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ORBITAL TUMOURSORBITAL TUMOURS
LocationLocation
A lesion in the orbit?
Decide whether it is an ocular lesion OR
 a non-ocular lesion, i.e. is it involving the globe
or involving the structures outside the globe.
If it is a non-ocular lesion, see the lesion is
involving which space.
INTRA ORBITAL SPACES
Intra-orbital pathology which is non-ocular is
either in the
Intraconal
conal
extraconal space.
Conal space
The ocular muscles within the orbit form a
muscle-cone.
These are connected via the annulus of Zin,
which is a fibrous connective tissue sheet.
The muscles and fascia together form the conal
space.
Intraconal space
within the muscle cone.
bounded by the cone formed by the extraocular
muscles.
Extraconal space
The extraconal space is the area outside the
muscle cone.
ORBITAL PATHOLOGIESORBITAL PATHOLOGIES
Intraconal space pathology:
Venous vascular malformation
Capillary hemangioma
Optic nerve lesions
◦ Optic neuritis
◦ Optic nerve glioma
◦ Optic nerve meningioma
Venous dilatation
◦ Carotid Cavernous Fistula
◦ Varices
Schwannoma of 3rd, 4th and 6th cranial nerve
Conal space pathology:
Thyroid eye disease
Pseudotumor
Enlargement of the extra-ocular muscles by
glycogen storage disease
 Lymphoma
Rhabdomyosarcoma
Extraconal space pathology:
Abscess due to sinusitis
Schwannoma of the trigeminal nerve
Dermoid
Bone lesions, Fibrous dysplasia of the sphenoid wing
Metastases
Diseases of the orbital appendages
ORBITAL LYMPHOMA
Presents in middle age with painless orbital
swelling progressing to proptosis.
Orbital lymphoma is of the B-cell variety
(NHL); Hodgkins disease of the orbit is rare.
Site of involvement
Any structure in the orbit may be affected.
The lacrimal gland is involved most frequently,
then the conal/intraconal compartment.
Superior rectus is the commonest extra-ocular
muscle involved.
RADIOLOGIC FEATURES:
CT findings:
a wide range of radiological findings.
It may be a well-defined hyperdense enhancing
mass OR
Can produce diffuse infiltration leading to
destruction of the normal anatomical
architecture.
Molds to the contour of the orbit without bone
destruction, unless it is very aggressive.
Enhanced axial CT image
demonstrates extension of
right orbital apex mass to the
right cavernous sinus (arrow)
via superior orbital fissure.
with proptosis. Enlargement
and abnormal enhancement
of right medial and lateral
rectus muscles could
represent
infiltration by tumour, but are
more likely due to venous
congestion from
cavernous sinus obstruction.
On MRI
lymphoma tends to be hypointense on T1, is
usually hyperintense on T2, and enhances.
Bilateral orbital masses suggest the diagnosis of
lymphoma.
T1-weighted MR image (A)
demonstrates proptosis
of right globe due to a large
intermediate signal intensity
lesion that involves the
lacrimal fossa and the right
lateral rectus muscle,with
extension posteriorly in the
extraconal compartment.
Postcontrast(B)
demonstrates homogeneous
enhancement.
(A) Axial T2, (B) axial T1, (C)
axial T1 MRI with
gadolinium, (D) coronal
T1 with gadolinium and fat
suppression.
A diffusely infiltrating mass in
the superior right orbit
,isointense to brain on T2-
& slightly hypointense on
T1sequence. The mass
extends outside the orbit
to involve the temporal
fossa. Following
gadolinium, there is
homogeneous signal
enhancement of the mass.
On coronal imaging, there
is thick meningeal
enhancement indicating
intracranial spread of
lymphoma.
RHABDOMYOSARCOMA
Most common primary malignant tumour of the
orbit in children.
A highly malignant tumour.
Av age of presentation:7 yrs.
M>F
presents with rapidly progressive
exophthalmos.
Originates from extra-ocular muscles,
nasopharynx, or paranasal sinuses.
Usually present in the superomedial orbit and
may produce bone destruction.
On CT,
A bulky aggressive-looking mass.
isodense or slightly hyperdense.
shows uniform enhancement.
Contrast-enhanced axial CT image through orbits demonstrates right
proptosis due to large, lobular, intraorbital mass.
Image at lower level demonstrates invasion of right maxillary sinus as well
as extension through lateral orbital wall,consistent with the aggressive
nature of this tumour.
(A) Axial and (B) coronal CT images with contrast medium.
There is a large mass in the superior right orbit which is difficult
to separate from the extra-ocular muscles. There is deformity of
the posterior wall of the globe and marked proptosis. The mass
shows uniform contrast enhancement.
On MRI
they are of intermediate signal intensity on both
T1 and T2 sequences.
