Chen H, Doppman J L, Chrousos G P, Norton J A, Nieman L K, Udelsman R
Division of Endocrine and Oncologic Surgery, Johns Hopkins Hospital, Baltimore, Md 21287, USA.
Surgery. 1995 Dec;118(6):988-94; discussion 994-5. doi: 10.1016/s0039-6060(05)80104-7.
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.
We report the largest series of patients with adrenocorticotropic hormone (ACTH)-secreting pheochromocytomas and review the world literature.
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.
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.
嗜铬细胞瘤的手术治疗要求切除受累肾上腺，若对侧肾上腺增大则需进行探查切除。我们描述了这一规则的一个例外情况。
我们报告了最大系列的分泌促肾上腺皮质激素（ACTH）的嗜铬细胞瘤患者，并回顾了世界文献。
4例患者表现出肾上腺皮质激素和儿茶酚胺过多的症状，以及血浆ACTH、尿间甲肾上腺素和尿游离皮质醇水平升高。腹部计算机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信号增强，提示嗜铬细胞瘤。2例患者接受了肾上腺静脉采血，将ACTH分泌定位到嗜铬细胞瘤。所有患者均接受了单侧肾上腺切除术，切除的是良性肿瘤，无并发症或死亡，对侧增生的肾上腺原位保留。术后所有患者血浆ACTH、尿间甲肾上腺素和尿游离皮质醇水平恢复正常，症状缓解。将我们的系列病例与先前报道的分泌ACTH的嗜铬细胞瘤病例相结合，几乎所有肿瘤都是良性的（21例中的20例），这与大多数分泌ACTH的肿瘤不同。
分泌ACTH的嗜铬细胞瘤是该规则的例外情况；单侧肾上腺切除术可治愈疾病，对侧增生的肾上腺可予保留。这种方法可使两种激素过多综合征均得到缓解，并保留长期肾上腺功能。
