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DR Eslam Mohamad Abd EL-Ghaffar

Ahmed Maher Teaching Hospital

Tumors that involve the orbit can be classified into two major
-primary tumors of the orbit
-tumors with other sites of origin that extend into the orbit.

The most frequent primary orbital tumors in adults include lymphoid

tumors, cavernous hemangiomas, and meningiomas,
whereas dermoid cysts, capillary hemangiomas, and
rhabdomyosarcoma predominate in children
The most common tumors that extend into the orbit are
meningiomas, followed by sinonasal carcinomas.

The most frequent initial symptom of an orbital

mass is proptosis, which occurs in 44% of patients.
Change in visual acuity is often a late finding or
indicates a tumor that is close to the orbital apex or
optic nerve.
Orbital tumors can also be divided into three
categories based on their location within the
intraconal (within the extraocular muscle cone),
(2) extraconal,
(3) intracanalicular (within the optic canal)

Intraconal tumors tend to cause early vision loss and

impairment of ocular motility, as well as axial proptosis.
These effects result from direct pressure on the optic
nerve and impingement on extraocular muscles.
Extraconal tumors cause proptosis as an early
Intracanalicular tumors cause early vision
loss,papilledema, and the appearance of optociliary
shunt vessels on the surface of the optic discs.
These tumors cause minimal or no proptosis


The orbit is a cone-shaped structure with an approximate volume

of 30 cm.the base of the cone is quadrangular, with its widest
dimension just posterior to the orbital rim. The apex is formed by
the optic canal (containing the optic nerve and ophthalmic artery)
and superior orbital fissure (gateway for the superior and inferior
divisions of the oculomotor nerve, the trochlear nerve, branches of
V1, the abducens nerve, the superior ophthalmic vein, and
sympathetic fibers from the cavernous sinus).
The orbit is composed of seven different bones. Its roof is part
of the frontal bone, and the floor consists of parts of the maxilla
and zygoma. Its lateral wall is formed by the zygoma and greater
sphenoid wing, and the medial wall has contributions from the
maxilla, lacrimal bone, and ethmoid bone. Medially, the sphenoid
and ethmoid sinuses border the inner aspect of the cone, as well
as the optic canal.

5% and 20% of orbital tumors
They can be purely intraorbital or within the
sphenoid wing or periorbita with secondary orbital
Typically manifest in women in their
fourth or fifth decade
they may be associated with
Periorbital meningiomas

Arise from the superolateralquadrant of

the orbit, usually the superior
orbital fissure.
Can extend intracranially.

Optic nerve sheath meningiomas

take origin within the dural sheath of the optic nerve
anywhere from the globe to the optic canal.
progressive visual loss and rarely proptosis.


Sphenoid wing meningiomas

surgical resection by the pterional approach.
If there is optic canal involvement, optic canal decompression
should be performed.
Excision of the involved bone and dura is important.
Postoperative irradiation for incompletely resected tumors will
retard regrowth of the tumor.

Optic nerve sheath meningioma

Patients with stable vision and anteriorly located or apical
meningioma are followed closely with imaging and visual studies.
With progressive visual loss, external beam radiation therapy (50
Gy) is recommended. There is evidence that radiation can arrest
the growth of optic nerve sheath meningiomas and preserve
Aggressive resection in sighted patients results in postoperative
visual loss!.
If the vision is lost completely, the tumor is resected
transcranially, especially if growth is demonstrated on


Optic nerve gliomas account for 2% to 5% of orbital tumors.
Typically occur in the first decade of life
Can be solitary or associated with neurofibromatosis type 1
When bilateral, they are pathognomonic of NF1.

Management of Optic Nerve Glioma

Without evidence of radiologic growth or clinical

deterioration, observation is the best management. Surgery
is recommended when there is significant loss of vision or
radiologic progression.
Surgery is resection of the optic nerve
Chiasmatic involement requires radiation therapy.
Malginant variant treated also by radiation therapy with

Slowly progressive
visual loss and
Histologically, they
are low-grade