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CLINICAL FEATURES AND MANAGEMENT OF RETINOBLASTOMA IN AFRICA

CLINICAL FEATURES AND MANAGEMENT OF RETINOBLASTOMA IN AFRICA. CLARE STANNARD Department of Radiation Oncology Groote Schuur Hospital and University of Cape Town, South Africa. CLINICAL FEATURES. Family history Squint Leucocoria Red eye Proptosis / orbital tumour Metastases.

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CLINICAL FEATURES AND MANAGEMENT OF RETINOBLASTOMA IN AFRICA

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  1. CLINICAL FEATURES AND MANAGEMENT OF RETINOBLASTOMA IN AFRICA CLARE STANNARD Department of Radiation Oncology Groote Schuur Hospital and University of Cape Town, South Africa INCTR 2004

  2. CLINICAL FEATURES • Family history • Squint • Leucocoria • Red eye • Proptosis / orbital tumour • Metastases INCTR 2004

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  6. EXAMINATION & INVESTIGATIONS • EUA + IO - establish diagnosis - no. size, site, +/- vit. seeds - examine 2nd eye • CT scan - calcification, intraoc. extent - optic n., extrascleral, orbit - brain metastases • Lumbar puncture • Bone marrow INCTR 2004

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  10. CAPE TOWN STAGING • Stage I: amenable to local therapy • Stage II: beyond conservative therapy but confined to the eye; subdivisions based on histology of eye • Stage III: locoregional - orbit, lymph nodes • Stage IV: metastases - CNS, haematogenous INCTR 2004

  11. SUBSTAGES of STAGE II • N0 = no nerve C0 = no choroid • N1 = ant. lam. crib. C1 = superficial ch. • N2 = post. lam. crib. C2 = deep choroid • N3 = resection line C3 = intrascleral C4 = extrascleral INCTR 2004

  12. STAGE in SOUTH AFRICA INCTR 2004

  13. TREATMENT: STAGE I • Laser • Transpupillary ThermoTherapy (TTT) • Cryotherapy • Plaques: I-125, Ru-106 • Whole eye applicator: I-125 • External beam radiotherapy (EBRT) • Chemotherapy (CT) INCTR 2004

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  23. EBRT for STAGE I • One eye: ½ Pb lateral Co-60/linac field lateral 80%, ant.E 20% + Pb for lens • Both eyes } opposing lat. ½ Pb Co-60/linac • 1 eye + 1 orbit} “ “ “ “ “ • Dose: 40Gy in 1.8-2Gy fractions, 5 x / week • NB. Delay 6-8 weeks if prior CT INCTR 2004

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  27. TREATMENT: STAGE II • Enucleation & histological evaluation • N0-1, C0-1: no further treatment • N2: ? orbital RT • C2-3: ? chemotherapy • N3: orbital RT, systemic CT (VEC) + IT x 3 ? cranial RT, ? spinal RT • C4: orbital RT, systemic CT (VEC) INCTR 2004

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  30. ORBITAL RT • Brachytherapy: Iodine-125 (Cape Town) Iridium-192 (Jo’burg) Dose: 40 - 45 Gy over 4 days • EBRT: Wedge pair or ant. electron field Dose: 40 - 45 Gy in 2Gy fractions INCTR 2004

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  37. TREATMENT: STAGE III • ORBIT: enucleation + RT + CT (syst + IT) or CT + RT (10Gy) + enucleation + RT + CT • RT: Brachytherapy or EBRT, 45Gy • CT: Vincristine, etoposide, carboplat (VEC) intrathecal methotrexate, ara-C, hydrocort. • NODES: CT + RT INCTR 2004

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  40. TREATMENT: STAGE IV • Symptomatic • Palliation with CT +/- RT INCTR 2004

  41. PROBLEMS in RB MANAGEMENT • Range of disease: early to late • Range of Rx: saving sight to saving eyes • Unilateral: enucleation +/- RT/CT (once off) • Bilateral: conservative Rx + monitoring possible further treatments for new and recurrent tumours INCTR 2004

  42. PROBLEMS in RB MANAGEMENT • Combined expertise required: ophthalmologist, radiation oncologist, paediatric oncologist, medical physicist, pathologist, radiologist, geneticist • Developed world trying to avoid EBRT a) cosmetic deformity b) 2nd non-ocular malignancy • Developing world needs EBRT INCTR 2004

  43. DEVELOPING WORLD • Late presentation of advanced disease • RB is low priority in blindness prevention programmes - low incidence & high cost • Education of patients & doctors in countries where facilities available, eg:TV advert-Brazil INCTR 2004

  44. Rx in DEVELOPING WORLD • Stage IV: Palliation only • Stage III: Need RB centres with RT & CT for good palliation / local control at least • Stage II: Hopefully attracted by RB centres, problem: reluctance to accept enucleation enucleation, histology +/- RT/CT • Stage I: EBRT or refer INCTR 2004

  45. PROBLEMS with REFERRAL • Cost • Time away from home • Not a once off treatment • Essential monitoring by ophthalmologist in home country preferably with cryotherapy & laser therapy INCTR 2004

  46. DOES REFERRAL WORK? • Not always; poor referral or delays • Namibia, Mauritius to South Africa • Algeria, Tunisia, Morocco to France • Need ophthalmologist to assess pts. • Need links between ophthalmologist & RB centre and referral centre INCTR 2004

  47. CAPE TOWN PATIENTS by STAGE & YEAR INCTR 2004

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