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Retinoblastoma
Staging system
International Classification System for Intraocular Retinoblastoma
(Murphree AL: Intraocular retinoblastoma: the case for a new group classification. Ophthalmol Clin North
Am 2005; 18:41-53.)
Investigations
Examination -Examination under anesthesia by ophthalmologist
-Audiology evaluation (hearing test) if systemic carboplatin is considered
Imaging studies -CT or MRI scan of brain and orbit Æshould include the pineal gland to
exclude trilateral retinoblastoma ( prefer MRI to avoid radiation exposure)
Laboratory evaluations -CBC
-BUN, Cr, electrolytes, Ca, Mg, P, LFT
-Calculated creatinine clearance
-Urine analysis
Diagnostic studies -Lumbar puncture only when there is radiographic or clinical suspicion of
CNS disease ie. optic nerve involvement or ≥ stage pT2
-Bone scan only with bone pain or other extraocular disease
-Bone marrow aspiration and biopsy only when there is abnormal blood
counts (without alternative explanation) or other extraocular disease
-Pathologic evaluation if enucleation is performed.
Indications of enucleation
1. Large tumor >50% of globe volume (ICRB group E, + D)
2. No potential for visions
3. Painful eyes
4. Optic nerve involvement
Laser photocoagulation and cryotherapy
For ICRB Group A: repeat every 3-4 weeks until evidence of tumor regression and inactivity (indirect
fundoscopy showing flat scar (or type IV regression pattern) along with the absence of new tumor foci,
evidence of tumor recurrence, or evidence of subretinal fluid, subretinal seeds, or vitreous seeds).
External beam radiation therapy (EBRT: lens sparing EBRT vs whole eye EBRT)
Standard dose is 40-45 Gy, preferably conformal, stereotactic, proton-beam or intensity-modulated
radiotherapy, and delayed to after 1 year of age with use of systemic adjuvant chemotherapy to avoid risk of
secondary malignancy
Family screening
Evaluation Schedule
Eye examination for -Parents and siblings of patients should have screening ophthalmic
parents and siblings examinations to exclude an unknown familial disease.
-Siblings should be under close surveillance until age 3 to 5 years (every 1-2
months during the first year, then every 2 months during the second year,
then every 4-6 months during the third year, then once or twice a year until
age 5, after that follow as general population ) or until confirmed not to have
a genetic mutation.
On the basis of the International Classification of Retinoblastoma, chemoreduction success is achieved in eye
survival rate 100% of group A, 93% of group B, 90% of group C, 47% of group D eyes, and 0% of group E.
Reference
Adapted from Carol L, Shields and Jerry A. Shields. Basic understanding of current classification and
management of retinoblastoma. Current Opinion in Ophthalmology 2006; 17: 228 – 234.
Retinoblastoma: Summary treatment strategy based on laterality and retinoblastoma grouping 213
Thai Pediatric Oncology Group
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Schematic treatment for intraocular retinoblastoma
Unilateral retinoblastoma
Cryotherapy Chemotherapy Protocol Chemotherapy Protocol High risk features: assessed by pathologist
and/or ThaiPOG-RB-I3-01 ThaiPOG-RB-13-02 -Anterior chamber seeding
Laser therapy (Carbo/VCR (Carbo/Eto/VCR) -Optic nerve tumor beyond lamina cribosa, but not to surgical margin
X 6 cycles) X 6 cycles for C -Choroidal involvement
-Intraocular hemorrhage
ThaiPOG-RB-13-03
-Posterior uveal involvement with any optic nerve disease (optic nerve
(Ifos/Carbo/Eto/VCR)
EBRT head, pre or postlaminar cribosa)
X 6-8 cycles for D
if not fully respond N Y
Low risk High risk
Enucleation or EBRT
if not fully responded
Schedule regular Chemotherapy Protocol
fundoscopic ThaiPOG-RB-13-02
examination (Carbo/VCR/Eto)
X 6 cycles
Retinoblastoma: Summary treatment strategy based on laterality and retinoblastoma grouping 214
Thai Pediatric Oncology Group
ชมรมโรคมะเร็งเด็ก
Schematic treatment for extraocular retinoblastoma
Extraocular retinoblastoma
Retinoblastoma: Summary treatment strategy based on laterality and retinoblastoma grouping 215
Thai Pediatric Oncology Group
ชมรมโรคมะเร็งเด็ก
Post-treatment evaluation
For patients with preserved eye
Evaluation Schedule Note
Eye examination under - Every 3-4 weeks until no active tumor on minimum 3 Patients should be
anesthesia (EUA) EUAs examined without
-Then every 6-8 weeks until 3 years of age anesthesia when old
-Then every 4-6 months until 10 years of age enough to cooperate.
