Retinoblastoma dr vandana

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Presented by Dr. VandanaCSMMU, LucknowEarlier King George Medical University.

Transcript of Retinoblastoma dr vandana

  • 1. Presented By:Dr. VandanaDept. of RadiotherapyCSMMU

2. Clinical Anatomy The eye is composed of threelayers. Outer fibrous layer formed by thesclera posteriorly and the corneaanteriorly. Inner layer , sensory retina withvision concentrated at the foveawhich is lateral to the optic nerveand directly posterior to the lens. In between these vascular layer the uvea or choroid whichsupplies the retina. The iris is theouter continuation of the vascularlayer Lens sits just behind iris,suspended from the ciliary body. No lymphatic drainage 3. Retinoblastoma Mid 18th century: 1st clinical report of RB was recognized 1920- Vernoff coined the term retinoblastoma 2.5-4% of paediatric malignancies Most common intraocular malignancy of childhood 2nd most common primary intraocular malignancy in any age group Tumor is of neuroepithelial origin & arises from unidentifiedprogenitor cell in nucleated layers of one or both eyes Accounts for 1:17,000-34,000 live births worldwide 4. Epidemiology Slight male preponderance 1.15:1.0 More than 90% of cases occur before age of 5 year Most common among blacks & Africans Age: average age in hereditary cases: 12-14 months, sporadic cases24-30 months Unilateral: 65-80% Bilateral: 20-35%Aetiology: unknown 5. Genetics Deletion of long arm of chromosome 13, 13q14, which is a tumorsuppressor gene termed as RB gene. Alfred Knudson hypothesis: 2 types HereditaryNon-hereditary FamilialSporadic Germline mutations2 hits Bilateral, multifocal Unilateral, unifocal Young age older age 6. Clinical features Family history: 10% Leukocoria (white pupillary reflex): 50% ,commonest Strabismus [esotropia]: 20% Ocular inflammation: due to necrosis of tumoror tumor cells may enter AC resemblinghyphema [pseudohypopyon] Secondary glaucoma: angle-closure Loss of vision Proptosis: extra ocular invasion Trilateral retinoblastoma; bil RB+pineoblastoma Distant metastases 7. Routes of spread Local spread; anteriorly- seeding of vitreous & aqueous; posteriorly,sub retinal space and choroids May spread through optic nerve Along the central retinal vessels: tumor cells pass through the laminacribrosa and enter subarachnoid space Distant metastases: CNS, skull, bones, lymph nodes, spinal cord, bonemarrow Orbital involvement 8. Staging 9. Reese-Ellsworth Classification 10. International Classification of RetinoblastomaGroup Features ASmall tumor: 3 mmB Large tumor: >3 mmMacular: 3 mm to foveolaJuxtapapillary: 1.5 mm to discSubretinal fluid: 3 mm from the margin CFocal seedsSubretinal seeds: 3 mmVitreous seeds: 3 mmBoth subretinal and vitreous seeds: 3 mm DDiffused seedsSubretinal seeds: >3 mmVitreous seeds: >3 mmBoth subretinal and vitreous seeds: >3 mmE Extensive retinoblastoma occupying more than 50% orneovascular glaucomaor opaque media from hemorrhage in anterior chamber, vitreous or subretinal spaceInvasion of postlaminar optic nerve, choroid (> 2mm), sclera, orbit, anterior chamber 11. AJCC Tumour Staging System for RBT1/p125 to 50% of retinaT3/Pt3 >50% of retina and/or intraocular beyond retinaT3a/pT3a >50% of retina and/or cells in vitreousT3bOptic diskpT3b Optic nerve up to lamina cribrosaT3cAnterior chamber and/or uveapT3c Anterior chamber and/or uvea and/or intrascleralT4/pT4 ExtraocularT4aOptic nervepT4a Beyond lamina cribrosa, not at resection lineT4bOther extraocularpT4b Other extraocular and/or at resection lineN1/pN1 RegionalMI Distant metastases 12. St. Judes Tumor Staging SystemStage I: Tumor (unifocal or multifocal) confined to retinaa. Occupying one quadrant or lessb. Occupying two quadrants or lessc. Occupying more than 50% of retinal surfaceStage II: Tumor (unifocal or multifocal) confined to globea. With vitreous seedingb. Extending to optic nerve headc. Extending to choroidd. Extending to choroid and optic nerve heade. Extending to emissariesStage III: Extraocular extension of tumora. Extending beyond cut end of optic nerve (including subarachnoid extension)b. Extending through sclera into orbital contentsc. Extending to choroid and beyond cut end of optic nerve (including subarachnoid extension)d. Extending through sclera into orbital contents and beyond cut end of optic nerve (including subarachnoid extension)Stage IV: Distant metastasesa. Extending through optic nerve to brainb. Blood-borne metastases to soft tissue(s) and bone(s)c. Bone marrow metastases 13. Diagnostic workup Diagnosis of retinoblastoma is made without pathologicconfirmation and is based on a clinical examination. Clinical history Physical examination: EUA Direct ophthalmoscopy: white reflex Indirect ophthalmoscopy: RB seen as projecting into vitreous, creamy white in color, neovascularisation seen onsurface, calcification gives cottage cheese appearance (glistening white) RB diagnosed owing to vitreous hemorrhage, RD, severe inflammatory reaction(A) A fundus photograph of an eye with retinoblastoma(B) The corresponding sketch of the disease in the eye diagram. 