammation due to
spillage of contents and fibrous scarring. That is why
you need to be careful when you excise the cyst,
capsule should be intact.
If capsule breaks, contents seep into the sutures
of the skull.
Must request for orbital CT scan to detect the size,
area involved and helps in surgery
SYRINGOMA
Solid nodules that resemble milia - benign
Common in middle-aged young females (30, 40, 50
years old) 35 up
Nodule may contain keratin
Probably eccrine sweat duct in origin, most of the
time is multiple
Located in periorbital area interior eyelid
Autosomal dominant
No predicting factor for having syringoma
Harmless but unsightly
TREATMENT
Excision/Cauterization
If single lesion, you can do excision
If multiple, do electrocauterization
Increase chance of recurrence
Carbon dioxide laser
HIDROCYSTOMA
Pseudoriferous cyst / sweat gland cyst
Apocrine or eccrine in origin
Cystic nodules arising from the eyelid: lower marginal
area or eyelid margin
Contains clear or milky fluid - through slit lamp
examination (clear cystic mass) depending on the
reaction inside the hidrocystoma
Usually along or above the grey line
Usually non-recurrent
Solitary usually but may occur bilaterally
Dome-shaped, soft
Translucent papule or nodule
Clear fluid filled hidrocystoma
TREATMENT
Excision / incision
If recurrent, marsupialization.
Sometimes due to financial constraints, doc does
puncturing as a trial and evacuates the content with
regular steroids they usually dont recur. If it does
recur, however, excision should be done.
XANTHELASMA
Most common form of cutaneous xanthoma
Middle age to older females
Flat, plaque like creamy yellow lesions as a result of
lipid deposition in the dermis
Young person with xanthelasma, do lipid profile
there is possibility that the cholesterol is high but it
may be just coincidental. Increased cholesterol levels
not true all the time.
Xanthelasma is associated with
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hypercholesterolemia. But, not all with
Xanthelasma have hyperlipidemia.
Sharply demarcated lesions
Common location medial aspect
Occurs on the anterior surface of the eyelid but can
sometimes grow inferiorly
Usually bilaterally near the inner angle of the eye.
Xanthelasma represents collections of lipid-containing
histiocytes in the dermis of the lid.
May occur in patients with hereditary hyperlipidemia
or with secondary hyperlipidemia.
In non-hereditary cases, it may predispose patients to
acquiring atherosclerosis later in life.
Approximately two-thirds of patients with
xanthelasma have normal serum lipids.
It is benign but can disfigure image of patient.
No direct correlation between xanthelasma and
hypercholesterolemia
Local fat metabolism disorder that produces
lipoprotein deposits.
Usually bilateral in the medial canthus
Post-menopausal women are most frequently
affected
Higher incidence observed in patients with diabetes,
increased levels of plasma lipoprotein, or bile duct
disorders
Soft yellow white plaques are sharply demarcated
Patients are asymptomatic
Only be removed surgically with high recurrence rate
Flat, plaque like, creamy yellow lesion arising from the
dermis, medial in location but sometimes going down
If your xanthelasma is this big you cannot do excision
entirely on this side because if you create a wedge
shape excision on this side and you do primary closure
you might cause disfigurement in the eyelid. Trapping
of eyelids leads to ectropion. So we do skin scraping?
Sorry hindi clear. Excise, scrape whats inside and put
back the skin in proper place so that you will get skin
that will cause scarring and ectropion.
TREATMENT
Small no need for surgery
If cosmetically disfiguring, excise. Tell the patient that
it returns most of the time.
Recurrence rate at 50% even with excision
Lesions can be excised, cauterized, or treated with
laser surgery.
When you excise deeper, make sure you dont
traverse the orbital septum or you can puncture the
ectropion.
Excision, electrophoric cauterization, carbon dioxide
or argon laser or chemical cauterization using
trichloroacetic acid.
Oculoplastic surgeon should appose skin to
prevent ectropion.
NEVUS (MOLE)
Benign lesions commonly seen in eyelid or eyelid
margins
Pigmented or non-pigmented
Flat or elevated
They initially may be relatively nonpigmented and
show enlargement and increased pigmentation
during adolescence
Many never acquire visible pigment, and many
resemble benign papillomas.
Maybe located in different levels of the skin.
Nevus Evolution Stages:
1. Junctional - lie between dermal and epidermal
junction
2. Compound - up to the dermis or down to the
dermis
3. Intradermal- do excision biopsy because of high
malignant degeneration
Nevus arise from nevus from nevus cells which are
nucleated.
A case of an elevated, pigmented nevus in the lid
margin ->easy to operate since it is <1/4 the length
and it is in the lower eyelid (in superior eyelid you
need to consider several factors: levator aponeurosis
& the size of tarsal plate ->large incision)
In this case, there may be a disruption in vision. So
you excise. You may do inverted House procedure
TREATMENT
Excision is a must (for fast growing and unsightly
tumors)
Depends on location. Remove if it hampers vision.
