ENN Chap 16

Presenter:
dr. Endra Wibisono
Advisor:
Dr. dr. Joni Wahyuhadi, SpBS (K)
Dr. dr. Rahadian Indarto S. SpBS (K)
dr. Irwan Barlian I. H, SpBS (K) 1
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16.1 Introduction
Orbital Tumor
Acquired
Developmental
Lession
Ilmiah Divisi Neuro-Onkologi

Departemen Bedah Saraf
FK UNAIR – RSUD dr Soetomo
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16.2 Epidemiology
Rare, incidence is less than 1 per 100,000

pop- ulation.
More than 50% of all orbital tumors

occurring between the fifth and sevent
No significant male or female

preponderance
Orbital neoplasms comprise about 20% of

all orbital conditions

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16.3 Orbital Anatomy
16.3.1 Dimensions
• It is pyramidal with four walls
• Narrowing posteriorly toward the apex,  many nerves and vessels
pass to the cranial cavity.
• The bony orbital volume averages about 27 mL, with the globe taking
up about 7–8 mL volume, the extraocular muscles about 5 mL, and
the fat about 10–13 mL.
• The optic canal is usually 40–45 mm behind the medial orbital rim

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16.3. Orbital Anatomy

16.3.2 Bony Walls
1. The orbital roof  the frontal and sphenoid
bones,
2. The medial wall  the ethmoid, lacrimal,
sphenoid, and maxillary bones
3. The orbital floor  the maxillary, zygomatic,
and palatine bones
4. lateral wall  comprising part of the zygoma,
sphenoid, and frontal bones
The bony orbital entrance is the strongest part of

the orbit, the weakest part being the orbital floor
and medial wall.

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Orbital Anatomy
16.3.3 Orbital Foramina and Fissures
• The supraorbital neurovascular  supraorbital notch ->

supraorbital foramen -> lacrimal branch + frontal
branch ophthalmic nerve
• The infraorbital neurovascular  infraorbital foramen
-> maxilarry branch N V
• The inferior orbital fissure,  fat, the infraorbital nerve,
and veins leaving the orbit for the pterygopalatine
fossa.
• Posteriosuperiorly, (junction of the lateral wall and the
anterior (a.l.c) and posterior lacrimal crests (p.l.c)
roof)  superior orbital fissure frontal process of the zygoma (f.p.z),
- the superior and inferior divisions of the oculomotor frontozygomatic suture (f.z.s).
supraorbital notch (s.o.n)
nerve lamina papyracea (l.p) of the ethmoid air cells
the anterior (a.e.f) and posterior ethmoidal foramina
- the trochlear nerve (p.e.f
infraorbital canal (i.o.c)
- the abducens nerve, inferior orbital fissure (i.o.f)
infraorbital foramen (i.f.fo).
- the first division of the trigeminal nerve The superior orbital fissure (s.o.f)
optic canal (o.c)

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Orbital Anatomy
• In the orbital apex (medial to the
superior orbital fissure)  Optic
foramen  optic nerve in its meninges
and the ophthalmic artery.
• The optic nerve :

- about 4 mm in diameter,
- takes an “S”-shaped course for its 30-
mm orbital portion
- about 10 mm of intracranial length
before joining the optic chiasm.
• The intracanalicular part is immobile as

its dural sheath is fused to the
periosteum of the optic canal
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Orbital Anatomy
16.3.4 Periorbita and Surgical Spaces
• The periorbita covers all bones of the orbit and is only loosely attached to the underlying bone
(except at the orbital rim and at suture lines).
• The orbit can be divided into four surgical spaces:

1. Subperiosteal (extraperiosteal) space,
2. Intraperiosteal extraconal space
3. Intraconal space,
4. Sub-Tenon’s space.

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Orbital Anatomy
3. Intraconal Space.
- Lies within the
1. The Subperiosteal - Contains the optic nerve, intraconal
(Extraperiosteal) Space. orbital fat, motor nerves, and some
- Existing only when created surgically blood vessels.
or when filled by a pathological process - During a lateral orbitotomy approach
- Between the periorbita and the bony
with bone resection, the intraconal
space is usually entered by passing
orbital walls.
below the lateral rectus muscle.
2. Extraconal Space. 4. Sub-tenon’s Space.

Structures in the extraconal space are - Located between the globe and the
lacrimal gland, the oblique muscles, anterior surface of Tenon’s capsule
trochlea, orbital fat, sensory nerves,
trochlear (motor) nerve, and some vessels. - Can be enlarged by inflammatory fluid
or infiltrated by extraocular growth of
intraocular tumors (e.g., with choroidal
melanoma).

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16.4 Symptoms and Signs

Signs
Proptosis—by a retrobulbar mass, such as intraconal cavernous

hemangiomas (Fig. 16.2a) or optic nerve tumors
The globe displaced vertically or horizontally, due to a lacrimal

gland mass (Fig. 16.3a) or intraorbital dermoid cyst.
.
Inferolateral displacement is typically due to frontoethmoidal
mucoceles (Fig. 16.4f), vascular lesion, neural tumors, or
dermoid cysts.

