Orbital TumorsJames M. Ridgway, MD

Presentation

• 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.• Neck: No LAD.

Work Up

• Labs ?• Imaging Studies?

Differential

• 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

• Intraconal space: – Contains CN II, ophthalmic artery, superior

division of CN III, nasociliary nerve (V1), inferior division of CN III, and CN VI.

• Extraconal space:– 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.

Bony Orbit• F: Frontal bone

with associated suture and notch.

• <- Trochlea• <- Optical Canal• S: Superior

orbital fissure• I: Inferior orbital

fissure• L: Lacrimal bone• E: Ethmoid bone.

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.

Schwannoma

• 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).

Lymphoma

• After inflammation and hemangioma this is the third most common cause of proptosis.

• Usually insidious onset, typically presents with proptosis, ptosis, diplopia, motility disorders.

• Of all orbital sites, the lacrimal gland is the most common site involved.

• Commonly will mold itself along the globe margin rather than invade. Bilateral occurrence is common.

Rhabdomyosarcoma

• Most common orbital tumor in children.• 90% of cases occur before age 16.• Rapidly progressive but painless exophthalmos, proptosis,

and ptosis.• Arises from

primative mesenchymal elements into 4 different types: embryonal >> pleomorphic, alveolar (worst prognosis) >> differentiated (best prognosis).

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.

Capillary Hemangioma

• One third are diagnosed at birth and over 90% are visible by 6 months of age.

• Bony erosion is not observed, although expansion of the walls is possible.

• Telangiectatic vessels typical create strawberry appearance.

• Frequently produce globe displacement and enlarge with crying.

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.

• In the pediatric population metastatic disease is far less common.

Diagnosis

• 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.

• With open biopsy a histological diagnosis is commonly made because enough of a specimen usually is obtained.

Surgical Considerations

• Some conditions (ex. Pseudotumor, capillary hemangioma) do not mandate surgical intervention.

• Surgical approach is tailored to the location of the lesion with minimization of infraorbital venous pressure.

• 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.