Spontaneous adrenocorticotropic hormone (ACTH) normalisation due to tumour regression induced by metyrapone in a patient with ectopic ACTH syndrome: case report and literature review
Abstract Background Ectopic adrenocorticotropic hormone (ACTH) syndrome (EAS) is caused by tumours releasing ACTH. Ectopic ACTH-producing tumour regression is rarely induced using steroidogenesis inhibitors. We presented a case of EAS in which ACTH production by a lung tumour was reduced by metyrapo...
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doaj-2a7cb05ff0eb4c72912272da5d808b572020-11-25T03:17:07ZengBMCBMC Endocrine Disorders1472-68232018-03-011811610.1186/s12902-018-0246-2Spontaneous adrenocorticotropic hormone (ACTH) normalisation due to tumour regression induced by metyrapone in a patient with ectopic ACTH syndrome: case report and literature reviewHideyuki Iwayama0Sho Hirase1Yuka Nomura2Tatsuo Ito3Hiroyuki Morita4Kazuo Otake5Akihisa Okumura6Junko Takagi7Department of Paediatrics, Aichi Medical University, School of MedicineDivision of Endocrinology and Metabolism, Department of Internal Medicine, Aichi Medical University, School of MedicineDivision of Endocrinology and Metabolism, Department of Internal Medicine, Aichi Medical University, School of MedicineDivision of Endocrinology and Metabolism, Department of Internal Medicine, Aichi Medical University, School of MedicineDivision of Endocrinology and Metabolism, Department of Internal Medicine, Aichi Medical University, School of MedicineDivision of Endocrinology and Metabolism, Department of Internal Medicine, Aichi Medical University, School of MedicineDepartment of Paediatrics, Aichi Medical University, School of MedicineDivision of Endocrinology and Metabolism, Department of Internal Medicine, Aichi Medical University, School of MedicineAbstract Background Ectopic adrenocorticotropic hormone (ACTH) syndrome (EAS) is caused by tumours releasing ACTH. Ectopic ACTH-producing tumour regression is rarely induced using steroidogenesis inhibitors. We presented a case of EAS in which ACTH production by a lung tumour was reduced by metyrapone (MTP) and also reviewed previous cases of ectopic ACTH production suppressed via steroidogenesis inhibition. Case presentation A 71-year-old female with general fatigue, central obesity and impaired glucose tolerance was diagnosed with Cushing’s syndrome due to elevated ACTH (192.9 pg/mL; normal range, 7.2–63.3 pg/mL), cortisol (73.1 μg/dL; 6.4–21.0 μg/dL) and 24-h urinary free cortisol (UFC) (6160 μg/day; 11.2–80.3 μg/day) levels. Chest computed tomography identified a solid 26.6 × 22.9 × 30.0 mm tumour with a cavity in the upper lobe of the left lung. There was no adrenal gland enlargement. Tumour markers were not significantly elevated; ACTH levels were not suppressed by 8-mg dexamethasone. A corticotropin-releasing hormone stimulation test revealed blunted ACTH response (basal ACTH, 204.6 pg/mL; highest ACTH level during the 120-min stimulation test, 214.0 pg/mL). She was diagnosed with EAS due to a lung lesion. MTP treatment was started to reduce cortisol production. ACTH levels and cortisol and UFC levels were normalised and the ACTH-producing lung tumour was ablated after MTP treatment. In several reported cases, plasma ACTH levels reduced during steroidogenesis inhibitor treatment for EAS. Among the 10 patients, three cases of pheochromocytoma, one of thymic carcinoid and one of islet cell carcinoma were reported. In four cases, the tumour was not detected. In our case, the pathology of the lung tumour was unknown because of lack of tumour cells in biopsy. The patients were treated with ketoconazole (KTZ) and/or MTP and exhibited ACTH and cortisol/UFC suppression, but tumour regression was observed only in our case. Conclusion MTP and/or KTZ may reduce ACTH and cortisol production. The tumour spontaneously regressed after MTP treatment, indicating that MTP may reduce the tumour size without surgery. The mechanisms of therapeutic effects of steroidogenesis inhibitors and prognosis of spontaneous remission should be elucidated further via molecular biology studies.http://link.springer.com/article/10.1186/s12902-018-0246-2Cushing’s syndromeEctopic hormone syndromeSteroidogenesis inhibitorMetyraponeTumour regression |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Hideyuki Iwayama Sho Hirase Yuka Nomura Tatsuo Ito Hiroyuki Morita Kazuo Otake Akihisa Okumura Junko Takagi |
