巨大囊性嗜铬细胞瘤/副神经节瘤诊治研究进展

Research progress on diagnosis and treatment of giant cystic pheochromocytoma/paraganglioma

嗜铬细胞瘤并不常见,而巨大囊性嗜铬细胞瘤更为少见,是嗜铬细胞瘤的一个亚组,大部分因为肿瘤坏死和出血过程形成囊性,极少为真囊肿。因为大部分儿茶酚胺在肿瘤内代谢或储存,当肿瘤发生囊性退变时,儿茶酚胺生成减少或储存在胞膜下,不易释放入血,导致其缺乏典型嗜铬细胞瘤的常见症状,生化检查儿茶酚胺代谢产物阴性亦不少见,加之影像学表现多样,不易与其他腹腔或腹膜后占位鉴别,早期诊断极困难。本文就巨大囊性嗜铬细胞瘤的发生机制、临床表现、诊治等方面综述其进展,以加深对本病的认识,提高临床诊断的准确率。

Pheochromocytoma is not common,while giant cystic pheochromocytoma is even more rare.It is a subgroup of pheochromocytoma.Most of them are cystic due to tumor necrosis and hemorrhagic process,and rarely are true cysts.Because most of the catecholamines are metabolized or stored in the tumor,when the tumor undergoes cystic degeneration,the production of catecholamines is reduced or stored under the cell membrane,which is not easy to release into the blood,resulting in a lack of common symptoms of typical pheochromocytoma.Biochemical examination of catecholamines metabolites is more likely to be negative.In addition to the diverse imaging findings,it is not easy to differentiate from other abdominal or retroperitoneal space,and early diagnosis is extremely difficult.This article reviews the progress of giant cystic pheochromocytoma in terms of its mechanism,clinical manifestations,diagnosis and treatment,in order to deepen the understanding of the disease and improve the accuracy of clinical diagnosis.