Orbital Tumors

Michael Underbrink, MD

Faculty Advisor: Shawn Newlands, MD, PhD

The University of Texas Medical Branch

Department of Otolaryngology

Grand Rounds Presentation

October 31, 2001

Anatomy - Bony

Anatomy - Bony

Anatomy - Bony

Anatomy - Bony

Anatomy - Bony

Anatomy – Fascial Compartments

Anatomy – Fascial Compartments

Anatomy – Eyelid

Anatomy – Eyelid

Anatomy – Blood Supply

Anatomy – Blood Supply

Anatomy – Lacrimal System

Secretory and excretory system

Secretory

Lacrimal gland

Conjunctival goblet cells, accessory subconjunctival

glands and meibomian glands

Excretory

Removes tears via contraction of the eyelids

Anatomy – Lacrimal System

Evaluation of Orbital Tumors

Good history and physical examination

Elicit history of allergies, sinus infection, epistaxis,

or nasal congestion

PMHx – thyroid?, autoimmune?

Ophthalmic exam essential

Look for visual acuity/fields, ocular motility and

pupillary responses (RAPD); palpate

Evaluation Continued

Exophthalmos – 90%

“Worm’s eye” view

Protrusion more than

21mm beyond rim

One globe displaced >

2mm relative to the other

Direction of displacement

important

Evaluation – laboratory and imaging

CBC, ESR, and TFT’s

Imaging most important to define extent and

location

Ultrasonography – inexpensive, safe, cystic vs. solid

CT scanning – most widely used, bony landmarks

MRI – useful for intracranial disease and vascular

lesions

Arteriography – good for certain vascular disease

Pediatric Orbital Tumors

Differs substantially from adult types

More often congenital lesions and infectious

Most common – cystic lesions (dermoids)

2nd most common – vascular lesions

Most common malignancy - rhabdomyosarcoma

Cystic Lesions – Dermoid Cyst

Most common

Preschool child

Superotemporal mass

Mobile and nontender

Well circumscribed on CT

with rare bony remodeling

Cystic Lesions – Dermoid Cyst

Deeper lesions usually

show bony abnormality

May present with

proptosis and visual c/o

Surgical excision at

around 1 year of age

Cystic Lesions - Teratoma

Rare congenital germ-cell

tumors

Ectodermal, mesodermal

and endodermal elements

Present at birth, usually

with significant morbidity

Massive proptosis with

large intraconal masses

Cystic Lesions - Teratoma

Cystic Lesions - Teratoma

Vasculogenic Lesions – Capillary

Hemangioma 1/3 diagnosed at birth

90% visible by 6 months

Most common presenting as superficial tumor that

develops “strawberry” appearance

Enlarge with Valsalva

CT/MRI show diffusely infiltrating non-

encapsulated mass

Capillary Hemangioma

Capillary Hemangioma

Usual course

Normal at birth – noticed at one month – enlarge till 1 to

2 years of age – spontaneous involution by age 4 to 8 yr

Cosmetic sequelae minimal

Visual complications – amblyopia or astigmatism

Major complications – superinfection, ulceration

Rare complications – Kasbach-Merrit, HO cardiac

Capillary Hemangioma - Outcome

Capillary Hemangioma - Treatment

Indications include any complication

Medical therapy – steroids (systemic, intralesional)

or interferon

Radiation therapy

Surgical resection for unresponsive or well-

encapsulated lesions

Capillary Hemangioma

Lymphangioma

Benign congenital vascular malformations

May involve conjunctiva, eyelids or deep orbit

Usually identified prior to teenage years

Usually slow enlargement and increasing proptosis

Sudden proptosis from hemorrhage into cyst

No enlargement with Valsalva

CT/MRI shows multi-compartmental nature

Lymphangioma

Lymphangioma

Lymphangioma

Treatment for significant proptosis, corneal

exposure or optic nerve compression

Debulking and cyst drainage usually

Complete removal often not possible

Rhabdomyosarcoma

Most common malignant tumor in children

Presents within 1st decade

Rapid unilateral proptosis and globe displacement

CT scan shows irregular margins and often bony

destruction

Excisional biopsy ASAP for diagnosis if suspected

Rhabdomyosarcoma

Rhabdomyosarcoma

Take as much tumor as

possible on biopsy

Disseminated or gross

residual disease after

biopsy carries 35% 5-year

survival rate

Chemotherapy and XRT

after biopsy (90% 5-yr for

localized disease)

Rhabdomyosarcoma

Optic Nerve Glioma

3rd most common in children

May occur randomly although often associated

with NF type I (up to 50%)

