Management of adrenocorticotropic hormone-secreting neuroendocrine tumors and the role of bilateral adrenalectomy in ectopic Cushing syndrome.
Management of adrenocorticotropic hormone-secreting neuroendocrine tumors and the role of bilateral adrenalectomy in ectopic Cushing syndrome.
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DOI：
10.1016/j.surg.2022.03.014
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发表时间：
2022-08
期刊：
影响因子：
3.8
通讯作者：
Perrier, Nancy D.
中科院分区：
文献类型：
--
作者：
Landry, Jace P.;Clemente-Gutierrez, Uriel;Pieterman, Carolina R. C.;Chiang, Yi-Ju;Waguespack, Steven G.;Jimenez, Camilo;Habra, Mouhammed A.;Halperin, Daniel M.;Fisher, Sarah B.;Graham, Paul H.;Perrier, Nancy D.
神经内分泌肿瘤（NET）可引起异位库欣综合征（ECS），大多数患者在诊断时已有转移性疾病。我们确定了结果的危险因素，评估了 ECS 管理，并探讨了双侧肾上腺切除术 (BA) 在该人群中的作用。回顾性研究，包括在我们的四级转诊中心治疗四十年（1980-2020）的 NET-ECS。七十六名患者被纳入其中。诊断时的平均年龄为 46.3±15.8 岁。大多数患者 (N=61, 80%) 在 ECS 诊断时已有转移。平均随访时间为 2.9±3.7 年（范围：4 个月至 17.2 年）。 ECS 之前患有 NET 的患者有更频繁的转移性疾病和 ECS 耐药。患有新发高血糖、NET分化差和转移性疾病的患者生存率较差。在非转移性疾病患者中，8 例 (53%) 在 NET 切除后获得 ECS 缓解，3 例 (20%) 得到药物控制，4 例 (27%) 接受 BA。在转移性 NET 患者中，92% 的皮质醇增多症最初得到了药物治疗，3% 立即接受 BA，2% 在原发性 NET 减瘤后得到控制，2% 失访。药物治疗使 7 名患者（13%）的荷尔蒙得到控制。在 49 例患有转移性疾病和耐药 ECS 的患者中，23 例最终通过 ECS 治愈获得 BA。 ECS 发展之前患有 NET 的患者更有可能发生转移，且生存率较差。新生高血糖和 NET 分化差预示着较差的预后。 NET-ECS 患者很难实现皮质醇增多症的药物控制。精心挑选的患者可能会在治疗方案的早期受益于 BA，而多学科管理对于这种复杂的疾病至关重要。我们确定了结果的预测危险因素，并表明异位库欣综合征患者很难实现皮质醇增多症的药物控制。精心挑选的患者可能会在治疗方案的早期受益于双侧肾上腺切除术。
Neuroendocrine tumors (NET) can cause ectopic Cushing syndrome (ECS) and most patients have metastatic disease at diagnosis. We identified risk factors for outcome, evaluated ECS management, and explored the role of bilateral adrenalectomy (BA) in this population. Retrospective study including NET-ECS treated at our quaternary referral center over a forty-year period (1980–2020). Seventy-six patients were included. Mean age at diagnosis was 46.3±15.8 years. Most patients (N=61, 80%) had metastases at ECS diagnosis. Average follow-up was 2.9±3.7 years (range, 4 months-17.2 years). Patients with NET prior to ECS had more frequent metastatic disease and resistant ECS. Patients with de novo hyperglycemia, poor NET differentiation, and metastatic disease had worse survival. Of those with non-metastatic disease, 8 (53%) had ECS resolution after NET resection, 3 (20%) were medically controlled, and 4 (27%) underwent BA. In patients with metastatic NET, hypercortisolism was initially medically managed in 92%, 3% underwent immediate BA, 2% had control after primary NET debulking, and 2% were lost to follow-up. Medical treatment resulted in hormonal control in 7 (13%) patients. Of the 49 patients with metastatic disease and medically resistant ECS, 23 ultimately had BA with ECS cure in all. Patients with NET prior to ECS development were more likely metastatic and had worse survival. De novo hyperglycemia and poor NET differentiation were predictive of worse prognosis. Medical control of hypercortisolism is difficult to achieve in patients with NET-ECS. Well-selected patients may benefit from BA early in the treatment algorithm, and multidisciplinary management is essential in this complex disease. We identified predictive risk factors for outcome and showed that medical control of hypercortisolism is difficult to achieve in patients with ectopic Cushing syndrome. Well-selected patients may benefit from surgical bilateral adrenalectomy early in the treatment algorithm.
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DOI：
10.1111/j.1749-6632.2002.tb04419.x
发表时间：
2002-01-01
期刊：
ENDOCRINE HYPERTENSION
影响因子：
--
作者：
Torpy, DJ;Mullen, N;Nieman, LK
通讯作者：
Nieman, LK
影响因子：
3.2
作者：
Valassi, Elena;Crespo, Iris;Webb, Susan M.
通讯作者：
Webb, Susan M.
DOI：
10.1007/s00464-011-2020-7
发表时间：
2012-04-01
影响因子：
3.1
作者：
Alberda, Wijnand J.;van Eijck, Casper H. J.;Burger, Jacobus W. A.
通讯作者：
Burger, Jacobus W. A.
影响因子：
2.6
作者：
Aniszewski, JP;Young, WF;van Heerden, JA
通讯作者：
van Heerden, JA
影响因子：
5.8
作者：
Ilias, I;Torpy, DJ;Nieman, LK
通讯作者：
Nieman, LK