Always send a specimen for histopathology
Small lid nevus- do surgery, you can do primary repair
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For big lesions, size is >1/4 the eyelid length -> Evaluate the base of the lesion because of its
reconstructive surgery especially in the young (very wide coverage.
tight skin unlike in adults who has elastic & redundant
skin)
Do surgery while it is small
PAPILLOMA
Most common benign epithelial tumor of the eyelid
Single/multiple
Tend to involve lid margin
Sessile or pedunculated
Different from verruca or warty papilloma TREATMENT
This is a true tumor Excision
Misdiagnosed as viral warts Do excision under magnification.
Finger-like projections containing fibrovascular cores. Surgery of upper lid is more dangerous than the
Two types: lower lid because it has more important function
1. Squamous Cell Papillomas like protecting your eyes from drying.
2. Seborrheic Keratoses - occur in middle-aged Some patients dont want to undergo surgery so
and older individuals. doc would just cut the horn every time it grows
They have a friable, verrucous surface and are often if you really want to eradicate this, you have to
pigmented because melanin accumulates in the remove the base
keratocytes. Daw sungay lang sang kanding nga kung gusto mo
In both, fibrovascular cores permeate thickened bali-on, bali-on mo lang
(acanthotic and hyperkeratotic) surface epithelium, BASAL CELL CARCINOMA
giving it a papillomatous appearance. Arises from pluripotent stem cells within the basal
layers of the epidermis and external root sheaths of
hair follicles.
Most common skin (EYELID) malignancy
It comprises about 90% of all eyelid malignancies.
The incidence of basal cell carcinoma of the eyelid
skin increases with age and there is a slight male
preponderance (3:2)
Common in lower lid (but can grow anywhere along
the lids) can also grow in other body parts
Manifest like warts, ragged edge, finger-like Lower lid involves all the lower orbital structures
projections Can be medial or temporal
Risk factors: sun exposure (long exposure), fair skin
TREATMENT (Caucasian) and age (older, 50 years old) are the risk
Excision biopsy especially if it increases in number, factors
they grows from single to multiple side by side Common in fisherman and farmers
Can do grafting or transposition depending on size Presents with: skin ulceration/bleeding, madarosis
INVERTED FOLLICULAR KERATOSIS (loss of eyelashes), telangiectasia, nonhealing skin
Cutaneous horn in the lid margin lesion, skin crusting
Grows rapidly RODENT ULCER Initially presents as a small
Common in older people ulcer with a slightly elevated nodule at the
It represents an irritated seborrheic keratosis periphery and a crater at the middle. After some
(precursor of SCC) time, the ulcer will deepen and not heal, with a
crusted surface. Then, a whitish material can be
Very soft and fragile and it easily breaks off but it found at the central core.
grows back ->better excise up to the its root and Tumor starts in the epithelium (hyperemic) and
submit for biopsy. extend to deeper structures
Edges are typically raised with central crater of pearl
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gray white tissue- rodent ulcer
Less aggressive compared to SCC but cause significant
morbidity if left attended
Basal cell carcinoma usually grows slowly and
painlessly as a nodule that may become ulcerated.
Rarely metastasizes.
Depending on their location, basal cell
carcinomas may produce ectropion, entropion,
lid notching or retraction, dimpling of the
overlying skin, or loss of eyelashes.
BCC is a frequent, moderately malignant,
fibroepithelial tumor that can cause severe local
tissue destruction but very rarely metastasizes
60% of cases are localized on the lower eyelid
Gender is not a predisposing factor
Typical characteristics include a firm, slightly raised
margin with a central crater and superficial
vascularization with an increased frequency to bleed
Loss of the eyelashes in the vicinity of the tumor
always suggests malignancy
This is a hard case because it is located in the medial
area, there are several structure that are involved (the
punctum up and down and the canaliculus up and down
and the lacrimal gland) elevation of the sides with a
small crater / ulcer at the center. There is
neovascularization at the periphery.
TREATMENT
Incisional biopsy - initially get small portion only
because it may be benign
After documented do wide excision by Mohs
micrographic technique (slice for evaluation until
there is space with no tumor) (note for deeper orbital
structure involvement)
Wide excision with margin control meaning you really
have to measure your excision size - extend beyond
1mm
Accomplished by frozen sectioning or Mohs surgery.
So every time you excise in that portion, pathologist
check edges if there are still cancer cells. If none you
can close wound by transposition, skin sampling.
Advancement flap using posteroauricular skin
Will usually do a 3cm lesion which is hard to close
thus you do skin grafting or transposition.
Monitor for recurrence
Lesion is treated by surgical excision. If a radical
procedure is not feasible, only remaining options are
radiation therapy or cryotherapy.
SQUAMOUS CELL CARCINOMA
Arises from the stratified squamous epithelium. It
tends to be locally invasive but rarely metastasizes. It
comprises 510% of all eyelid malignancies, being
much less common than basal cell carcinoma.
