Retinoblastoma: Review of Current Management

Abstract

After completing this course, the reader will be able to:1. Discuss the need for a multidisciplinary approach to the management of children with retinoblastoma.2. Identify the patient factors that need to be considered when choosing the most appropriate initial and subsequent treatment for a child with retinoblastoma.3. Describe the role of genetics in the follow-up of retinoblastoma patients.Access and take the CME test online and receive 1 AMA PRA Category 1 Credit ™ at CME.TheOncologist.com CME CME ABSTRACTThe most common ocular cancer in children is retinoblastoma. It affects approximately 300 children in the U.S. every year. It can affect one or both eyes and the disease can be inherited. Altered discoloration of the pupil and strabismus are the usual symptoms that lead to medical attention. Subsequent appropriate diagnostic studies and care provided by a multidisciplinary team, including an ophthalmologist, a pediatric oncologist, a radiation oncologist, and a geneticist, among others, often result in optimal short-term and long-term care. The best initial and subsequent treatments are based on whether the child has unilateral or bilateral disease, the stage of the disease, and the age of the child. Enucleation, chemotherapy, and various forms of radiation therapy along with local ophthalmic therapies can be used in the treatment of retinoblastoma. Cure rates are high in children when the tumor is confined to the eye and has not spread systemically or into the orbit or brain. Children with the heritable form of retinoblastoma are at high risk for developing subsequent malignancies, most commonly sarcomas. This risk is greater for those children with the heritable form of the disease who were exposed to ionizing radiation at age <1 year. Exciting discoveries using animal models are providing new insights into the development of this disease and opening new avenues for targeted therapies that may lead to high cure rates with minimal toxicities. EPIDEMIOLOGYRetinoblastoma is the most common malignant ocular tumor in childhood and affects approximately 1 in 18,000 children Ͻ5 years of age in the U.S. [1]. The incidence is higher in developing countries, and in some countries in Central and South America retinoblastoma is one of the most common solid tumor malignancies in children [2]. The reason for this higher incidence is not clear. Lower socioeconomic status and the presence of human papilloma virus sequences in the retinoblastoma tissue have been implicated [3]. A higher risk for diseases like retinoblastoma in children born through in vitro fertilization was described previously, but a recent large study does not support this association [4]. Approximately 80% of children with retinoblastoma are diagnosed before 3 years of age. The diagnosis of retinoblastoma in children 6 years or older is extremely rare. Children with bilateral retinoblastoma constitute about 20%-30%. Patients with bilateral disease usually present at a younger age (14 -16 months) than patients with unilateral disease (29 -30 months) [...

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“…CT or MRI of the orbit and brain are the standard imaging modalities used for diagnosing and evaluating disease extent in retinoblastoma (5). To the best of our knowledge, the role of 18 F-FDG PET/CT in retinoblastoma has not been reported in the published literature.…”

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Immunohistochemical analysis of retinoblastoma cell phenotype using neuronal and glial cell markers

“…Retinoblastoma (RB) is the most common intraocular tumor in childhood (1,2) , and the vast majority of cases (about 80%) are diagnosed before the age of three (3) . Retinoblastoma originates from a neuro sensitive retina, and despite extensive study for many decades, its origin remains controversial (4,5) .…”