嗜铬细胞瘤和副神经节瘤（PPGL）是分泌儿茶酚胺的罕见肿瘤，起源于肾上腺髓质或肾上腺外交感神经节。这些肿瘤分泌肾上腺素和去甲肾上腺素，但副神经节瘤通常只产生去甲肾上腺素，因为缺乏苯乙醇胺 N-甲基转移酶 (PNMT) 表达。复合副神经节瘤是由多种类型的神经母细胞组成的复杂肿瘤，极为罕见。我们介绍了一名 46 岁女性的病例，她的儿茶酚胺谱不典型，她在术前被诊断为嗜铬细胞瘤。然而术后病理显示，患者患有肾上腺外副神经节瘤并伴有神经节神经瘤，从而诊断为复合肿瘤。有趣的是，PNMT 在副神经节瘤和神经节神经瘤中都有表达。此外，我们回顾了报道的复合性副神经节瘤，并将其与复合性嗜铬细胞瘤的临床特征进行了比较。我们还讨论了复合性副神经节瘤病因学的各个方面以及 PNMT 在肿瘤中表达的机制。

Pheochromocytomas and paragangliomas (PPGLs) are rare tumors that secrete catecholamines and arise from the adrenal medulla or extra-adrenal sympathetic ganglia. These tumors secrete adrenaline and noradrenaline, but paragangliomas usually produce only noradrenaline because of the lack of phenylethanolamine N-methyltransferase (PNMT) expression. Composite paragangliomas, which are complex tumors consisting of multiple types of neuroblastic cells, are extremely rare. We present the case of a 46-year-old woman with an atypical catecholamine profile who was preoperatively diagnosed with pheochromocytoma. However, postoperative pathology revealed that the patient had an extra-adrenal paraganglioma accompanied by a ganglioneuroma, which led to the diagnosis of a composite tumor. Interestingly, PNMT is expressed in both paragangliomas and ganglioneuromas. In addition, we reviewed reported composite paragangliomas and compared their clinical features with those of composite pheochromocytomas. We also discuss various aspects of the etiology of composite paragangliomas and the mechanism by which PNMT is expressed in tumors.