orbital tumors 2 edited.pptx medicine ophthal

Tumors

1. ORBITAL METASTATIC TUMORS

2. Metastasis to the orbit mainly occurs by hematogenous route. The primary tumors causing metastasis to the orbit aredifferent in children and in adults. ADULTS :m/c primary tumor metastatising to the orbit are carcinomas of breast ,prostrate gland,lungs and gastro-intestinal tract.Rarely cutaneous and uveal melanoma can metastatize to the orbit. CHILDREN : orbital metastasis can occur from adrenal neuroblastoma , wilms tumor,ewings tumor.

3. PRESENTING FEATURES :- -They depend on the exact location pf the tumor in the orbit and the type of primay neoplasm. -Some of these lesions are confined to the extraocular muscles while others occur as solitary intraconal or extraconal mass producing rapidly progressive proptosis ,pain diplopia,ptosis , eyelid,swelling , -Enophthalmos is seen in tumors causing fibrosis of the orbital tumors Which cause fibrosis of the orbital tissues,namely scirrhous caecinoma of breast and stomach.

4. IMAGING : -Thorough history,systemic examinationand whole body imaging is undertaken to diagnose the primary tumor.Imaging of the orbit for the size and location of the tumor is to be mandated.The diagnosis should be confirmed by an orbital incisional biopsy.

5. MANAGEMENT: Once the tumor is proven metastatic ,further management should involve medical and surgical oncologists.The orbital tumor may subsequently require radiation therapy.

6. SECONDARY ORBITAL TUMORS These are primary tumours causing secondary orbital involvement .

7. An orbital secondary is a malignant tumour that has extended into the orbit from the adjacent tissues around the orbit from adjacent tissues around the orbit including eyelid ,conjunctiva ,intraocular structures,sinuses ,nasopharynx and brain .

8. Eyelid tumors can involve the orbit secondarily include sebaceous gland carcinoma ,squamous cell carcinoma,basal cell carcinoma. Conjuctival tumors extending into the orbit include squamous cell carcinoma ,melanoma. Intraocular tumors include uveal melanoma and retinoblastoma. Sinus tumors like carcinoma of ethmoid and maxillary sinus. Intracranial tumors like sphenoid wing meningiomaalso invade orbit.

9. BCC (Basal Cell) SCC (Squamous Cell) SGC (Sebaceous Gland)Frequency Most common eyelid malignancy 2nd most common Rare but aggressiveSite (commonest0 Lower lid, medial canthus Lower lid Upper lid (meibomian glands) Slow, locally invasive Faster, more aggressive Aggressive, often multicentris Metastasis Rare Common (lymph nodes/distant) Common (regional & systemic) Pearly nodule, rolled edges Indurated ulcer, lash loss “Great masquerader”, pagetoid spread Excellent if excised completely Worse, due to metastasis Poor (high recurrence, mortali

10. INTRAOCULAR TUMORS

11. Clinical Features : -Often asymptomatic initially. -Blurred vision, visual field defects, photopsia, floaters. -Visible pigmented intraocular mass, sometimes dome-shaped or mushroom-shaped (if breaks Bruch’s membrane). -Secondary retinal detachment. -Iris melanoma → visible pigmented mass, distorted pupil. Investigations : -Fundus exam – pigmented choroidal mass. -Ultrasound B-scan – dome/mushroom shaped mass, low internal reflectivity. -FAF/FA (Fluorescein angiography) – double circulation. -OCT – retinal detachment. -MRI/CT – extent of tumor. -Liver imaging & LFTs – common site of metastasis.

12. Images of uveal MELANOMA

13. RETINOBLASTOMA . Definition : -Malignant tumor of primitive retinal neuroblastic cells. -Most common primary intraocular malignancy in children. Epidemiology : -Onset: usually before age 5 years (90% cases). -Sporadic and hereditary forms. Genetic s: -Mutation in RB1 tumor suppressor gene (chromosome 13q14). -Heritable form: bilateral, multifocal, autosomal dominant inheritance with high penetrance. -Sporadic form: unilateral, unifocal.