Risk factors are the same: UV radiation and fair skin
Risk factors for eyelid squamous cell carcinoma of the
skin include ultraviolet light/actinic damage, chronic
exposure to arsenic, hydrocarbons, radiation, or
immunosuppressive drugs, genetic disorders such as
albinism and xeroderma pigmentosum, and chronic
skin lesions.
more aggressive: penetrates deeper, to the side and
to the bones
clinically you cant differentiate both lesions on initial
exam because they will present with ulceration and
bleeding but this one is located more on the superior
eyelid it is a differentiating factor but not specific
just a matter of localization
occurs more commonly in the conjunctiva
common location superior eyelid when it affects the
lids
greater potential for local extension and regional
metastasis
occurs in older patients almost the same risk factor
with basal cell ca
actinic keratosis is the most common precursor
Potential for local extension and regional metastasis
Grow slowly and painlessly
Often starting as a hyperkeratotic nodule that
may become ulcerated
Benign inflammatory tumors such as
keratoacanthomas may closely resemble
carcinomas
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OCULAR AND ORBITAL TUMORS
SCC is the 2nd most frequently encountered canthus
malignant eyelid tumor
Arises from the epidermis
Grows rapidly and destroys tissue
Metastasizes into the regional lymph nodes
Treatment of choice is complete surgical removal
TREATMENT
Incisional biopsy
Wide excision with close monitoring
If deep tumor, remove everything. Patient may then
wear eye prosthesis or glasses with drawing of eyes.
Surgical excision of the entire lesion whenever In this case, it is located along the limbus or in
possible, either by conventional methods or Mohs the conjunctivo-corneal junction (inferio-laterally) ->
micrographic surgery, followed by reconstruction of lamellar keratectomy meaning partial thickness
the defect. Focal radiation therapy is used excision of the cornea going to the conjunctiva and
occasionally to treat perineural invasion into bone or sclera. This cannot be totally removed because
the orbit, and exenteration is generally performed in sometimes it can go deeper, you just do debulking in
cases with extensive eyelid destruction or massive this cases. It can irritate the eye because there are
orbital invasion. hair that grows (under slit lamp white hair follicles -
Radiologic examination: CT SCAN and MRI prior >daw may puling). Usual indication is more of
excision to document bone involvement. discomfort not of visual problem since the patient
Histopath : Squamous cell CA can still see in this case. In addition to this you do
CONJUNCTIVAL TUMORS lamellar keratectomy/excision because of irritation &
Common benign it is big. Everytime you blink you will have a foreign
Dermolipoma body sensation.
Pyogenic Granuloma TREATMENT
Squamous cell CA most common in conjunctiva as Cautious debulking for very big dermolipoma
compared in lids especially in children as it may cause high astigmatism
DERMOLIPOMA and the visual pathway will not mature
development of amblyopia (lazy eye)
Choristomas - because the conjunctiva is devoid of
dermis and fat tissues Total excision is contraindicated as it can penetrate
beyond the conjunctiva and may cause more
Solid and non-movable
damage/ severe complication because it can
Surface is covered by hair (epidermal in origin)
penetrate beyond the conjunctiva
Smooth solid mass containing hair follicles and fatty
Lamellar keratectomy/excision
deposits
If it involves the cornea- can grow towards the center
Inferotemporally (most of the time)
thus it can distract the visual access going to the pupil
In the slit lamp microscopy, this tumor contains fat,
(threatens the vision) very hard to do surgery
skin, hair that is keratinized
excise (keratoplasty) the cornea and do corneal
Associated with Goldenhars syndrome (a congenital
transplant
condition in which colobomas of the upper eyelid,
If in the limbus or at the periphery do lamellar
epibulbar dermoids, bilateral accessory auricular
keratoplasty & excision by slicing of sclera and
appendages anterior to the ears, and vertebral
conjunctiva
anomalies are associated with asymmetrical facies,
If dermolipoma is behind the limbus, it may
prominent frontal bossing, low hairline, low set ears.
encroach the cornea.
Triad of pre-auricular tags, vertebral abnormality &
Usually not indicated, but at least partial removal may
dermolipoma)
be indicated if the growth is enlarging or is
Dermolipoma is a benign condition.
cosmetically disfiguring.
Its surface is keratinized.
Posterior dissection must be undertaken with
Common congenital tumor that usually appears as a
extreme care (if at all) since this lesion is frequently
smoothly rounded growth in the upper temporal
continuous with orbital fat and extraocular muscles;
quadrant of the bulbar conjunctiva near the lateral
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orbital derangement may cause scarring and conjunctiva that encroaches the cornea sometimes)
complications far more serious than the original
lesion.
Even with debulking-choice of treatment, it can still
grow again.
PYOGENIC GRANULOMA
Pedunculated, arising from the conjunctiva
Can be bulbar or palpebral
Coated with whitish mucus plaque
Big type of SCC. It involves the cornea. Easy to operate as
Associated with a ruptured chalazion
it goes with dissection. Careful cauterization is done and
Manifests with foreign body sensation, frequent
dissection offer good result.
tearing and discharges (mucopurulent)
Mass of exuberant granulation tissue
From trauma, ruptured chalazion/hordeolum ->
granuloma formation and sometimes with whitish
discharges at the surface, becoming a pyogenic
granuloma like an abscess, related to Staph infection
& its surface may be cheese-like (this case is common
in children) or surgery
Small, nodular, delimited aggregation of mononuclear Another SCC with corneal involvement. Notice the
inflammatory cells, or a similar collection of vascularity - feeding vessels. When you scrape, its like
epitheliod cells; usually surrounded by lymphocytes taking scales off the fish.