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Signs
eyelid asymmetry
conjunctival inflammation and swelling (chemosis)

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Funduscopy
optic nerve head swelling
choroidal folds (Fig. 16.2b),

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Fig. 16.2 (a–c) Adult onset proptosis of slow progression
(a) Slowly progressive left proptosis with reduced visual acuity

and
(b) choroidal folds
(c) these changes being caused by the slow growth of a well-
defined, round, intraconal mass, most commonly a cavernous
hemangioma

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16.5 Clinical History and Orbital Examination
Current state Previous sinus

of general disease or
health, surgery
Endocrine
Malignancies
dysfunction
infections injuries,

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16.5.2 Examination
• General examination
• Facial asymmetry, for displacement of
the globe, or for orbital or periorbital
masses.
• Skin changes, such as discoloration, may
indicate an underlying vascular anomaly
or neurofibroma (Fig. 16.8b, c)
• Asymmetry or changes of the eyelids—
such as swelling, erythema, ptosis, loss of
the eyelid sulcus, or skin crease

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Examination
Palpation
• The orbital margin -> notches, or foreign body

• Shape, size, surface texture, and attachment of any mass
• well or ill-defined, a round or irregular shape, soft or hard, and non-tender or painful.
• Palpation of the preauricular, submandibular, and clavicular lymph node regions.

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Examination
Examination of Globe Position.
• Proptosis, axial anterior displacement of the globe,

formally measured by exophthalmometry.
• Any readings greater than 22 mm are probably
abnormal.

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Examination
• Proptosis is mainly due to retrobulbar masses

• It is important to differentiate between real
proptosis and “pseudo-proptosis,”
• The elongated myopic globe leads to pseudo-
proptosis.

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Examination
Extraocular Muscle and Periorbital Sensory Functions.
- Extraocular muscle function is assessed by asking the patient to follow a fingertip

- Double vision most commonly due to mechanical restriction and more rarely to
neurological deficit.

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Examination
Assessment of Visual Functions.
• A normal acuity does not exclude orbital disease

• Color testing, often gives an early indication of optic neuropathy.
• The test for a relative afferent pupillary defect (RAPD) is the least subjective method and a very
sensitive test for optic neuropathy.

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16.5.3 Additional Examinations
Visual Field Assessment
- Confrontational visual field testing

- Goldmann perimetry
- Tumors of the orbit, optic canal, or chiasm frequently
impair visual function and cause visual field defects
- Disease progression or improvement monitoring
Optic nerve sheath meningioma.

Visual field testing was possible only
on the left eye (d) and shows gross
impairment

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16.5.4 Imaging
Orbital Ultrasonography.
• Provides useful information about the location, size, shape,

tissue characteristics, and vascular features of orbital
tumors
• a non- invasive technique
• very helpful for long-term follow-up assessments.
• For confirming the cystic nature of a mass

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Imaging
Computed Tomography.
• Superb bone detail
• Very sensitive for tissue calcification or radio-opaque foreign bodies.
• With its high resolution, thin-slice (0.6–1 mm) CT with contrast should be the first choice for imaging adult orbital disease
• However, after intravenous injection of X-ray contrast media, CT density increases rapidly in tissue with increased (neo-)
vascularization
Malignant Pleomorphic Adenoma.

Orbital Lymphona. CT shows the mass arising from the right lacrimal
maging may then show bone
gland and cloaking the globe both posteriorly (c) and superiorly (d)
invasion and destruction (e)

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Imaging
Magnetic Resonance Imaging Magnetic Resonance Imaging
Pros: • Rarely specific for a diagnosis and is not routinely

required for orbital patients.
• Free of ionizing radiation / no harmful
radiation
• Specific indications
• able to provide soft-tissue differentiation 1. evaluation of optic nerve lesions
• can provide images in several planes 2. of suspected radiolucent orbital foreign bodies,
3. the extent of sphenoid wing meningiomas
Cont : 4. Specific indications for MRI of the orbit include
known or suspected disease of the optic nerve,
• Expensive optic chiasm, meningeal membranes (Fig. 16.5c),
• time-consuming or the central nervous system and the search for
non-metallic foreign bodies which are not
• extremely liable to acts due to motion during delineated by computed tomography.
image acquisition.
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Magnetic Resonance Imaging
• Combinations of different sequences are usually applied for MRI of the orbit.
• T2-weighted MR images, which show water with high-signal intensity, fat with intermediate
signal intensity, and muscle with low signal intensity, distinguish, e.g., cerebro- spinal fluid in
the optic nerve sheath from soft tissue.
• T1-weighted MR images are susceptible to contrast change after intravenous administra- tion
of gadolinium-based contrast media. Soft tissue shows intermediate to low signal intensity
in unenhanced T1-weighted MR images. However, after intravenous injection of contrast
media, signal intensity increases rapidly in tissue with increased (neo-)vascularization, e.g.,
inflammatory tissue and many malignant tumors.
• Since adipose tissue shows with bright contrast at T1-weighted imaging, fat signal
suppression may improve the delineation of inflammatory or tumor tissue.
• Diffusion- weighted imaging, is not (yet) routinely used.

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Imaging
Positron-Emission Tomography
and Combined Computed
Tomography.
• PET/CT combines morphological

and metabolic information in a
single examination with excellent
sensitivity and specificity.
• Positron-emission tomography
and combined computed
tomography (PET-CT) has
recently shown its value in the
investigation of orbital diseases
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Imaging
Positron-Emission Tomography and Combined Computed Tomography.
• Two different PET tracers with very different prop- erties have been
successfully applied to specific orbital diseases.
• Radioactively labelled glucose (18F-fluorodeoxyglucose, or FDG)
accumulates in tissue with increased cell energy metabolism. Although
increased energy metabolism is not specific to any disease, FDG
accumulation is particularly strong in malignant tumors, e.g.,
lymphoma, or squamous cell carcinoma.
• Besides localizing sites of disease inside and around the orbit, FDG
localizes concomitant primary or metastatic foci elsewhere in the body.
Specific binding to soma- tostatin receptors (SSR)

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16.6 An Approach to Differential Diagnosis
• The rate of onset can be considered

1. Acute (minutes to hours)
2. Subacute (days to weeks)
3. Chronic (over months)
4. Acute on chronic, the latter being a chronic condition with recent
dramatic acceleration.

