INCTR

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

2
CLINICAL FEATURES

• Family history
• Squint
• Leucocoria
• Red eye
• Proptosis / orbital tumour
• Metastases

3
(No Transcript)
4
(No Transcript)
5
(No Transcript)
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

7
(No Transcript)
8
(No Transcript)
9
(No Transcript)
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

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

12
STAGE in SOUTH AFRICA
Cape Town E London Pmaritsburg
Period 1953-2004 1988-2001 1987-2003
No. of pts. 411 53 187
No./year 10 4 11
Bilateral 38 23
Stage I 6 0.5 2
Stage II 72 33
Stage III 9 16 98
Stage IV 13 50
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)

14
(No Transcript)
15
(No Transcript)
16
(No Transcript)
17
(No Transcript)
18
(No Transcript)
19
(No Transcript)
20
(No Transcript)
21
(No Transcript)
22
(No Transcript)
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

24
(No Transcript)
25
(No Transcript)
26
(No Transcript)
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)

28
(No Transcript)
29
(No Transcript)
30
ORBITAL RT

• Brachytherapy Iodine-125 (Cape Town)
• Iridium-192 (Joburg)
• Dose 40 - 45 Gy over 4 days
• EBRT Wedge pair or ant. electron field
• Dose 40 - 45 Gy in 2Gy fractions

31
(No Transcript)
32
(No Transcript)
33
(No Transcript)
34
(No Transcript)
35
(No Transcript)
36
(No Transcript)
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

38
(No Transcript)
39
(No Transcript)
40
TREATMENT STAGE IV

• Symptomatic
• Palliation with CT /- RT

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

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

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, egTV advert-Brazil

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

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

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

47
CAPE TOWN PATIENTS by STAGE YEAR
Bilat. I I / II II III / IV
53-73 33 1 17 68 13
74-83 31 5 19 39 36
84-93 35 6 20 50 24
94-03 51 15 37 34 14
48
(No Transcript)