Advances in Adrenal and Extra-adrenal Paraganglioma: Practical Synopsis for Pathologists.

IF 5.1 2区医学 Q1 PATHOLOGY Advances In Anatomic Pathology Pub Date : 2023-01-01 DOI:10.1097/PAP.0000000000000365

Carl Christofer Juhlin, Ozgur Mete

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引用次数: 5

Abstract

Adrenal paraganglioma (or "pheochromocytoma") and extra-adrenal paraganglioma, collectively abbreviated PPGL, are rare but spectacular nonepithelial neuroendocrine neoplasms. These are the most inheritable neoplasia of all, with a metastatic potential in a varying degree. As of such, these lesions demand careful histologic, immunohistochemical, and genetic characterization to provide the clinical team with a detailed report taking into account the anticipated prognosis and risk of syndromic/inherited disease. While no histologic algorithm, immunohistochemical biomarker, or molecular aberration single-handedly can identify potentially lethal cases upfront, the combined analysis of various risk parameters may stratify PPGL patients more stringently than previously. Moreover, the novel 2022 WHO Classification of Endocrine and Neuroendocrine Tumors also brings some new concepts into play, not least the reclassification of special neuroendocrine neoplasms (cauda equina neuroendocrine tumor and composite gangliocytoma/neuroma-neuroendocrine tumor) previously thought to belong to the spectrum of PPGL. This review focuses on updated key diagnostic and prognostic concepts that will aid when facing this rather enigmatic tumor entity in clinical practice.

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肾上腺和肾上腺外副神经节瘤的研究进展:病理学家实用指南。

肾上腺副神经节瘤(或“嗜铬细胞瘤”)和肾上腺外副神经节瘤，统称PPGL，是罕见但壮观的非上皮性神经内分泌肿瘤。这些是所有肿瘤中最具遗传性的，具有不同程度的转移潜力。因此，这些病变需要仔细的组织学、免疫组织化学和遗传学特征，以便为临床团队提供详细的报告，同时考虑到预期的预后和综合征/遗传性疾病的风险。虽然没有任何组织学算法、免疫组织化学生物标志物或分子畸变可以单独识别潜在的致命病例，但各种风险参数的联合分析可能比以前更严格地对PPGL患者进行分层。此外，新的2022年WHO内分泌和神经内分泌肿瘤分类也带来了一些新的概念，尤其是对以前认为属于PPGL谱系的特殊神经内分泌肿瘤(马尾神经内分泌肿瘤和复合神经节细胞瘤/神经-神经内分泌肿瘤)进行了重新分类。这篇综述的重点是更新的关键诊断和预后概念，将有助于在临床实践中面对这种相当神秘的肿瘤实体。

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来源期刊

Advances In Anatomic Pathology 医学-病理学

CiteScore

10.30

自引率

3.00%

发文量

审稿时长

>12 weeks

期刊介绍： Advances in Anatomic Pathology provides targeted coverage of the key developments in anatomic and surgical pathology. It covers subjects ranging from basic morphology to the most advanced molecular biology techniques. The journal selects and efficiently communicates the most important information from recent world literature and offers invaluable assistance in managing the increasing flow of information in pathology.