TREATMENT
Excision
Mitomycin C - patients are responsive to this drug. As
per docs practice no recurrence to his patients
CONJUNCTIVAL LYMPHOMA
Salmon colored patches
Usually primary extranodal B cell neoplasms
Most prevalent subtype is Mucosa Associated
Lymphoid Tissue (MALT)
In this case this is big, you can evert and cut with Presence of salmon colored conjunctival thickening
scissors using a slit lamp machine. Eye patch to avoid in the inferior fornix is often the first sign of
bleeding. Give massive topical steroids. lymphatic disease.
TREATMENT May range from benign lymphoid hyperplasia to
Topical steroids (sometimes intralesional steroids - it malignant lymphomas
will shrink the lesion) Treatment is a combination of radiation and
Excision chemotherapy
Incision
Topical antibiotic
Warm compress
Any part of the body that is cut, send for biopsy.
SQUAMOUS CELL CARCINOMA
Most common primary malignancy of the
conjunctiva
Associated with UV exposure, smoking, polycyclic
hydrocarbons
Initially presents as hyperemia with a flat to slightly
elevated fixed and nontender mass
Mimics a pterygium (wing like fleshy mass in the Patient with primary conjunctival lymphoma. Sometimes
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you cannot excise the entire tumor. observe as long as it doesnt affect the vision or
TREATMENT create ptosis.
Excision biopsy Tender, swells on crying (because of feeder vessels, it
You cannot get all of the tumor, submit sample grows just like in lymphangioma; initially or before
for histochemical testing involution/resolution)
It is confined to the conjunctiva so no systemic With regression (changes color from strawberry red
chemotherapy needed to pale) becomes less vascular, small and fibrotic
Refer to oncologist to evaluate for other malignancies Feeder vessels always present
as it is usually a metastatic extension of another Can be superficial or deep
malignancy - sometimes this can be an initial Abnormal proliferation of endothelial cells forming
manifestation of an abdominal pathology, or any part capillary like vessels
of the body pathology Present few months in life
External beam radiation therapy or Mitomycin C Spontaneous regression over time thus NO
ORBITAL TUMORS hemorrhage.
Hemangioma vascular orbital tumors (cavernous vs Superficial lesions are reddish (strawberry nevus), and
capillary) deeper lesions are more bluish. -Over 90% become
Rhabdomyosarcoma most common primary apparent before the age of 6 months.
malignant tumor in the orbit Enlarge rapidly in the first year of life and regress
Optic Nerve Glioma slowly over 6-7 years.
All orbital tumors displace the globe and cause Lesions within the orbit may cause strabismus or
exophthalmos that is frequently associated with proptosis.
limited ocular motility Involvement of the eyelids may induce astigmatism or
CAPILLARY HEMANGIOMA occlude vision, resulting in amblyopia.
Hemangiomas are the most common benign orbital
tumors in both children and adults
Usually occur in a nasal superior location
Cavernous Hemangioma well encapsulated and
confined
Common in adults
Capillary Hemangiomaspreads sideward without
definite edges
Capillary Hemangioma is the most common
primary orbital and cutaneous tumor in
childhood.
Common in children
Can be treated with cortisone or low dose radiation
therapy
abnormal proliferation of endothelial cells forming Eyelid droops because of the hemangioma, you can still
capillary like vessels observe. Cutaneous capillary like hemangioma. No
Hamartomas - mature normal cells present in normal defined edges. The gauge to treat is if there is severe
locations. Example blood vessels in the skin ptosis and the pupil is covered (that is worse as there is
Can be cutaneous or orbital no stimulation of visual pathway and the patient will
With cutaneous affects eyebrow and upper lid develop amblyopia). Treat before seven years old as
most common primary orbital and cutaneous amblyopia may be treated with eye patching.
tumor in childhood
A case of deeper capillary orbital hemangioma. You have
present few months in life (not visible most of
to intervene as the eyes have closed. Give intralesional
the time after birth because it can be very small
steroids but avoid injecting intravascularly as it may
until few month it will look like strawberry red
cause embolization. We can give steroids from time to
nevus)
time. It depends on how fast the hemangioma responds.
Rapid growth for 6-12 months with gradual resolution
Most regress in 3 years, no need to intervene. Just
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Before intralesional steroids. In this case, because of the
pressure effect of the mass to your cornea, it causes
astigmatism->high astigmatism, you have high visual
disturbance ->will not stimulate the pathway well and
later leads to sensory deprivation amblyopia.
After intralesional steroids, so the inflammation
subsided.
TREATMENT
Needed in severe proptosis or astigmatism or
occlusion
Observe (majority regresses after 3 yrs time)
Indications for surgical intervention before 3 years:
If there is a danger of amblyopia because of
obstruction in the pupillary axis (sensory
deprivation amblyopia) -> sometimes you can
still do
Intralesional steroid (will take few months
before it involute unlike in excision (immediate
effect) do this before the age of 5 years old. In
big hemangioma it takes more the 3 years to
regress spontaneously
If with danger of amblyopia
Intralesional steroid (danger is that you can
inject intravascular steroid -> toxic effect, so use
fine needles and be very careful) Corticosteroids
are thought to have an antiangiogenic effect that
inhibits capillary proliferation and induces
vascular constriction.