Mean age – 8 years

Proptosis and visual symptoms

Headache and pain with intracranial extension

Diagnosis clinically and radiographically

Optic Nerve Glioma

Optic Nerve Glioma

CT/MRI shows fusiform enlargement of optic nerve

MRI for intracranial extension

Significant mortality once into chiasm

Must be excised while confined to nerve, esp. if blind or proptotic

Fibrous Dysplasia

Most often fibro-osseous

tumor

Occurs in 1st two decades

Replacement of normal

bone with immature

woven bone

Polyostotic (Albright’s)

and monostotic types

Fibrous Dysplasia

Fibrous Dysplasia

Usually stabilize after puberty

Conservative treatment the rule

Complete resection preferable for significant

cosmetic deformity or vision loss

Craniofacial reconstruction with neurosurgeon

Metastatic Tumor: Neuroblastoma

Most frequent in kids

Neuroblastoma accounts for 10% of all childhood malignancies

Primary: usually adrenal

Bilateral metastasis with eyelid ecchymoses and proptosis common

Survival rate – 15%

Adult Orbital Tumors

Vary significantly from children

Most common

Carcinomas

Pseudotumor

Lacrimal gland tumors

Lymphomas

Cysts, meningiomas, vascular tumors

Paranasal Sinus Masses

Masses of the paranasal sinus potentially can

spread to involve the orbit

Most common: mucocele

Neoplasms of this area are uncommon, but

frequently involve orbit

Benign tumors push periorbita, malignant invade

Mucoceles

Obstruction of ostium in a

sinus

Enlarging fluid filled sinus

Erodes through bony orbit

wall

Most arise from frontal

and ethmoid

CT – homogenous mass

Mucoceles

Neoplasms of Paranasal Sinus

Uncommon

Most common – SCCa

Orbital invasion in 2/3 of patients with SCCa

Glandular malignancies from minor salivary

glands or respiratory epithelium

Orbital extensive gives poor prognosis

Biopsy to Dx; radical resection to treat

Neoplasms of Paranasal Sinus

Orbital Pseudotumor

Idiopathic orbital inflammation

1905 by Birch-Hirschfiel first described

Excludes systemic diseases (sarcoid, thyroid,

autoimmune and Wegener’s)

2nd to 7th decade

Multifocal involvement of any orbital structure

Orbital Pseudotumor

Proptosis – acute onset of a few days

Eyelid swelling, chemosis and diplopia also common

Visual loss with optic nerve involvement

CT findings – hazy enlargement of affected structures

Treatment – Steroids, immunosuppresive meds, radiation therapy when steroids adverse

Orbital Pseudotumor

Lacrimal Gland Tumors

Enlargement of lacrimal fossa with displacement

of globe and no inflammatory signs

50% epithelial, 50% lymphoproliferative

CT scan – lymphoid show smooth enlargement of

gland, epithelial are irregular

Primary epithelial neoplasms of lacrimal gland are

rare

Lacrimal Tumor – Pleomorphic

Adenoma Benign mixed tumor

Most common of these

20 to 50 years

Painless proptosis with

inferior/medial globe

displacement

Many months or years

Excisional biopsy (total)

Lacrimal Tumor – Adenoid Cystic

Carcinoma Most common malignant

of these

Progressive onset of symptoms

Pain and numbness

CT with bony destruction and infiltration

50% mortality, requires aggressive surgical Tx

Lymphoid Tumors

Incidence between 4 to 13 % of all orbital tumors

Primary or secondary to systemic disease

Most patients who present with localized orbital

disease will develop systemic lymphoma

Presents between age 50 and 70

Anterior, “salmon patch” mass causing progressive

painless proptosis

Lymphoid Tumors

Lymphoid Tumors

Generous biopsy needed to make diagnosis

Systemic workup necessary

Localized orbital lymphoma – XRT

Systemic lymphoma – XRT + chemotherapy

Consultation of oncologist should be obtained

Orbital Meningiomas

4th to 7th decade of life, rare in children

Most (70%) invade from cranium

Primary orbital meningiomas may arise from optic

nerve

Proptosis, visual disturbances, headache and

diplopia

CT/MRI fusiform enlargement of optic nerve

Orbital Meningiomas

Schwannomas

Neurilemoma – benign, non-invasive peripheral

nerve tumor, from any nerve in orbit

Rare, ages 20 to 70 years

CT/MR show well circumscribed ovoid mass

Most commonly intraconal, may be extraconal

(trochlear, supraorbital nerves)

Schwannomas

Cavernous Hemangioma

a.k.a encapsulated venous malformation

Most common vascular lesion of adults

Peak incidence – middle age (40 years)

Women > men

Slowly progressive painless proptosis over several

years

do not enlarge with Valsalva, but grow slowly

Cavernous Hemangioma

CT/MRI reveals well-

defined mass, oval

Homogeneous with

increased density – CT

MRI – isointense to

muscle

Treatment –surgical

excision, recur rarely

Cavernous Hemangioma

Metastatic Tumors

8% of all orbital tumors

Most common in women – breast (& overall)

Most common in men – prostate & lung

Symptoms – proptosis, diplopia, pain, vision loss

Presents in 7th decade

Prognosis is very poor (avg. survival 10 months)

XRT usual; Chemo and Hormonal occasional

Metastatic Tumor - Breast

Conclusions

Orbital anatomy very complex with close

association to sinuses and cranial vault

Broad range of diseases and tumors

Important to recognize the signs of possible orbital

malignancy

Ophthalmologic consultation always

Often need multi-specialty cooperation