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Orbital tumors refer to benign or malignant neoplasms arising from or extending into the bony orbit and its contents, including the globe, extraocular muscles, nerves, vessels, lacrimal gland, fat, or bone. They may be primary, secondary (extension from adjacent structures), or metastatic.

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Classification


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WHO Tumor Classification (5th edition)

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Anatomical classification


Compartment Common Tumors
Intraconal (within muscle cone) ‣ ,‣, ‣
Extraconal (outside muscle cone) Dermoid cyst, ‣, ‣
Intracanalicular/optic nerve sheath
Bone ‣ , metastases, ‣
Globe (intraocular tumors) ‣ , ‣ , uveal metastases

![Soft-tissue orbital compartments on CT and MRIa Axial and (b) coronal NCCT images in a normal 7-year-old male illustrating the location and appearance of the soft-tissue orbital compartments. c Axial and (d) coronal T2-weighted MR images in a normal 5-year-old male illustrating the location and appearance of the soft-tissue orbital compartments. The soft-tissue orbital compartments shown include the ocular space (O, contains the globe), optic nerve-sheath complex (ONS, contains the optic nerve, nerve sheath, cerebrospinal fluid, ophthalmic artery, central retinal artery and vein), intraconal space (IS, contains orbital fat, ophthalmic artery, cranial nerves 2, 3, 4, and V1), myofascial cone (MC, contains extraocular muscles, cranial nerve 6), and extraconal space (ES, contains fat, lacrimal gland and sac, superior ophthalmic vein, cranial nerve V1—lacrimal and frontal nerves)

Gerrie, S.K., Rajani, H., Navarro, O.M. et al. Pediatric orbital lesions: non-neoplastic extraocular soft-tissue lesions. Pediatr Radiol 54, 910–921 (2024). https://doi.org/10.1007/s00247-024-05892-x](attachment:e8a307ea-9eba-403d-82e3-2f8c742c30ea:image.png)

Soft-tissue orbital compartments on CT and MRIa Axial and (b) coronal NCCT images in a normal 7-year-old male illustrating the location and appearance of the soft-tissue orbital compartments. c Axial and (d) coronal T2-weighted MR images in a normal 5-year-old male illustrating the location and appearance of the soft-tissue orbital compartments. The soft-tissue orbital compartments shown include the ocular space (O, contains the globe), optic nerve-sheath complex (ONS, contains the optic nerve, nerve sheath, cerebrospinal fluid, ophthalmic artery, central retinal artery and vein), intraconal space (IS, contains orbital fat, ophthalmic artery, cranial nerves 2, 3, 4, and V1), myofascial cone (MC, contains extraocular muscles, cranial nerve 6), and extraconal space (ES, contains fat, lacrimal gland and sac, superior ophthalmic vein, cranial nerve V1—lacrimal and frontal nerves)

Gerrie, S.K., Rajani, H., Navarro, O.M. et al. Pediatric orbital lesions: non-neoplastic extraocular soft-tissue lesions. Pediatr Radiol 54, 910–921 (2024). https://doi.org/10.1007/s00247-024-05892-x

Demographic classification:


Children:

Tumor Notes
Capillary hemangioma Most common benign orbital tumor in infants
Rhabdomyosarcoma Most common malignant orbital tumor in children
Lymphangioma Venolymphatic malformation; may hemorrhage acutely
Optic nerve glioma Often associated with NF1
Neuroblastoma metastasis Common in younger children; “raccoon eyes”
Leukemia Orbital involvement as chloroma

Adults:

Tumor Notes
Lymphoma (MALT) Most common orbital tumor in adults
Cavernous hemangioma Most common benign adult orbital tumor
Meningioma Arises from optic nerve sheath
Metastasis Breast, prostate, lung, kidney common sources
Pleomorphic adenoma, adenoid cystic carcinoma

Clinical Features


Symptom Description
Proptosis Axial or non-axial, painless (commonly) or painful (if aggressive or inflamed)
Globe displacement Depending on tumor location (e.g., inferomedial with lacrimal gland mass)
Diplopia From mass effect or extraocular muscle involvement
Vision loss If optic nerve compressed or infiltrated
Pain Usually absent unless inflammatory or aggressive lesion
Lid swelling / palpable mass Common with superficial lesions

Radiology


Modality Utility
CT Bone detail: Excellent; detects bony remodeling or destruction
Calcification: Common in optic nerve sheath meningioma, retinoblastoma
Density of mass: Cystic, solid, or fat-containing components
MR T1: Defines anatomy; fat is hyperintense
T2: Fluid-sensitive; cystic or vascular tumors are hyperintense
Post-contrast T1FS: Identifies enhancement patterns (e.g., homogenous in lymphoma)
DWI/ADC: Restricted diffusion suggests high cellularity (e.g., lymphoma, metastasis)