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Familial Cancer - In high-risk individuals participating in a pancreatic cancer surveillance program, worrisome features warrant for intensified surveillance or, occasionally, surgery. Our...  相似文献   
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It is important to adequately and timely identify individuals with cancer worries amongst participants in a pancreatic ductal adenocarcinoma (PDAC) surveillance program, because they could benefit from psychosocial support to decrease distress. Therefore, the aim of this study was to assess both psychosocial and clinical factors associated with cancer worries. High-risk individuals participating in PDAC-surveillance were invited to annually complete a cancer worry scale (CWS) questionnaire which was sent after counseling by the clinical geneticist (T0), after intake for participation in PDAC-surveillance (T1), and then annually after every MRI and endoscopic ultrasonography (EUS) (T2 and further). Analyses were performed to identify factors associated with cancer worries in the second year of surveillance (T3). We found a significant intra-individual decrease in cancer worries (β = ?0.84, P < 0.001), nevertheless, 33 % of individuals had a CWS-score ≥14 at T3. We found one factor significantly associated with cancer worries at T3: having a family member affected by PDAC <50 years of age (β = 0.22, P = 0.03). The detection of a cystic lesion, a shortened surveillance interval, or undergoing pancreatic surgery did not lead to more cancer worries (P = 0.163, P = 0.33, and P = 0.53, respectively). In conclusion, this study identified ‘a family history of PDAC <50 years of age’ as the only predictor of cancer worries experienced after 2 years of surveillance in individuals at high risk of developing PDAC. This knowledge could help clinicians to timely identify individuals ‘at risk’ for high levels of cancer worries who would likely benefit from psychosocial support.  相似文献   
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PALB2-mutation carriers not only have an increased risk for breast cancer (BC) but also for pancreatic cancer (PC). Thus far, PALB2 mutations have been mainly found in PC patients from families affected by both PC and BC. As it is well known that the prevalence of gene mutations varies between different populations, we studied the prevalence of PALB2 mutations in a Dutch cohort of non-BRCA1/2 familial PC (FPC) families and in non-BRCA1/2 familial BC (FBC) families with at least one PC case. Mutation analysis included direct sequencing and multiplex ligation-dependent probe amplification (MLPA) and was performed in a total of 64 patients from 56 distinct families (28 FPC families, 28 FBC families). In total, 31 patients (48%) originated from FPC families; 24 were FPC patients (77%), 6 had a personal history of BC (19%) and 1 was a suspected carrier (3.2%). The remaining 33 patients (52%) were all female BC patients of whom 31 (94%) had a family history of PC and 2 (6.1%) had a personal history of PC. In none of these 64 patients a PALB2 mutation was found. Therefore, PALB2 does not have a major causal role in familial clustering of PC and BC in non-BRCA1/2 families in the Dutch population.  相似文献   
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