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
