Risk Stratification on Pheochromocytoma and Paraganglioma from Laboratory and Clinical Medicine.
Risk Stratification on Pheochromocytoma and Paraganglioma from Laboratory and Clinical Medicine.
复制标题
实验室和临床医学的嗜铬细胞瘤和paraganglioma的风险分层。
DOI：
10.3390/jcm7090242
复制
发表时间：
2018-08-27
影响因子：
3.9
通讯作者：
Naruse M
中科院分区：
文献类型：
--
作者：
Kimura N;Takekoshi K;Naruse M
嗜铬细胞瘤（PCC）和交感神经副神经节瘤（PGL）是罕见的神经内分泌肿瘤，其特征是肾上腺髓质和肾上腺外副神经节产生儿茶酚胺。PCC和PGL（PPGL）伴转移者称为恶性PPGL。然而，“良性”和“恶性”PPGL之间的区别一直存在争议。目前，所有PPGL都被认为具有一定的转移潜力，并被WHO内分泌器官分类（2017年，第4版）指定为恶性肿瘤（ICD-0/3）。因此，先前的良性和恶性PPGL类别已被删除，有利于风险分层方法。肾上腺嗜铬细胞瘤和副神经节瘤分级（GAPP）是预测转移和患者预后的危险分层工具。至少30%的PPGL是遗传性的，其中20个基因被鉴定，基因型-表型相关性被阐明。在这些基因中，VHL、RET和NF 1已被充分研究，并且是双侧PCC的主要原因。此外，琥珀酸脱氢酶基因亚基SDHB和SDHD突变与肾上腺外部位、年龄小、肿瘤多发、转移和预后差密切相关。通过儿茶酚胺表型和分子谱进行的疾病分层与通过GAPP进行的组织学分级相关。应根据临床、生化、分子和病理学数据全面了解PPGL，以便患者护理。并附有病理诊断流程图。
Pheochromocytoma (PCC) and sympathetic paraganglioma (PGL) are rare neuroendocrine tumors characterized by catecholamine production in the adrenal medulla and extra-adrenal paraganglia. PCC and PGL (PPGL) with metastasis was termed malignant PPGL. However, the distinction between “benign” and “malignant” PPGLs has been debated. Currently, all PPGLs are believed to have some metastatic potential and are assigned malignant tumors (ICD-O/3) by the WHO Classification of Endocrine Organs (2017, 4th edition). Therefore, the previous categories benign and malignant PPGL have been eliminated in favor of risk stratification approach. The Grading of Adrenal Pheochromocytoma and Paraganglioma (GAPP) is a tool for risk stratification for predicting metastasis and the prognosis of patients. At least 30% of PPGLs are hereditary, with 20 genes identified and genotype-phenotype correlations clarified. Of these genes, VHL, RET and NF1 have been well investigated and are the primary cause of bilateral PCC. In addition, mutation of succinate dehydrogenase gene subunits SDHB and SDHD are strongly correlated with extra-adrenal location, younger age, multiple tumors, metastasis and poor prognosis. Disease stratification by catecholamine phenotype and molecular profiling correlates with histological grading by GAPP. PPGLs should be understood comprehensively based on clinical, biochemical, molecular and pathological data for patient care. A flow chart for pathological diagnosis is included.
登录
查看更多内容
影响因子：
3.7
作者：
Koh JM;Ahn SH;Kim H;Kim BJ;Sung TY;Kim YH;Hong SJ;Song DE;Lee SH
通讯作者：
Lee SH
DOI：
10.1007/bf03017207
发表时间：
2008-05-01
期刊：
CANADIAN JOURNAL OF ANAESTHESIA-JOURNAL CANADIEN D ANESTHESIE
影响因子：
--
作者：
Bellingham, Geoff A.;Dhir, Achal K.;Luke, Patrick P.
通讯作者：
Luke, Patrick P.
影响因子：
3.9
作者：
Kimura, Noriko;Takayanagi, Ryoichi;Naruse, Mitsuhide
通讯作者：
Naruse, Mitsuhide
HIF1Alpha调节环将缺氧和线粒体信号联系起来。
影响因子：
4.5
作者：
Dahia, PLM;Ross, KN;Wright, ME;Hayashida, CY;Santagata, S;Barontini, M;Kung, AL;Sanso, G;Powers, JF;Tischler, AS;Hodin, R;Heitritter, S;Moore, F;Dluhy, R;Sosa, JA;Ocal, IT;Benn, DE;Marsh, DJ;Robinson, BG;Schneider, K;Garber, J;Arum, SM;Korbonits, M;Grossman, A;Pigny, P;Toledo, SPA;Nosé, V;Li, C;Stiles, CD
通讯作者：
Stiles, CD
影响因子：
30.8
作者：
Comino-Mendez, Inaki;Gracia-Aznarez, Francisco J.;Cascon, Alberto
通讯作者：
Cascon, Alberto