Anatomy - Bony

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1 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

2 Anatomy - Bony

3 Anatomy - Bony

4 Anatomy - Bony

5 Anatomy - Bony

6 Anatomy - Bony

7 Anatomy Fascial Compartments

8 Anatomy Fascial Compartments

9 Anatomy Eyelid

10 Anatomy Eyelid

11 Anatomy Blood Supply

12 Anatomy Blood Supply

13 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

14 Anatomy Lacrimal System

15 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

16 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

17 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

18 Pediatric Orbital Tumors Differs substantially from adult types More often congenital lesions and infectious Most common cystic lesions (dermoids) 2 nd most common vascular lesions Most common malignancy - rhabdomyosarcoma

19 Cystic Lesions Dermoid Cyst Most common Preschool child Superotemporal mass Mobile and nontender Well circumscribed on CT with rare bony remodeling

20 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

21 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

22 Cystic Lesions - Teratoma

23 Cystic Lesions - Teratoma

24 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

25 Capillary Hemangioma

26 Usual course Capillary Hemangioma 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

27 Capillary Hemangioma - Outcome

28 Capillary Hemangioma - Treatment Indications include any complication Medical therapy steroids (systemic, intralesional) or interferon Radiation therapy Surgical resection for unresponsive or wellencapsulated lesions

29 Capillary Hemangioma

30 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

31 Lymphangioma

32 Lymphangioma

33 Lymphangioma Treatment for significant proptosis, corneal exposure or optic nerve compression Debulking and cyst drainage usually Complete removal often not possible

34 Rhabdomyosarcoma Most common malignant tumor in children Presents within 1 st decade Rapid unilateral proptosis and globe displacement CT scan shows irregular margins and often bony destruction Excisional biopsy ASAP for diagnosis if suspected

35 Rhabdomyosarcoma

36 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)

37 Rhabdomyosarcoma

38 Optic Nerve Glioma 3 rd 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

39 Optic Nerve Glioma

40 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

41 Fibrous Dysplasia Most often fibro-osseous tumor Occurs in 1 st two decades Replacement of normal bone with immature woven bone Polyostotic (Albright s) and monostotic types

42 Fibrous Dysplasia

43 Fibrous Dysplasia Usually stabilize after puberty Conservative treatment the rule Complete resection preferable for significant cosmetic deformity or vision loss Craniofacial reconstruction with neurosurgeon

44 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%

45 Adult Orbital Tumors Vary significantly from children Most common Carcinomas Pseudotumor Lacrimal gland tumors Lymphomas Cysts, meningiomas, vascular tumors

46 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

47 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

48 Mucoceles

49 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

50 Neoplasms of Paranasal Sinus

51 Orbital Pseudotumor Idiopathic orbital inflammation 1905 by Birch-Hirschfiel first described Excludes systemic diseases (sarcoid, thyroid, autoimmune and Wegener s) 2 nd to 7 th decade Multifocal involvement of any orbital structure

52 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

53 Orbital Pseudotumor

54 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

55 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)

56 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

57 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

58 Lymphoid Tumors

59 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

60 Orbital Meningiomas 4 th to 7 th 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

61 Orbital Meningiomas

62 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)

63 Schwannomas

64 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

65 Cavernous Hemangioma CT/MRI reveals welldefined mass, oval Homogeneous with increased density CT MRI isointense to muscle Treatment surgical excision, recur rarely

66 Cavernous Hemangioma

67 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 7 th decade Prognosis is very poor (avg. survival 10 months) XRT usual; Chemo and Hormonal occasional

68 Metastatic Tumor - Breast

69 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

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