Acinar Cell Carcinoma of the Pancreas: Overview of Clinicopathologic Features and Insights into the Molecular Pathology.

Acinar Cell Carcinoma of the Pancreas: Overview of Clinicopathologic Features and Insights into the Molecular Pathology.
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DOI:
10.3389/fmed.2015.00041
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发表时间:
2015
影响因子:
3.9
通讯作者:
Capella C
Capella C
中科院分区:
医学3区
文献类型:
--
作者:
La Rosa S;Sessa F;Capella C

文献摘要

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胰腺腺泡细胞癌(ACC)是一种罕见的胰腺肿瘤,在成人中约占胰腺肿瘤的1-2%,在儿科受试者中约占15%。它们表现出不同的临床症状,不同的形态学特征,不同的结局和不同的分子改变。这种异质性的临床病理谱可能会引起临床和病理诊断的困难,从而产生治疗和预后的影响。ACC发生和发展的分子机制尚未完全清楚,尽管近年来已经进行了几次尝试来阐明ACC生物学中涉及的分子机制。在本文中,我们将审查的主要临床病理和分子特征的胰腺ACC的成人和儿童的科目,让读者全面概述这种罕见的肿瘤类型。
Acinar cell carcinomas (ACCs) of the pancreas are rare pancreatic neoplasms accounting for about 1–2% of pancreatic tumors in adults and about 15% in pediatric subjects. They show different clinical symptoms at presentation, different morphological features, different outcomes, and different molecular alterations. This heterogeneous clinicopathological spectrum may give rise to difficulties in the clinical and pathological diagnosis with consequential therapeutic and prognostic implications. The molecular mechanisms involved in the onset and progression of ACCs are still not completely understood, although in recent years, several attempts have been made to clarify the molecular mechanisms involved in ACC biology. In this paper, we will review the main clinicopathological and molecular features of pancreatic ACCs of both adult and pediatric subjects to give the reader a comprehensive overview of this rare tumor type.