There is bone destruction in 40 per cent of
cases and frequent distortion of the globe.
ORBITAL METASTASIS
6% of orbital tumours.
Most retrobulbar metastases are extraconal in
location,
subsequently encroach on the intraconal
compartment as they increase in size.
when large,produce infiltrating poorly
marginated masses.
originate mostly from the greater wing of the
sphenoid, resulting in bone destruction.
 In children, the primary lesions are most
commonly Ewing's sarcoma and neuroblastoma.
In Ewing's sarcoma, proptosis is usually
unilateral with sudden onset & accompanying
hemorrhage.
The presentation in neuroblastoma is similar;
however, it is bilateral in 50% of cases.
Other pediatric malignancies that metastasize
to the orbit are testicular tumors and
leukemias.
In adults, the primary tumor is usually breast or
lung carcinoma.
Tumor metastasizes more frequently to eye
than the orbit (8:1 ratio).
The orbital metastases may be the initial
manifestation of the lung, GI, thyroid, or renal
Ca.
 In adults, an infiltrative retrobulbar mass and
enophthalmos is characteristic of scirrhous
carcinoma of the breast.
RADIOLOGIC FINDINGS
Metastases often are diffusely infiltrating and
have indistinct margins. Less frequently, they are
well circumscribed.
On CT, these lesions are isodense or
hyperdense, and enhance.
Metastatic prostate carcinoma. Axial CT image (A) through
orbits demonstrates small lytic lesion of left lateral orbital wall in a patient
with prostate carcinoma. Soft-tissue windows (B) demonstrate contiguous
extension of soft tissue into lateral extraconal compartment with
medial displacement of the lateral rectus muscle.
Metastasis diffusely involving medial rectus muscle.Metastasis diffusely involving medial rectus muscle.
On MRI,
the signal intensity is low on T1-weighted
images and high on T2-weighted images.
These lesions enhance with contrast.
Ocular metastasis from systemic lymphoma. (A) Axial T2, (B) axial T1,
(C) axial T1 MRI with IV gadolinium and fat suppression. There is thickening of
the wall of the globe with soft tissue and enhancement extending into the
vitreous and retrobulbar space.
Coronal T1-weighted, fat-saturated MRI shows infiltration of the retrobulbar fat on right
and infiltration of the superior orbit on left. B,C, Axial T1-and T2 MRI show swelling and
infiltration by metastasis of the left orbit and eyelid.
DERMOID CYSTS
usually occur in children and make up 4% to 6%
of orbital tumors.
Painless mass, free from the skin, with variable
ocular displacement.
Mostly located near the lacrimal fossa or nasal
bone.
Grow slowly, remodeling adjacent bones or
sutures.
RADIOLOGIC FEATURES
The intensity of dermoid cyst is similar to that
of fat on MRI and CT, making diagnosis easy.
On CT
appear as well defined low attenuating (fat
density) lobulated masses. Calcifications may be
present in the wall. Enhancement is uncommon.
The central cavity may appear heterogeneous
as a result of keratin and other cystic debris.
This is a coronal CT scan demonstrating a dumbbell dermoid that straddles
the right lateral orbital wall. A bony channel in the lateral orbital wall connects
the two lobes. Note that the deep lobe displaces many of the lateral
intraorbital structures.
This coronal CT image without contrast demonstrates a lateral dermoid cyst
with the characteristic hyperdense cyst wall and hypodense cyst cavity.
On MRI
T1 -> typically hyperintense
droplets in the subarachnoid space may be
visible if rupture has occurred
T1 C+ (Gd) -> typically do not enhance
T2 - variable signal ranging from hypo to hyper
intense.
THANK YOU

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Orbital tumours

  • 2. LocationLocation A lesion in the orbit? Decide whether it is an ocular lesion OR  a non-ocular lesion, i.e. is it involving the globe or involving the structures outside the globe. If it is a non-ocular lesion, see the lesion is involving which space.
  • 3.
  • 4.
  • 5. INTRA ORBITAL SPACES Intra-orbital pathology which is non-ocular is either in the Intraconal conal extraconal space.
  • 6.
  • 7. Conal space The ocular muscles within the orbit form a muscle-cone. These are connected via the annulus of Zin, which is a fibrous connective tissue sheet. The muscles and fascia together form the conal space.
  • 8. Intraconal space within the muscle cone. bounded by the cone formed by the extraocular muscles.
  • 9.
  • 10.
  • 11. Extraconal space The extraconal space is the area outside the muscle cone.
  • 12.
  • 13.
  • 14.
  • 15.
