Cushing's syndrome due to phaeochromocytoma secreting the precursors of adrenocorticotropin
Adrenal phaeochromocytoma rarely causes ectopic ACTH syndrome. We describe a 44-yr-old hypertensive woman who was Cushingoid and markedly pigmented. Laboratory studies indicated severe hypokalaemia, abnormal liver function tests, and random serum cortisols greater than 1660 nmol/L. Urinary catechola...
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Published in | The journal of clinical endocrinology and metabolism Vol. 85; no. 12; pp. 4771 - 4775 |
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Main Authors | , , , , , |
Format | Journal Article |
Language | English |
Published |
Bethesda, MD
Endocrine Society
01.12.2000
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Subjects | |
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Abstract | Adrenal phaeochromocytoma rarely causes ectopic ACTH syndrome. We describe a 44-yr-old hypertensive woman who was Cushingoid and markedly pigmented. Laboratory studies indicated severe hypokalaemia, abnormal liver function tests, and random serum cortisols greater than 1660 nmol/L. Urinary catecholamines were markedly increased. An abdominal computed tomography scan showed a 4-cm left adrenal mass and an hypertrophied right adrenal. ACTH levels were elevated at 200 pmol/L, but ACTH precursors, which cross-react in the ACTH assay, were more highly elevated at 1625 pmol/L. The tumor cells cultured in vitro also secreted ACTH precursors, whereas ACTH levels were undetectable. Because the patient was highly pigmented, we measured circulating concentrations of alpha-MSH, which were undetectable and certainly insufficient to stimulate melanogenesis, suggesting that tumorderived ACTH precursors or ACTH were responsible for the pigmentation. A laparoscopic adrenalectomy resulted in remission of the Cushing's syndrome and dramatic reduction in the pigmentation. Before operation, treatment of the patient with metyrapone and replacement dexamethasone decreased cortisol from more than 1660 to less than 20 nmol/L. Surprisingly, this resulted in a decrease in ACTH precursors to 100 pmol/L and ACTH to 9.0 pmol/L. In vitro treatment of the tumor cells with dexamethasone for 24 or 40 h increased ACTH precursor secretion. In summary, this phaeochromocytoma causing Cushing's syndrome secreted primarily ACTH precursors, which seemed to cause the marked pigmentation. In vivo and in vitro evidence suggests that glucocorticoids induced ACTH precursor secretion. |
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AbstractList | Adrenal phaeochromocytoma rarely causes ectopic ACTH syndrome. We describe a 44-yr-old hypertensive woman who was Cushingoid and markedly pigmented. Laboratory studies indicated severe hypokalaemia, abnormal liver function tests, and random serum cortisols greater than 1660 nmol/L. Urinary catecholamines were markedly increased. An abdominal computed tomography scan showed a 4-cm left adrenal mass and an hypertrophied right adrenal. ACTH levels were elevated at 200 pmol/L, but ACTH precursors, which cross-react in the ACTH assay, were more highly elevated at 1625 pmol/L. The tumor cells cultured in vitro also secreted ACTH precursors, whereas ACTH levels were undetectable. Because the patient was highly pigmented, we measured circulating concentrations of alpha-MSH, which were undetectable and certainly insufficient to stimulate melanogenesis, suggesting that tumorderived ACTH precursors or ACTH were responsible for the pigmentation. A laparoscopic adrenalectomy resulted in remission of the Cushing's syndrome and dramatic reduction in the pigmentation. Before operation, treatment of the patient with metyrapone and replacement dexamethasone decreased cortisol from more than 1660 to less than 20 nmol/L. Surprisingly, this resulted in a decrease in ACTH precursors to 100 pmol/L and ACTH to 9.0 pmol/L. In vitro treatment of the tumor cells with dexamethasone for 24 or 40 h increased ACTH precursor secretion. In summary, this phaeochromocytoma causing Cushing's syndrome secreted primarily ACTH precursors, which seemed to cause the marked pigmentation. In vivo and in vitro evidence suggests that glucocorticoids induced ACTH precursor secretion. |
Author | BEVAN, J. S THODY, A. J WHITE, A ABRAHAM, P RAY, D. W TALBOT, A |
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Keywords | Endocrinopathy Human Corticosteroid Dexamethasone Pheochromocytoma Adrenal cortex diseases Adrenal glands Treatment efficiency Cushing syndrome Metyrapone Hyperadrenocorticism Ectopic secretion Prohormone Secretory tumor Case study Chemotherapy Adrenocorticotropic hormone Adenohypophyseal hormone Etiology Surgery Adrenal gland diseases Adult Female |
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Snippet | Adrenal phaeochromocytoma rarely causes ectopic ACTH syndrome. We describe a 44-yr-old hypertensive woman who was Cushingoid and markedly pigmented. Laboratory... |
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SubjectTerms | Adrenal Gland Neoplasms - complications Adrenal Gland Neoplasms - metabolism Adrenal Gland Neoplasms - surgery Adrenalectomy Adrenals. Adrenal axis. Renin-angiotensin system (diseases) Adrenocorticotropic Hormone - metabolism Adult alpha-MSH - metabolism Anti-Inflammatory Agents - pharmacology Biological and medical sciences Cushing Syndrome - etiology Cushing Syndrome - surgery Dexamethasone - pharmacology Endocrinopathies Female Humans Hydrocortisone - metabolism Medical sciences Metyrapone - pharmacology Non tumoral diseases. Target tissue resistance. Benign neoplasms Pheochromocytoma - complications Pheochromocytoma - metabolism Pheochromocytoma - surgery Tumor Cells, Cultured - metabolism |
Title | Cushing's syndrome due to phaeochromocytoma secreting the precursors of adrenocorticotropin |
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