Walther M M, Keiser H R, Linehan W M
Urologic Oncology Branch, DCS/NCI/NIH, Bethesda, MD 20892-1501, USA.
World J Urol. 1999 Feb;17(1):35-9. doi: 10.1007/s003450050102.
Pheochromocytoma is a catecholamine-producing tumor of the sympathetic nervous system. Signs and symptoms are generally related to catecholamine excess; these include hypertension, sweating, palpitatione, headaches, and anxiety attacks. Abdominal imaging and 24-h urine collection for catecholamines are usually be sufficient for diagnosis. Catecholamine blockade with phenoxybenzamine and metyrosine generally ameliorates symptoms and is necessary to prevent hypertensive crisis during surgery. Standard treatment is laparoscopic adrenalectomy, although partial adrenalectomy is gaining enthusiastic support in familial forms of pheochromocytoma. Pheochromocytomas have been estimated to be present in approximately 0.3% of patients undergoing evaluation for secondary causes of hypertension [41]. Pheochromocytomas are usually curable if diagnosed and treated properly, but they can be fatal if they are not diagnosed or are managed inappropriately. Autopsy series suggest that many pheochromocytomas are not clinically suspected and that the undiagnosed tumor can be associated with morbid consequences [42].
嗜铬细胞瘤是一种起源于交感神经系统的能产生儿茶酚胺的肿瘤。其体征和症状通常与儿茶酚胺过量有关,包括高血压、出汗、心悸、头痛和焦虑发作。腹部影像学检查以及收集24小时尿儿茶酚胺通常足以确诊。使用酚苄明和甲酪氨酸进行儿茶酚胺阻滞通常可改善症状,并且对于预防手术期间的高血压危象是必要的。标准治疗方法是腹腔镜肾上腺切除术,尽管在家族性嗜铬细胞瘤中,部分肾上腺切除术正获得广泛支持。据估计,在因继发性高血压病因接受评估的患者中,约0.3%存在嗜铬细胞瘤[41]。如果诊断和治疗得当,嗜铬细胞瘤通常是可治愈的,但如果未被诊断或处理不当,可能会致命。尸检系列研究表明,许多嗜铬细胞瘤在临床上未被怀疑,未被诊断的肿瘤可能会导致不良后果[42]。