_tumors university of texas medical branch眼眶肿瘤德克萨斯大学医学分校课件

Orbital Tumors Michael Underbrink, MD Shawn Newlands, MD, PhD 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 vSecretory and excretory system vSecretory vLacrimal gland vConjunctival goblet cells, accessory subconjunctival glands and meibomian glands vExcretory vRemoves tears via contraction of the eyelids Anatomy Lacrimal System Evaluation of Orbital Tumors vGood history and physical examination vElicit history of allergies, sinus infection, epistaxis, or nasal congestion vPMHx thyroid?, autoimmune? vOphthalmic exam essential vLook for visual acuity/fields, ocular motility and pupillary responses (RAPD); palpate Evaluation Continued vExophthalmos 90% v“Worms eye” view vProtrusion more than 21mm beyond rim vOne globe displaced 2mm relative to the other vDirection of displacement important Evaluation laboratory and imaging vCBC, ESR, and TFTs vImaging most important to define extent and location vUltrasonography inexpensive, safe, cystic vs. solid vCT scanning most widely used, bony landmarks vMRI useful for intracranial disease and vascular lesions vArteriography good for certain vascular disease Pediatric Orbital Tumors vDiffers substantially from adult types vMore often congenital lesions and infectious vMost common cystic lesions (dermoids) v2nd most common vascular lesions vMost common malignancy - rhabdomyosarcoma Cystic Lesions Dermoid Cyst vMost common vPreschool child vSuperotemporal mass vMobile and nontender vWell circumscribed on CT with rare bony remodeling Cystic Lesions Dermoid Cyst vDeeper lesions usually show bony abnormality vMay present with proptosis and visual c/o vSurgical excision at around 1 year of age Cystic Lesions - Teratoma vRare congenital germ-cell tumors vEctodermal, mesodermal and endodermal elements vPresent at birth, usually with significant morbidity vMassive proptosis with large intraconal masses Cystic Lesions - Teratoma Cystic Lesions - Teratoma Vasculogenic Lesions Capillary Hemangioma v1/3 diagnosed at birth v90% visible by 6 months vMost common presenting as superficial tumor that develops “strawberry” appearance vEnlarge with Valsalva vCT/MRI show diffusely infiltrating non- encapsulated mass Capillary Hemangioma Capillary Hemangioma vUsual course Normal at birth noticed at one month enlarge till 1 to 2 years of age spontaneous involution by age 4 to 8 yr vCosmetic sequelae minimal vVisual complications amblyopia or astigmatism vMajor complications superinfection, ulceration vRare complications Kasbach-Merrit, HO cardiac Capillary Hemangioma - Outcome Capillary Hemangioma - Treatment vIndications include any complication vMedical therapy steroids (systemic, intralesional) or interferon vRadiation therapy vSurgical resection for unresponsive or well- encapsulated lesions Capillary Hemangioma Lymphangioma vBenign congenital vascular malformations vMay involve conjunctiva, eyelids or deep orbit vUsually identified prior to teenage years vUsually slow enlargement and increasing proptosis vSudden proptosis from hemorrhage into cyst vNo enlargement with Valsalva vCT/MRI shows multi-compartmental nature Lymphangioma Lymphangioma Lymphangioma vTreatment for significant proptosis, corneal exposure or optic nerve compression vDebulking and cyst drainage usually vComplete removal often not possible Rhabdomyosarcoma vMost common malignant tumor in children vPresents within 1st decade vRapid unilateral proptosis and globe displacement vCT scan shows irregular margins and often bony destruction vExcisional biopsy ASAP for diagnosis if suspected Rhabdomyosarcoma Rhabdomyosarcoma vTake as much tumor as possible on biopsy vDisseminated or gross residual disease after biopsy carries 35% 5-year survival rate vChemotherapy and XRT after biopsy (90% 5-yr for localized disease) Rhabdomyosarcoma Optic Nerve Glioma v3rd most common in children vMay occur randomly although often associated with NF type I (up to 50%) vMean age 8 years vProptosis and visual symptoms vHeadache