  • 16. ORBITAL PATHOLOGIESORBITAL PATHOLOGIES Intraconal space pathology: Venous vascular malformation Capillary hemangioma Optic nerve lesions ◦ Optic neuritis ◦ Optic nerve glioma ◦ Optic nerve meningioma
  • 17. Venous dilatation ◦ Carotid Cavernous Fistula ◦ Varices Schwannoma of 3rd, 4th and 6th cranial nerve
  • 18. Conal space pathology: Thyroid eye disease Pseudotumor Enlargement of the extra-ocular muscles by glycogen storage disease  Lymphoma Rhabdomyosarcoma
  • 19. Extraconal space pathology: Abscess due to sinusitis Schwannoma of the trigeminal nerve Dermoid Bone lesions, Fibrous dysplasia of the sphenoid wing Metastases Diseases of the orbital appendages
  • 20. ORBITAL LYMPHOMA Presents in middle age with painless orbital swelling progressing to proptosis. Orbital lymphoma is of the B-cell variety (NHL); Hodgkins disease of the orbit is rare.
  • 21. Site of involvement Any structure in the orbit may be affected. The lacrimal gland is involved most frequently, then the conal/intraconal compartment. Superior rectus is the commonest extra-ocular muscle involved.
  • 22. RADIOLOGIC FEATURES: CT findings: a wide range of radiological findings. It may be a well-defined hyperdense enhancing mass OR
  • 23. Can produce diffuse infiltration leading to destruction of the normal anatomical architecture. Molds to the contour of the orbit without bone destruction, unless it is very aggressive.
  • 24. Enhanced axial CT image demonstrates extension of right orbital apex mass to the right cavernous sinus (arrow) via superior orbital fissure. with proptosis. Enlargement and abnormal enhancement of right medial and lateral rectus muscles could represent infiltration by tumour, but are more likely due to venous congestion from cavernous sinus obstruction.
  • 25.
  • 26. On MRI lymphoma tends to be hypointense on T1, is usually hyperintense on T2, and enhances. Bilateral orbital masses suggest the diagnosis of lymphoma.
  • 27. T1-weighted MR image (A) demonstrates proptosis of right globe due to a large intermediate signal intensity lesion that involves the lacrimal fossa and the right lateral rectus muscle,with extension posteriorly in the extraconal compartment. Postcontrast(B) demonstrates homogeneous enhancement.
  • 28. (A) Axial T2, (B) axial T1, (C) axial T1 MRI with gadolinium, (D) coronal T1 with gadolinium and fat suppression. A diffusely infiltrating mass in the superior right orbit ,isointense to brain on T2- & slightly hypointense on T1sequence. The mass extends outside the orbit to involve the temporal fossa. Following gadolinium, there is homogeneous signal enhancement of the mass. On coronal imaging, there is thick meningeal enhancement indicating intracranial spread of lymphoma.
  • 29. RHABDOMYOSARCOMA Most common primary malignant tumour of the orbit in children. A highly malignant tumour. Av age of presentation:7 yrs. M>F
  • 30. presents with rapidly progressive exophthalmos. Originates from extra-ocular muscles, nasopharynx, or paranasal sinuses. Usually present in the superomedial orbit and may produce bone destruction.
  • 31. On CT, A bulky aggressive-looking mass. isodense or slightly hyperdense. shows uniform enhancement.
  • 32. Contrast-enhanced axial CT image through orbits demonstrates right proptosis due to large, lobular, intraorbital mass. Image at lower level demonstrates invasion of right maxillary sinus as well as extension through lateral orbital wall,consistent with the aggressive nature of this tumour.
  • 33. (A) Axial and (B) coronal CT images with contrast medium. There is a large mass in the superior right orbit which is difficult to separate from the extra-ocular muscles. There is deformity of the posterior wall of the globe and marked proptosis. The mass shows uniform contrast enhancement.
  • 34. On MRI they are of intermediate signal intensity on both T1 and T2 sequences. There is bone destruction in 40 per cent of cases and frequent distortion of the globe.
  • 35. ORBITAL METASTASIS 6% of orbital tumours. Most retrobulbar metastases are extraconal in location, subsequently encroach on the intraconal compartment as they increase in size.
  • 36. when large,produce infiltrating poorly marginated masses. originate mostly from the greater wing of the sphenoid, resulting in bone destruction.
  • 37.  In children, the primary lesions are most commonly Ewing's sarcoma and neuroblastoma. In Ewing's sarcoma, proptosis is usually unilateral with sudden onset & accompanying hemorrhage.
  • 38. The presentation in neuroblastoma is similar; however, it is bilateral in 50% of cases. Other pediatric malignancies that metastasize to the orbit are testicular tumors and leukemias.
  • 39. In adults, the primary tumor is usually breast or lung carcinoma. Tumor metastasizes more frequently to eye than the orbit (8:1 ratio). The orbital metastases may be the initial manifestation of the lung, GI, thyroid, or renal Ca.
