Ectopic ACTH-syndrome caused by neuroendocrine tumor of lung

The authors report a case of an ectopic ACTH-syndrome that resulted in severe hypercortisolism, hypokalemia, diabetes mellitus and osteoporosis. The ACTH-secreting tumor tissue was localized in the lung. The tumor was removed by segmentectomy and histological evaluation revealed an ACTH-secreting neuroendocrine tumor. After surgery, however, plasma cortisol and ACTH levels failed to decrease significantly due to subtotal tumor removal. Long-acting somatostatin analogue therapy resulted in a normalization of both plasma cortisol and ACTH levels and the clinical symptoms improved significantly. Residual tumor was removed by repeat surgery and the patient was permanently cured. Orv. Hetil., 2011, 152, 403–406.

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Ectopic ACTH syndrome caused by hepatic metastases from previously non-functional pancreatic neuroendocrine tumor

Ibnosina Journal of Medicine and Biomedical Sciences ◽

10.4103/1947-489x.210224 ◽

2016 ◽

Vol 8 (4) ◽

pp. 120

Author(s):

AbdulwahabM Elbarsha ◽

RafikR El Mehdawi ◽

Najat Buzaid ◽

Adel Attia ◽

Maisoon El Hemri

Keyword(s):

Neuroendocrine Tumor ◽

Hepatic Metastases ◽

Pancreatic Neuroendocrine Tumor ◽

Ectopic Acth Syndrome ◽

Ectopic Acth

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Ectopic ACTH Syndrome Caused by a Bronchial Neuroendocrine Tumor

Practical Clinical Endocrinology ◽

10.1007/978-3-030-62011-0_45 ◽

2021 ◽

pp. 447-457

Author(s):

Géza Nagy

Keyword(s):

Neuroendocrine Tumor ◽

Ectopic Acth Syndrome ◽

Ectopic Acth

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Ectopic ACTH Syndrome Emerging 5 Years after the Diagnosis of Neuroendocrine Tumor

Case Reports in Endocrinology ◽

10.1155/2019/6583467 ◽

2019 ◽

Vol 2019 ◽

pp. 1-5

Author(s):

Minghao Liu ◽

Diane Hamele-Bena ◽

John Ausiello ◽

Gabrielle Page-Wilson

Keyword(s):

Neuroendocrine Tumor ◽

Serum Cortisol ◽

Urinary Free Cortisol ◽

Ectopic Acth Syndrome ◽

Ectopic Acth ◽

Free Cortisol ◽

Metastatic Lung ◽

Suppression Test ◽

Dexamethasone Suppression ◽

Retrospective Examination

Ectopic ACTH syndrome (EAS) arising years after the diagnosis of a neuroendocrine tumor (NET) is exceedingly rare. We describe a case of EAS occurring five years after the diagnosis of a metastatic lung NET in a 61-year-old woman. She presented with severe hypokalemia but was not overtly Cushingoid on exam. Serum cortisol was 61mcg/dL after an overnight 1mg dexamethasone suppression test (<1.8mcg/dL) and urinary free cortisol was 7544 mcg/24h (<45mcg/24h), establishing the diagnosis of Cushing’s syndrome. Plasma levels of peptides which have been associated with EAS, Agouti-related peptide (AgRP) and the ACTH precursors POMC (31-kDa) and pro-ACTH (22-kDa), were elevated. Metyrapone was initiated, but hypercortisolism persisted and the patient succumbed to pneumonia shortly after presentation. Retrospective examination of biopsy tissues showed rare ACTH immunoreactivity at the time of initial diagnosis, followed by staining in a greater proportion of cells as the disease progressed, consistent with EAS arising years after the diagnosis of NET. Given the increase in mortality associated with EAS, this unusual case highlights the importance of early detection and raises the possibility that early immunohistochemical stains for ACTH and measurements of ACTH precursors may facilitate the identification of NETs at high risk for EAS.

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