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16.6.1 Acute Onset

1. Orbital hemorrhage
- Overnight onset, resolves only slowly, and bruising may appear a few days later
- Children -> vascular anomaly; adults  arteriopathic and tak-ing antiplatelet drugs.
- CT (if there is diagnostic doubt) and visual monitoring
- drainage indicated if there is major visual impairment, significant progression, or persistent proptosis.
2. Acute infective orbital cellulitis

- progression over hours,
- prodro- mal illness (headache, nasal discharge, and fever) .
- The orbit is painful, tense, and active, and passive ocular motility shows marked impairment.
- May be visual loss.
- an ophthalmo- logical emergency
- needs immediate treatment intravenous antibiotics (prior to imaging), close monitoring of vision, and then
orbital CT to confirm the diagnosis.
- Dranage is indicated for acute compressive optic neuropathy or persistent orbital abscess.

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Fig. 16.6 (a–d) Childhood orbital lesions of acute onset. (a)

Acute orbital cellulitis in an unwell child due to spread of
bacterial infection from the right ethmoid sinus. (b) Overnight
onset of massive left orbital hemorrhage from a
lymphangiomas/varix; MRI demonstrates a lobulated mass (c)
throughout the upper part of the orbit, with evi- dence of a
fluid level with the characteristics of layering blood (d). Clinical
photographs from the Moorfields Eye Hospital, London

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16.6.1 Acute Onset

3. Arteriovenous shunts,
- generally in patients with arterial disease
- develop within minutes to hours and are characterized by a painles
protrusion, acute “red eye” with chemosis, and a global reduction of
orbital functions.
- CT and ultrasound echography should be per- formed, visual acuity
and intraocular pressure monitored

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Subacute Onset
1. Childhood malignancies
- Characterized by subacute onset
- often in an otherwise well child
- May present with a tense, inflamed, and painful orbit
- Open incisional biopsy is always indicated, and the appropriate therapy depends on the
histologi- cal diagnosis.
- Rhabdomyosarcomas typically are well defined and do not occur in extraocular muscles.
• 2. Childhood capillary hemangiomas

- Develop over months
- the lesions being soft and causing only mild impairment of orbital functions.
- Often there is an expansion of the hemangioma when the child cries or catches a cold.
Childhood malignancies
Fig. 16.4 (a–f) Orbital lesion of subacute onset. (a) Slight

painless swelling of a child’s right upper lid, which developed
into a significant superonasal quadrant mass within a month of
onset (b). The mass, an embryonal rhabdomyosarcoma, was
excised intact (c), and after adju- vant chemotherapy, the child
was still disease-free 5 years later (d). (e) Within days,
increasing left upper lid redness and swelling due to a chronic
frontoethmoidal mucocele erupting into the left orbit; the
mass, originating within the sinuses, is shown on coronal CT (f)
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Subacute Onset
3. Orbital infections
- in adults may progress over weeks if due to low-grade or unusual organ- isms—such as fungi, parasites as in
hydatid disease, or tuberculosis—
- Infections are often painless with only mild inflammatory signs.
4. Adult metastatic disease

- may show a subacute, relentless progression over weeks, and about 75% of patients will have a known sys-
temic malignancy.
- The clinical picture is typically one of painful proptosis
- sometimes with inflammatory signs,
- a marked reduction of orbital functions.
- CT scan and systemic tests are obligatory,
- open biopsy indicated in most cases,
- treatment will often involve orbital radiotherapy and systemic chemotherapy or hormonal therapy.

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Chronic Onset
1. Childhood benign tumors,
- such as retrobulbar dermoid or optic nerve tumors
- present as slowly progressive proptosis with mild changes in orbital function.
- Some may be found by chance while imaging for other conditions, such as neurofibromatosis.
- Imaging may be advisable in some cases, and monitoring of visual development is compulsory.
2. Sinus mucoceles or tumors

- may progress slowly and present late,
- may be a history of sinus surgery or facial trauma.
- Inferior displacement of the globe is common, with some limitation of globe movements.
3. Osseous disease,
- such as fibrous dysplasia, osteoma, or sphenoid wing meningioma
- develops slowly at any age
- being manifest as facial asymmetry or optic neuropathy (Fig. 16.5).

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Chronic Onset
4. Adult benign tumors
- Show a chronic progression over years,
- globe displacement that may be axial or non-axial (Figs. 16.2 and 16.8).
- Generally, these tumors are painless,
- often there is only a mild change in orbital functions.
- Observation may be appropriate if orbital function is normal,
- CT shows a well-defined mass, but otherwise the mass should be excised.
5. Adult malignant tumors

- such as low- grade lymphomas or some sarcomas
- may show a painless, slow progression
- without significant loss of orbital function (Fig. 16.7).
- All ill-defined, infiltrative orbital masses should be biopsied.
Adult Benign Tumor.Cavernous Hemangioma.
Fig. 16.2 (a–c) Adult onset proptosis of slow progression: (a)
Slowly progressive left proptosis with reduced visual acuity and
hypermetropic refractive shift due to choroidal folds (b), these
changes being caused by the slow growth of a well-defined,
round, intraconal mass (c), most commonly a cavernous
hemangioma
Adult Malignant Tumor. Orbital lymphoma

