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| |  Most Significant Advance in Retinoblastoma Treatment in 40 Years LOS ANGELES, Nov. 12, 1996 -- Doctors and researchers at Childrens Hospital Los Angeles (CHLA) have pioneered the most significant advance in the treatment of retinoblastoma in more than 40 years, according to Dr. A. Linn Murphree, Director of the Ocular Oncology Center at CHLA, and a professor of Ophthalmology and Pediatrics at USC School of Medicine. One in 15,000 infants will develop retinoblastoma, a potentially fatal or blinding eye cancer. The tumor is present in both eyes of 40% of the affected patients. Unchanged since the 1950s, the standard treatment of this tumor has required removal of the eye with the more advanced disease, and radiation therapy to one or both eyes. Radiation causes an unacceptable facial deformity of the bone around the eyes. In addition, this treatment increases the risk of these children dying of a radiation induced cancer over their lifetime from a baseline of 6%, to approximately 35%. Dr. Murphree and Judith Villablanca, M.D., Clinical Director of CHLA's Brain Tumor Program, and an assistant professor of Pediatrics at USC, worked to redesign a radiation-free treatment regimen consisting of a three-month course of systemic chemotherapy for which they coined the term -- chemoreduction -- followed by SALT (Sequential Aggressive Local Therapy). Their treatment protocol began in 1990 and the results and outcomes were published in this month's issue of the journal Archives of Ophthalmology. Their protocol and approach is being used now in major pediatric cancer centers around the world in an effort to avoid radiation-induced second cancers. In the treatment of more than 170 patients with retinoblastoma during the six-year period (1990-1995), radiation therapy was not necessary as primary treatment in a single case. Both removal of the eye and facial deformity was significantly decreased. Most of what is known about the cause of retinoblastoma was learned in the last 15 years. By the mid-1960s, it was known that children with bilateral retinoblastoma were at great risk to develop other primary cancers in addition to retinoblastoma, but the reason was unknown. An answer began to emerge when CHLA's Clayton ocular oncology researchers led by Dr. Murphree and Yuen K.T. Fung, Ph.D., isolated and cloned the retinoblastoma (RBI) gene in early 1987. This RBI gene, the first human cancer gene to be cloned, was found to be a "suppressor of cell growth," or a "tumor suppressor" gene. Its presence prevented cells from escaping controls on cell growth, thus becoming cancer cells. Radiation therapy tends to damage the DNA of this and other growth suppressor genes, increasing the risk of cancer. Dr. Murphree is listed in both the national directory, "The Best Doctors in America," and in Los Angeles magazine's "Best Doctors in Los Angeles." Other Childrens Hospital Los Angeles doctors who participated in the study include Charles Gomer, Ph.D., Marcio Malagolowski, M.D., and Anita Fisher, Ph.D. Founded in 1901, Childrens Hospital Los Angeles is a global resource for advanced pediatric care, research and medical education. Each year, the nonprofit 318-bed hospital treats nearly 200,000 patients from newborn to 18 years of age with specialized, multidisciplinary care in pediatric and adolescent medicine. CHLA also houses an internationally recognized research program and trains pediatric specialists through its affiliation, since 1932, with the USC School of Medicine. For seven consecutive years -- 1990 to 1996 -- U.S. News & World Report magazine has named CHLA one of the top five pediatric facilities in the country, and the best in the West.
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