Syafril Umar Lubis

RETINOBLASTOMA (RB) is the most common primary intraocular malignancy of infancy and childhood
The incidence of RB : 1 : 14000 -20000 LIVE BIRTHS

SPORADICALLY OR INHERITED
Mutation of the RB1gene, the q14band of chromosome 13

 RB

: present with advance diseases in developing countries Eye enlargement is a common finding

Family history of RB 45-50% have child with RB

Onset : first year of life in bilateral and family history, between 1 and 3 in sporadic unilateral cases

THE GOAL WITH RETINOBLASTOMA

Early detection to maximize the visual outcome The quality of life of the affected child

Leukocoria also known as amaurotic cats eye

The most common sign

Dilated pupil

Normal red reflex

Red reflex absent

Red reflex abnormal

Strabismus, or decrease vision.

Less common : Vitreous hemorrhages Hyphema Ocular / periocular inflammation Glaucoma Proptosis Hypopyon

o
o o o o o

Screening for RB should be part of well baby screening for new borns during the first three

months of life

The red reflex The corneal red reflex / Hirshberg test Eye examination

IF THE EYE EXAMINATION IS ABNORMAL :

o CT Scan o Magnetic Resonance Imaging (MRI)

Can help define the structure abnormalities & reveal any calcium deposit

USG

CAN HELP DEFINE THE HEIGHT & THICKNESS OF THE TUMOR

o o

BONE MARROW EXAMINATION / LUMBAR PUNCTION

TO DETERMINE METASTASES TO BONES OR THE BRAINS

BLOOD TEST

GENETIC and/or DNA TESTING

The large white mass with prominent feeder vessels is located at the macula A smaller tumor is located in the inferolateral part of the retina.

Reese-Ellsworth. DD disc diameter

A I II III IV V

Solitary tumor <4 DD Multiple tumors, none >4 DD Solitary tumor, 4-10 DD Multiple tumors, 4-10 DD Any lesion anterior to equator Solitary tumor > 10 DD Multiple tumors, some > 10 DD Any lesion anterior OS Tumor involving 50% of retina Vitreous seeding

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International Classification
Group A Characteristics Small tumors, 3 mm, 3 mm from foveola, 1.5 mm from disc

B
C

Tumor > 3 mm, subretinal fluid < 3 mm from the base of tumor
Vitreous seeding (C1) 3 mm,or subretinal seeding (C2) 3 mm from tumor, or both (C3)

Vitreous seeding (D1) > 3 mm, or subretinal seeding (D2) > 3 mm from tumor, or both (D3)

E No visual potential, poor prognosis, tumor in anterior segment/ cilliary body/ iris neovascularization/ neovascular glaucoma/ vitreous hemorrhage/ phthisis bulbi/ tumor necrosis/ orbital cellulitis-like presentation/ optic nerve/ extraocular tumor

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Cataract Congenital
Persistent hyperplastic primary vitreous (PHVP)

Coats Disease
Retinopathy of Prematurity (ROP)

Early Diagnosis

Recent Advanced Management

Prognosis
Survival rate nearly 95 % in last decade (USA)

TREATMENT

VARIES FROM COUNTRY TO COUNTRY

The priority : life of child

The

Vision Minimize

complication / side effect of

treatment

The exact course of treatment individual case

TREATMENT
Chemotherapy Cryotheraphy External

Beam Radiotheraphy

Enucleation

EXTERNAL BEAM RADIOTHERAPY

Moderately advanced unilateral or bilateral RB Diffuse vitreous seeding Failure of chemotherapy or other modilities

Enucleation
2nd & 3nd management RB Massive unilateral RB, Reese-Ellsworth group V or International clssification groups D&E Enucleationhigh risk histopatology chemotherapy reduce the risk of metastase

The combination of ectoposide phosphate, vincristine sulfate and carboplastine is infused every four week 6 month Indicated for children whose enucleated globes show features high risk metastasis

Can Retinoblastoma be found early ?

All babies should have general eye

exam at birth six months

Newborns with family history of this cancer eye exam a few day after birth, at six weeks, once every three months until age two

A blood test to determine if the

mutation is present

THAN K YOU