Department of Surgical Oncology, University of Texas MD Anderson Cancer Center, Houston, TX 77030-4008, USA.
J Am Coll Surg. 2013 Feb;216(2):280-9. doi: 10.1016/j.jamcollsurg.2012.10.012.
The ideal surgical management of hereditary pheochromocytomas includes planning for a potential metachronous bilateral presentation and the possibility of lifelong steroid dependence if bilateral adrenalectomy is needed. An intact and viable cortical remnant after bilateral pheochromocytoma resection can eliminate the necessity for steroid dependency, but can increase the risk of pheochromocytoma recurrence.
We retrospectively reviewed outcomes of all patients with a diagnosis of hereditary pheochromocytomas treated at our tertiary cancer institution from 1962-2011, with subset analysis of patients undergoing a cortical-sparing procedure in the setting of bilateral adrenalectomy.
Of the ninety-six patients who underwent adrenalectomy for hereditary pheochromocytomas, 47 presented with bilateral disease. In 15 of the 49 patients (30%) who originally underwent unilateral adrenalectomy, pheochromocytoma developed in the contralateral gland at a median of 8.2 years (range 1 to 20 years) after the initial diagnosis. There were 4 recurrences in 55 cortical-sparing remnants (7%) and 3 recurrences in the adrenal bed after 101 intended total adrenal resections (3%) (p = 0.24). Total bilateral adrenalectomy was performed in 25 patients and acute adrenal insufficiency developed in 5 (20%) of those patients. An intended cortical-sparing adrenalectomy was performed in 39 patients and acute adrenal insufficiency developed in 1 (3%). Of these patients with adequate follow-up, 21 of 27 (78%) were steroid independent at 3-year follow-up. Sex, median age, adrenal vein preservation, metachronous adrenal resection, and bilateral cortical-sparing procedures did not predict steroid independence at 3 years.
Cortical-sparing adrenalectomy avoids long-term corticosteroid dependence in the majority of patients with hereditary pheochromocytoma with minimal risk of acute adrenal insufficiency. Recurrence occurs in approximately 7% of adrenal remnants.
遗传性嗜铬细胞瘤的理想手术治疗包括计划潜在的双侧病变，并在需要双侧肾上腺切除术时考虑终生类固醇依赖的可能性。双侧嗜铬细胞瘤切除后完整且有活力的皮质残肢可以消除类固醇依赖的必要性，但会增加嗜铬细胞瘤复发的风险。
我们回顾性分析了 1962 年至 2011 年间在我们的三级癌症机构治疗的遗传性嗜铬细胞瘤患者的所有结果，并对双侧肾上腺切除术时进行皮质保留手术的患者进行了亚组分析。
在因遗传性嗜铬细胞瘤接受肾上腺切除术的 96 名患者中，47 名患者双侧病变。在最初接受单侧肾上腺切除术的 49 名患者中的 15 名（30%）中，在初次诊断后中位数为 8.2 年（范围 1 至 20 年）的时间里，对侧腺体发生了嗜铬细胞瘤。在 55 个皮质保留残肢中发生了 4 次复发（7%），在 101 个预期的全肾上腺切除术后发生了 3 次复发（3%）（p = 0.24）。25 名患者接受了双侧肾上腺全切除术，其中 5 名（20%）发生急性肾上腺功能不全。39 名患者接受了预期的皮质保留肾上腺切除术，其中 1 名（3%）发生急性肾上腺功能不全。在有足够随访的这些患者中，27 名中的 21 名（78%）在 3 年随访时无需使用类固醇。性别、中位年龄、肾上腺静脉保留、双侧病变的肾上腺切除术和皮质保留手术在 3 年内均不能预测类固醇的独立性。
皮质保留肾上腺切除术可避免大多数遗传性嗜铬细胞瘤患者长期使用皮质类固醇，且急性肾上腺功能不全的风险极小。肾上腺残肢的复发率约为 7%。
