Mamilla Divya, Araque Katherine A, Brofferio Alessandra, Gonzales Melissa K, Sullivan James N, Nilubol Naris, Pacak Karel
Section on Medical Neuroendocrinology, Eunice Kennedy Shriver, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA.
Adult Endocrinology Department, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, MD 20892, USA.
Cancers (Basel). 2019 Jul 3;11(7):936. doi: 10.3390/cancers11070936.
Pheochromocytomas and paragangliomas (PPGLs) are rare catecholamine-secreting neuroendocrine tumors of the adrenal medulla and sympathetic/parasympathetic ganglion cells, respectively. Excessive release of catecholamines leads to episodic symptoms and signs of PPGL, which include hypertension, headache, palpitations, and diaphoresis. Intraoperatively, large amounts of catecholamines are released into the bloodstream through handling and manipulation of the tumor(s). In contrast, there could also be an abrupt decline in catecholamine levels after tumor resection. Because of such binary manifestations of PPGL, patients may develop perplexing and substantially devastating cardiovascular complications during the perioperative period. These complications include hypertension, hypotension, arrhythmias, myocardial infarction, heart failure, and cerebrovascular accident. Other complications seen in the postoperative period include fever, hypoglycemia, cortisol deficiency, urinary retention, etc. In the interest of safe patient care, such emergencies require precise diagnosis and treatment. Surgeons, anesthesiologists, and intensivists must be aware of the clinical manifestations and complications associated with a sudden increase or decrease in catecholamine levels and should work closely together to be able to provide appropriate management to minimize morbidity and mortality associated with PPGLs.
嗜铬细胞瘤和副神经节瘤（PPGLs）分别是肾上腺髓质和交感/副交感神经节细胞分泌儿茶酚胺的罕见神经内分泌肿瘤。儿茶酚胺的过度释放会导致PPGL的发作性症状和体征，包括高血压、头痛、心悸和多汗。术中，通过对肿瘤的处理和操作，大量儿茶酚胺会释放到血液中。相反，肿瘤切除后儿茶酚胺水平也可能突然下降。由于PPGL的这种双重表现，患者在围手术期可能会出现令人困惑且极具破坏性的心血管并发症。这些并发症包括高血压、低血压、心律失常、心肌梗死、心力衰竭和脑血管意外。术后还可见其他并发症，如发热、低血糖、皮质醇缺乏、尿潴留等。为了患者的安全护理，此类紧急情况需要精确的诊断和治疗。外科医生、麻醉师和重症监护医生必须了解与儿茶酚胺水平突然升高或降低相关的临床表现和并发症，并应密切合作，以便能够提供适当的管理，将与PPGLs相关的发病率和死亡率降至最低。
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