Abstract | BACKGROUND: METHODS: RESULTS: Seventy-six patients were included. Mean age at diagnosis was 46.3 ± 15.8 years. Most patients (N = 61, 80%) had metastases at ectopic Cushing syndrome diagnosis. Average follow-up was 2.9 ± 3.7 years (range, 4 months-17.2 years). Patients with neuroendocrine tumors before ectopic Cushing syndrome had more frequent metastatic disease and resistant ectopic Cushing syndrome. Patients with de novo hyperglycemia, poor neuroendocrine tumor differentiation, and metastatic disease had worse survival. Of those with nonmetastatic disease, 8 (53%) had ectopic Cushing syndrome resolution after neuroendocrine tumor resection, 3 (20%) were medically controlled, and 4 (27%) underwent bilateral adrenalectomy. In patients with metastatic neuroendocrine tumors, hypercortisolism was initially medically managed in 92%, 3% underwent immediate bilateral adrenalectomy, 2% had control after primary neuroendocrine tumor 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 ectopic Cushing syndrome, 23 ultimately had bilateral adrenalectomy with ectopic Cushing syndrome cure in all. CONCLUSION:
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Authors | Jace P Landry, Uriel Clemente-Gutierrez, Carolina R C Pieterman, Yi-Ju Chiang, Steven G Waguespack, Camilo Jimenez, Mouhammed A Habra, Daniel M Halperin, Sarah B Fisher, Paul H Graham, Nancy D Perrier |
Journal | Surgery
(Surgery)
Vol. 172
Issue 2
Pg. 559-566
(08 2022)
ISSN: 1532-7361 [Electronic] United States |
PMID | 35437162
(Publication Type: Journal Article, Research Support, N.I.H., Extramural)
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Copyright | Copyright © 2022 Elsevier Inc. All rights reserved. |
Chemical References |
- Adrenocorticotropic Hormone
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Topics |
- ACTH Syndrome, Ectopic
(complications, diagnosis)
- Adrenalectomy
(adverse effects)
- Adrenocorticotropic Hormone
- Cushing Syndrome
(diagnosis, etiology, surgery)
- Humans
- Hyperglycemia
(complications)
- Neuroendocrine Tumors
(complications, surgery)
- Retrospective Studies
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