with most cases occurring at the average age of 17 months. The majority of
cases of retinoblastoma are spontaneous, but a few cases are associated with
an autosomal-dominant pattern of inheritance with survivors of the disease at
risk for being carriers to their children.Signs and Symptoms
Common assessment findings:- History of trauma to leg (client related pain to trauma and delayed
trip to doctor) - Taller than average height due to rapid bone growth
- Pain, warmth, and swelling at the tumor site
- Pathologic fracture of the bone—as tumor invades and weakens bone
Test Results
Refer child to ophthalmologist for evaluation.
Indirect ophthalmoscopy performed with scleral indentation for diagnosis.Staging
Few cases have metastasis.
Classification by Reese-Ellsworth used.- Group I: Very favorable with solitary tumor <4 disc diameters (DD)
at or behind the equator or multiple tumors at or behind the equator
all <4 DD. - Group II: Favorable with solitary tumor 4 to 10 DD at or behind the
equator, or multiple tumors at or behind the equator 4 to 10 DD. - Group III: Doubtful with solitary tumor >10 DD behind the equator,
or any tumors anterior to the equator. - Group IV: Unfavorable with multiple tumors some larger than10 DD
at or any tumors anterior to the ora serrata. - Group V: Very unfavorable: Massive tumors involving more than
half the retina and vitreous seeding.
Metastasis is seldom noted; invasive staging procedures such as aspira-
tion or biopsy not performed.
Treatments
Localized tumor treatment:- Plaque brachytherapy (implantation of a radiation applicator on
sclera (iodine-125) until maximum dose delivered to the tumor. - Photocoagulation (laser beam used to destroy blood supply to
tumor). - Cryotherapy (freezing of the tumor to destroy microcirculation to
and cells of the tumor).
With advanced tumor growth with involvement of the optic nerve, enu-
cleation of the affected eye is treatment of choice.
32(^170) Pediatric Nursing Demystified