Journal
PEDIATRIC CLINICS OF NORTH AMERICA
Volume 62, Issue 1, Pages 201-+Publisher
W B SAUNDERS CO-ELSEVIER INC
DOI: 10.1016/j.pcl.2014.09.014
Keywords
Retinoblastoma; Chemotherapy; Germline mutation; Second malignant neoplasms
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Retinoblastoma is the most common neoplasm of the eye in childhood, and represents 3% of all childhood malignancies. Retinoblastoma is a cancer of the very young; two-thirds are diagnosed before 2 years of age and 95% before 5 years. Retinoblastoma presents in 2 distinct clinical forms: (1) a bilateral or multifocal, heritable form (25% of all cases), characterized by the presence of germline mutations of the RB1 gene; and (2) a unilateral or unifocal form (75% of all cases), 90% of which are nonhereditary. The treatment of retinoblastoma is multidisciplinary and is designed primarily to save life and preserve vision.
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