Retinoblastoma

By Tessa Rodgers

Anatomy & Physiology

Lymphatic drainage
- Eyelid submaxillary lymph nodes
- Anterior lymph channels of eye internal maxillary lymph nodes
- Posterior channels of eye subdural & subarachnoid spaces of brain
Retina
- Thin layer of tissue lining inside back of eye
- Converts received light to neural signals
- Sends neural signals to brain via optic nerve

Epidemiology
Most common primary intraocular tumor in pediatrics
Equally common amongst African Americans/Caucasians &
Females/Males
1 in 15,000 live births in US and Northern Europe
Birth 14 years old
- 95% < 5 years old at diagnosis
1/3 of cases are bilateral
- Younger

Etiology
40% Genetic Mutation
- RB1 tumor suppressor gene (chromosome 13)
- Bilateral
- Multifocal retinoblastoma
- Higher risk of developing secondary malignancy

60% Sporadic
- Unilateral

Staging

Reese Ellsworth Classification

- Groups 1-5
International Classification of Retinoblastoma
- Groups A-E
AJCC
-TNM

Presenting Symptoms
Leukocoria
Strabismus
Glaucoma
Poor vision
Irritability
Failure to eat
Low fever

Metastases
Optic Nerve Brain
Layers of eyeball Eye Socket
Eye Lid
Lymph nodes Liver
Bones
Bone Marrow

Common Treatment
Brachytherapy
- Up to 16 mm in diameter & 8 mm in thickness
- Eye plaque (Gold or Lead)
- Sewn into place
-

Iodine-125
Palladium-103
5 to 7 days
4000 6000 cGy

Cryotherapy
Singular tumors
Up to 4 mm in diameter & 2 mm in thickness
Small metal probe cooled with liquid nitrogen
Probe placed on outer surface of eye directly behind tumor
Frozen and thawed several times
- Creates intracellular crystals causing tumor cells to rupture
2-3 treatments each 1 month apart

Proton beam therapy

Bilateral disease & multifocal RB
Tumors unresponsive to chemotherapy
Versus Photon Beam
Radiation induced secondary malignancies
Dose to critical structures
No retardation of facial bone growth
180 cGy/fx
Total dose = 4500 cGy
Single lateral field, single AP field, AP and lateral fields, or oblique and AP
fields

Comparison of Side Effects

Brachytherapy
Damage to retina or optic nerve resulting in problems with
vision months/years later

Cryotherapy
Temporary swelling of eye and eyelid, damage to retina
resulting in blind spots

Proton Beam Therapy

Skin irritation, fatigue , no known late effects, very small
chance of RT induced secondary malignancies, vision

Prognosis and Survival Rates

5-year survival in children 014 years of age is 97%
Prognostic Factors
- Extraocular
- Metastatic
- Older children
- Glaucoma

My
Personal
Opinion

Aunt Tessa, Im

extremely skeptical
about what treatment I
should choose What do
you think I should do?

BRACHYTHERAPY
Shortest treatment duration
Least amount of times put under anesthesia
Not waiting months to see how tumor responds

References
Abramson DH, Ellsworth RM, Rozakis GW. Cryotherapy for Retinoblastoma. Arch
Ophthalmol. 1982; 100(8): 1253-1256.
Ammiryan ABeiki-Ardakani A, Galllie B. The American Brachytherapy Society
consensus guidelines for plaque brachytherapy of uveal melanoma and
retinoblastoma. Brachytherapy An International Multidisciplinary Journal. 2014;
13(1): 14.
Krengli, Marco et al. Proton radiation therapy for retinoblastoma: Comparison of
various intraocular tumor locations and beam arrangements. International
Journal of Radiation Oncology. 2005; 61(2): 583-593.