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胰腺具有甲状腺滤泡样特征的腺泡细胞癌:一种新的具有诊断挑战性的亚型的首次描述。

Acinar cell carcinoma of the pancreas with thyroid-like follicular features: first description of a new diagnostic challenging subtype.

机构信息

Institute of Pathology, Lausanne University Hospital and University of Lausanne, Lausanne, Switzerland.

Department of Gastroenterology, Lausanne University Hospital and University of Lausanne, Lausanne, Switzerland.

出版信息

Virchows Arch. 2019 Dec;475(6):789-794. doi: 10.1007/s00428-019-02628-3. Epub 2019 Jul 23.

DOI:10.1007/s00428-019-02628-3
PMID:31338587
Abstract

Acinar cell carcinomas (ACCs) of the pancreas are a heterogeneous group of neoplasms showing a wide spectrum of morphological features including acinar, solid, glandular, and trabecular architecture. In addition, uncommon cytological aspects have recently been described and include oncocytic, spindle, clear, and pleomorphic cell types. This wide histological spectrum represents a challenge in the diagnostic task for pathologists. Molecular mechanisms involved in the onset and progression of ACCs are not completely known, but, in general, they differ from those observed in ductal adenocarcinomas or neuroendocrine neoplasms of the pancreas and frequently include alterations in the APC/β-catenin pathway. In the present paper, we describe a new variant of ACC showing thyroid-like follicular features and CTNNB1 mutation. This phenotype needs to be included in the spectrum of morphological presentation of ACC.

摘要

胰腺腺泡细胞癌 (ACCs) 是一组异质性肿瘤,表现出广泛的形态学特征,包括腺泡、实性、腺样和小梁状结构。此外,最近还描述了一些不常见的细胞学特征,包括嗜酸细胞性、梭形、透明和多形性细胞类型。这种广泛的组织学谱给病理学家的诊断任务带来了挑战。ACCs 发生和进展中涉及的分子机制尚不完全清楚,但一般来说,它们与在胰腺导管腺癌或神经内分泌肿瘤中观察到的机制不同,并且经常包括 APC/β-连环蛋白途径的改变。在本文中,我们描述了一种具有甲状腺样滤泡特征和 CTNNB1 突变的新型 ACC 变体。这种表型需要包含在 ACC 的形态表现谱中。

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本文引用的文献

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Genome-wide genetic and epigenetic analyses of pancreatic acinar cell carcinomas reveal aberrations in genome stability.对胰腺腺泡细胞癌的全基因组遗传和表观遗传学分析揭示了基因组稳定性的异常。
Nat Commun. 2017 Nov 6;8(1):1323. doi: 10.1038/s41467-017-01118-x.
2
Primary Hepatic Neuroendocrine Tumor with Unusual Thyroid Follicular-Like Morphologic Characteristics.具有不寻常甲状腺滤泡样形态特征的原发性肝神经内分泌肿瘤
Case Rep Pathol. 2017;2017:7931975. doi: 10.1155/2017/7931975. Epub 2017 Feb 20.
3
Mixed Neuroendocrine-Nonneuroendocrine Neoplasms (MiNENs): Unifying the Concept of a Heterogeneous Group of Neoplasms.
混合性神经内分泌-非神经内分泌肿瘤(MiNENs):统一一组异质性肿瘤的概念
Endocr Pathol. 2016 Dec;27(4):284-311. doi: 10.1007/s12022-016-9432-9.
4
CTNNB1 (β-Catenin)-altered Neoplasia: A Review Focusing on Soft Tissue Neoplasms and Parenchymal Lesions of Uncertain Histogenesis.CTNNB1(β-连环蛋白)改变的肿瘤形成:聚焦软组织肿瘤和组织发生不确定的实质病变的综述
Adv Anat Pathol. 2016 Jan;23(1):1-12. doi: 10.1097/PAP.0000000000000104.
5
TP53 alterations in pancreatic acinar cell carcinoma: new insights into the molecular pathology of this rare cancer.胰腺腺泡细胞癌中的TP53改变:对这种罕见癌症分子病理学的新见解
Virchows Arch. 2016 Mar;468(3):289-96. doi: 10.1007/s00428-015-1882-9. Epub 2015 Nov 19.
6
Acinar Cell Carcinoma of the Pancreas: Overview of Clinicopathologic Features and Insights into the Molecular Pathology.胰腺腺泡细胞癌:临床病理特征概述及对分子病理学的深入了解。
Front Med (Lausanne). 2015 Jun 15;2:41. doi: 10.3389/fmed.2015.00041. eCollection 2015.
7
Acinar cell carcinomas of the pancreas: a molecular analysis in a series of 57 cases.胰腺腺泡细胞癌:57例病例的分子分析
Virchows Arch. 2014 Dec;465(6):661-72. doi: 10.1007/s00428-014-1657-8. Epub 2014 Oct 9.
8
Comprehensive genomic profiling of pancreatic acinar cell carcinomas identifies recurrent RAF fusions and frequent inactivation of DNA repair genes.对胰腺腺泡细胞癌进行全面的基因组分析,确定了 RAF 融合的反复出现和 DNA 修复基因的频繁失活。
Cancer Discov. 2014 Dec;4(12):1398-405. doi: 10.1158/2159-8290.CD-14-0617. Epub 2014 Sep 29.
9
APC alterations are frequently involved in the pathogenesis of acinar cell carcinoma of the pancreas, mainly through gene loss and promoter hypermethylation.APC 改变常涉及胰腺腺泡细胞癌的发病机制,主要通过基因缺失和启动子过度甲基化。
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