Majumder Monica, Chiang Cherie, Kong Grace, Michael Michael, Sachithanandan Nirupa, Boehm Emma
Endocrinology and Diabetes Department, St Vincent's Hospital, Melbourne, Victoria, Australia.
Department of Medicine, The University of Melbourne, Fitzroy, Victoria, Australia.
Clin Endocrinol (Oxf). 2025 Jul;103(1):21-35. doi: 10.1111/cen.15235. Epub 2025 Mar 24.
Managing gastrointestinal symptoms in patients with phaeochromocytoma and paraganglioma (PPGL) is challenging due to the risk of catecholaminergic crisis with many commonly prescribed medications, especially in functional tumours. We reviewed gastrointestinal symptom management and outcomes in PPGL patients at our centre and developed recommendations based on a literature review and our experience.
DESIGN, PATIENTS, MEASUREMENT: A single-centre retrospective analysis of the management of gastrointestinal symptoms in patients with PPGL between 2019 and 2024 was completed. A literature review of gastrointestinal manifestations in PPGL was undertaken.
Twenty-four individuals with PPGL admitted for radionuclide therapy, chemotherapy, surgery or other medical illness were included. Eighteen (75%) had metastatic disease. Fifty administration events of antiemetics for nausea or vomiting occurred. Two patients had acute colonic pseudo-obstruction. Dopamine antagonists (metoclopramide) and corticosteroids (dexamethasone) were administered to 10 and 9 patients, respectively, the majority of whom were alpha-blocked (n = 7) or had a dopaminergic/biochemically silent phenotype (n = 10). A patient with noradrenergic PPGL experienced a hypertensive episode following high-dose dexamethasone. No patients with biochemically negative/dopaminergic phenotypes or on alpha blockade experienced an antiemetic-related adverse event. Published evidence of dopamine antagonists and corticosteroids precipitating catecholaminergic crisis was mostly limited to case reports. While low-risk antiemetics (serotonin, histamine or neurokinin antagonists) are preferable, we found higher-risk antiemetics (dexamethasone and metoclopramide) can be cautiously administered in patients with a biochemically negative/dopaminergic phenotype or in those on adequate alpha blockade. Limited case reports demonstrated anti-cholinergic agents were beneficial for the management of acute colonic pseudo-obstruction.
Optimal management of gastrointestinal symptoms in PPGL should consider disease characteristics such as primary location, secretory profile, alpha blockade and medication profile.
由于许多常用药物存在引发儿茶酚胺能危象的风险，尤其是在功能性肿瘤中，因此管理嗜铬细胞瘤和副神经节瘤（PPGL）患者的胃肠道症状具有挑战性。我们回顾了本中心PPGL患者的胃肠道症状管理及结果，并基于文献综述和我们的经验制定了建议。
设计、患者、测量：完成了一项对2019年至2024年间PPGL患者胃肠道症状管理的单中心回顾性分析。对PPGL的胃肠道表现进行了文献综述。
纳入了24例因放射性核素治疗、化疗、手术或其他疾病入院的PPGL患者。18例（75%）有转移性疾病。发生了50次用于恶心或呕吐的止吐药给药事件。2例患者出现急性结肠假性梗阻。分别有10例和9例患者使用了多巴胺拮抗剂（甲氧氯普胺）和皮质类固醇（地塞米松），其中大多数患者已接受α受体阻滞剂治疗（n = 7）或具有多巴胺能/生化无活性表型（n = 10）。一名去甲肾上腺素能PPGL患者在大剂量地塞米松治疗后发生高血压发作。没有生化阴性/多巴胺能表型的患者或接受α受体阻滞剂治疗的患者发生与止吐药相关的不良事件。关于多巴胺拮抗剂和皮质类固醇引发儿茶酚胺能危象的已发表证据大多限于病例报告。虽然低风险止吐药（5-羟色胺、组胺或神经激肽拮抗剂）更可取，但我们发现高风险止吐药（地塞米松和甲氧氯普胺）可在生化阴性/多巴胺能表型的患者或接受充分α受体阻滞剂治疗的患者中谨慎使用。有限的病例报告表明抗胆碱能药物对急性结肠假性梗阻的管理有益。
PPGL患者胃肠道症状的最佳管理应考虑疾病特征，如原发部位、分泌特征、α受体阻滞剂治疗情况和用药情况。
