Finding the Culprit: Cushing Syndrome Secondary to Lung Carcinoid Tumor

Background/Objective: Cushing syndrome resulting from ectopic adrenocorticotropic hormone (ACTH) secretion (EAS) is a rare condition, and its occurrence in adolescents is even more uncommon, representing <1% of cases. We describe a case of EAS from a lung carcinoid tumor leading to Cushing syndro...

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Main Authors: Richard Vaca, MD, Nirali A. Shah, MD
Format: Article
Language:English
Published: Elsevier 2025-01-01
Series:AACE Clinical Case Reports
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Online Access:http://www.sciencedirect.com/science/article/pii/S2376060524001007
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author Richard Vaca, MD
Nirali A. Shah, MD
author_facet Richard Vaca, MD
Nirali A. Shah, MD
author_sort Richard Vaca, MD
collection DOAJ
description Background/Objective: Cushing syndrome resulting from ectopic adrenocorticotropic hormone (ACTH) secretion (EAS) is a rare condition, and its occurrence in adolescents is even more uncommon, representing <1% of cases. We describe a case of EAS from a lung carcinoid tumor leading to Cushing syndrome in a young woman, which was successfully treated with excision of the tumor. Case Report: An 18-year-old woman presented with mood disturbances, weight gain, and fatigue for 6 months. Workup revealed high levels of urinary free cortisol (>900 μg/dL; normal range, <45 μg/dL) and midnight salivary cortisol (0.755 μg/dL; normal range, <0.09 μg/dL). The ACTH and cortisol levels remained elevated after a low-dose dexamethasone suppression test. Magnetic resonance imaging of the pituitary gland did not reveal any adenoma and inferior petrosal sinus sampling showed no central-to-peripheral gradient. A diagnosis of EAS was made. Subsequent body imaging noted a 1-cm lung nodule. Due to symptoms of severe hypercortisolism including hypokalemia and worsening mood changes, the patient was started on metyrapone as a bridge to surgery. A few weeks later, the patient underwent successful surgical resection, after which symptoms promptly resolved. Pathology report later confirmed an atypical lung carcinoid tumor. The patient remained in remission at 1-year follow-up. Discussion: Medical therapy aids in managing severe hypercortisolism in ACTH-secreting tumors until definitive surgical treatment can be undertaken. Conclusion: This case underscores the critical importance of promptly recognizing EAS and the resulting severe hypercortisolism symptoms because early surgical intervention can lead to a cure.
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spelling doaj-art-6beda3fd06024dbc8a60368b6a13fbe92025-01-15T04:11:43ZengElsevierAACE Clinical Case Reports2376-06052025-01-011111013Finding the Culprit: Cushing Syndrome Secondary to Lung Carcinoid TumorRichard Vaca, MD0Nirali A. Shah, MD1Division of Internal Medicine, Icahn School of Medicine at Mount Sinai, Mount Sinai Beth Israel, New York, New YorkDivision of Endocrinology, Diabetes and Bone Disease, Department of Medicine, Icahn School of Medicine at Mount Sinai, New York, New York; Address correspondence to Dr Nirali A. Shah, Division of Endocrinology, Diabetes and Bone Disease, Department of Medicine, Icahn School of Medicine at Mount Sinai, One Gustave L. Levy Place, Box 1055, New York, NY 10029.Background/Objective: Cushing syndrome resulting from ectopic adrenocorticotropic hormone (ACTH) secretion (EAS) is a rare condition, and its occurrence in adolescents is even more uncommon, representing <1% of cases. We describe a case of EAS from a lung carcinoid tumor leading to Cushing syndrome in a young woman, which was successfully treated with excision of the tumor. Case Report: An 18-year-old woman presented with mood disturbances, weight gain, and fatigue for 6 months. Workup revealed high levels of urinary free cortisol (>900 μg/dL; normal range, <45 μg/dL) and midnight salivary cortisol (0.755 μg/dL; normal range, <0.09 μg/dL). The ACTH and cortisol levels remained elevated after a low-dose dexamethasone suppression test. Magnetic resonance imaging of the pituitary gland did not reveal any adenoma and inferior petrosal sinus sampling showed no central-to-peripheral gradient. A diagnosis of EAS was made. Subsequent body imaging noted a 1-cm lung nodule. Due to symptoms of severe hypercortisolism including hypokalemia and worsening mood changes, the patient was started on metyrapone as a bridge to surgery. A few weeks later, the patient underwent successful surgical resection, after which symptoms promptly resolved. Pathology report later confirmed an atypical lung carcinoid tumor. The patient remained in remission at 1-year follow-up. Discussion: Medical therapy aids in managing severe hypercortisolism in ACTH-secreting tumors until definitive surgical treatment can be undertaken. Conclusion: This case underscores the critical importance of promptly recognizing EAS and the resulting severe hypercortisolism symptoms because early surgical intervention can lead to a cure.http://www.sciencedirect.com/science/article/pii/S2376060524001007Cushing syndromeectopic ACTH secretioncarcinoid tumorhypercortisolism
spellingShingle Richard Vaca, MD
Nirali A. Shah, MD
Finding the Culprit: Cushing Syndrome Secondary to Lung Carcinoid Tumor
AACE Clinical Case Reports
Cushing syndrome
ectopic ACTH secretion
carcinoid tumor
hypercortisolism
title Finding the Culprit: Cushing Syndrome Secondary to Lung Carcinoid Tumor
title_full Finding the Culprit: Cushing Syndrome Secondary to Lung Carcinoid Tumor
title_fullStr Finding the Culprit: Cushing Syndrome Secondary to Lung Carcinoid Tumor
title_full_unstemmed Finding the Culprit: Cushing Syndrome Secondary to Lung Carcinoid Tumor
title_short Finding the Culprit: Cushing Syndrome Secondary to Lung Carcinoid Tumor
title_sort finding the culprit cushing syndrome secondary to lung carcinoid tumor
topic Cushing syndrome
ectopic ACTH secretion
carcinoid tumor
hypercortisolism
url http://www.sciencedirect.com/science/article/pii/S2376060524001007
work_keys_str_mv AT richardvacamd findingtheculpritcushingsyndromesecondarytolungcarcinoidtumor
AT niraliashahmd findingtheculpritcushingsyndromesecondarytolungcarcinoidtumor