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- W1978234885 abstract "Among the high proportion of Li-Fraumeni syndrome (LFS) patients who are diagnosed with cancer, 15% and 4% develop secondary and third cancers, respectively. Prior studies have shown that radiation therapy (RT) raises the risk of malignancy in LFS patients, but this risk needs to be better characterized. The purpose of this literature review is to determine the proportion and patterns of subsequent malignancies in LFS patients who receive RT. A PubMed search of articles from 1998 to 2012 pertaining to LFS patients who received RT was performed using a combination of keywords that included Li-Fraumeni syndrome, TP53 mutation, radiation therapy, irradiation, familial, cancer, and neoplasm. To be included in this review, patients had to be diagnosed with LFS (classic definition) and prior RT exposure. Additionally, it was necessary that the initial tumor type, the site of RT, and follow-up information were documented. We identified 10 articles and case reports with a combined 23 patients who met the inclusion criteria. Gender was female in 16, male in 5, and unknown in 2. The median age was 26.5 years at diagnosis of the initial cancer (0.7-48 years). The most common primary tumors treated with RT were breast cancer 9 (39%), non-rhabdomyosarcoma soft tissue sarcoma (NRSTS) 5 (22%) and rhabdomyosarcoma 3 (13%). The median post-RT follow-up was 6 years (1.3-12 years). Of the 23 LFS patients who received RT, 16 (70%) developed a secondary and 5 (22%) developed a tertiary malignancy. Eleven (48%) developed a secondary and 2 (13%) developed a tertiary malignancy in the RT field. The median time to develop a secondary malignancy in the RT field was 9 years (2-22 years). The most common cancers in the RT field included soft tissue sarcomas 7 (54%), lung cancer 2 (15%) and osteosarcoma 2 (15%) while the most common secondary cancers outside the RT field were breast cancer 5 (38%), soft tissue sarcoma 3 (23%), and pancreatic cancer 2 (15%). In LFS patients who received RT for their primary cancer, 48% developed a secondary malignancy in the RT field. Soft tissue sarcoma was the predominant type of secondary cancer in the RT field, while breast cancer was most common outside of the RT field." @default.
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- W1978234885 date "2013-10-01" @default.
- W1978234885 modified "2023-10-18" @default.
- W1978234885 title "Subsequent Malignancies in Patients With Li-Fraumeni Syndrome Treated With Radiation Therapy" @default.
- W1978234885 doi "https://doi.org/10.1016/j.ijrobp.2013.06.186" @default.
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