Intra-orbital tumors - Diagnosis & Management Part 1

Orbital tumors are abnormal growths in the tissues of the eye socket, or orbit. They can be benign (noncancerous) or malignant (cancerous).
Types of orbital tumors
Primary tumors: Begin in the orbit, and can be benign or malignant
Metastatic tumors: Spread from another part of the body
Cysts: A common type of orbital tumor
Vascular lesions: Grow from blood vessels
Lymphomas: A common type of orbital tumor in adults
Neurogenic tumors: Grow from nerves
Secondary tumors: Spread from the sinuses or cranium
Symptoms Proptosis (displacement of the eye), Pain, Diplopia (double vision), and Vision loss.
Treatment Surgery to remove the tumor, Radiation therapy, and Chemotherapy.
Diagnosis
History, examination, and neuroimaging (CT, MRI)
Biopsy to confirm
Prognosis
Most benign tumors can be removed and rarely recur
Some tumors in children are life-threatening and require advanced treatment
Certain rare tumors require removal of the eye and orbital contents

1. Intra-orbital tumors Dr. RACHIT RASTOGI PG JR3 OPHTHALMOLOGY GMC HALDWANI

2. Anatomy

3. Orbital spaces Intraconal space Extraconal space EOM Sub periosteal space Tenons capsule Extraorbital space

4. Pandora’s Box >50% of orbital tumors are malignant, with life threatning potential. Primary Goal – Life salvage Secondary Goal- Eye salvage Tertiary Goal- Vision & cosmesis

5. Approach to a case Clinical History Regional Examination (Orbit, Nose & regional LN) Systemic examination Imaging Specific Investigation Surgery HPE

6. History & Examination - 6 P P ain- Characterise pain Severity - Dull ache / Severe Constant / Intermittent Change with movement of eye, etc P rogression- Acute(days), Subacute (weeks), Chronic (Months-years) Rapid, slow, indolent. P roptosis P alpation P ulsation P eriorbital changes Special attention Visual acuity + color vision Schirmers test Pupil (RAPD grading) VFA EOM Globe position (Dystopia) Fundus examination Oral and nasal examination

7. Proptosis Hertels Exophthalmometer - standard Leudes Exophthalmometer - transparent Naugles Exophthalmometer - frontal and maxillary bones as its reference Hilal and Trokel method- on CT scan Terminology Proptosis/exophthalmos- forward displacement / protrusion of the globe. Exorbitism - angle between the lateral orbital walls >90°(associated with shallow orbital depth) Hyperglobus or hypoglobus - Vertical dispalcemnt . Enophthalmos - Retrodisplacement d/t volume expansion of the orbit (fracture/silent sinus syndrome) Orbital varix Secondary to sclerosing orbital tumors( metastatic breast carcinoma )

8. Types Axial displacement- (intraconal mass) – Cavernous venous malformation Optic nerve glioma Primary optic nerve sheath meningioma Metastasis Arteriovenous malformation Nonaxial displacement(extraconal mass)- Superior displacement- maxillary sinus tumors Inferomedial displacement - orbital dermoid cysts, lacrimal gland tumors . Inferolateral displacement - frontoethmoidal mucoceles, abscesses, osteomas, and ethmoid sinus carcinomas.

9. Adult Well cicumscribed lesion Cavernous hemangioma Neurilemmoma Neurofibroma Fibrous histiocytoma Axial Proptosis Cavernous hemangioma Lymphangioma ONG Optic nerve sheath meningioma Abaxial Proptosis Lymphoproliferative lesions (M/C/C) Neurilemmoma Lacrimal gland tumors Other Structural tumors Triradiate lesions Sphenoid wing meningioma Eosinophilic granuloma Osteosarcoma Ewing’s sarcoma Metastasis

10. Child + Axial Proptosis Optic Nerve Glioma Child + Abaxial Proptosis Subacute- Rhabdomyosarcoma ( M/C primary orbital malignancy in children ) Leukemia Primary Neuroectodermal tumor (PNET) Chronic Lymphangioma Dermoid

