Health Behavior and Cancer Prevention among Adults with Li-Fraumeni Syndrome and Relatives in Germany-A Cohort Description

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.

Medienart:

E-Artikel

Erscheinungsjahr:

2022

Erschienen:

2022

Enthalten in:

Zur Gesamtaufnahme - volume:29

Enthalten in:

Current oncology (Toronto, Ont.) - 29(2022), 10 vom: 15. Okt., Seite 7768-7778

Sprache:

Englisch

Beteiligte Personen:

Nees, Juliane [VerfasserIn]
Kiermeier, Senta [VerfasserIn]
Struewe, Farina [VerfasserIn]
Keymling, Myriam [VerfasserIn]
Maatouk, Imad [VerfasserIn]
Kratz, Christian P [VerfasserIn]
Schott, Sarah [VerfasserIn]

Links:

Volltext

Themen:

Cancer predisposition
Cancer prevention
Journal Article
Li-Fraumeni syndrome
MEDAS
Pathogenic TP53 germline variant
Physical activity
Research Support, Non-U.S. Gov't
SF-12
Tumor Suppressor Protein p53

Anmerkungen:

Date Completed 27.12.2022

Date Revised 11.01.2023

published: Electronic

Citation Status MEDLINE

doi:

10.3390/curroncol29100614

funding:

Förderinstitution / Projekttitel:

PPN (Katalog-ID):

NLM348052960