Endocrine Unit, Department of Medicine, University Malaya Medical Centre, Lembah Pantai, Kuala Lumpur 59100, Malaysia.
Singapore Med J. 2010 May;51(5):e89-93.
Most functional phaeochromocytomas/paragangliomas produce noradrenaline and/or adrenaline. Those that produce dopamine are rare. We describe the distinguishing clinical features of dopamine-secreting phaeochromocytomas and paragangliomas from those that secrete noradrenaline/adrenaline and the impact on their management. We present a case of a dopamine-secreting paraganglioma from our institution and review 14 case reports of dopamine-secreting phaeochromocytomas/paragangliomas published between 1984 and 2008. As observed in the literature, 80% of the tumours were extra-adrenal. Most patients presented with non-specific symptoms or mass effect without the classical presentation of catecholamine excess. The majority were diagnosed with urinary or plasma dopamine. Five patients had malignant tumours and 12 patients underwent surgical resection of the primary tumours. Unlike noradrenaline/adrenaline-secreting phaeochromocytomas/paragangliomas, dopamine-secreting tumours lack a classical presentation, are extra-adrenal and have a higher malignant potential. A routine inclusion of urinary or plasma dopamine as part of catecholamine screening in all suspected phaeochromocytomas and paragangliomas is recommended.
大多数功能性嗜铬细胞瘤/副神经节瘤产生去甲肾上腺素和/或肾上腺素。那些产生多巴胺的是罕见的。我们描述了多巴胺分泌性嗜铬细胞瘤和副神经节瘤与分泌去甲肾上腺素/肾上腺素的那些的区别临床特征及其对其管理的影响。我们介绍了一个来自我们机构的多巴胺分泌性副神经节瘤的病例，并回顾了 1984 年至 2008 年间发表的 14 例多巴胺分泌性嗜铬细胞瘤/副神经节瘤的病例报告。如文献中观察到的，80%的肿瘤是肾上腺外的。大多数患者表现为非特异性症状或肿块效应，而没有儿茶酚胺过多的典型表现。大多数患者通过尿液或血浆多巴胺进行诊断。5 名患者患有恶性肿瘤，12 名患者接受了原发性肿瘤的手术切除。与去甲肾上腺素/肾上腺素分泌性嗜铬细胞瘤/副神经节瘤不同，多巴胺分泌性肿瘤缺乏典型表现，是肾上腺外的，恶性潜能更高。建议在所有疑似嗜铬细胞瘤和副神经节瘤的儿茶酚胺筛查中常规包括尿液或血浆多巴胺。