Fig. 16.7 Slowly progressive proptosis in
adulthood, due to orbital lymphoma. (a) Subtle,
right, painless proptosis due to a pink
subconjunctival mass of lymphomatous tis-sue ((b)
viewed from above). CT shows the mass arising
from the right lacrimal gland and cloaking the 38
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Chronic Onset
6. Adult vascular lesions

- arising from an “evolution” of venous anomalies or enlargement of
arterial anomalies,
- may cause chronic pain and a moderate loss of function.
- CT scan and Doppler ultrasonography are of diagnostic help,
- MRI angiogram may be indicated.
- Venous anomalies should be observed where possible
- Arteriovenous malformations usually need treatment by
interventional radiology.
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Acute-on-Chronic Onset
1. A sudden acceleration
of a previously very
slowly progressive
proptosis suggests
the malignant
transformation of a
formerly benign
tumor. Pleomorphic adenomas should be excised intact (c), as they
carry a risk of later malignant transformation (so-called
malignant mixed tumor), when the patient will present with an
2. O accelerated history (d), and imaging may then show bone
invasion and destruction (e)

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Acute-on-Chronic Onset
1. Venous anomalies
- may undergo spontaneous thrombosis,
- causing acute pain, signs of inflammation, and a dramatic increase in
proptosis with a marked loss of function.
- a surgical intervention or, in some cases, anticoagulants and medical
therapy may be required if orbital functions are severely impaired.

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16.7. Common Orbital Tumors
Onset
• The prevalence of various orbital tumors varies with age,
• structural anomalies and benign tumors being more common in
childhood
• acquired benign or malignant neoplasms being typical of adulthood.

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16.7.1. Benign Orbital Tumors
Childhood benign lesions

• Relatively common, the most frequent being dermoid or epidermoid cysts (up to
about one third of refer- rals) and capillary hemangiomas in up to about 15% of
cases.
• In contrast, dermoid cysts very rarely remain occult until adulthood
Benign tumors in adults

• tend to be acquired lesions
• such as cavernous hemangiomas, benign peripheral nerve sheath tumors, optic
nerve tumors,
• distinctly rare, lacrimal gland and mesenchymal tumors.
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16.7.1.1 Orbital Cystic Tumors
• Generally arise from developmental sequestration of epithelium within the orbit.
• Congenital dermoid and epidermoid cysts,

- often noted shortly after birth
- Most commonly located near the orbital rim at the zygomatico- frontal suture
- Firm, smooth preseptal masses that may be mobile over the bone or fixed deeply
• Mobile, anteriorly situated, and characteristic lesions do not require radiological investiga-
tion and should be excised through an upper lid skin crease incision or through an incision
hidden on the brow hairs;
• Fixed dermoid cysts do not necessitate imaging if the surgeon is adequately experienced to
follow the lesion to its limits.
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16.7.1.1 Orbital Cystic Tumors
• Intraoperative rupture of a dermoid cyst may lead to a marked

postoperative inflammation, and any spilt contents should be
removed and residual oily debris “floated out” from the operative field
by copious saline irriga- tion.
• Incomplete excision of the epithelial lining will lead to recurrent
inflammation with forma- tion of a discharging cutaneous fistula
through the operative incision.

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16.7.1.2 Orbital Vascular Tumors

• Many vascular anomalies, such as varices, lymphangiomas, and
arteriovenous anomalies, are rather diffuse within the orbit and do not
form discrete, tumorlike masses.
• Childhood capillary hemangiomas and cavernous hemangiomas in
adults do, however, present as well-defined tumors with appropriate
orbital signs.

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16.7.1.2 Orbital Vascular Tumors

Infant
• Capillary hemangiomas occur in up to 2% of infants and typically appear soon after birth,
enlarge, and then undergo a spontaneous involu- tion, with most having resolved by 7
years of age. Unlike rapidly growing malignancies of infancy (such as rhabdomyosarcoma),
capillary hemangiomas show multiple vessels with very high flow rates—generally above
50 cm/s—and arterial waveform. Unless the rare necessity for surgical resection is being
considered, CT or MRI is only rarely needed for the diagnosis but generally shows a rather
irregular lesion with marked contrast enhancement. Affected children should be moni-
tored and treated for their visual development, and if the visual impairment is due to
tumor bulk, these hemangiomas are usually treated with systemic or topical β-blockers
(e.g., systemic propranolol) or, more rarely, with intralesional steroids or resection.
Adult
• The most common adult benign orbital tumor, cavernous hemangiomas, usually arise in
retrobulbar tissues and present as painless, very slowly progressive proptosis in the
fourth or fifth decades; vision may be reduced due to induced presbyopia, choroidal folds,
or optic nerve com- pression.
• CT scanning reveals a well- defined, round intraconal tumor, with only very slow and
patchy contrast enhancement, which commonly displaces the optic nerve medially.