Excision
Laser photocoagulation
During surgery you may opt to use laser scalpel or
harmonics to lessen the bleeding at the same time
and at the same time when you cut, there is good
coagulation -> lesser bleeding
Surgery is very bloody
Treatment is only indicated where the tumor
threatens to occlude the visual axis with
resulting amblyopia or where there is a risk of
compressive optic neuropathy
CAVERNOUS HEMANGIOMA
Encapsulated benign mass of dilated vascular spaces
separated by connective tissue stroma
Usually located intraconically
Benign well circumscribed intraconal lesions (at the
apex of the orbit which is at the optic canal, there are
muscles that spread up and so it looks like a cone -
usually it is located within this cone and it is near the
optic nerve)
Intraconal lesion pushes the eyeball forward so the
patient manifests with axial proptosis
Usually located deep
Slowly progressive
Presents as:
Proptosis (axial proptosis if there is intraconal
cavernous hemangioma, due to direct pressure
to the globe - protrusion is straight forward;
usually in adults since it is slowly progressive),
Diplopia (cannot move eyeball well)
Orbital pain
Optic nerve compression (causes visual acuity
disturbance)
Eyeball distortion (because of the pressure)
Optic nerve swelling - papilledema
Motility abnormality
Choroidal folds
Hyperopia (up to +200) mass is pushing the eyeball so
the tendency of the eyeball it will be compressed, the
anterior-posterior diameter will shorten, shortened
eyeball will cause hyperopia.
In fundoscopy examination, using the slit portion
of your fundoscopy when you swing right and
left you will notice bowing or distortion of your
slit. Curving of the slit means that there
something at the back of the eyeball pushing the
retina. This pushing effect causes hyperopia or a
short eyeball.
CT scan shows low flow vascular lesions (because of
absence of feeder vessels compared to capillary
hemangioma)
Retinal striations
Without CT scan and + hyperopia & retinal striations -
suspect cavernous hemangioma
In CT, it is hyperluscent. With black spots. Optic
glioma is solid.
Decreased visual acuity
Usually become symptomatic in middle life.
Unlike capillary hemangiomas, they do not tend to
regress spontaneously.
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OCULAR AND ORBITAL TUMORS
TREATMENT Non- classic location, superio-temporal mass ->
Surgical removal is the treatment displacement is inferio-medial, but if it was a superior-
Less bleeding absence of feeder vessels medial mass, displacement is inferio-temporal. The
But since it is deep, its a bit difficult - can approach it mass pushed the eyeball downward and forward.
from above or at the sides. If its really big do lateral Looks like dermoid cyst but unlike dermoid cyst, it is
orbitotomy then get a portion of the bone to access fast growing.
the lesion. Then return & suture the bone after Usually hyperemic (reddish) while dermoid cyst is no
removing the lesion. color or blackish surface.
Surgical excision is usually successful and is indicated
if the patient is symptomatic.
RHABDOMYOSARCOMA
Most common primary (intraorbital) malignancy of
childhood
RETINOBLASTOMA- most common intraocular
malignancy in children
Tumor arises from pluripotential mesenchyme that
differentiates into striated muscle
Most common type is the embryonal type
Rapidly growing (Presentation is before age 10, and
rapid growth is characteristic)
Manifests with exophthalmos (lateral, downward,
medial, upward displacement depending on location),
There is involvement of the posterior portion and note
swelling and eyelid injection
the possibility of erosion on the medial area up to the
Most commonly found in the superomedial area
ethmoid sinus.
(specifically the ethmoid area)
TREATMENT
when you palpate its really deep, as small as a
Treatment is immediate excision and biopsy (never
corn kernel in early childhood and progresses up
do fine needle incisional biopsy instead do complete
to the size of a small lanzones later on, early
excision because incisional biopsy will result to the
detection for higher chance of survival
spillage and seeding of cancer cells outside that area -
chance of survival depends on how early you
proliferate more and invade the unaffected area)
detect the case and treat
Irradiation and chemotherapy (by radiologist)
May erode the sinuses - ethmoid, frontal (most of the
time) as seen on MRI Overall survival rate is 95% (in early stage and well
Active in regional and direct metastasis manage case)
All cases should be referred to oncologist for staging
The tumor may destroy adjacent orbital bone and
and workup for metastasis before commencing
spread into the brain
treatment
CT scan / MRI shows bony lytic lesions
Patients with distant metastasis - chemotherapy;
The commonest primary malignant tumor in children
patients without distant metastasis - irradiation
Grows very rapidly
Because of accompanying inflammation, a Exenteration for resistant and recurrent cases (last
differential diagnosis should exclude orbital cellulitis resort; simple exenteration - leave the eyelids, up and
down, remove the whole eyeball including the
muscles and nerves. Total exenteration - includes the
eyelids, up and down leaving the bone)
OPTIC NERVE GLIOMA
Affects the optic nerve, benign
Opposite of meningioma. Meningioma in adult has
good survival but poor if it happens in children.
Opposite with optic nerve glioma.
Preschoolers (most common)
Approximately 75% of symptomatic optic nerve
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gliomas become apparent before age 10
Initially complains of visual acuity disturbance hard
or axial proptosis in very large tumors
Loss of vision is hard to catch until you notice the
patient to bump on one side since one eye is a
seeing eye
Loss of vision, gradual axial proptosis, papilledema
(on funduscopy) leading to optic atrophy (chronic
stage)
Relative afferent pupillary defect (RAPD) on flashlight
test and strabismus (because of visual disturbance, so
if your patient have optic nerve glioma on the right,
you have optic atrophy and will not be able to see
well then it cannot fixate -> eye will deviate either
medially or laterally)
Nuclear ganglion and nearby muscles are
affected leading to symptoms such as loss of
vision and strabismus.