11. Palpation Tenderness (Mild, moderate,severe ) Location(Quadrant) Mobility(Both directions, Free/limited/fixed) Compressibility(assess rebound) Retropulsion(resistance, grade) Shape( regular/irregular, describe shape) Surface( Smooth,nodular,lobulated ) Consistency (Cystic, soft, firm, hard) Orbital margin( Regulatr , irregular) Pulsation, thrill, bruit (Auscultation)

12. Periorbital changes

13. Imaging CT MRI USG/ color doppler Sterotactic navigation

14. Causes of Proptosis Congenital Infection Inflammatory Traumatic Neoplastic Primary Benign Malignant Secondary

15. Primary Tumors Cystic lesions Vascular tumors Peripheral Nerve Tumors Optic Nerve & Meningeal tumors Myogenic tumors Fibrous connective tissue tumors Osseous & Fibroosseous tumors Histiocytic tumors Primary Melanocytic tumors Epithelial tumors of Lacrimal gland

16. Infantile (Capillary) Hemangioma at birth / within the first few weeks of life. Enlarge over the first 6–12 months 75% - resolve by 3–7 years . Female sex, low birth weight, prematurity, and historically maternal chorionic villus sampling. Superficial - bright red, soft mass with a dimpled texture. Subcutaneous - bluish. Deeper within the orbit - progressively enlarging mass without any overlying skin change. MRI - fine intralesional vascular channels and high blood flow. Doppler ultrasound - numerous blood vessels within the mass and abundant blood flow Criteria for management- amblyopia, strabismus, and anisometropia Beta- blockers (Topical Timolol, Oral Propranolol ) Steroid (Intra- lesional Triamcinolone) Surgical Excision Post 6 wks Propranolol treatment

17. Lymphangioma low- flow orbital lesions Can occur in conjunctiva , eyelids, oropharynx and sinuses Congenital, become apparent in the 1 st / 2 nd decade of life macrocystic (cysts ≥ 2 cm), microcystic (cysts < 2 cm), or mixed macrocystic /microcystic Enlarge during URTI, sudden proptosis d/t spontaneous intralesional hemorrhage (chocolate cysts) MRI - multiple grapelike cystic lesions with fluid– fluid layering of serum and red blood cells ( Pathognomic - confirmatory) Management- Obsevation Debulking+ Sclerosing agent Sclerosants - morrhuate sodium, bleomycin , polidocanol, OK-432, Thrombosing agents - fibrin glue Embolizing agents - cyanoacrylate glues, ethylene vinyl alcohol copolymer

18. Distensible Venous Malformations(Orbital Varices) Low flow lesions Enophthalmos Initially Enlarge with a Valsalva maneuver/ tilting head down . Rest all ophthalmic examination is often unremarkable Confirmatory Diagnosis- MRI or contrast-enhanced rapid spiral CT during a Valsalva Characteristic enlargement of the engorged veins Management- Observation Combined embolization + excision – If Pain or vision threatning compressive Optic Neuropathy

19. Cavernous Hemangioma Non-distensible , low- flow vascular lesions. M/C type of primary orbital lesion in adults 4 TH – 5 TH decades of life. Women- 60% of cases; circulating estrogen and progesterone helps progression, more in pregnancy. slowly progressive, painless proptosis Other finding- Retinal striae, Hyperopia, optic nerve compression, increased IOP, Strabismus Contrast- enhanced orbital imaging - stippled pattern in the early phase of contrast, homogeneous staining in the late phase. HPE – encapsulated; composed of large, cavernous spaces with walls of smooth muscle containing red blood cells

20. Cavernous Hemangioma Management- Surgical excision . encapsulated , isolated from the surrounding tissue- easier to remove Radiotherapy - lesions located deep within the orbital apex

21. Arterio-venous Malformation high- flow developmental anomalies. Dilated corkscrew episcleral vessels + pulsatile proptosis Vascular steal / shunting from the orbit - ischemic changes. CT - diffuse enhancement MRI - flow voids . Management – Preoperative selective angiography with embolization followed by resection of the nidus Failure to completely resect the nidus - Recurrence Uncontrolled arterial hemorrhage - unsuspected lesions without preoperative embolization.