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16.7.1.3 Benign Lacrimal Gland Lesions

• Lacrimal gland masses can be due to chronic dac- ryoadenitis or benign neoplasms
• Mismanagement of benign conditions can lead to serious consequences as with, for
example, malignant recurrence after biopsy of a benign pleomorphic adenoma.
• Pleomorphic adenomas are a rare, benign, epithelial neoplasm of the lacrimal gland,
typically arising in the orbital lobe and presenting in the fourth or fifth decades as a
slow onset of painless proptosis and inferomedial displacement of the globe.
• To prevent inadvertent biopsy, it is imperative to diagnose the tumor based on clinical
history and CT scanning.
• The key to treatment of pleomorphic adenomas is preoperative recognition, with avoid-
ance of biopsy. Because of the risk of late malignant transformation, tumors of the
orbital lobe should be excised intact—sometimes requiring lateral orbitotomy—and
breach of the “pseudocapsule” of compressed tissues avoided; to this end, the tumor is
handled at all times with a malleable retractor and not with any sharp-edged
instruments.
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16.7.1.4 Benign Optic Nerve Tumors

• Primary optic nerve meningiomas or gliomas (juvenile pilocytic astrocytoma) are usually benign and present in
children or young adults.
Glioma
• Glioma is the most common optic nerve tumor but comprises only 3% of orbital tumors.
• One third of gliomas are related to type I neurofibromatosis, these having a better visual prognosis than those in
patients without NF1.
• Gliomas generally cause painless proptosis and visual loss ranging from mild to severe.
• In the early stages, fundus examination may show a swollen optic disc, which later may become pale with the
appearance of a retinochoroidal shunt vessel on the margin of the disc.
• Imaging shows a fusiform enlargement of the optic nerve, often with a characteristic intraorbital kink, and MRI is
especially useful for detailing the intracanalicular and intracranial portions of the nerve.
• Chemotherapy, radiotherapy, and neurosurgical resection should be considered if an optic nerve glioma is showing
progression with a threat to the chiasm, whereas a transcanthal resection of the orbital tumor—sparing the eye—
may be considered for gross proptosis.
• The prognosis is generally good for solely orbital glioma and the mortality, other than with intrace- rebral disease,
less than 5%.

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16.7.1.4 Benign Optic Nerve Tumors

Orbital meningiomas
• are benign neoplasms arising from the meninges,
• two distinct forms—optic nerve sheath meningioma (Fig. 16.9) and sphenoid wing meningiomas (Fig. 16.5)
• which are both frequent in middle- aged women.
• Optic nerve meningiomas cause minimal proptosis but profoundly affect vision due to impairment of optic
nerve perfusion.
• The affected eye presents with a swollen or atrophic optic disc, occasionally with retinochoroidal shunt
vessels.
• CT scan typically shows a diffuse expansion of the optic nerve and, in some cases, a “train-track” parallel
calcification of the optic nerve sheath.
• MRI may demonstrate a normal or small optic nerve passing through an enlarged sheath.
• The therapy of optic nerve sheath meningioma is conservative, since surgical excision invari- ably leads to
blindness.
• High-dose radiotherapy should be considered for optic nerve meningiomas with clinical progression and,
in most patients, will slow tumor growth and stabilize or improve vision.

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The right optic disc (b) is atrophic, with marked optociliary

shunt vessels, and the left disc (c) shows temporal atro- phy;
visual field testing was possible only on the left eye
(d) and shows gross impairment. Bilateral calcified optic
nerves, pathognomonic of optic nerve sheath meningioma, are
clearly shown on CT (e); a slight flattening of the posterior pole
of both globes (especially the left) is also evident due to a
“splinting” effect of the optic nerve tumors

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16.7.1.5 Benign Tumors of the Peripheral Nerve, Bone, or Mesenchyme
Schwanoma
• Solitary benign nerve sheath tumors, such as neurilemmoma
(schwannoma) and neurofibroma, arise from peripheral nerves,
comprising about 4% of orbital neoplasms.
• They present either in the intraconal space (with an imaging appearance
like cavernous hemangiomas) or as a sausage- like mass along the orbital
roof, causing slowly progressive proptosis and hypoglobus.
• occurs in middle-aged adults and causes painless proptosis with
symptoms similar to those of cavernous hemangioma; they are readily
cured by surgery.
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Sphenoid wing meningioma

• tends to present in middle age with chronic variable lid swelling, chemosis, and mild proptosis.
• CT scan shows hyperostosis of the greater wing of the sphenoid with en-plaque soft tissue on
the lateral wall of the orbit, the temporalis fossa, or the middle cranial fossa
• A rapidly progressing tumor should probably undergo biopsy to exclude metastatic disease
with a view to radiotherapy or neurosurgical resection if shown to be meningioma.
• As progestogens may act as a stimulus to the growth of sphenoid wing meningiomas, it is pru-
dent to discourage the use of hormone replace- ment therapy in postmenopausal women with
this condition.
• Benign mesenchymal tumors of the orbit, such as solitary fibrous tumors or hemangio-
pericytomas, are very rare and typically present as painless proptosis with diplopia. The
masses, generally well defined but cloaking normal orbital structures, are often located in the
superonasal quadrant of the orbit and may be en-plaque with the orbital periosteum. These
tumors should, where possible, be excised intact, as they carry a significant risk of perva- sive
tumor recurrence with piecemeal primary excision.
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Hemangiopericytomas
• Benign mesenchymal tumors of the orbit
• very rare
• painless proptosis with diplopia.
• well defined
• often located in the superonasal quadrant of the orbit and may be en-
plaque with the orbital periosteum.
• These tumors should, where possible, be excised intact, as they carry a
significant risk of perva- sive tumor recurrence with piecemeal primary
excision.

