Ectopic Cushing’s syndrome from an ACTH-producing pheochromocytoma with a non-functioning pituitary adenoma
An adrenocorticotropic hormone (ACTH)-producing pheochromocytoma (PCC)/paraganglioma is the cause of ectopic Cushing’s syndrome (CS) in 5.2% of cases reported in the literature. We present a previously healthy 43-year-old woman admitted to our hospital with cushingoid features and hypertensive urgen...
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Bioscientifica
2022-03-01
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| Series: | Endocrinology, Diabetes & Metabolism Case Reports |
| Online Access: | https://edm.bioscientifica.com/view/journals/edm/2022/1/EDM21-0189.xml |
| _version_ | 1828880394900996096 |
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| author | David Kishlyansky Gregory Kline Amita Mahajan Konstantin Koro Janice L Pasieka Patrick Champagne |
| author_facet | David Kishlyansky Gregory Kline Amita Mahajan Konstantin Koro Janice L Pasieka Patrick Champagne |
| author_sort | David Kishlyansky |
| collection | DOAJ |
| description | An adrenocorticotropic hormone (ACTH)-producing pheochromocytoma (PCC)/paraganglioma is the cause of ectopic Cushing’s syndrome (CS) in 5.2% of cases reported in the literature. We present a previously healthy 43-year-old woman admitted to our hospital with cushingoid features and hypertensive urgency (blood pressure = 200/120 mmHg). Her 24-h urinary free cortisol was >4270 nmol/day (reference range (RR) = 100–380 nmol/day) with a plasma ACTH of 91.5 pmol/L (RR: 2.0–11.5 pmol/L). Twenty-four-hour urinary metanephrines were increased by 30-fold. Whole-body CT demonstrated a 3.7-cm left adrenal mass with a normal-appearing right adrenal gland. Sellar MRI showed a 5-mm sellar lesion. MIBG scan revealed intense uptake only in the left adrenal mass. She was managed pre-operatively with ketoconazole and phenoxybenzamine and underwent an uneventful left laparoscopic adrenalectomy, which resulted in biochemical resolution of her hypercortisolemia and catecholamine excess. Histology demonstrated a PCC (Grading System for Adrenal Pheochromocytoma and Paraganglioma score 5) with positive ACTH staining by immunohistochemistry. A PCC gene panel showed no mutations and there has been no evidence of recurrence at 24 months. This case highlights the difficult nature of localizing the source of CS in the setting of a co-existing PCC and sellar mass. |
| first_indexed | 2024-12-13T09:46:52Z |
| format | Article |
| id | doaj.art-ef6dc88ac5bc42f3920c727aa4569ab3 |
| institution | Directory Open Access Journal |
| issn | 2052-0573 |
| language | English |
| last_indexed | 2024-12-13T09:46:52Z |
| publishDate | 2022-03-01 |
| publisher | Bioscientifica |
| record_format | Article |
| series | Endocrinology, Diabetes & Metabolism Case Reports |
| spelling | doaj.art-ef6dc88ac5bc42f3920c727aa4569ab32022-12-21T23:52:01ZengBioscientificaEndocrinology, Diabetes & Metabolism Case Reports2052-05732022-03-01111810.1530/EDM-21-0189Ectopic Cushing’s syndrome from an ACTH-producing pheochromocytoma with a non-functioning pituitary adenomaDavid Kishlyansky0Gregory Kline1Amita Mahajan2Konstantin Koro3Janice L Pasieka4Patrick Champagne5Division of Internal Medicine, Department of Medicine, University of Calgary, Calgary, Alberta, CanadaDivison of Endocrinology and Metabolism, Department of Medicine, University of Calgary, Calgary, Alberta, CanadaDivison of Endocrinology and Metabolism, Department of Medicine, University of Calgary, Calgary, Alberta, CanadaDepartment of Pathology and Laboratory Medicine, University of Calgary, Calgary, Alberta, CanadaDivison of Endocrine surgery, Surgical Oncology and Endocrinology, Department of Surgery, University of Calgary, Calgary, Alberta, CanadaDepartment of Cardiac Sciences, University of Calgary, Calgary, Alberta, CanadaAn adrenocorticotropic hormone (ACTH)-producing pheochromocytoma (PCC)/paraganglioma is the cause of ectopic Cushing’s syndrome (CS) in 5.2% of cases reported in the literature. We present a previously healthy 43-year-old woman admitted to our hospital with cushingoid features and hypertensive urgency (blood pressure = 200/120 mmHg). Her 24-h urinary free cortisol was >4270 nmol/day (reference range (RR) = 100–380 nmol/day) with a plasma ACTH of 91.5 pmol/L (RR: 2.0–11.5 pmol/L). Twenty-four-hour urinary metanephrines were increased by 30-fold. Whole-body CT demonstrated a 3.7-cm left adrenal mass with a normal-appearing right adrenal gland. Sellar MRI showed a 5-mm sellar lesion. MIBG scan revealed intense uptake only in the left adrenal mass. She was managed pre-operatively with ketoconazole and phenoxybenzamine and underwent an uneventful left laparoscopic adrenalectomy, which resulted in biochemical resolution of her hypercortisolemia and catecholamine excess. Histology demonstrated a PCC (Grading System for Adrenal Pheochromocytoma and Paraganglioma score 5) with positive ACTH staining by immunohistochemistry. A PCC gene panel showed no mutations and there has been no evidence of recurrence at 24 months. This case highlights the difficult nature of localizing the source of CS in the setting of a co-existing PCC and sellar mass.https://edm.bioscientifica.com/view/journals/edm/2022/1/EDM21-0189.xml |
| spellingShingle | David Kishlyansky Gregory Kline Amita Mahajan Konstantin Koro Janice L Pasieka Patrick Champagne Ectopic Cushing’s syndrome from an ACTH-producing pheochromocytoma with a non-functioning pituitary adenoma Endocrinology, Diabetes & Metabolism Case Reports |
| title | Ectopic Cushing’s syndrome from an ACTH-producing pheochromocytoma with a non-functioning pituitary adenoma |
| title_full | Ectopic Cushing’s syndrome from an ACTH-producing pheochromocytoma with a non-functioning pituitary adenoma |
| title_fullStr | Ectopic Cushing’s syndrome from an ACTH-producing pheochromocytoma with a non-functioning pituitary adenoma |
| title_full_unstemmed | Ectopic Cushing’s syndrome from an ACTH-producing pheochromocytoma with a non-functioning pituitary adenoma |
| title_short | Ectopic Cushing’s syndrome from an ACTH-producing pheochromocytoma with a non-functioning pituitary adenoma |
| title_sort | ectopic cushing s syndrome from an acth producing pheochromocytoma with a non functioning pituitary adenoma |
| url | https://edm.bioscientifica.com/view/journals/edm/2022/1/EDM21-0189.xml |
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