22. Arterio-Venous Fistula/ Carotico - Cavernous Fistula

23. Management- Endovascular treatment (coils or glue) – to block the fistula . Small indirect carotid- cavernous fistulas – Observation, close spontaneously. direct carotid- cavernous fistulas- high- flow lesions- usually require intervention

24. Optic Nerve Glioma uncommon, usually benign Children – 1 ST decade of life. gradual, painless, unilateral axial proptosis + vision loss + RAPD Other- optic atrophy, optic nerve head swelling, nystagmus, strabismus 50% - assoc. with Neurofibromatosis. CT/MRI- fusiform enlargement of the optic nerve, stereo typical kinking of the nerve. Gross pathology - smooth, fusiform intradural lesion Microscopic examination - benign tumors in children are considered to be juvenile pilocytic (hairlike) astrocytomas . Other histologic findings include arachnoid hyperplasia, muco-substance, and Rosenthal fibers

25. Management- Observation - Good Vision, Regular follow-up, Some pt. never require surgery Surgical Excision- Transcranial approach Chemotherapy - Combination chemotherapy (dactinomycin, vincristine, etoposide, and other) for progressive chiasmal-hypothalamic gliomas. Delay need for radiation therapy. long- term risks of blood cancers . Radiotherapy - Indication - tumor cannot be resected (lesions of the chiasm/ optic tract) and if symptoms, particularly neurologic, progress after chemotherapy. S/E- intellectual disability, growth retardation, secondary tumors, and malignant transformation within the radiation field

26. Neurofibroma Proliferating Schwann cells within the nerve sheaths. HPE- Axons, endo-neural fibroblasts, and mucin Plexiform neurofibromas - well- vascularized infiltrative lesions, making complete surgical excision difficult. Discrete neurofibromas are less common than the plexiform type and can usually be excised without recurrence. Surgery - tumors that compromise vision or produce disfigurement. NF1 AD gene with incomplete penetrance. Hamartomas involving the skin, eye, central nervous system, and viscera, it is classified as a phakomatosis . Orbital features – plexiform neurofibromas of the lateral aspect of the upper eyelid that result in an S- shaped contour of the eyelid margin, pulsatile proptosis secondary to sphenoid bone aplasia optic nerve glioma . Café-au-lait spots Axillary freckles Lisch nodules S shaped lid

27. Optic Nerve Sheath Meningioma Origin- arachnoid of the optic nerve sheath Female, 3 rd or 4 th decade gradual, painless, unilateral loss of vision + RAPD Proptosis and ophthalmoplegia ONH - normal, atrophic, or swollen, and tortuous vessels may be visible. CT and MRI show diffuse tubular enlargement of the optic nerve with contrast enhancement. (CT can show calcification aka tram- tracking) Management- Observation - vision is minimally affected and no intracranial extension Fractionated stereotactic radiotherapy - confined to the orbit and vision loss is significant or progressive, Surgery - reserved for patients with severe vision loss and profound proptosis.

28. Schwannoma/ Neurilemomas Proliferations of Schwann cells that are encapsulated by perineurium. Characteristic biphasic pattern of solid areas nuclear palisading ( Antoni A pattern ) myxoid areas ( Antoni B pattern ). well encapsulated, can be excised with relative ease. Hypercellular schwannomas sometimes Rarely undergo malignant transformation.

29. Rhabdomyosarcoma Rhabdomyosarcoma is the most common primary orbital malignancy of childhood Origin - undifferentiated pluripotent mesenchymal elements in the orbital soft tissues, not from the extraocular muscles Onset- 5–7 years Sudden onset, rapid progression of unilateral proptosis. Marked adnexal response, with edema and discoloration of the eyelids Palpable mass- particularly in the superonasal quadrant of the eyelid. Regional metastasis- Palpate Cervical & Pre-auricular LN Distant Metastasis- Chest radiography, bone marrow aspiration and biopsy, and lumbar puncture Urgent workup CT and MRI can be used to define the location and extent of the tumor Biopsy - anterior orbitotomy approach.

