Department of Medicine, Division of Endocrinology, Leiden University Medical Center, Albinusdreef 2, 2333 ZA, Leiden, The Netherlands.
Center for Endocrine Tumors Leiden (CETL), Leiden University Medical Center, Albinusdreef 2, 2333 ZA, Leiden, The Netherlands.
Hormones (Athens). 2021 Jun;20(2):395-403. doi: 10.1007/s42000-021-00274-6. Epub 2021 Feb 11.
Pheochromocytoma/paraganglioma (PPGL)-induced catecholamine crisis is a rare endocrine emergency leading to life-threatening hemodynamic instability causing end-organ damage or dysfunction. As it is associated with a significant mortality rate of approximately 15%, recognizing the signs and symptoms and making the appropriate diagnosis are critical. For this purpose, we report the clinical course of the crisis in four out of a total of six patients with a PPGL crisis from a cohort of 199 PPGL patients of a single tertiary referral center for PPGL patients in the Netherlands diagnosed between 2002 and 2020. Successful treatment of a PPGL crisis demands prompt diagnosis, vigorous pharmacological therapy, and emergency tumor removal if the patient continues to deteriorate.
嗜铬细胞瘤/副神经节瘤（PPGL）引起的儿茶酚胺危象是一种罕见的内分泌急症，可导致危及生命的血流动力学不稳定，从而导致终末器官损伤或功能障碍。由于其死亡率约为 15%，因此识别其体征和症状并做出适当的诊断至关重要。为此，我们报告了荷兰一家专门的 PPGL 转诊中心在 2002 年至 2020 年间诊断的 199 例 PPGL 患者队列中，6 例 PPGL 危象患者中的 4 例患者的危象临床经过。及时诊断、强有力的药物治疗以及在患者持续恶化的情况下进行紧急肿瘤切除，是成功治疗 PPGL 危象的关键。
