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Pediatr Clin North Am. 2015 Feb;62(1):201-23. doi: 10.1016/j.pcl.2014.09.014.

Retinoblastoma.

Author information

1
Department of Pediatric Oncology, Dana-Farber/Boston Children's Cancer and Blood Disorders Center, Harvard Medical School, 450 Brookline Avenue, D3-133, Boston, MA 02215, USA. Electronic address: carlos_rodriguez-galindo@dfci.harvard.edu.
2
Department of Radiology, Boston Children's Hospital, Harvard Medical School, 300 Longwood Avenue, Boston, MA 02215, USA.
3
Department of Ophthalmology, Boston Children's Hospital, Harvard Medical School, 300 Longwood Avenue, Boston, MA 02215, USA.

Abstract

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.

KEYWORDS:

Chemotherapy; Germline mutation; Retinoblastoma; Second malignant neoplasms

PMID:
25435120
DOI:
10.1016/j.pcl.2014.09.014
[Indexed for MEDLINE]

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