25% to 50% are associated with neurofibromatosis 1
(von Recklinghausen disease -> caf au lait spot,
sphenoid bone defects& nerve glioma)
They are low-grade astrocytomas.
Referred to ophthalmology to know presence of optic
nerve glioma or Lisch nodules in the iris
CT scan will show fusiform enlargement of optic
nerve
Well-circumscribed astrocytic tumors derived from
oligodendroglia, interstitial cells and astroglia - if with
cystic areas can expand rapidly
They are low-grade astrocytomas
Those anterior to the chiasm behave in a benign
fashion and may regress spontaneously; those in and
posterior to the chiasm may be more aggressive.
Visual loss and optic atrophy are the most common
signs.
7 y.o, non-classic because it has downward proptosis.
Inability to close eyes causes drying and desiccation
keratitis.
In children, this is the 2nd most common potentially
malignant orbital tumor
In 25% of patients, the optic nerve glioma is
associated with neurofibromatosis (Recklinghausen
disease)
Shows fusiform or a big optic nerve is involved,
pushing the eyeball forward and downward. Egg like
mass pushing eye forward
TREATMENT
Controversial management observe (small lesion
since its benign) or aggressive (if the eyes are already
going outside)
Surgical- excision or debulking
Irradiation or chemotherapy is still controversial as it
is a benign tumor
If with extension towards the brain -> teamwork with
a neurosurgeon, sometimes you will just transect the
optic nerve since its already a blind eye and to
reposition the eyeball. Exposed eyeballs leads to
melting of the cornea and so the eye will burst
Complications: Exposure keratitis and corneal
melting
If progressive tumor growth and visual loss can be
clearly documented, radiotherapy is often effective in
stabilizing or even improving vision. There is a risk of
secondary damage to the central nervous system
such that chemotherapy is advocated as a better
option, but there is little long-term follow-up data. In
blind eyes with marked proptosis, the patients
cosmetic appearance can often be improved by
excising the tumor through lateral orbitotomy.
MENINGIOMA
Lesions arising from meningothelial cap cells of
arachnoid villi
Affects middle aged women
Slowly growing painless exophthalmos type of tumor
Manifests as visual disturbances associated with
minimal proptosis (unilateral), headache, diplopia,
optic nerve changes, Marcus Gunn pupil
Pathologic origin: arachnoid cap cells as evidenced by
histopath with arachnoid villi
Primary from optic nerve sheath
Secondary from inner and outer aspects of the
sphenoid wing
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OCULAR AND ORBITAL TUMORS
Sometimes they will only complain of color vision
abnormality and visual field changes (such
enlargement of blind spot or contracted visual field
(smaller visual field)
On funduscopy: optic nerve usually edematous and
there is engorged papillary and peripapillary vessels
leading to optic atrophy patient will be blind
CT scan - detect intracranial involvement
May appear as tubular, fusiform or globular lesion
Psammomatous meningioma are calcified and
demonstrate as railroad track sign (lucent optic
nerve surrounded by dense tumor) or tram track sign
in axial view and donut sign if coronal view
Sphenoid wing meningioma most common
intracranial meningioma NF2 meningioma from inner
and outer aspect of sphenoid wing
Case of optic nerve sheath meningioma.
Manifest with hyperemia, protrusion of eyeball and
blurring of vision
A meningioma can proceed from the optic nerve or
from within the cranium.
Symptoms can vary: exophthalmos, limited motility,
and compressive optic neuropathy
Hyperostoses are frequent findings in radiographic
studies
16% of meningiomas are associated with
neurofibromatosis
Average age of is 32; 20% are younger than 20
You cannot really differentiate it from orbital
inflammatory syndrome or orbital psuedotumor -> its
important to collate all history and signs and symptoms
Translucent optic nerve with thick and dense tumor.
Tram sign / Rail road track sign: two lines of translucency
and center vacant, tram sign
TREATMENT
Management depends on the size and functional
involvement after biopsy
You can do endovascular embolization
preoperatively
observe (if small) - surgical (if big which causes
visual dysfunction)
Surgical or radiosurgery gamma knife (if vision is
affected)
Radiotherapy (slowing progression) as adjuvant
therapy and primary treatment (decreasing
recurrence)
usually for deep lesions or after incomplete
excision
LYMPHOID TUMORS
Most common primary malignancy in adults
Be alert of lymphoma elsewhere like in the abdominal
cavity
Presents with painless mass grows slowly
Insidious
Less inflammatory changes
Can present as fleshy mass in the orbit (conjunctiva
most of the time)
Lymphomatous tumors of the orbit are divided into
Malignant lymphomas- can have associated
benign reactive lesions, reactive lymphoid
hyperplasia, or pseudolymphoma
Benign polyclonal lesions can have small clones
of B lymphocytes
Monoclonal tumors often remain localized and
behave in a benign fashion.
Manifest with bulging of inferior fornix and
conjunctiva.