spellingShingle |
Hideyuki Iwayama Sho Hirase Yuka Nomura Tatsuo Ito Hiroyuki Morita Kazuo Otake Akihisa Okumura Junko Takagi Spontaneous adrenocorticotropic hormone (ACTH) normalisation due to tumour regression induced by metyrapone in a patient with ectopic ACTH syndrome: case report and literature review BMC Endocrine Disorders Cushing’s syndrome Ectopic hormone syndrome Steroidogenesis inhibitor Metyrapone Tumour regression |
author_facet |
Hideyuki Iwayama Sho Hirase Yuka Nomura Tatsuo Ito Hiroyuki Morita Kazuo Otake Akihisa Okumura Junko Takagi |
author_sort |
Hideyuki Iwayama |
title |
Spontaneous adrenocorticotropic hormone (ACTH) normalisation due to tumour regression induced by metyrapone in a patient with ectopic ACTH syndrome: case report and literature review |
title_short |
Spontaneous adrenocorticotropic hormone (ACTH) normalisation due to tumour regression induced by metyrapone in a patient with ectopic ACTH syndrome: case report and literature review |
title_full |
Spontaneous adrenocorticotropic hormone (ACTH) normalisation due to tumour regression induced by metyrapone in a patient with ectopic ACTH syndrome: case report and literature review |
title_fullStr |
Spontaneous adrenocorticotropic hormone (ACTH) normalisation due to tumour regression induced by metyrapone in a patient with ectopic ACTH syndrome: case report and literature review |
title_full_unstemmed |
Spontaneous adrenocorticotropic hormone (ACTH) normalisation due to tumour regression induced by metyrapone in a patient with ectopic ACTH syndrome: case report and literature review |
title_sort |
spontaneous adrenocorticotropic hormone (acth) normalisation due to tumour regression induced by metyrapone in a patient with ectopic acth syndrome: case report and literature review |
publisher |
BMC |
series |
BMC Endocrine Disorders |
issn |
1472-6823 |
publishDate |
2018-03-01 |
description |
Abstract Background Ectopic adrenocorticotropic hormone (ACTH) syndrome (EAS) is caused by tumours releasing ACTH. Ectopic ACTH-producing tumour regression is rarely induced using steroidogenesis inhibitors. We presented a case of EAS in which ACTH production by a lung tumour was reduced by metyrapone (MTP) and also reviewed previous cases of ectopic ACTH production suppressed via steroidogenesis inhibition. Case presentation A 71-year-old female with general fatigue, central obesity and impaired glucose tolerance was diagnosed with Cushing’s syndrome due to elevated ACTH (192.9 pg/mL; normal range, 7.2–63.3 pg/mL), cortisol (73.1 μg/dL; 6.4–21.0 μg/dL) and 24-h urinary free cortisol (UFC) (6160 μg/day; 11.2–80.3 μg/day) levels. Chest computed tomography identified a solid 26.6 × 22.9 × 30.0 mm tumour with a cavity in the upper lobe of the left lung. There was no adrenal gland enlargement. Tumour markers were not significantly elevated; ACTH levels were not suppressed by 8-mg dexamethasone. A corticotropin-releasing hormone stimulation test revealed blunted ACTH response (basal ACTH, 204.6 pg/mL; highest ACTH level during the 120-min stimulation test, 214.0 pg/mL). She was diagnosed with EAS due to a lung lesion. MTP treatment was started to reduce cortisol production. ACTH levels and cortisol and UFC levels were normalised and the ACTH-producing lung tumour was ablated after MTP treatment. In several reported cases, plasma ACTH levels reduced during steroidogenesis inhibitor treatment for EAS. Among the 10 patients, three cases of pheochromocytoma, one of thymic carcinoid and one of islet cell carcinoma were reported. In four cases, the tumour was not detected. In our case, the pathology of the lung tumour was unknown because of lack of tumour cells in biopsy. The patients were treated with ketoconazole (KTZ) and/or MTP and exhibited ACTH and cortisol/UFC suppression, but tumour regression was observed only in our case. Conclusion MTP and/or KTZ may reduce ACTH and cortisol production. The tumour spontaneously regressed after MTP treatment, indicating that MTP may reduce the tumour size without surgery. The mechanisms of therapeutic effects of steroidogenesis inhibitors and prognosis of spontaneous remission should be elucidated further via molecular biology studies. |
topic |
Cushing’s syndrome Ectopic hormone syndrome Steroidogenesis inhibitor Metyrapone Tumour regression |
url |
http://link.springer.com/article/10.1186/s12902-018-0246-2 |
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