14. Ophthalmic USG: Non-invasive, safe,repeatable,andimmediatelyinterpretable. B-scan USG reveals a 2D cross-sectional view, confirms presence and therelationship, the size and shape of the tumors. Orbital involvement, opticnerve invasion can be seen, extrascleral extension, and calcification..B-scan of an eye with retinoblastoma 15. CT scan, dense heterogenous lesion with hyper dense foci corresponding tocalcification. for assessing extraocular extension and invasion of the optic nerve. A computed tomography scan of a large calcified retinoblastoma in the right eye. 16. MRI Instead, as part of an extent-of-disease work-up, MRI is routinely performed. Involvement of the optic nerve, extraocular extension, and intracranialmidline neoplasm in trilateral retinoblastoma are best detected. Used in differentiating retinoblastoma from simulating lesions. 17. Other tests Anterior chamber para-centesis: to assay LDH. Elevated ratio of aqueousLDH5/LDH1 iso-enzymes, elevated ratio of aqueous LDH/ serum LDH Fluorescein angiography: tumor blush CSF cytology Bone marrow biopsy &aspiration Bone scan Lab tests: Hemogram, Blood chemistries, KFT, LFT 18. Treatment of Retinoblastoma Primary goal to ensure the survival of children. retention of eyes and vision. Avoidance of side effects- second malignancies, facial bonydeformities, or other physical changes that can affect functionalwell-being. Treatment approaches are guided by the presence ofintraocular or extraocular disease. 5 yr. DFS > 90% for intraocular disease pts., but < 10% forextra-ocular disease. 19. Treatment Modalities 20. Surgery 21. Enucleation Procedure: removal of globe after severing the rectus muscles, optic nerve is cut(10-20mm) near its exit from the socket Indications: Unilateral RB with blind eye Bilateral RB with both eyes blind- bilateral enucleation Uni/bilateral RB with glaucoma (rubeosis iridis) with visual loss Local recurrence after conservative measures fail bilateral retinoblastoma in which the previously mentioned conditions exist in only oneeye a tumor present in the anterior chamber retinoblastoma unresponsive to other forms of local therapy cases with permanent vision loss in which intraocular tumor is suspected. 22. Exenteration Procedure: removal of globe, extra ocular muscles, lids , nerves and orbitalfat Indications: Extensive local tumor breaching the globe Recurrence of tumor in socket after enucleation 23. Local therapies Used for small tumors < 3 6 mm Usually in patients with bi-lateral disease and In combination with Chemo- Radiation. 24. CryotherapyProcedure: tumor is localized (by indirect ophthalmoscope), indented trans-sclerally with nitrous oxide cryoprobe, freeze is applied (-80c),Indications: Small tumor anterior to equator (4-7 mm in size) Small recurrence or tumor persisting after radiotherapy In conjunction with chemotherapy (may increase the intravitreal penetrationof carboplatin)Side effects: Can induce acute retinal edema Accumulation of sub retinal fluid retinal detachment 25. PhotocoagulationProcedure: obliteration of retinal vessels by creating retinal burn with laserbeamIndications: Tumor 4.5mm at base and 2.5mm thick Away from macula or disc Small tumor recurrence after prior irradiation Contraindication: vitreous seedingLaser hyperthermiaProcedure: generated by Diode laser (810 nm) on continuous mode Single spot 0.8-2 mm placed on center of tumor Tumor is heated for 10-30 min per session. Central tumor temperature 460cand decreases by 20c for each mm outside the temperature spot 26. Radioactive plaque application Isotopes used: Co-60, Ir-192, I-125, 106Rh (emitter) Co-60: circular, crescentic to fit around optic nerve I-125: seeds glued within a carrier and gold shield, circular or notched configuration Procedure: 1st USG of eye done: for tumordimensions: maximum basal diameter, maxheight surgical exploration applicators areapplied over sclera overlying the tumor 1.5-2mm margin on either side of basal diameter retention sutures Rx Re-exploration forremoval of plaques 27. Indications: Solitary lesion 2-16 mm basal diameter Unifocal lesions Located greater than 3 mm from optic nerve or fovea Thickness