Pain is more common with benign inflammatory
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OCULAR AND ORBITAL TUMORS
processes than with malignant lymphomas. Pseudotumor is usually unilateral; when both orbits
Usually associated with other organ lymphoma. are involved, it is more often a manifestation of
Excellent prognosis. vasculitis.
Orbital pseudotumor is a lumphocytic rbital tumor of
unknown origin
Painful, moderately severe inflammatory reaction
with eyelid swelling, chemosis, and unilateral or
bilateral exophthalmos
Involvement of the ocular muscles results in limited
motility with diplopia
CT and MRI images will show diffuse soft tissue
Palpebral conjunctival lymphoma; a thick Salmon pink
swelling.
color tissue. May present just like a chronic inflammatory
A biopsy is required to confirm diagnosis
reaction because of conjunctivitis or because of allergic
reaction -> needs a very keen eye to identify.
TREATMENT
Excision (whole tumor) / incision biopsy (no
occurrence of spillage or seeding)
Stage and identify the lymphoid lesion (histochemical
test) different type of tumor have different protocol
for treatment
Orbital lymphoma (radiation)
With systemic involvement (chemotherapy)
Regular follow up for possibility of recurrence
Patients with orbital tumors can extend to the
lacrimal gland or behind the orbit Presents with chemosis or swelling of conjunctiva, lid
If disease is confined to the orbit, treatment for both swelling, and proptosis.
monoclonal and polyclonal lesions is with radiation.
NON TUMOR LESIONS BUT PRESENT AS CASES OF
TUMORS
Orbital Pseudotumor
Graves Orbitopathy may manifest like meningioma
They mimic the presentation of a true tumor
ORBITAL PSEUDOTUMOR
Wide spectrum of inflammatory syndromes (can
affect any part of the orbit) waste basket diagnosis
AKA idiopathic orbital pseudotumor
Vary in location in the orbit and in onset
Manifests with severe pain, proptosis, swelling and lid
injection of globe/lid (lid hyperemia of the eyelid or
the eyeball)
Can affect any part of the orbit (sclera muscles fats On orbital CT there is enlargement of the medial rectus
periorbital) muscle with edema on the orbital contents pushing the
eyebrow forward. The whole length is involved from
Looks like meningioma
insertion to origin (arrow) is enlarged. Positive scleral
A frequent cause of proptosis in adults and children is
ring sign.
inflammatory pseudotumor.
In contrast with thyroid ophthalmopathy, only the
Pseudotumor" indicate a nonneoplastic process that
muscle belly is affected, a muscle fusiform
produces the sentinel sign of an orbital neoplasm.
enlargement. In orbital pseudotumor, it involves the
Site of inflammation is usually diffuse and not
origin and insertion.
amenable to excision.
Onset is usually rapid, and pain is often present. Possible ocular findings: uveitis, scleritis, papillitis
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(optic nerve head inflammation), and exudative
retinal detachment because the choroid is also
inflamed, you can develop swelling and detachment
of the cord.
Sometimes you will not see muscle enlargement but
the patient will complain of pain and proptosis- do
incisional biopsy
sometimes it is difficult to differentiate from
lymphoma
CT scan (orbital) - only way to determine the
diagnosis
TREATMENT
Responsive to steroids (oral or IV for 3-7 days)
Topical antibiotics for conjunctival involvement
Sometimes in chronic state, even with steroid you will
develop fibrosis/scarring -> the muscles are frozen
Biopsy
If non-responsive: biopsy the orbital contents only, no
muscle involvement.
Surgery tends to exacerbate the inflammatory
reaction
Orbital radiation therapy or surgical intervention
may be indicated in patients with no response to
treatment
GRAVES ORBITOPATHY / OPHTHALMOPATHY
Lid retraction, exophthalmos, hyperemic conjunctiva and
lids. The danger here in infrequent blinking & dry eyes.
Patient may be euthyroid, hypothyroid, or
hyperthyroid
Since condition is not correlated with amount of
thyroid hormone but instead an autoimmune
disease that involves the extraocular muscle
Swelling, proptosis, lid retractin, corneal exposure
Autoantibodies target the fibroblasts in the eye
muscles- differentiate into adipocytes muscles and
fat expand and become inflamed, so its not the
thyroid level but more of the immune response.
Most common cause of proptosis (both uni- and
bilateral) in adults
Autoimmune mediated process involving orbital
inflammation and most of the time the end stage is
scarring
Only the belly muscle is affected, spares the
insertion and origin.
4x common in middle aged women
Manifests as fixed eye (4 recti muscles are affected
and fibrotic)
90% have or had systemic thyroid abnormality
Some cases, patients with thyroid related
eorbitopathy will be euthyroid because this is an
autoimmune disorder, not all patients with TRO are
hyperthyroid (normal T3, T4 and TSH), it is good to
check for LATS (long-acting thyroid stimulator test) or
TRIs since they are usually elevated in patients with
thyroid related ophthalmopathy (TRO)
TRO characterized by chronic orbital inflammation
Autoimmune process results in infiltration of EOM
and orbital fat by inflammatory cells (short term)
active stage/inflammatory stage: best stage to give
steroids (responsive and shrinks fast) by
mucopolysaccharide and collagen deposition (long
term) fibrosis and scarring (muscles no longer moves)
Early manifestation is subtle hyperemia, chemosis,
irritation, eyelid swelling
Lid retraction (lid lag), corneal exposure (cornea will
dry infection and ulcer formation leading to
infrequent blinking and dry eyes
Corneal perforation and rupture of the eyeball. EOM
dysfunction (inferior rectus commonly affected ask
patient to look down minimal movement downward
and when asked to look up will never go upwards
because of restriction of the inferior muscle. Because
if its scarred it will pull the action of the superior
rectus and at the same time if its enlarged it will be
stronger than the superior rectus), optic nerve
compression, VA (visual acuity), color vision, VF
(visual filed), RAPD (relative afferent pupillary defect),
monitored to rule out optic nerve compression
6 24 months active stage time to treat steroids.
