Lenders Jacques W M, Eisenhofer Graeme, Mannelli Massimo, Pacak Karel
Department of Internal Medicine, Division of General Internal Medicine, Radboud University Nijmegen Medical Centre, Nijmegen 6525GA, Netherlands.
Lancet. 2005;366(9486):665-75. doi: 10.1016/S0140-6736(05)67139-5.
Phaeochromocytomas are rare neuroendocrine tumours with a highly variable clinical presentation but most commonly presenting with episodes of headaches, sweating, palpitations, and hypertension. The serious and potentially lethal cardiovascular complications of these tumours are due to the potent effects of secreted catecholamines. Biochemical testing for phaeochromocytoma is indicated not only in symptomatic patients, but also in patients with adrenal incidentalomas or identified genetic predispositions (eg, multiple endocrine neoplasia type 2, von Hippel-Lindau syndrome, neurofibromatosis type 1, and mutations of the succinate dehydrogenase genes). Imaging techniques such as CT or MRI and functional ligands such as (123)I-MIBG are used to localise biochemically proven tumours. After the use of appropriate preoperative treatment to block the effects of secreted catecholamines, laparoscopic tumour removal is the preferred procedure. If removal of phaeochromocytoma is timely, prognosis is excellent. However, prognosis is poor in patients with metastases, which especially occur in patients with large, extra-adrenal tumours.
嗜铬细胞瘤是罕见的神经内分泌肿瘤，临床表现高度多变，但最常见的症状是头痛、出汗、心悸和高血压发作。这些肿瘤严重且可能致命的心血管并发症是由于分泌的儿茶酚胺的强效作用所致。嗜铬细胞瘤的生化检测不仅适用于有症状的患者，也适用于肾上腺偶发瘤患者或已确定有遗传易感性的患者（如2型多发性内分泌腺瘤、冯·希佩尔-林道综合征、1型神经纤维瘤病以及琥珀酸脱氢酶基因突变）。CT或MRI等成像技术以及（123）I-间碘苄胍等功能性配体用于定位经生化证实的肿瘤。在使用适当的术前治疗来阻断分泌的儿茶酚胺的作用后，腹腔镜肿瘤切除术是首选的手术方式。如果嗜铬细胞瘤切除及时，预后良好。然而，有转移的患者预后较差，转移尤其发生在肿瘤较大的肾上腺外肿瘤患者中。