and pain with intracranial extension vDiagnosis clinically and radiographically Optic Nerve Glioma Optic Nerve Glioma vCT/MRI shows fusiform enlargement of optic nerve vMRI for intracranial extension vSignificant mortality once into chiasm vMust be excised while confined to nerve, esp. if blind or proptotic Fibrous Dysplasia vMost often fibro-osseous tumor vOccurs in 1st two decades vReplacement of normal bone with immature woven bone vPolyostotic (Albrights) and monostotic types Fibrous Dysplasia Fibrous Dysplasia vUsually stabilize after puberty vConservative treatment the rule vComplete resection preferable for significant cosmetic deformity or vision loss vCraniofacial reconstruction with neurosurgeon Metastatic Tumor: Neuroblastoma vMost frequent in kids vNeuroblastoma accounts for 10% of all childhood malignancies vPrimary: usually adrenal vBilateral metastasis with eyelid ecchymoses and proptosis common vSurvival rate 15% Adult Orbital Tumors vVary significantly from children vMost common vCarcinomas vPseudotumor vLacrimal gland tumors vLymphomas vCysts, meningiomas, vascular tumors Paranasal Sinus Masses vMasses of the paranasal sinus potentially can spread to involve the orbit vMost common: mucocele vNeoplasms of this area are uncommon, but frequently involve orbit vBenign tumors push periorbita, malignant invade Mucoceles vObstruction of ostium in a sinus vEnlarging fluid filled sinus vErodes through bony orbit wall vMost arise from frontal and ethmoid vCT homogenous mass Mucoceles Neoplasms of Paranasal Sinus vUncommon vMost common SCCa vOrbital invasion in 2/3 of patients with SCCa vGlandular malignancies from minor salivary glands or respiratory epithelium vOrbital extensive gives poor prognosis vBiopsy to Dx; radical resection to treat Neoplasms of Paranasal Sinus Orbital Pseudotumor vIdiopathic orbital inflammation v1905 by Birch-Hirschfiel first described vExcludes systemic diseases (sarcoid, thyroid, autoimmune and Wegeners) v2nd to 7th decade vMultifocal involvement of any orbital structure Orbital Pseudotumor vProptosis acute onset of a few days vEyelid swelling, chemosis and diplopia also common vVisual loss with optic nerve involvement vCT findings hazy enlargement of affected structures vTreatment Steroids, immunosuppresive meds, radiation therapy when steroids adverse Orbital Pseudotumor Lacrimal Gland Tumors vEnlargement of lacrimal fossa with displacement of globe and no inflammatory signs v50% epithelial, 50% lymphoproliferative vCT scan lymphoid show smooth enlargement of gland, epithelial are irregular vPrimary epithelial neoplasms of lacrimal gland are rare Lacrimal Tumor Pleomorphic Adenoma vBenign mixed tumor vMost common of these v20 to 50 years vPainless proptosis with inferior/medial globe displacement vMany months or years vExcisional biopsy (total) Lacrimal Tumor Adenoid Cystic Carcinoma vMost common malignant of these vProgressive onset of symptoms vPain and numbness vCT with bony destruction and infiltration v50% mortality, requires aggressive surgical Tx Lymphoid Tumors vIncidence between 4 to 13 % of all orbital tumors vPrimary or secondary to systemic disease vMost patients who present with localized orbital disease will develop systemic lymphoma vPresents between age 50 and 70 vAnterior, “salmon patch” mass causing progressive painless proptosis Lymphoid Tumors Lymphoid Tumors vGenerous biopsy needed to make diagnosis vSystemic workup necessary vLocalized orbital lymphoma XRT vSystemic lymphoma XRT + chemotherapy vConsultation of oncologist should be obtained Orbital Meningiomas v4th to 7th decade of life, rare in children vMost (70%) invade from cranium vPrimary orbital meningiomas may arise from optic nerve vProptosis, visual disturbances, headache and diplopia vCT/MRI fusiform enlargement of optic nerve Orbital Meningiomas Schwannomas vNeurilemoma benign, non-invasive peripheral nerve tumor, from any nerve in orbit vRare, ages 20 to 70 years vCT/MR show well circumscribed ovoid mass vMost commonly intraconal, ma