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Study examines risk of soft tissue sarcomas in hereditary retinoblastoma survivors

increase in soft-tissue sarcomas among hereditary retinoblastoma survivors compared with the general population and a 390-fold increase in cases of leiomyosarcomas.

Only 20 percent of the soft tissue sarcomas were diagnosed within 10 years of the retinoblastoma diagnosis; 45 percent of the sarcomas arose 30 or more years after diagnosis. In addition, 18 of the 23 (78 percent) leiomyosarcomas were diagnosed 30 or more years after the diagnosis of retinoblastoma. Almost all sarcomas were diagnosed in retinoblastoma survivors who had been treated with radiation. However, 18 of the 69 sarcomas developed outside the original radiation treatment field, and leiomyosarcoma was diagnosed more often outside the radiation field than in it.

The authors note that some of their calculations are based on few cases of these rare cancers. In addition, classification schemes for some sarcomas changed over the study period, so some cancer subtypes may have been classified differently depending on the year they were diagnosed.

"Given the excellent survival of retinoblastoma patients, it is important that survivors continue to undergo regular medical surveillance for sarcomas in their adult years," the authors write.

In an editorial, Anna Meadows, M.D., of The Children's Hospital of Philadelphia, highlights the advances in treatment for retinoblastoma in the last several decades, and she notes that some of these findings may be applicable only to patients who received treatments that are no longer used. "Improved treatment can be expected to reduce the incidence of soft tissue (and bone) sarcomas associated with radiation," Meadows writes. "The importance of this [new] report lies in its emphasis on leiomyosarcoma, a tumor that occurs in RB-1 gene carriers whether or not they were treated with radiation and that can be expected to continue to occur in older survivors, who will require more careful follow-up."


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Source:Journal of the National Cancer Institute


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