Pomares F J, Cañas R, Rodriguez J M, Hernandez A M, Parrilla P, Tebar F J
Department of Endocrinology, Hospital Universitario Virgen de la Arrixaca, Murcia, Spain.
Clin Endocrinol (Oxf). 1998 Feb;48(2):195-200. doi: 10.1046/j.1365-2265.1998.3751208.x.
Phaeochromocytoma may be either sporadic or part of a familial cancer syndrome. We have investigated whether there are differences between sporadic and MEN 2A phaeochromocytomas.
A retrospective study. We analysed age at presentation, sex, mode of presentation, clinical data, laboratory and imaging techniques, treatment, pathology and follow-up.
Forty-six patients diagnosed with phaeochromocytoma between 1979 and 1995 (23 sporadic and 23 familial) at the Hospital Universitario Virgen de la Arrixaca (Murcia, Spain), a tertiary referral centre.
Mean age at presentation was 47 +/- 16 years for sporadic and 38 +/- 11 years for familial phaeochromocytoma (P < 0.05). The most common clinical feature and the presenting feature in cases of isolated phaeochromocytoma was hypertension. Of the MEN 2A patients, 52% were asymptomatic at diagnosis and only 35% presented with hypertension. Levels of adrenaline, noradrenaline, dopamine and metanephrines in a 24-hour urine connection confirmed the diagnosis in 95% of the isolated and 91% of the familial phaeochromocytomas. Computed tomography located 100% of the sporadic and 76% of the familial phaeochromocytomas (P < 0.02). All the MEN 2A phaeochromocytomas were bilateral compared with none of the sporadic phaeochromocytomas (P < 0.001). All the patients with familial phaeochromocytoma underwent bilateral adrenalectomy. The patients with sporadic phaeochromocytoma had the affected gland or the tumour, if extraadrenal, removed.
In comparing sporadic and MEN 2A phaeochromocytoma we found differences in age at presentation, mode of presentation, clinical data, duration of clinical features, imaging findings bilaterality and type of surgical treatment required.
嗜铬细胞瘤可以是散发性的，也可以是家族性癌症综合征的一部分。我们研究了散发性嗜铬细胞瘤与MEN 2A嗜铬细胞瘤之间是否存在差异。
一项回顾性研究。我们分析了就诊年龄、性别、就诊方式、临床数据、实验室及影像学检查技术、治疗、病理及随访情况。
1979年至1995年间在西班牙穆尔西亚的阿利克斯卡圣母大学医院（一家三级转诊中心）被诊断为嗜铬细胞瘤的46例患者（23例散发性和23例家族性）。
散发性嗜铬细胞瘤的平均就诊年龄为47±16岁，家族性嗜铬细胞瘤为38±11岁（P<0.05）。孤立性嗜铬细胞瘤最常见的临床特征及就诊表现为高血压。在MEN 2A患者中，52%在诊断时无症状，仅有35%表现为高血压。24小时尿儿茶酚胺及其代谢产物水平检测确诊了95%的孤立性嗜铬细胞瘤和91%的家族性嗜铬细胞瘤。计算机断层扫描定位了100%的散发性嗜铬细胞瘤和76%的家族性嗜铬细胞瘤（P<0.02）。所有MEN 2A嗜铬细胞瘤均为双侧性，而散发性嗜铬细胞瘤无一例为双侧性（P<0.001）。所有家族性嗜铬细胞瘤患者均接受了双侧肾上腺切除术。散发性嗜铬细胞瘤患者则切除了患侧肾上腺或肿瘤（若为肾上腺外肿瘤）。
比较散发性和MEN 2A嗜铬细胞瘤时，我们发现两者在就诊年龄、就诊方式、临床数据、临床特征持续时间、影像学表现、双侧性及所需手术治疗类型方面存在差异。