30. Management- Based on Guidelines by Intergroup Rhabdomyosarcoma Study Group Radiation therapy and systemic chemotherapy 4500 to 6000 cGy , given over a period of 6 weeks survival rates are better than 90% if the orbital tumor has not invaded or extended beyond the bony orbital walls Recurrent cases- Exentration

31. Other Mesenchymal Tumors Fibrous Histiocytoma Solitary Fibrous Tumor Fibrous dysplasia (McCune- Albright syndrome) Osteoma Malignant mesenchymal tumors Liposarcoma Fibrosarcoma Chondrosarcoma Osteosarcoma

32. Lymphoma Most orbital lymphomas (98%) are derived from B cells. B- cell lymphoma is divided into Hodgkin and non- Hodgkin tumors(MC) 4 most common types Extranodal marginal zone B- cell lymphoma, EMZL ( 57% ) Diffuse large B- cell lymphoma (DLBCL) (15%) Follicular Lymphoma (11%) Mantle Cell Lymphoma (8%) Clinical Presentation gradually progressive, painless mass that results in proptosis, a visible periocular mass, or ptosis anteriorly in the orbit or beneath the conjunctiva, where they may show the typical salmon- patch appearance mold to surrounding orbital structures rather than invading them EOM & V/A- Normal Regional LN- Palpable

33. Diagnosis Orbital imaging - characteristic puttylike molding of the tumor to normal structures. 50% of orbital lymphoproliferative lesions - arise in the fossa of the lacrimal gland 17% of orbital lymphoid lesions - occur bilaterally Incisional biopsy. Light microscopy & molecular diagnostics (flow cytometry, immunohistochemistry, and genomic analysis) Management EMZL and FL- Radiotherapy (10- year survival rates - 92% and 71%, respectively) DLBCL and MCL - poorer prognosis(10- year survival rates of 41% and 32%, respectively). Treatment of non- EMZL – chemotherapy + immunomodulation + Radiotherapy Targeted therapies ( Rituximab ) - CD20- positive lymphomas.

34. Langerhans cell histiocytosis Rare disorders of the mononuclear phagocytic system abnormal immune regulation All subtypes are characterized by an accumulation of proliferating dendritic histiocytes. Children, Peak age- 5-10 yrs Newer names- unifocal and multifocal eosinophilic granuloma of bone and diffuse soft tissue histiocytosis Diagnosis- CT imaging- lytic defect (most frequent presentation) affects the superotemporal orbit or sphenoid wing and causes relapsing episodes of orbital inflammation initially misdiagnosed as infectious orbital cellulitis. MRI - aids in visualization of the soft- tissue component Histopathology - foamy histiocytes with abundant eosinophilic cytoplasm and irregular nuclei that are immunoreactive for antibodies against CD1a

35. Management - Survival rate - 50% (< 2 years at presentation) Survival rate - 87%. (> 2 yrs of presentation) Localized orbital disease - confirmatory biopsy with debulking, f/b intralesional steroid injection / low- dose radiation therapy . Spontaneous remission has also been reported. Although destruction of the orbital bone may be extensive at the time of presentation, the bone usually reossifies completely. Systemic disease - aggressive chemotherapy.

36. Secondary Orbital Conditions Globe - choroidal melanomas and retinoblastomas Eyelid - sebaceous gland carcinoma, squamous cell carcinoma, and basal cell carcinoma Nose & PNS- Mucoceles and mucopyoceles of the sinuses Silent sinus syndrome Squamous cell carcinoma and adenocarcinoma of the sinuses Non epithelial tumors- osteomas, fibrous dysplasia, and miscellaneous sarcomas

37. Metastatic Tumor Children Burkitt lymphoma Leukemia Neuroblastoma- ecchymotic proptosis Ewing sarcoma, Wilms tumor (nephroblastoma). Adults- Bronchogenic Carcinoma (MCC of Orbital mets in Men) Breast Cancer (MCC of Orbital mets in Women) Prostate cancer - osteoblastic bone lesion

38. …TO BE CONTINUED Congenital cystic lesion Lacrimal gland tumors Orbitotomy & its various approches

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