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16.7.2 Malignant Orbital Tumors
1. Can affect all ages
2. should be considered
wher- ever there is
rapidly or relentlessly
progressive disease

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16.7.2.1 Malignant Orbital Tumors of Childhood

• Rhabdomyosarcoma is the most common primary orbital malignancy of
childhood
• arises from pluripotent mesenchyme that normally dif- ferentiates into
striated muscle cells.
• a peak incidence at about 7 years of age,
• The tumor mass may be located anywhere in the orbital soft tissues, most
commonly in the superome- dial quadrant, and typically does not arise in
the extraocular muscles (Fig. 16.4a–c).
• Imaging will usually demonstrate a fairly well-defined, round mass with
moderate contrast enhancement, arising within the orbital fat and
flattening the globe; expansion of the thin bone of the childhood orbit is
quite common.
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16.7.2.2 Orbital Lymphoma in Adults 58
• Orbital lymphocytic lesions display a spectrum from benign morphology, showing a well- organized follicular
pattern (so-called reactive lymphoid hyperplasia), through the rare “atypi- cal lymphoid hyperplasia” with
poorly organized or disrupted follicles, to frankly malignant lymphoma
• Orbital lymphomas typically present in those over 50 with a slowly progressive, painless, pink subconjunctival
mass or—if deeper in the orbit— with eyelid swelling, globe displacement, or diplopia (Fig. 16.7).
• CT scan commonly shows a moderately well-defined soft-tissue mass, which may be bilateral, cloaking the
globe and other orbital structures;
• tumor calcification and bone destruction are distinctly rare.
• Biopsy is mandatory, as the CT and MRI characteristics of lymphomas are indistinguishable from orbital
inflammation.
• All patients with lymphoid lesions should undergo investigation for systemic disease, including whole-body PET-
CT scan and bone marrow biopsy if the lymphoma is of higher grades.
• low-grade orbital lymphomas respond very well to about 2400 cGy fractionated radiotherapy or to oral
chemotherapy.
• Patients with high-grade lymphomas have, however, a much higher chance of systemic disease and usually
require multiple cycles of more aggressive chemotherapy, and adjunctive orbital radiotherapy (often to 3500
cGy) may be used to accelerate resolution of the orbital disease.

59
16.7.2.3 Lacrimal Gland Carcinomas

• With a peak incidence in the fourth decade, adenoid cystic carcinoma is the most common epithelial malignancy of the lacrimal gland,
and other carcinomas (primary adenocarcinoma, mucoepidermoid carcinoma, squamous carcinoma, or malignant mixed tumors;
Fig. 16.3d, e) are much rarer; malignant mixed tumors arise within a long-standing pleomorphic adenoma or in recurrent tumor after
incomplete resection of a benign pleomorphic adenoma. The diagnosis of lacrimal carcinoma is suggested by persistent periocular
ache, ocular displacement, and upper lid swelling progressing over a few months and a non-tender lacrimal gland mass. CT scan
shows an enlarged gland molding to the globe, flecks of calcification in about one third of tumors, extension along the lateral orbital
wall with medial displacement of lateral rectus (the “lateral wedge sign”), and—in more advanced cases—erosion of the cortical bone
in the lacrimal gland fossa.
• Biopsy of suspected lacrimal gland malignancy should be through an upper lid skin crease incision, and adenoid cystic carcinoma is
com- posed of cords of malignant epithelial cells, often with cystic spaces giving a “Swiss cheese” pattern. Adenoid cystic carcinoma
has a tendency to perineural spread (into the cavernous sinus and pterygopalatine fossa) and also tends to infiltrate beyond the
macroscopic boundaries evident at surgery or radiologically. As recurrent lacrimal carcinoma may not present for more than a
decade after primary therapy, treatment cannot realistically be considered a “cure” until at least 20 years have elapsed without
disease. The opti- mum treatment for lacrimal carcinoma appears to be local macroscopic debulking—without resection of the bone
—followed by high-dose radiotherapy (55–60 Gy) to the lateral part of the orbit, the orbital roof, the superior orbital fissure, and the
anterior part of the ipsilateral cavernous sinus; major cranio-orbital resection (“super- exenteration”) appears to worsen the prognosis
for this disease. Deliberate surgical breach of an intact lateral orbital wall should be avoided, as this may encourage tumor seeding
into the cranial diploe and a relentless, fatal recurrence of local disease. Implantation brachytherapy has been used to deliver a high
radiation dosage to the tumor bed while relatively sparing the globe, but it does not treat the cavernous sinus or pterygopalatine
fossa, areas in which tumor recurrence occurs after perineural invasion. Intracarotid chemotherapy may have a role as an adjunct to
radiotherapy in advanced disease.
• The long-term prognosis is probably one of the worst of all orbital tumors with a 10-year sur- vival rate of approximately 50%.

16.7.2.4 60
Secondary Orbital Malignancy from the Eyelids, Paranasal Sinuses,

or Globe
• Orbital exenteration, with craniofacial resection in some cases, is generally required where there is extensive orbital
involvement by secondary spread of tumors from the globe or from sites around the orbit.
• Extensive meibomian gland carcinoma and neglected basal cell or squamous carcinomas tend to invade the orbit and
conjunctival fornices, causing diplopia, and tumor fixation to underlying bone suggests advanced disease. Painful peri-
neural invasion, usually from forehead tumors with infiltration along the frontal nerve, is most common with squamous cell
carcinoma and may not be associated with a significant orbital mass. Likewise, sebaceous (meibomian gland) carcinoma
may show intraepithelial pagetoid invasion across the conjunctiva or skin remote from an apparently localized eyelid mass.
• Squamous carcinoma from the paranasal sinuses or pharynx is the most common secondary epithelial neoplasm of the
orbit, either with direct bone destruction or microscopic perineural spread through, for example, the ethmoid foramina or
the inferior orbital fissure. Management involves diagnostic biopsy, wide surgical clearance, and later radiotherapy and
chemotherapy.
• Other rare tumors of the paranasal sinuses that may involve the orbit include adenoid cystic carcinoma, adenocarcinoma,
esthesioneuroblas- toma, and melanoma.
• Uveal malignant melanoma is the most common primary intraocular tumor of adulthood, and orbital extension probably
occurs through the emissary veins, although aggressive tumors may reach the orbit by direct scleral invasion or through the
optic nerve head. As there is often coexistent systemic disease, orbital extension of uveal melanoma carries a poor
prognosis, although future advances in tumor-directed chemotherapy may improve this outlook. Extraocular extension of
retinoblastoma—the most common childhood ocular malignancy—occurs in about 8% of cases and carries a poor prognosis,
despite systemic chemotherapy and local radiotherapy