  • 40.  In adults, an infiltrative retrobulbar mass and enophthalmos is characteristic of scirrhous carcinoma of the breast.
  • 41. RADIOLOGIC FINDINGS Metastases often are diffusely infiltrating and have indistinct margins. Less frequently, they are well circumscribed. On CT, these lesions are isodense or hyperdense, and enhance.
  • 42. Metastatic prostate carcinoma. Axial CT image (A) through orbits demonstrates small lytic lesion of left lateral orbital wall in a patient with prostate carcinoma. Soft-tissue windows (B) demonstrate contiguous extension of soft tissue into lateral extraconal compartment with medial displacement of the lateral rectus muscle.
  • 43. Metastasis diffusely involving medial rectus muscle.Metastasis diffusely involving medial rectus muscle.
  • 44. On MRI, the signal intensity is low on T1-weighted images and high on T2-weighted images. These lesions enhance with contrast.
  • 45. Ocular metastasis from systemic lymphoma. (A) Axial T2, (B) axial T1, (C) axial T1 MRI with IV gadolinium and fat suppression. There is thickening of the wall of the globe with soft tissue and enhancement extending into the vitreous and retrobulbar space.
  • 46. Coronal T1-weighted, fat-saturated MRI shows infiltration of the retrobulbar fat on right and infiltration of the superior orbit on left. B,C, Axial T1-and T2 MRI show swelling and infiltration by metastasis of the left orbit and eyelid.
  • 47. DERMOID CYSTS usually occur in children and make up 4% to 6% of orbital tumors. Painless mass, free from the skin, with variable ocular displacement.
  • 48. Mostly located near the lacrimal fossa or nasal bone. Grow slowly, remodeling adjacent bones or sutures.
  • 49. RADIOLOGIC FEATURES The intensity of dermoid cyst is similar to that of fat on MRI and CT, making diagnosis easy.
  • 50. On CT appear as well defined low attenuating (fat density) lobulated masses. Calcifications may be present in the wall. Enhancement is uncommon. The central cavity may appear heterogeneous as a result of keratin and other cystic debris.
  • 51. This is a coronal CT scan demonstrating a dumbbell dermoid that straddles the right lateral orbital wall. A bony channel in the lateral orbital wall connects the two lobes. Note that the deep lobe displaces many of the lateral intraorbital structures.
  • 52. This coronal CT image without contrast demonstrates a lateral dermoid cyst with the characteristic hyperdense cyst wall and hypodense cyst cavity.
  • 53.
  • 54. On MRI T1 -> typically hyperintense droplets in the subarachnoid space may be visible if rupture has occurred T1 C+ (Gd) -> typically do not enhance T2 - variable signal ranging from hypo to hyper intense.
  • 55.

Editor's Notes

  1. divided into four main compartments, Intraconal space Extraconal space Optic nerve/sheath complex Globe.
  2. Although small, the orbits are a complex anatomical space. Determining which compartment/s pathology arises from, is important in reducing the differential diagnoses.
  3. Normal anatomy. Coronal CT reformat
  4. Normal anatomy - Coronal T1W MR
  5. The orbital spaces are important when considering different pathologies: globe subdivided into anterior and posterior chambers by the lens optic nerve-sheath complex optic nerve ophthalmic artery central retinal artery and vein surrounding sheath of meninges as an extension of the cerebral meninges
  6. conal space extraocular muscles interconnecting fascia
  7. intraconal space orbital fat ophthalmic artery superior ophthalmic vein cranial nerves II, III, IV, V1, VI
  8. axial CT section through the orbit (Blue - intra conal space, Red - Globe)
  9. extraconal space fat lacrimal gland and sac portion of the superior ophthalmic vein
  10. Orbital lymphoma comprises 55% of orbital malignancies. Unilateral and bilateral presentations can occur within any orbital compartment, although the lacrimal gland, (found within the extraconal compartment) is the most common. The CT appearance is of a hyperdense, enhancing mass which may have a spectrum of appearances from well defined to diffusely infiltrative. On MRI there is low to intermediate signal on T2 weighted images.
  11. Bilateral lymphoma - Coronal CT reformat
  12. extraconal tumour Rhabdomyosarcoma is a highly malignant, extraconal tumour of childhood, often with rapidly progressing proptosis. The most common age of development is between 5 and 10 years. The CT presentation is of a well-defined but irregular, muscle-like density mass in the extraconal space, although intraconal extension is possible. The MRI appearance is of a hyperintense T2 mass, hypointense T1 (relative to muscle) with marked uniform enhancement
  13. Angular dermoid
  14. intracranial dermoids have more variable signal characteristics