TY  - JOUR
A1  - Nees, Juliane
A1  - Kiermeier, Senta
A1  - Struewe, Farina
A1  - Keymling, Myriam
A1  - Maatouk, Imad
A1  - Kratz, Christian P.
A1  - Schott, Sarah
T1  - Health behavior and cancer prevention among adults with Li-Fraumeni syndrome and relatives in Germany — a cohort description
JF  - Current Oncology
N2  - Li-Fraumeni-syndrome (LFS) is a rare, highly penetrant cancer predisposition syndrome (CPS) caused by pathogenic variants (PVs) in TP53. Physical activity (PA) and a Mediterranean diet lead to cancer reduction or survival benefits and increased quality of life (QoL), but this is yet unstudied among LFS. TP53 PV carriers (PVC) and their relatives were questioned on dietary patterns (Mediterranean Diet Adherence Screener), PA (Freiburg Questionnaire), QoL (Short-form-Health-Survey-12), smoking, alcohol consumption and perception of cancer risk in a German bi-centric study from March 2020–June 2021. The study enrolled 70 PVC and 43 relatives. Women compared to men (6.49 vs. 5.38, p = 0.005) and PVC to relatives (6.59 vs. 5.51; p = 0.006) showed a healthier diet, associated with participation in surveillance (p = 0.04) and education (diet p = 0.02 smoking p = 0.0003). Women smoked less (2.91 vs. 5.91 packyears; p = 0.03), psychological well-being was higher among men (SF-12: males 48.06 vs. females 41.94; p = 0.004). PVC rated their own cancer risk statistically higher than relatives (72% vs. 38%, p < 0.001) however, cancer risk of the general population was rated lower (38% vs. 70%, p < 0.001). A relative’s cancer-related death increased the estimated personal cancer risk (p = 0.01). The possibilities of reducing cancer through self-determined health behavior among PVC and relatives has not yet been exhausted. Educating families with a CPS on cancer-preventive behavior requires further investigation with regard to acceptance and real-life implementation.
KW  - pathogenic TP53 germline variant
KW  - Li-Fraumeni syndrome
KW  - cancer prevention
KW  - physical activity
KW  - cancer predisposition
KW  - SF-12
KW  - MEDAS
Y1  - 2022
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-290432
SN  - 1718-7729
VL  - 29
IS  - 10
SP  - 7768
EP  - 7778
ER  -