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Abstract：
BACKGROUND: Cowden syndrome is a cancer predisposition syndrome caused by pathogenic variants in PTEN. The affected patients possess an increased risk of breast, thyroid, renal, colorectal, endometrial cancers as well as malignant melanoma. Thus prophylactic surveillance and follow up is crucial for these patients.
METHODS: A review of the literature including existing guidelines from the years 1996 until 2017 was carried out. In total, 2078 scientific papers were identified through database searches on Cowden syndrome. Among these, 11 manuscripts were included based on scientific relevance and quality. Expert consensus was reached to define management guidelines.
RESULTS: The literature revealed a high risk of cancer in specific organs for patients diagnosed with Cowden Syndrome. Alternative management guidelines were proposed and discussed.
CONCLUSIONS: Here we propose a revised set of management guidelines for patients with Cowden syndrome in Denmark to address the increased risk of various cancer types.
METHODS: A review of the literature including existing guidelines from the years 1996 until 2017 was carried out. In total, 2078 scientific papers were identified through database searches on Cowden syndrome. Among these, 11 manuscripts were included based on scientific relevance and quality. Expert consensus was reached to define management guidelines.
RESULTS: The literature revealed a high risk of cancer in specific organs for patients diagnosed with Cowden Syndrome. Alternative management guidelines were proposed and discussed.
CONCLUSIONS: Here we propose a revised set of management guidelines for patients with Cowden syndrome in Denmark to address the increased risk of various cancer types.
摘要：
背景：Cowden综合征是一种由PTEN致病变异引起的癌症易感综合征。受影响的患者患乳房的风险增加，甲状腺，肾,结直肠,子宫内膜癌和恶性黑色素瘤.因此，预防性监测和随访对这些患者至关重要。
方法：对1996年至2017年的文献进行了回顾，包括现有指南。总的来说,通过对Cowden综合征的数据库搜索，确定了2078篇科学论文。其中,根据科学相关性和质量，包括11份手稿。在确定管理准则方面达成了专家共识。
结果：文献显示，对于被诊断为Cowden综合征的患者，特定器官的癌症风险很高。提出并讨论了替代管理指南。
结论：在这里，我们为丹麦的Cowden综合征患者提出了一套修订的管理指南，以解决各种癌症类型风险增加的问题。
方法：对1996年至2017年的文献进行了回顾，包括现有指南。总的来说,通过对Cowden综合征的数据库搜索，确定了2078篇科学论文。其中,根据科学相关性和质量，包括11份手稿。在确定管理准则方面达成了专家共识。
结果：文献显示，对于被诊断为Cowden综合征的患者，特定器官的癌症风险很高。提出并讨论了替代管理指南。
结论：在这里，我们为丹麦的Cowden综合征患者提出了一套修订的管理指南，以解决各种癌症类型风险增加的问题。
