Porcaro Antonio Benito, Novella Giovanni, Ficarra Vincenzo, D'Amico Antonio, Antoniolli Stefano Zecchini, Curti Pierpaolo
Department of Urology, University Hospital, Verona, Italy.
Arch Ital Urol Androl. 2003 Dec;75(4):217-25.
Adrenal masses discovered by imaging techniques for reasons unrelated to adrenal diseases are called incidentalomas with pheochromocytomas accounting for about 20%. The aim of this study was to report on our experience of 5 patients operated for incidentally discovered adrenal pheochromocytoma and update the literature concerning this subject.
From September 1976 to December 2000 we operated on 28 for adrenal incidentaloma, and pheochromocytoma was detected in 5 patients (19%). Average age was 61.4 years (range 54-65). All patients underwent adrenal hormonal as well as imaging investigation. Preoperative care for pheochromocytoma was performed in 3 patients. All patients were followed up.
Average tumor diameter, as detected by CT, was 5.5 cm (range 3-10). Urinary 24/h excretion of VMA resulted elevated in 2 cases (40%). Two patients during adrenalectomy developed paroxysmal hypertension which was readily controlled by intravenous administration of nitroprussiate and trandate. Postoperative outcome was uneventful. Average tumor size, as detected by pathology, resulted 8.9 cm (range 3-12). Mean follow-up was 60.4 months (range 32-122) and overall survival rate was 60%. Three patients are alive and disease-free and 2 died after a follow-up of 32 and 36 months for reasons unrelated to the primary disease.
Every incidentally discovered adrenal mass should be investigated for pheochromocytoma since accounting for about 20%. Undetected pheochromocytomas undergoing surgery without preoperative care for avoiding cardiovascular disturbances confer a high risk for morbidity and mortality, especially in pregnancy and childhood. Nonfunctional and small tumors sized 3 cm or lesser may be closely followed up by imaging and hormonal investigation. Surgery is indicated when tumors are functional or larger than 3 cm. Actually, laparoscopy is the new gold-standard in treating adrenal pheochromocytomas. Adrenal sparing surgery as well as autotransplantation of adrenal tissue are both effective and safe in treating patients with bilateral pheochromocytomas, thus improving quality of life since avoiding lifelong steroid dependency. When managed appropriately, the outlook for pheochromocytoma is excellent with a free survival rate of 92% and 80% at 5 and 10 years respectively. Unfortunately, malignant pheochromocytomas are still difficult to treat.
通过成像技术发现的与肾上腺疾病无关的肾上腺肿块被称为偶发瘤，其中嗜铬细胞瘤约占20%。本研究的目的是报告我们对5例因偶然发现的肾上腺嗜铬细胞瘤而接受手术治疗的患者的经验，并更新有关该主题的文献。
1976年9月至2000年12月，我们对28例肾上腺偶发瘤患者进行了手术，其中5例（19%）检测出嗜铬细胞瘤。平均年龄为61.4岁（范围54 - 65岁）。所有患者均接受了肾上腺激素及影像学检查。3例患者进行了嗜铬细胞瘤的术前护理。所有患者均进行了随访。
CT检测到的肿瘤平均直径为5.5厘米（范围3 - 10厘米）。24小时尿香草扁桃酸（VMA）排泄量在2例（40%）患者中升高。2例患者在肾上腺切除术中出现阵发性高血压，通过静脉注射硝普钠和三甲噻方可迅速控制。术后恢复顺利。病理检测到的肿瘤平均大小为8.9厘米（范围3 - 12厘米）。平均随访时间为60.4个月（范围32 - 122个月），总生存率为60%。3例患者存活且无疾病，2例在随访32个月和36个月后因与原发性疾病无关的原因死亡。
由于嗜铬细胞瘤约占偶然发现的肾上腺肿块的20%，因此应对每一个偶然发现的肾上腺肿块进行嗜铬细胞瘤的检查。未检测到的嗜铬细胞瘤在未进行术前护理以避免心血管紊乱的情况下进行手术，会带来较高的发病和死亡风险，尤其是在妊娠和儿童期。对于无功能且大小为3厘米或更小的肿瘤，可通过影像学和激素检查进行密切随访。当肿瘤有功能或大于3厘米时则需手术治疗。实际上，腹腔镜手术是治疗肾上腺嗜铬细胞瘤的新金标准。肾上腺保留手术以及肾上腺组织自体移植在治疗双侧嗜铬细胞瘤患者中均有效且安全，从而避免了终身依赖类固醇，提高了生活质量。如果处理得当，嗜铬细胞瘤的预后极佳，5年和10年的无瘤生存率分别为92%和80%。不幸的是,恶性嗜铬细胞瘤仍然难以治疗。