14. Clinical Features : -Leukocoria (white pupillary reflex) – most common sign. -Strabismus. -Pain, redness, secondary glaucoma. -Proptosis if orbital extension. -Rare: hypopyon, vitreous seeding. Types (growth pattern): -Endophytic : grows into vitreous, vitreous seeds. -Exophytic : grows towards subretinal space → retinal detachment. -Diffuse infiltrating : rare, flat infiltration without mass. Investigations: Fundoscopy: cream-white mass, chalky calcification. Ultrasound B-scan: intraocular mass with calcification (diagnostic). CT scan: detects calcification, orbital extension. MRI: better for optic nerve and intracranial extension. Genetic testing for RB1 mutation in suspected hereditary cases.

15. Spread : Local: sclera, orbit. Hematogenous: liver (most common), lungs, bone. Lymphatic spread rare (eye lacks lymphatics). Management : Small tumors: observation, laser photocoagulation, transpupillary thermotherapy. Medium tumors: plaque brachytherapy (Iodine-125, Ruthenium) Large tumors: enucleation. Orbital exenteration if extraocular spread. Systemic chemo/immunotherapy – limited benefit, used in metastasis. Prognosis: 5-year survival: ~70–80%. Poor prognosis with: large size, ciliary body involvement, epithelioid cell type, extraocular extension, liver metastasis.

16. Managemen t: Eye-saving approaches (for small/moderate tumors) Focal: laser photocoagulation, cryotherapy, thermotherapy Intravitreal / intra-arterial chemotherapy. Systemic chemotherapy ( chemoreduction ). Advanced disease / no visual potential: Enucleation. Extraocular spread: Radiotherapy + systemic chemotherapy. Lifelong genetic counseling and screening of siblings. Prognosis : Survival in developed countries: >95%. Worse in developing countries due to late diagnosis. Poor prognosis with: optic nerve invasion, extraocular extension, trilateral RB. Risk of second malignancy (osteosarcoma, soft tissue sarcoma) in hereditary form, especially after radiotherapy.

17. Staging of retinoblastoma

18. Feature Uveal Melanoma (Adult) Retinoblastoma (Child) Age group 5th–6th decade <5 years Origin Uveal tract (choroid > ciliary > iris) Retinal neuroblasts (RB1 gene) Common symptom Blurred vision, visual field loss Leukocoria, strabismus Spread Hematogenous (→ liver) Optic nerve, CNS, lymph, bone Imaging hallmark B-scan: mushroom/dome mass B-scan/CT: calcification Treatment Brachytherapy, enucleation Chemotherapy, focal therapy, enucleation Prognosis 70–80% 5-yr survival, poor if metastatic >95% survival in developed nations

19. Images of retinoblastoma

20. CLINICAL FEATURES: -It is a secondary orbital tumor depending upon the type and location of the primary neoplasm,with proptosis being the most common symptom. -Sometimes ,the tumoris detected accidentally during follow up for a previous eye and ocular adnexal tumour .

21. DIAGNOSIS : -A detailed history,clinical exam,imagingand orbital biopsy are needed to establish the diagnosis. MANAGEMENT : depends on the extent of the lesion in the orbit. -excisional biopsy : for smaller tumors -orbital exenteration : for larger tumors -subsequent chemotherapy and radiation therapy

22. ORBITAL MUCOCELE Orbital mucocele is an expansile lesion resulting from the accumulation of mucoidal secretion of a chronically inflamed paranasal sinus secondary to the obstruction of the sinus ostium.

23. It is generally painless and has rapid growth It is genrally seen in adults but is seen in children with cystic fibrosis Most commonly frontal sinus is involved which produces proptosis which tends to displace globe downwards Ethmoidal mucocele is less common and maxillary and sphenoidal sinus involvement is very rare The mass is generally visible and it is fluctuant to palpation beneath the orbital rim When it becomes infected it becomes mucopyocele

24. On imaging :Mucocele demonstrates opacificationon the affected sinus and thickening of mucocele lining with erosion of adjacent bones HISTOPATHOLOGY:it is lined by pseudostratified columnar cells with infiltration of inflammatory cells.the lumen contains mucous or pus MANAGEMENT:complete excision of mucosal wall with marsupialization to avoid recurrence . Antibiotics to cover both aerobic and anaerobic bacteriashould be administered before and after surgery.