Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content +++ Key Features ++ Most common intraocular tumor in pediatric patients and causes 5% of cases of childhood blindness 90% of the tumors diagnosed before age 5 years Bilateral involvement occurs in 20–30% of children and typically is diagnosed at a younger age (median age 14 months) than unilateral disease (median age 23 months) +++ Clinical Findings ++ Leukocoria (white pupillary reflex) is the most common sign (found in 60% of patients) Parents may note an unusual appearance of the eye or asymmetry of the eyes in a photograph Strabismus is seen when the tumor involves the macula and central vision is lost Rarely (in 7% of patients), a painful red eye with glaucoma, a hyphema, or proptosis is the initial manifestation A single focus or multiple foci of tumor may be seen in one or both eyes at diagnosis Bilateral involvement occurs in 20–30% of children +++ Diagnosis ++ A detailed ophthalmologic examination under general anesthesia is required when retinoblastoma is suspected Diagnosis is made by the appearance of the tumor within the eye without pathologic confirmation A white to creamy pink mass protruding into the vitreous matter suggests the diagnosis; intraocular calcifications and vitreous seeding are virtually pathognomonic A CT scan of the orbits and MRI of the orbits/brain Detects intraocular calcification Evaluates the optic nerve for tumor infiltration Detects extraocular extension of tumor Metastatic disease of the marrow and meninges can be ruled out with bilateral bone marrow aspirates and biopsies plus CSF cytology +++ Treatment ++ Choice of therapy depends on the size, location, and number of intraocular lesions Absolute indications for enucleation include No vision Neovascular glaucoma Inability to examine the treated eye Inability to control tumor growth with conservative treatment External beam irradiation has been the mainstay of therapy; a total dose of 35–45 Gy is administered Cryotherapy, photocoagulation, and radioactive plaques can be used for local tumor control Patients with metastatic disease receive chemotherapy Your Access profile is currently affiliated with [InstitutionA] and is in the process of switching affiliations to [InstitutionB]. Please select how you would like to proceed. Keep the current affiliation with [InstitutionA] and continue with the Access profile sign in process Switch affiliation to [InstitutionB] and continue with the Access profile sign in process Get Free Access Through Your Institution Learn how to see if your library subscribes to McGraw Hill Medical products. Subscribe: Institutional or Individual Sign In Error: Incorrect UserName or Password Username Error: Please enter User Name Password Error: Please enter Password Sign in Forgot Password? Forgot Username? Sign in via OpenAthens Sign in via Shibboleth You already have access! Please proceed to your institution's subscription. Create a free profile for additional features.