After treatment, they usually quiet down but if you
miss the diagnosis and you did not give the anti-
inflammatory immediately or their already fibrosis
and scarring all complications will appear
In cases with multiple recti muscle involvement/
enlargement (4 muscles) optic nerve compression
leading to blindness treating patient with steroids
Most common cause of unilateral or bilateral
proptosis in adults or children is Graves' disease.
Severe infiltrative orbital myopathy with significant
proptosis and restricted motility occurs in about 5%
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OCULAR AND ORBITAL TUMORS
of cases of Graves' disease
Can occur in hypothyroid, euthyroid, and
hyperthyroid
CT scan -> 2 muscle involvement -> spindle shaped
enlargement of medial and lateral rectus sparing
the spindles
TREATMENT
Observation (mild TRO)
Lubrication (for dry eyes)
Education (emphasize to the patient the etiology,
possible complications, what to do and when to
follow up)
Monitor thyroid status ( most of the time patients
with TRO are hyperthyroid although 10% are
euthyroid)
Steroids during congestion stage, if not responsive
proceed to radiotherapy for muscles to be smaller
Radiotherapy (for early stage who are resistant to
steroids)
Surgery
Tarsorrhapy if with associated lid problem;
close the lid temporarily to prevent corneal
desiccation and thinning of the tear film until you
manage the condition, Mller's muscles are
more sensitive to the sympathetic
subcutaneously to close the eyelids
Orbital decompression to create more space. The
orbital floor is removed to expand the maxillary
sinus to release optic nerve compression since if
untreated it entraps the inferior rectus, limits
upward eye movement, and causes diplopia on
up gaze. Timely decompression will save visual
acuity.
Last surgery done when there is scarring because
the result is unpredictable - only do this if
there is already scarring or >24 mos)
Myomectomy in fibrotic stage the muscles cant
move, cut medial rectus to contract the lateral
rectus to straighten the visual axis
Myotomy- to relax / weaken the muscles
strabismus surgery - adjust muscle movement
Assess corneal involvement
Antithyroglobulin, antimicrosomal, and other
antibodies can usually be demonstrated, but their
role in pathogenesis is in question.
Complication
Fibrotic muscle making manifestations permanent
NOT DISCUSSED
NON-TUMOR LESIONS
PTERYGIUM
A fleshy, triangular encroachment of a pinguecula
onto the cornea, usually on the nasal side bilaterally.
Thought to be an irritative phenomenon due to
ultraviolet light, drying, and windy environments,
since it is common in persons who spend much of
their lives out of doors in sunny, dusty, or sandy,
windblown surroundings.
The pathologic findings in the conjunctiva are the
same as those of pinguecula. In the cornea, there is
replacement of Bowman's layer by hyaline and elastic
tissue.
TREATMENT
If the pterygium is enlarging and encroaches on the
pupillary area, it should be removed surgically along
with a small portion of superficial clear cornea
beyond the area of encroachment.
Conjunctival autografts combined with surgical
excision have been shown to reduce the risk of
recurrent disease.
LYMPHOID TUMOR
CONJUNCTIVAL LYMPHOMA: thick colored mass
behind the conjunctiva.
Often manifests with lymphoma on other parts of the
body.
The clinical appearance of benign lymphoid
hyperplasia and malignant lymphoma can be similar;
therefore, biopsy is essential to establish a diagnosis.
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OCULAR AND ORBITAL TUMORS
DIAGNOSTICS
Magnetic resonance imaging or computed
tomography may be required to determine the true
extent of the tumor.
Most primary conjunctival lymphomas are low-
grade B cell lymphomas (MALT lymphoma).
TREATMENT
Both benign and malignant lesions is best
accomplished with radiotherapy.
SQUAMOUS CELL CARCINOMA
Rapidly progressing invading the corneal epithelium.

TREATMENT
Excision; responsive to chemotherapeutic agents

REFERENCES
Upper Class Notes
Lectures Audio
Ophthalmology Pocket Book Atlas

Previous Year Quiz

1. What is the most common skin malignancy in the
eyelid? Basal Cell CA
2. What is a lesion composed of normal matured tissue
in a abnormal location? Choristoma
3. Most common cause of unilateral proptosis in adults?
Grave's Disease
4. Neurofibromatosis is most likely associated with?
Optic Glioma
5. A six year old child was seen with axial proptosis,
decreased in visual acuity, and retrobulbar pain. An
intraconal lesion was suspected? What is the most likely
diagnosis? Optic Nerve Glioma

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