Anesthesia and Pheochromocytoma
Anesthesia and Pheochromocytoma
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DOI：
10.1097/aia.0b013e3181ff4db0
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发表时间：
2011-03-01
影响因子：
0.6
通讯作者：
Markan, Sandeep
中科院分区：
文献类型：
--
作者：
Jugovac, Izabela;Antapli, Mursel;Markan, Sandeep
嗜铬细胞瘤是一种起源于肾上腺髓质产生儿茶酚胺的嗜铬细胞的肿瘤，是一种肾上腺内的副神经节瘤。1肾上腺外交感神经（产生儿茶酚胺）和副交感神经（很少产生儿茶酚胺）副神经节的密切相关的肿瘤被归类为肾上腺外副神经节瘤。实际上，所有的嗜铬细胞瘤产生的儿茶酚胺在其含量相当大的变化，这取决于生物合成酶的表达。大多数肾上腺外嗜铬细胞瘤主要产生去甲肾上腺素（NE）。许多肾上腺肿瘤产生NE和肾上腺素（EPI），或少数主要产生EPI（例如，多发性内分泌腺瘤病2型和神经纤维瘤病1型）。嗜铬细胞瘤组织中的儿茶酚胺浓度是巨大的。显著的喷发导致称为“攻击”的儿茶酚胺风暴。2肾上腺素受体是大多数嗜铬细胞瘤患者体内过量儿茶酚胺的最终靶点。EPI和NE对不同器官和系统的α和β肾上腺素能受体的作用是相互重叠的，但作用方式不同。
DiscussionPheochromocytoma is a tumor arising from catecholamine-producing chromaffin cells in the adrenal medulla—an intra-adrenal paraganglioma. 1 Closely related tumors of extra-adrenal sympathetic (catecholamine producing) and parasympathetic (rarely catecholamine producing) paraganglia are classified as extra-adrenal paragangliomas. Practically, all pheochromocytomas produce catecholamines with a considerable variation in their content, depending on the expression of biosynthetic enzymes. Most extra-adrenal pheochromocytomas produce predominantly norepinephrine (NE). Many adrenal tumors produce either NE and epinephrine (EPI), or few rarely produce predominantly EPI (eg, in patients with multiple endocrine neoplasia type 2 and neurofibromatosis type 1). The concentrations of catecholamines in pheochromocytoma tissues are enormous. Significant eruptions result in a catecholamine storm called “attacks.” 2 The adrenoceptors are the final targets for catecholamines that are found in excess in most patients with pheochromocytoma. Both EPI and NE have overlapping but different effects on α and β adrenoceptors on various organs and systems.