Ectopic ACTH-secreting syndrome: a single-center experience
| Autor: | Rajeev Kasaliwal, Padmavathy S. Menon, Nalini S. Shah, Anurag R. Lila, Sweta Budyal, Amol Bukan, Tushar Bandgar, Shruti Khare, Harshal Kakade, Varsha S. Jagtap |
|---|---|
| Rok vydání: | 2013 |
| Předmět: |
Adult
Male medicine.medical_specialty Adolescent Hydrocortisone Corticotropin-Releasing Hormone Endocrinology Diabetes and Metabolism medicine.medical_treatment Gallium Single Center Lesion Basal (phylogenetics) Young Adult Endocrinology Adrenocorticotropic Hormone Medicine Humans Thyroid Neoplasms Child Cushing Syndrome Retrospective Studies Transsphenoidal surgery medicine.diagnostic_test business.industry Bronchial Neoplasms Retrospective cohort study Magnetic resonance imaging General Medicine Thymus Neoplasms Middle Aged Magnetic Resonance Imaging Inferior petrosal sinus sampling Surgery Pancreatic Neoplasms ACTH Syndrome Ectopic Treatment Outcome Positron emission tomography Pituitary Gland Female Radiology medicine.symptom business Tomography X-Ray Computed Biomarkers |
| Zdroj: | Endocrine practice : official journal of the American College of Endocrinology and the American Association of Clinical Endocrinologists. 19(6) |
| ISSN: | 1934-2403 |
| Popis: | Objective Ectopic adrenocorticotropic hormone (ACTH)-secreting syndrome (EAS) is a rare cause of ACTH-dependent endogenous hypercortisolism. The objective of this study was to analyze clinical, biochemical, and imaging characteristics; management strategies; and outcomes of EAS patients. Method We screened the records (1993-2012) of ACTH-dependent endogenous hypercortisolism cases managed at a tertiary care center. Results Of the 218 patients, 17 were diagnosed with EAS. The median 8:00 AM serum cortisol was 36 μg/dL (11.4-82.7 μg/dL), and the median basal plasma ACTH was 156 pg/mL (53.5-468 pg/mL). Notably, ACTH levels below 100 pg/mL were found in 4 patients. Suspicious microadenoma was found on magnetic resonance imaging (MRI) of the pituitary in 5 patients, and all of them underwent transsphenoidal surgery (TSS). Inferior petrosal sinus sampling (IPSS) was performed in 8 patients, and the results were suggestive of a peripheral source in all 8. Computed tomography (CT) localized the lesion in 15/17 patients. In 2 patients with negative CTs, gallium DOTATATE positron emission tomography (PET) scans localized the lesion. Despite difficulties localizing bronchial carcinoids, the cure rate was high (72%). In contrast, thymic carcinoids were easily localized but had poor outcomes. Conclusion EAS cannot be ruled out on the basis of marginally elevated ACTH. In cases with an equivocal MRI pituitary finding, prior IPSS can help avoid unnecessary TSS. CT is a useful modality for localization of an ectopic source. Functional imaging may help in cases where anatomical imaging fails. (Endocr Pract. 2013;19:1007-1014) |
| Databáze: | OpenAIRE |
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