Orbital Tumors

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 – 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  Ultrasonography

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 nonencapsulated 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 wellencapsulated 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

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  Most

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 – 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  Proptosis

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 – 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)  Neurilemoma

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 welldefined 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