Orbital tumors

• HPI: Patient is a 63 year old female with a one year history of a right orbital mass that has mildly enlarged during this time period.. There is a hard 1 cm mass present on the superi

Orbital Tumors

James M Ridgway, MD

• CC: Right eye mass

• HPI: Patient is a 63 year old female with a one year history

of a right orbital mass that has mildly enlarged during this time period The patient states a history of trauma to the given area 1.5 years ago, but did not note any sequelae

during the following six months She recently went to her PCP physician who subsequently referred to patient to UCI

Detailed Medical History

• PMH: DM, hypothyroidism, and a CVA at 36

years of age with right sided paralysis (full

recovery) Hospitalized for 7 days approximately three years ago for “thyroid problems”.

• PSH: Cystectomy, ilieostomy, distal

pancreatectomy and total colectomy Minor

cysts removed from left arm and leg.

• Meds: Metoprolol, ASA, Synthroid.

• Allergies: Demerol (hives).

• FH: Colon polyps, breast cancer.

• SH: - EtOH, +TOB (1ppd X 40 years).

Physical Exam

• General: NAD, A&OX3, normal weight, afebrile.

• Ears: TM are clear and mobile bilaterally.

• Eyes: EOMI, PERRL, globes are in correct

position and proportion (no ptosis,

exophthalmos, dystopia, epiphoria, diplopia, or change in visual acuity) There is a hard 1 cm

mass present on the superior-medial aspect of the right orbital rim extending to the right upper eyelid The overlying tissues are not adherent.

• Nose: No mucosal lesions.

• OC/OP: No observed masses/lesions.

Work Up

• Labs ?

• Imaging Studies?

• V: cavernous hemangioma (venous malformation), capillary

hemangioma, lymphangioma, venous varix, AVM, hematic cyst.

• I: sinusitis (including invasive fungal), cavernous sinus

thrombosis, osteomyelitis of orbital bones, infection of ocular

adnexa, phlebitis of facial veins, dental infections, Whipple’s

disease, angiolymphoid hyperplasia with eosinophilia.

• T: hematoma, carotid cavernous fistula, foreign body.

• A: polyarteritis nodosa, orbital myositis, Wegener granulomatosis

• M: thyroid opthalmopathy.

• I: idiopathic orbital inflammatory disease (pseudotumor),

sarcoidosis, amyloidosis, sickle cell anemia.

• N: schwannoma, neurofibroma, meningioma, lymphoma,

histiocytosis X [Letterer-Siwe], leukemia, metastatic carcinoma, retinoblastoma, rhabdomyosarcoma, fibrous dysplasia, paranasal sinus tumors, lacrimal gland tumors.

• C: dermoid cysts, teratoma.

CT

Gardner’s Syndrome

• Familial adenomatous polyposis combined with

extraintestinal manifestations of sebacous cysts, osteomas (particularly of the mandible skull and long bones), and

desmoid tumors The mutation arises in the adenomatous polyposis coli (APC) 5q gene

Spaces of the Retrobulbar Orbit

• Cone:

– Composed of the four rectus muscles and

the thin intramuscular membrane which

joins them and extends posteriorly to the

insertion of the muscle tendons at the

orbital apex.

Spaces of the Retrobulbar Orbit

– Contains ophthalmic vein, lacrimal nerve

(V1), CN IV and frontal nerve (V1).

Other Locations.

• The lacrimal gland and

lacrimal sac as well as

the potential for

multiple compartment

involvement.

Evaluation - Review

• Detailed recording of onset, duration and

progression of the orbital disease.

• History of allergies, sinus infections, epistaxis, nasal discharge, and tearing to be reviewed to rule out sinonasal orgin.

• Review of systemic diseases (ex thyroid,

granulomatous and autoimmune) as well.

• PE: visual acuity, visual fields, pupillary

responses, ocular motility, globe surface,

exophthalmos, and direction of displacement.

• Complete head and neck evaluation.

• Lab/Imaging.

Separation of Lesions by Anatomic

Subsite

Cavernous Hemangioma

• Hamartomas contained within a fibrous capsule with large vascular

channels, but no definite feeding vessels.

• Most common benign tumor.

• Peak between 20-40 years.

• Slow growing, but easily enlarge with stress

proptosis.

• CT – sharp, well circuscribed, dense mass.

• Intra and extraconal.

• Arising from any nerve braches within the orbit – most

common V1

• Account for 1-6% of all orbital masses

• Slow growing, well circumscribed, ovid and homogenous

• Antoni A (spindle shaped cells), Antoni B (foamy cells)

• Most common orbital tumor in children

• 90% of cases occur before age 16

• Rapidly progressive but painless exophthalmos, proptosis, and ptosis

Dermoid Cyst

• Represent the most common

congenital lesion of the orbit

(1/3 of all childhood orbital

tumors)

• Arise as a sequestration of

ectoderm within the suture

lines of the orbital bones

• Commonly observed as a

painless mass in the

superiotemporal area at the

lateral portion of the

eyebrow

• Classified into juxtasutural,

sutural and soft tissue types

Carotid Cavernous Fistula

• Acute or delayed onset of post-traumatic diplopia with

proptosis and chemosis

• Venous flow reversal

• Orbital presentation is secondary to prominent anterior

venous drainage

Orbital Varix

• May be either congenital or acquired (thrombosis is common).

• Not neoplastic, but simple focal dilation that may be enlarged with increased venous pressure

• May be associated with intraorbital/ intracranial AVM or simply result from wall weakness.

Metastasis to the Orbit

• Accounts for approximately 10% of all orbital neoplasms

(5% hematogenous, 5% from adjacent structures)

• What is the most common tumor to spread to the orbit?

– Breast Cancer (42%).

– Lung Carcinoma (11%).

– Unknown Primary Cancer (11%).

– Prostate (8%).

– Melanoma (5%) Average survival after dx is a dismal 9 months.

– Metastatic neuroblastoma is second only to primary

retinoblastoma as the most frequent malignant tumor in childhood.

common.

• FNA is a minimally invasive technique that may be used for

diagnosing orbital lesions

• Differentiation between benign and malignant lesions

reaches an accuracy of 95%

• Coupled with clinical and radiological finding, proper

diagnosis is made in 80% of cases

• Disadvantages include poor cellular yield, difficulty in

interpreting the specimen, and the possible need for

another biopsy procedure

• Open biopsy of an orbital tumor is the common method of

obtaining tissue from the orbital lesion It also may be

necessary if FNA is inadequate

• Orbitotomy can be performed in anterior, lateral, medial,

cornal and even intracranial dissections can be made to gain inferior, supertemporal, posterior, or central access

• Close intraoperative monitoring of the pupil size and

administered medications in addition to post-operative

evaluation with regards to vision, bleeding, and pain is

essential is the treatment of these lesions.

• Finally, a thorough explanation of the procedure and the risks, benefits, and alternatives should be clearly explained and

documented The patient should be cognizant of the exact

procedure and it is imperative that the patient understands the possibility of orbital exenteration if indicated