-Then yearly
MRI brain and orbit -Every 6-12 months until 5 years of age
For patients after enucleation and/or received EBRT
Evaluation Schedule Note
Eye examination under -At 4-6 weeks post treatment Patients should be
anesthesia (EUA) -Then every 2-3 months in year 1 examined without
-Then every 3-4 months in year 2 anesthesia when old
-Then every 6 months until 5 years of age enough to cooperate.
-Then yearly
For patients with extraocular disease
Evaluation Schedule Note
CT or MRI of the brain and Every 6 months in first 3 years then every year until 5 - prefer MRI to avoid
orbits years after off treatment radiation exposure
CSF profiles and cytology Every 6 months in first 3 years then every year until 5
years after off treatment
Bone marrow aspiration and If unexplained cytopenia or bone scan positive
biopsy
Bone scan -Every 1 year for first 3 years
For patients with bilateral retinoblastoma, esp. whom diagnosed before 1 year of age or positive family
history
Evaluation Schedule Note
CT or MRI of the brain and Every 6 months from the end of treatment until 5 years - Screen for trilateral
orbits of age retinoblastoma
- prefer MRI to avoid
radiation exposure
For patients who received chemotherapy and/or radiation therapy
Evaluation Schedule Note
History and physical -Every 3 months until 2 years off treatment - Late effects of treatment
examination -Then every 6 months until 5 years off - Growth and development
treatment - Surveillance for second malignant
-Then yearly neoplasms (osteosarcoma, soft tissue
sarcomas, skin cancers, breast
cancers)
Visual acuity assessment -Yearly
CBC -Yearly for 10-15 years from exposure of -Surveillance for secondary leukemia
chemotherapy
Inclusion criteria
Retinoblastoma ICRB group B
Inclusion criteria
Retinoblastoma ICRB group C
Retinoblastoma post enucleation with high risk features
Anterior chamber seeding Optic nerve tumor, but not to surgical margin
Choroidal involvement Intraocular hemorrhage
Posterior uveal involvement with any optic nerve disease
Inclusion criteria
Retinoblastoma ICRB group D
Retinoblastoma ICRB group E
10
11
12
Inclusion criteria
Disease at the surgical margin Orbital recurrence (orbital mass)
Tumor in an emissary canal (intrascleral involvement) Episcleral disease
Positive pre-auricular lymph nodes
Chemotherapy schedule
Week 0 3 6 9 12 15 18 21
Course A1 B1 A2 B2 A3 B3 A4 B4
Date
***** Start G-CSF at 24 -48 hr after completion each course of chemotherapy *****
Course A
Given dose Drug Dosage Day
__________mg Vincristine 0.05 mg/kg or 1.5 mg/m2 IV slowly push 1
__________mg Idarubicin 0.33 mg/kg or 10 mg/m2 IV infusion in 60 min 1
__________mg Cyclophosphamide* 65 mg/kg or 2,000 mg/m2 IV drip in 30-60 min 1
__________mg Mesna 30 mg/kg or 1,000 mg/m2 IV drip in in15 min 1
at 0,3 hr after CTX (total 2 doses)
In patient BW < 12 kg, use doses per kg
* hydration following High dose CTX guideline
Course B
Given dose Drug Dosage Day
__________mg Etoposide 3.3 mg/kg or 100 mg/m2 IV drip in 60 min 1-3
__________mg Carboplatin 18.6 mg/kg or 560 mg/m2 IV drip in 15-30 min 1-2
In patient BW < 12 kg, use doses per kg
A1
B1
A2
B2
A3
B3
A4
B4
Inclusion criteria
Retinoblastoma with CNS/ bone/ bone marrow involvement
Retinoblastoma with isolated meningeal disease
Ectopic intracranial retinoblastoma