ADRENOCORTICOTROPIC HORMONE-SECRETING PHEOCHROMOCYTOMAS - THE EXCEPTION TO THE RULE
ADRENOCORTICOTROPIC HORMONE-SECRETING PHEOCHROMOCYTOMAS - THE EXCEPTION TO THE RULE
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DOI：
10.1016/s0039-6060(05)80104-7
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发表时间：
1995-12-01
期刊：
影响因子：
3.8
通讯作者：
UDELSMAN, R
中科院分区：
文献类型：
--
作者：
CHEN, H;DOPPMAN, JL;UDELSMAN, R
背景嗜铬细胞瘤的手术治疗要求切除受累的肾上腺，如果扩大则探查切除对侧肾上腺。我们描述了一个例外，这个规则。我们报告了最大的一组肾上腺皮质激素（ACTH）分泌性嗜铬细胞瘤患者，并回顾了世界文献。4例患者出现肾上腺皮质激素和儿茶酚胺过量，以及血浆ACTH、尿变肾上腺素和尿游离皮质醇水平升高。腹部电脑断层扫描显示双侧肾上腺增生，磁共振成像扫描显示单侧肾上腺肿块，T2信号明亮，提示嗜铬细胞瘤。两名患者接受肾上腺静脉取样定位ACTH分泌的嗜铬细胞瘤。所有患者均接受单侧肾上腺切除术治疗良性肿瘤，无发病或死亡，对侧肾上腺原位增生。手术后所有患者的血浆ACTH、尿肾上腺素和尿游离皮质醇水平均恢复正常，症状消失。结合我们的系列与以前报道的病例ACTH分泌嗜铬细胞瘤，几乎所有的良性（20/21），而大多数ACTH分泌肿瘤。分泌ACTH的嗜铬细胞瘤是例外，单侧肾上腺切除术是治愈性的，对侧增生的肾上腺可以保留。这种方法可以解决激素过多的两种综合征，并保持长期的肾上腺功能。
Background. Operative management of pheochromocytomas dictates resection of the involved adrenal and exploration-resection of the contralateral gland if enlarged. We describe an exception to this rule.Methods. We report the largest series of patients with adrenocorticotropic hormone (ACTH)-secreting pheochromocytomas and review the world literature.Results. Four patients presented with findings of adrenocorticoid and catecholamine excess, as well as elevated levels of plasma ACTH, urinary metanephrines, and urinary free cortisol. Abdominal computed tomography scans revealed bilateral adrenal hyperplasia, and magnetic resonance imaging scans showed a unilateral adrenal mass with a bright T2 signal suggesting a pheochromocytoma. Two patients underwent adrenal venous sampling localizing ACTH secretion to the pheochromocytoma. All underwent unilateral adrenalectomy for a benign tumor without morbidity or death, leaving the contralateral hyperplastic adrenal in situ. After operation all patients experienced normalization of their levels of plasma ACTH, urinary metanephrines, and urinary free cortisol with resolution of symptoms. Combining our series with previously reported cases of ACTH-secreting pheochromocytomas, almost all are benign (20 of 21) in contrast to most ACTH-secreting tumors.Conclusions. ACTH-secreting pheochromocytomas are the exception to the rule; unilateral adrenalectomy is curative and the contralateral hyperplastic adrenal can be preserved. This approach results in resolution of both syndromes of hormone excess and preserves long-term adrenal function.