Intraconal Lesion with Optic Nerve Involvement

1.   Optic nerve glioma

2.   Optic nerve sheath meningioma (10% of orbital neoplasm)

3.   Optic neuritis

4.   Inflammatory pseudotumor (may surround optic nerve)

5.   Sarcoidosis

6.   Intraorbital lymphoma (may surround optic nerve, older patient)

7.   Elevated intracranial pressure

→  distension of optic sheath

√  bilateral tortuous enlarged optic nerve-sheath complex

OPTIC NERVE TRAM-TRACK SIGN

1.   Optic nerve sheath meningioma

2.   Orbital pseudotumor

3.   Perioptic neuritis

4.   Perioptic hemorrhage

5.   Sarcoidosis

6.   Lymphoma / leukemia

7.   Metastasis

8.   Erdheim-Chester disease = systemic xanthogranulomatosis

Intraconal Lesion without Optic Nerve Involvement

1.   Cavernous hemangioma

2.   Orbital varix

3.   Carotid-cavernous fistula

4.   Arteriovenous malformation

least common of orbital vascular malformations (congenital, idiopathic, traumatic)

√  irregularly shaped intensely enhancing mass of enlarged vessels

√  associated with dilated superior / inferior ophthalmic v.

5.   Hematoma

6.   Lymphangioma

7.   Neurilemmoma

√  commonly adjacent to superior orbital fissure, inferior to optic nerve

√  local bone erosion

8.   Rhabdomyosarcoma (mostly extraconal)

Extraconal lesion

Extraconal-intraorbital Lesion

A.  BENIGN TUMOR

1.   Dermoid cyst

2.   Teratoma

<  1% of all pediatric orbital tumors

√  ± areas of fat, cartilage, bone

√  expansion of bony orbit ± bone defect

3.   Capillary hemangioma

4.   Lymphangioma

5.   Plexiform neurofibroma

6.   Inflammatory orbital pseudotumor

7.   Histiocytosis X

lesion usually arises from bone

B.  MALIGNANT TUMOR

1.   Lymphoma / leukemia

2.   Metastasis

3.   Rhabdomyosarcoma

mnemonic:   MOLD

Metastasis

Others (rhabdomyosarcoma, lymphangioma, sinus lesion)

Lymphoma, Lacrimal gland tumor

Dermoid

Extraconal-extraorbital Lesion

A.  FROM SINUS

maxillary / sphenoid sinuses are rare locations of origin

1.   Tumor:

squamous cell carcinoma (80%), lymphoma, adenocarcinoma, adenoid cystic carcinoma

2.   Mucocele

3.   Paranasal sinusitis:

◊  Most common cause of orbital infection!

Origin:   from ethmoid sinuses (in children), from frontal sinus (in adolescence)

√  preseptal / orbital edema / cellulitis

√  subperiosteal / orbital abscess

√  mucormycosis (in diabetics) destroys bone and extends into cavernous sinus

B.  FROM SKIN

1.   Orbital cellulitis

C.  FROM LACRIMAL GLAND

√  mass arising from superolateral aspect of orbit

Dilatation of Superior Ophthalmic Vein

1.   Carotid-cavernous sinus fistula

2.   Cavernous sinus thrombosis

3.   Venous varix

4.   Graves disease

5.   Normal variant

ORBITAL MASS

Orbital Mass in Adults

(a)  vasculogenic orbital mass

1.   Cavernous malformation

2.   Hemangiopericytoma

(b)  lymphoproliferative orbital lesion

1.   Typical / atypical lymphoid hyperplasia

2.   Lymphoma of orbit

(c)  lacrimal gland lesion (5–14% of biopsied orbital masses)

›  epithelial (largely neoplastic) lesion (40–50%)

1.   Pleomorphic adenoma

2.   Adenoid cystic carcinoma

›  nonepithelial lesion

1.   Dacryoadenitis

2.   Lacrimal gland lymphoma

(d)  optic nerve & meningeal lesions

1.   Optic pathway glioma

2.   Optic nerve sheath meningioma

(e)  peripheral nerve sheath lesions

1.   Schwannoma (rare)

2.   Neurofibroma

3.   Malignant peripheral nerve sheath tumor

(f)  Primary orbital melanoma

(g)  Metastasis to orbit

Orbital Mass in Childhood

mnemonic:   LO VISHON

Leukemia, Lymphoma

Optic nerve glioma

Vascular malformation: hemangioma, lymphangioma

Inflammation

Sarcoma: ie, rhabdomyosarcoma

Histiocytosis

Orbital pseudotumor, Osteoma

Neuroblastoma

Osseous Orbital Lesion in Children

(a)  development of the osseous orbit

1.   Dermoid inclusion cyst

2.   Epidermoid inclusion cyst

(b)  primary bone lesions

1.   Fibrous dysplasia

2.   Juvenile ossifying fibroma

3.   Cementifying fibroma

4.   Langerhans cell histiocytosis

(c)  malignant tumors involving multiple sites

1.   Granulocytic sarcoma

2.   Hematogenous metastasis: neuroblastoma (most common primary tumor to involve orbit)

(d)  primary malignant bone tumor

1.   Osteosarcoma

Nonosseous Lesion of Extraocular Orbit in Children

1.   Rhabdomyosarcoma

2.   Infantile hemangioma

3.   Lymphangioma

4.   Infantile fibromatosis

Primary Malignant Orbital Tumors

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