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© The Author 2007. Published by Oxford University Press.
EDITORIAL |
Retinoblastoma Survivors: Sarcomas and Surveillance
Correspondence to: Anna T. Meadows, MD, Division of Pediatric Oncology, Children's Hospital of Philadelphia, 3400 Civic Center Blvd., Philadelphia, PA 19104 (e-mail: meadows@email.chop.edu).
| The first 10% of the full text of this article appears below. |
Modern therapy for childhood cancer began in 1970, and when, in the 1980s, cure rates increased and survivors were sufficiently numerous, reports of second cancers began to emerge. Despite its rarity among children with cancer (3%), retinoblastoma was the most frequent first neoplasm in a large cohort of survivors with second cancers (1,2). During that same period, our knowledge of the genetics of retinoblastoma increased, and the RB-1 gene, cloned from a secondary sarcoma, was found to be a critical regulator of cell proliferation in all cells (3,4). This raised a question: why did mutations in this gene predispose carriers to retinoblastoma specifically? We now know that the gene was not specific for this cancer and, in fact, other cancers were also part of the predisposition.
As early as
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