61
16.7.2.5 Orbital Metastases in Adults

• Occur more commonly in the uveal tract
• Occur by hematological spread in the absence of orbital lymphatics
• Form 2–3% of all orbital tumors and may arise from an occult primary tumor.
• The most common primary sites are the breast, pros- tate, lung, kidney, and the gastrointestinal tract
• Present with painful proptosis and diplopia, orbital inflammation.
• Malignancy should be considered whenever an orbital disease progresses despite treatment.
• A multidisciplinary approach involving the ophthalmologist, family physician, patholo- gist, and oncologist is
essential for an adequate management of these patients.
• Treatment from an ophthalmologic standpoint includes preservation of vision and relief of pain.
Radiotherapy, chemotherapy, and hormonal therapy can often achieve these goals.
• After possibly debulking the tumor, the mainstay of therapy is local treatment with about 5500 cGy
fractionated radiotherapy.
• Radical surgery is contraindicated except in rare cases, when exenteration may be considered if the orbit is
the sole metastasis (e.g., carcinoid, renal carcinoma). Most treatments are palliative, with avoidance of
discomfort and preservation of vision (if possible), but dry eye and troublesome diplopia are major
problems, particularly after radiation.

62
16.7.2.6 Rare Adulthood Malignancies of Mesenchymal or Neural Origin
• Sarcomas of the orbit are extremely rare. The highly malignant osteosarcoma is often secondary to childhood
orbital radiotherapy in genetically predisposed individuals (with prior retinoblastoma), and even with radical
clearance, the tumor is almost uniformly fatal within 2 years. Children may present with metastatic Ewing’s
sarcoma or Wilms’ tumor within the orbit and will require systemic therapy after diagnosis.
• Fibrosarcomas arise as a primary orbital tumor or as a secondary tumor from adja- cent sinuses or the site of
prior radiotherapy. Exenteration is often necessary for wide clearance or palliation with radiotherapy and che-
motherapy. The prognosis for vision and life is variable, but it is best for primary juvenile fibrosarcomas.
• Several rare orbital tumors present with a spectrum of disease from benign to malignant. With a poor prognosis,
malignant fibrous histio- cytomas generally present with a well-defined mass in the superonasal quadrant, and
even after wide excision, recurrence of these radio- resistant and chemoresistant tumors is common.
Hemangiopericytoma, likewise, has a spectrum of malignancy and should be treated by wide and, if possible,
intact resection.
• Leiomyosarcoma, a tumor of smooth muscle, and liposarcomas of various degrees of differentiation present
considerable diagnostic difficul- ties and have been reported to involve the orbit very rarely.
• Of Schwann cell origin, the extremely rare malignant neurilemmoma may arise spontane- ously or in association
with neurofibromatosis. It presents as a slowly progressive lid mass or proptosis. CT scan shows an ill-defined
mass, and management involves wide surgical clearance with adjunctive radiotherapy or chemotherapy. The
prognosis is poor, as these tumors tend to invade the middle cranial fossa and develop pul- monary metastases.

63
16.8 Principles of Surgical Management
• Orbital tumors generally require excision.
• It is possible to approach all areas of the orbit through cosmetically “hidden” incisions
• there is almost no indication for using transcranial approaches for solely orbital disease.
• Cranioorbitotomy should be reserved for cases where there is a need to remove both an
intracranial and an orbital mass

64
16.8.1 Principles of Anterior Orbitotomy
• A skin incision of about 3 cm, generally the upper eyelid skin crease
or lower eyelid “tear trough,”
• The underlying orbicularis oculi muscle cauterized and divided at the
midpoint of the skin incision.
• The points of a pair of scissors are inserted through the defect,
opened widely along the line of the muscle to separate the fibers by
blunt dis- section.
• The septum is likewise divided along the line of incision to expose
the orbital fat,
• The direction of the orbital mass ascertained by analysis of the
imaging and by palpation. .
• When the abnormal tissue is located, a relatively large biopsy should
be taken using a number 11 blade or noncrushing biopsy forceps.

65
16.8.2 Principles of Lateral Orbitotomy
• An upper lid skin crease incision is extended laterally to

about 1 cm below the lateral canthus,
• The periosteum incised 6 mm outside the rim from the
lateral one third of the supraorbital ridge to the level of
the zygomatic arch.
• the fragment is then out-fractured and trimmed, swung
laterally on temporalis, and the periosteum opened to
provide access for the intraorbital procedure.
•
• Complications
1. diplopia fairly common with surgery near the orbital
apex and superior orbital fissure
2. Postoperative mydriasis—probably due to
denervation at the ciliary ganglion—is relatively
common and may be permanent,
3. Total loss of vision is a distinct risk with any surgery
involving the posterior half of the orbit.

