Metyrapone-responsive ectopic acth-secreting pheochromocytoma with a vicious cycle via a glucocorticoid-driven positive-feedback mechanism

Minako Inoue, Ken Okamura, Chie Kitaoka, Fumio Kinoshita, Ryo Namitome, Udai Nakamura, Masaki Shiota, Kenichi Goto, Toshio Ohtsubo, Kiyoshi Matsumura, Yoshinao Oda, Masatoshi Eto, Takanari Kitazono

研究成果: ジャーナルへの寄稿記事

2 引用 (Scopus)

抄録

In ectopic ACTH-secreting pheochromocytoma, combined ACTH-driven hypercortisolemia and hypercatecholaminemia are serious conditions, which can be fatal if not diagnosed and managed appropriately, especially when glucocorticoid-driven positive feedback is suggested with a high ACTH/cortisol ratio. A 46-year-old man presented with headache, rapid weight loss, hyperhidrosis, severe hypertension and hyperglycemia without typical Cushingoid appearance. Endocrinological examinations demonstrated elevated plasma and urine catecholamines, serum cortisol and plasma ACTH. Moreover, his ACTH/cortisol ratio and catecholamine levels were extremely high, suggesting catecholamine-dominant ACTH-secreting pheochromocytoma. Computed tomography revealed a large right adrenal tumor.18F-FDG positron emission tomography showed uptake in the area of the adrenal tumor, while123I-metaiodobenzylguanidine scintigraphy showed no accumulation. His plasma ACTH level paradoxically became elevated after a dexamethasone suppression test. After metyrapone administration, not only serum cortisol but also plasma ACTH levels were exponentially decreased almost in parallel, suggesting a glucocorticoid-driven positive-feedback regulation in this rapidly exacerbated ectopic ACTH-producing pheochromocytoma. Interestingly enough, plasma catecholamine levels were also decreased by metyrapone, although they remained extremely high. He became severely dehydrated due to hypoadrenalism requiring hydrocortisone supplementation. His clinical signs and symptoms were improved, and right adrenalectomy was performed uneventfully, resulting in complete remission of pheochromocytoma and Cushing’s syndrome. A glucocorticoid-driven positive-feedback regulation in this ectopic ACTH-secreting pheochromocytoma created a vicious cycle with rapid exacerbation of both hypercortisolemia and hypercatecholaminemia with extremely elevated plasma ACTH level. Metyrapone was clinically effective to stop this vicious cycle; nonetheless, great care must be taken to avoid hypoadrenalism especially when hypercatecholaminemia remained.

元の言語英語
ページ(範囲)755-767
ページ数13
ジャーナルEndocrine Journal
65
発行部数7
DOI
出版物ステータス出版済み - 1 1 2018

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Metyrapone
Pheochromocytoma
Adrenocorticotropic Hormone
Glucocorticoids
Hydrocortisone
Catecholamines
Adrenal Insufficiency
Hyperhidrosis
Glandular and Epithelial Neoplasms
Cushing Syndrome
Adrenalectomy
Serum
Radionuclide Imaging
Hyperglycemia
Positron-Emission Tomography
Dexamethasone
Signs and Symptoms
Headache
Weight Loss
Tomography

All Science Journal Classification (ASJC) codes

  • Endocrinology, Diabetes and Metabolism
  • Endocrinology

これを引用

Metyrapone-responsive ectopic acth-secreting pheochromocytoma with a vicious cycle via a glucocorticoid-driven positive-feedback mechanism. / Inoue, Minako; Okamura, Ken; Kitaoka, Chie; Kinoshita, Fumio; Namitome, Ryo; Nakamura, Udai; Shiota, Masaki; Goto, Kenichi; Ohtsubo, Toshio; Matsumura, Kiyoshi; Oda, Yoshinao; Eto, Masatoshi; Kitazono, Takanari.

:: Endocrine Journal, 巻 65, 番号 7, 01.01.2018, p. 755-767.

研究成果: ジャーナルへの寄稿記事

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abstract = "In ectopic ACTH-secreting pheochromocytoma, combined ACTH-driven hypercortisolemia and hypercatecholaminemia are serious conditions, which can be fatal if not diagnosed and managed appropriately, especially when glucocorticoid-driven positive feedback is suggested with a high ACTH/cortisol ratio. A 46-year-old man presented with headache, rapid weight loss, hyperhidrosis, severe hypertension and hyperglycemia without typical Cushingoid appearance. Endocrinological examinations demonstrated elevated plasma and urine catecholamines, serum cortisol and plasma ACTH. Moreover, his ACTH/cortisol ratio and catecholamine levels were extremely high, suggesting catecholamine-dominant ACTH-secreting pheochromocytoma. Computed tomography revealed a large right adrenal tumor.18F-FDG positron emission tomography showed uptake in the area of the adrenal tumor, while123I-metaiodobenzylguanidine scintigraphy showed no accumulation. His plasma ACTH level paradoxically became elevated after a dexamethasone suppression test. After metyrapone administration, not only serum cortisol but also plasma ACTH levels were exponentially decreased almost in parallel, suggesting a glucocorticoid-driven positive-feedback regulation in this rapidly exacerbated ectopic ACTH-producing pheochromocytoma. Interestingly enough, plasma catecholamine levels were also decreased by metyrapone, although they remained extremely high. He became severely dehydrated due to hypoadrenalism requiring hydrocortisone supplementation. His clinical signs and symptoms were improved, and right adrenalectomy was performed uneventfully, resulting in complete remission of pheochromocytoma and Cushing’s syndrome. A glucocorticoid-driven positive-feedback regulation in this ectopic ACTH-secreting pheochromocytoma created a vicious cycle with rapid exacerbation of both hypercortisolemia and hypercatecholaminemia with extremely elevated plasma ACTH level. Metyrapone was clinically effective to stop this vicious cycle; nonetheless, great care must be taken to avoid hypoadrenalism especially when hypercatecholaminemia remained.",
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AU - Inoue, Minako

AU - Okamura, Ken

AU - Kitaoka, Chie

AU - Kinoshita, Fumio

AU - Namitome, Ryo

AU - Nakamura, Udai

AU - Shiota, Masaki

AU - Goto, Kenichi

AU - Ohtsubo, Toshio

AU - Matsumura, Kiyoshi

AU - Oda, Yoshinao

AU - Eto, Masatoshi

AU - Kitazono, Takanari

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Y1 - 2018/1/1

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