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家族性胰腺癌

Familial pancreatic cancer.

作者信息

Del Nero Lorenzo, Dabizzi Emanuele, Ceglie Antonella De, Ziola Sebastiano, Zerbi Alessandro, Baron Todd H, Conio Massimo

机构信息

Gastroenterology Department, Santa Corona Hospital, Pietra Ligure, ASL 2 Savonese, Italy.

Gastroenterology and Interventional Endoscopy Unit, AUSL Bologna, Surgical Department, Bologna, Italy.

出版信息

Clin Res Hepatol Gastroenterol. 2023 Mar;47(3):102079. doi: 10.1016/j.clinre.2023.102079. Epub 2023 Jan 18.

Abstract

Pancreatic cancer (PC) carries a poor prognosis with an overall 5-year survival of less than 10%. Early diagnosis, though cumbersome, is essential to allow complete surgical resection. Therefore, primary and secondary prevention are critical to reduce the incidence and to potentially prevent mortality. Given a relatively low lifetime risk of developing PC, identification of high-risk individuals is crucial to allow identification of pre-malignant lesions and small, localized tumors. Although 85-90% of PC cases are sporadic, we could consider risk stratification for the 5-10% of patients with a family history and the 3-5% of cases due to inherited genetic syndromes. These high-risk populations should be considered for screening and surveillance of PC. MRI/MRCP and EUS are the preferred modalities, due to their high sensitivity in lesion detection. Surveillance should be personalized, considering genetics and family history, and assessment of risk factors that may increase cancer risk. Screening programs should be limited to tertiary referral center, with high-volumes and adequate facilities to manage these patients.

摘要

胰腺癌(PC)预后较差,总体5年生存率低于10%。早期诊断虽繁琐,但对于实现完整手术切除至关重要。因此,一级和二级预防对于降低发病率并可能预防死亡至关重要。鉴于患胰腺癌的终生风险相对较低,识别高危个体对于识别癌前病变和小的局限性肿瘤至关重要。虽然85% - 90%的胰腺癌病例为散发性,但对于5% - 10%有家族史的患者以及3% - 5%由遗传综合征导致的病例,我们可以考虑进行风险分层。应考虑对这些高危人群进行胰腺癌的筛查和监测。MRI/MRCP和EUS是首选的检查方式,因为它们在病变检测方面具有高灵敏度。监测应个性化,考虑遗传学和家族史以及可能增加癌症风险的危险因素评估。筛查项目应限于三级转诊中心,这些中心具备大量病例和足够的设施来管理这些患者。

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