PDF(Pubmed)
Abstract：
Pancreatic cancer is one of the deadliest malignancies in humans and it is expected to play a bigger part in cancer burden in the years to come. Pancreatic ductal adenocarcinoma (PDAC) represents 85% of all primary pancreatic malignancies. Recently, much attention has been given to PDAC, with significant advances in the understanding of the mechanisms underpinning disease initiation and progression, along with noticeable improvements in overall survival in both localized and metastatic settings. However, given their rarity, rare histological subtypes of pancreatic cancer have been underappreciated and are frequently treated as PDAC, even though they might present non-overlapping molecular alterations and clinical behavior. While some of these rare histological subtypes are true variants of PDAC that should be treated likewise, others represent separate clinicopathological entities, warranting a different therapeutic approach. In this review, we highlight clinical, pathological, and molecular aspects of rare histological types of pancreatic cancer, along with the currently available data to guide treatment decisions.
Should rare subtypes of pancreatic cancer be treated as its most common variant (ductal adenocarcinoma)? The most common type of pancreatic cancer is ductal adenocarcinoma. While much attention has been given to the molecular aspects and treatment aspects of this disease, rare variants of pancreatic cancer have been underappreciated. Some of them present unique molecular features that suggest different treatment approaches could lead to better outcomes. In this review, we summarize information on the clinical, pathological, and molecular features of rare subtypes of pancreatic cancer, along with subtype-specific data on treatment.
Should rare subtypes of pancreatic cancer be treated as its most common variant (ductal adenocarcinoma)? The most common type of pancreatic cancer is ductal adenocarcinoma. While much attention has been given to the molecular aspects and treatment aspects of this disease, rare variants of pancreatic cancer have been underappreciated. Some of them present unique molecular features that suggest different treatment approaches could lead to better outcomes. In this review, we summarize information on the clinical, pathological, and molecular features of rare subtypes of pancreatic cancer, along with subtype-specific data on treatment.
摘要：
胰腺癌是人类最致命的恶性肿瘤之一，预计在未来几年将在癌症负担中发挥更大的作用。胰腺导管腺癌（PDAC）占所有原发性胰腺恶性肿瘤的85％。最近,PDAC受到了很多关注，随着对疾病发生和进展的基础机制的理解取得重大进展，在局部和转移性环境中，总生存率均有显着改善。然而,鉴于它们的稀有性，胰腺癌的罕见组织学亚型被低估，经常被视为PDAC，即使它们可能存在非重叠的分子改变和临床行为。虽然这些罕见的组织学亚型中的一些是PDAC的真正变体，应该同样治疗，其他代表独立的临床病理实体，保证采用不同的治疗方法。在这次审查中,我们强调临床，病态,和罕见的胰腺癌组织学类型的分子方面，以及目前可用的数据来指导治疗决策。
胰腺癌的罕见亚型应该作为其最常见的变异体（导管腺癌）吗？最常见的胰腺癌类型是导管腺癌。虽然人们对这种疾病的分子方面和治疗方面给予了很大的关注，胰腺癌的罕见变异被低估了.其中一些表现出独特的分子特征，表明不同的治疗方法可能会导致更好的结果。在这次审查中,我们总结了临床信息，病态,和罕见的胰腺癌亚型的分子特征，以及亚型特异性治疗数据。
胰腺癌的罕见亚型应该作为其最常见的变异体（导管腺癌）吗？最常见的胰腺癌类型是导管腺癌。虽然人们对这种疾病的分子方面和治疗方面给予了很大的关注，胰腺癌的罕见变异被低估了.其中一些表现出独特的分子特征，表明不同的治疗方法可能会导致更好的结果。在这次审查中,我们总结了临床信息，病态,和罕见的胰腺癌亚型的分子特征，以及亚型特异性治疗数据。
