Mannelli M, Ianni L, Cilotti A, Conti A
Department of Clinical Physiopathology, Endocrine Unit, University of Florence, Viale Pieraccini 6, 50139 Florence, Italy.
Eur J Endocrinol. 1999 Dec;141(6):619-24. doi: 10.1530/eje.0.1410619.
To conduct an epidemiological study on pheochromocytoma in Italy.
Data on 284 patients with pheochromocytoma observed between 1978 and 1997 were collected from 18 Italian centers through a questionnaire reporting epidemiological, clinical, laboratory, radiological and surgical data.
53.6% of the patients were females and 46.4% were males. Thirty-two tumors were discovered as incidental adrenal masses. The most frequent referred symptoms were palpitations (58.1%), headache (51.9%), sweating (48. 8%) and anxiety (35.3%). Their association was present only in 15.5% of patients. Paroxysmal symptoms were reported in 67.1% and hypertensive crises in 59.7% of patients. Normal blood pressure (systolic and diastolic) was present both in the supine and upright positions in 21.1% of patients. Among laboratory assays, urinary vanylmandelic acid (VMA) was the most widely used (58.1%) and was the least sensitive (25% of false negative results). Basal plasma catecholamines were found to be normal in 11.3% of patients but were always elevated when sampled during a hypertensive paroxysm. A clonidine suppression test was performed in 38 patients with no adverse side effects. It gave a false negative response in 2 patients. A glucagon test was performed in 21 patients. It was interrupted for acute hypertension in 52.4% of patients. Only 5/21 patients were normotensive and had normal basal plasma catecholamines. In these patients the test gave a positive response in four (80%). CT (79.6%) and I-MIBG scintigraphy (68.5%) were the most widely used methods for tumor localization. CT sensitivity was 98.9% for intra-adrenal and 90.9% for extra-adrenal tumors. MIBG sensitivity was 88.5%. In the 263 patients who underwent surgery, the tumor was intra-adrenal in 89.4%, extra-adrenal in 8.5%, intra- and extra-adrenal in 2.1%, and bilateral in 11.0% of patients. Malignancy was reported in 9.9% of cases. Surgery caused remission of hypertension in 59.3%, improvement in 26.8%, and no changes in 13. 9% of patients. In the last group the interval between initial symptoms and diagnosis was significantly longer.
The present study confirms that the clinical presentation of pheochromocytoma is variable and aspecific. Normotension is often present and often the tumor is discovered incidentally. An indication for the routine use of screening methods more sensitive than urinary VMA is strongly suggested. The clonidine test was found to be safe and should be preferred to the glucagon test which has to be restricted to very selected patients. CT and MIBG scintigraphy are almost always successful in localizing the tumor. Reversal of hypertension by surgery seems to depend on an early diagnosis.
在意大利开展一项嗜铬细胞瘤的流行病学研究。
通过一份报告流行病学、临床、实验室、放射学及手术数据的问卷，从意大利18个中心收集了1978年至1997年间观察到的284例嗜铬细胞瘤患者的数据。
53.6%的患者为女性，46.4%为男性。32个肿瘤是作为肾上腺意外肿块被发现的。最常见的症状是心悸（58.1%）、头痛（51.9%）、出汗（48.8%）和焦虑（35.3%）。这些症状仅在15.5%的患者中同时出现。67.1%的患者有阵发性症状，59.7%的患者有高血压危象。21.1%的患者仰卧位和立位血压均正常。在实验室检测中，尿香草扁桃酸（VMA）使用最为广泛（58.1%），但敏感性最低（假阴性结果占25%）。11.3%的患者基础血浆儿茶酚胺正常，但在高血压发作时采样时总是升高。对38例患者进行了可乐定抑制试验，未出现不良反应。2例患者出现假阴性反应。对21例患者进行了胰高血糖素试验。52.4%的患者因急性高血压试验中断。仅5/21例患者血压正常且基础血浆儿茶酚胺正常。在这些患者中，试验在4例（80%）中呈阳性反应。CT（79.6%）和I-MIBG闪烁扫描（68.5%）是最广泛用于肿瘤定位的方法。CT对肾上腺内肿瘤的敏感性为98.9%，对肾上腺外肿瘤为90.9%。MIBG敏感性为88.5%。在接受手术的263例患者中，89.4%的肿瘤位于肾上腺内，8.5%位于肾上腺外，2.1%为肾上腺内外均有，11.0%为双侧。9.9%的病例报告为恶性。手术使59.3%的患者高血压缓解，26.8%的患者病情改善，13.9%的患者无变化。在最后一组中，初始症状与诊断之间的间隔明显更长。
本研究证实嗜铬细胞瘤的临床表现多样且缺乏特异性。常出现血压正常情况，且肿瘤常为偶然发现。强烈建议常规使用比尿VMA更敏感的筛查方法。发现可乐定试验安全，应优先于胰高血糖素试验，后者应仅限于非常特定的患者。CT和MIBG闪烁扫描几乎总能成功定位肿瘤。手术使高血压逆转似乎取决于早期诊断。
