Kopetschke Robert, Slisko Mario, Kilisli Aylin, Tuschy Ulrich, Wallaschofski Henri, Fassnacht Martin, Ventz Manfred, Beuschlein Felix, Reincke Martin, Reisch Nicole, Quinkler Marcus
Clinical Endocrinology, Charité Campus Mitte, Charité University Medicine Berlin, Charitéplatz 1, D 10117 Berlin, Germany.
Eur J Endocrinol. 2009 Aug;161(2):355-61. doi: 10.1530/EJE-09-0384. Epub 2009 Jun 4.
Adrenal and extra-adrenal phaeochromocytoma are chromaffin cell-derived tumours that are discovered due to classical symptom triad with headache, sweating and palpitations combined with persistent or paroxysmal hypertension. However, an increasing proportion of phaeochromocytoma seems to be discovered incidentally upon abdominal imaging.
To specify the exact circumstances of discovery of adrenal and extra-adrenal phaeochromocytoma.
Four German endocrine centres participated in this retrospective study. MEDICAL: records of 201 patients with adrenal and extra-adrenal phaeochromocytoma who were diagnosed between 1973 and 2007 were analyzed.
The typical triad of symptoms was found only in 10% of cases. Ten percent of patients presented were without clinical symptoms and 6.1% were normotensive. Documented blood pressure peaks occurred in 44.1% of cases. In 24 patients (12.2%), phaeochromocytoma was malignant. Before 1985, <10% of cases were incidentally discovered, whereas thereafter the frequency was >25% (29.4% of the total study population). Patients with incidentally detected phaeochromocytoma were significantly older (53.1+/-1.9 vs 47.0+/-1.3 years; P<0.05) and often had less blood pressure peaks (37.0 vs 70.7%; P<0.001) than patients in whom the diagnosis was suspected on clinical grounds. Of phaeochromocytomas 94.4% were intra-adrenal tumours, of which 12.9% were bilateral. Bilateral tumours were significantly smaller than unilateral tumours (36.6+/-14.7 vs 52.5+/-34.3 mm; P<0.05), whereas extra-adrenal tumours had a mean diameter of 52.6+/-28.7 mm.
Owing to better availability and accessibility of imaging procedures, the number of incidentally discovered phaeochromocytoma is increasing and reaches nearly 30% in our study population. Every adrenal incidentaloma should be investigated for the presence of phaeochromocytoma.
肾上腺嗜铬细胞瘤和肾上腺外嗜铬细胞瘤是起源于嗜铬细胞的肿瘤,因其具有头痛、出汗和心悸的典型症状三联征,并伴有持续性或阵发性高血压而被发现。然而,越来越多的嗜铬细胞瘤似乎是在腹部影像学检查时偶然发现的。
明确肾上腺嗜铬细胞瘤和肾上腺外嗜铬细胞瘤的具体发现情况。
四个德国内分泌中心参与了这项回顾性研究。医学:分析了1973年至2007年间诊断的201例肾上腺嗜铬细胞瘤和肾上腺外嗜铬细胞瘤患者的病历。
仅10%的病例出现典型症状三联征。10%的患者无临床症状,6.1%血压正常。44.1%的病例记录到血压峰值。24例患者(12.2%)的嗜铬细胞瘤为恶性。1985年前,偶然发现的病例不到10%,而此后这一比例超过25%(占研究总人群的29.4%)。偶然发现嗜铬细胞瘤的患者年龄显著较大(53.1±1.9岁对47.0±1.3岁;P<0.05),且血压峰值往往较少(37.0%对70.7%;P<0.001),而基于临床怀疑诊断的患者则相反。94.4%的嗜铬细胞瘤为肾上腺内肿瘤,其中12.9%为双侧肿瘤。双侧肿瘤明显小于单侧肿瘤(36.6±14.7mm对52.5±34.3mm;P<0.05),而肾上腺外肿瘤的平均直径为52.6±28.7mm。
由于影像学检查的可用性和可及性提高,偶然发现的嗜铬细胞瘤数量正在增加,在我们的研究人群中接近30%。每一例肾上腺意外瘤均应检查是否存在嗜铬细胞瘤。