66
16.8.3 Principles of Orbital Exenteration
• Orbital Exenteration,  the complete removal of

the eyeball, retrobulbar soft tissues, and, in some
cases, the eyelids.
• The skin incision should be placed well clear of the

malignancy
• The anterior and posterior ethmoidal vessels
should be cauterized and divided, along with the
nasolacrimal duct and any vessels crossing
• The ophthalmic artery should also be cauterized
with bipolar diathermy, and any persistent bleeding
from the bones should be plugged with bone wax.
• With skin-sparing exenteration, the orbicularis of
the residual upper and lower lid flaps are sutured
using buried 5/0 absorbable sutures, and the skin
closed with continuous 6/0 nylon

Suggested Reading
1. Harris GJ, Logani SC (1999) Eyelid crease incision for lateral orbitotomy. Ophthalmic Plast Reconstr Surg 15:9–16
2. HendersonJW(1994)Orbitaltumors,3rdedn.Raven Press, New York
3. Klingenstein A, Haug AR, Miller C, Hintschich C (2015) Ga-68-DOTA-TATE PET/CT for discrimina- tion of tumors of the optic pathway. Orbit 34(1):16–22
4. Lacey B, Chang W, Rootman J (1999) Nonthyroid causes of extraocular muscle disease. Surv Ophthalmol 44:187–213
5. Lacey B, Rootman J, Marotta TR (1999) Distensible venous malformations of the orbit: clinical and hemo- dynamic features and a new technique for management.
Ophthalmology 106:1197–1209
6. McNabAA,WrightJE(1990)Lateralorbitotomy—a review. Aust N Z J Ophthalmol 18:281–286
7. Moreiras JVP, Prada MC, Coloma J, Beverra EP (2004) Orbit: examination, diagnosis, microsurgery and pathology, 1st edn. Highlights of Ophthalmology International, Panama
8. Rootman J (1999) Diseases of the orbit. A multidisciplinary approach, 2nd edn. J.B. Lippincott, Philadelphia
9. Rootman J, Kao SC, Graeb DA (1992) Multidisciplinary approaches to complicated vascular lesions of the orbit. Ophthalmology 99:1440–1446
10. Rootman J, Stewart B, Goldberg RA (1995) Orbital surgery. A conceptual approach, 1st edn. Lippincott- Raven, Philadelphia
11. Rose GE (1996) Clinical examination in orbital disease. In: Bosniak S (ed) Principals and practice of ophthalmic plastic and reconstructive surgery, 1st edn. W.B. Saunders,
Philadelphia, pp 860–873
12. Rose GE, Wright JE (1992) Pleomorphic adenomas of the lacrimal gland. Br J Ophthalmol 76:395–400
Rose GE, Wright JE (1994) Trigeminal sensory loss and orbital disease. Br J Ophthalmol 78:427–429
13. Wright JE, McNab AA, McDonald WI (1989) Primary optic nerve sheath meningioma. Br J Ophthalmol 73:960–966
14. Wright JE, McNab AA, McDonald WI (1989) Optic nerve glioma and the management of optic nerve tumors of the young. Br J Ophthalmol 73:967–974
15. Wright JE, Rose GE, Garner A (1992) Primary malignant neoplasms of the lacrimal gland. Br J Ophthalmol 76:401–407
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Presenter:

dr. Endra Wibisono

Ilmiah Divisi Neuro-Onkologi

Rare, incidence is less than 1 per 100,000

More than 50% of all orbital tumors

No significant male or female

Orbital neoplasms comprise about 20% of

Ilmiah Divisi Neuro-Onkologi

16.3 Orbital Anatomy

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16.3. Orbital Anatomy

The bony orbital entrance is the strongest part of

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• The supraorbital neurovascular  supraorbital notch ->

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• The optic nerve :

• The intracanalicular part is immobile as

• The orbit can be divided into four surgical spaces:

Ilmiah Divisi Neuro-Onkologi

2. Extraconal Space. 4. Sub-tenon’s Space.

Ilmiah Divisi Neuro-Onkologi

Ilmiah Divisi Neuro-Onkologi

Proptosis—by a retrobulbar mass, such as intraconal cavernous

The globe displaced vertically or horizontally, due to a lacrimal

Ilmiah Divisi Neuro-Onkologi

conjunctival inflamma- tion and swelling (chemosis)

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optic nerve head swelling

choroidal folds (Fig. 16.2b),

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Fig. 16.2 (a–c) Adult onset proptosis of slow progres- sion

(a) Slowly progressive left proptosis with reduced visual acuity

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Current state Previous sinus

Ilmiah Divisi Neuro-Onkologi

Ilmiah Divisi Neuro-Onkologi

• The orbital margin -> notches, or foreign body

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Examination of Globe Position.

• Proptosis, axial anterior displacement of the globe,

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• Proptosis is mainly due to retrobulbar masses

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Extraocular Muscle and Periorbital Sensory Functions.

- Extraocular muscle function is assessed by asking the patient to follow a fingertip

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Assessment of Visual Functions.

• A normal acuity does not exclude orbital disease

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Visual Field Assessment

- Confrontational visual field testing

Optic nerve sheath meningioma.

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• Provides useful information about the location, size, shape,

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Malignant Pleomorphic Adenoma.

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Pros: • Rarely specific for a diagnosis and is not routinely

Magnetic Resonance Imaging

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• PET/CT combines morphological

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16.6 An Approach to Differential Diagnosis

• The rate of onset can be consid- ered