Retinoblastoma Flashcards
(25 cards)
Is a
PNET that develops in the retina.
Specialized by
Light sensitive tissue at back of the eye that detects light and color
It is the most
Common intraoccular tumor in young children
It is a disease of the
Very young, 2/3 are diagnosed before 2 years and 95% are before 5
How many patients presenting with a germline mutation
25-35%
What gene is mutated?
RB1 on chromosome 13
Atleast
65% of retinoblastomas are unilateral and without evidence of inheritance
Often discovered as a
Result of abnormal retinal light reflex called leukocoria
Leukocoria
White pupil rather than red, cats eye reflex
Biopsy is typically
Contraindicated because of risk of virtuous seeding
Ultra sound
Useful for location and size
For advanced cases
The CsF may be assessed with lumbar puncture and bone marrow biopsy
Small focal tumors away from optic disc and macula
Photocoagulation, laser hyperthermia, cryosurgery can result in disease control
Local therapies are commonly used in
Conjunction with chemo therapy
External beam radiation
Cases of bilateral or inoperable unilateral disease
Challenge of EBrt
Difficult to treated entire retina while sparing cornea and lens
Modern treatment methods include
1.) suction to displace globe
2.) 3d/IMRT
3.) conformal proton therapy
If tumor masses are less extensive
Radiation implants plaques can be used.
What is the most widely ysed radio include
Iodine 125
Cure rate of more than 80% and visual preservation
Because it’s a small blue cell tumor of neuroectodermal origin
A good response to chemo is expected
Long term sequence include
Altered growth of bony orbit
Decreased vision
Cataract
Dry eye
2nd malignancy
If enucleation is chosen
Child needs several prodthetic eyes as growth occurs
Because EBRT uses doses of 4000
Facial growth is likely impaired
Cured tumors can leave
Blind spots
Cataracts
Dry eyes
