Case Report: A Case of ACTH-Producing Pheochromocytoma

Sakurai, Haruhisa; Yoshiike, Yasuhiro; S, Isahaya; Matsushita, Hirooki; Yamanaka, Kazushi; Ōkubo, Takao; Kanamori, Hironori; Takahashi, H; Fu, Tong

doi:10.1097/00000441-198710000-00008

Cited by 7 publications

(2 citation statements)

References 13 publications

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“…Both catecholamine and glucocorticoid excess have complex effects on adipogenesis and lipolysis. The plasma (4,448 pg/mL) and urinary (2,590 μg/day) adrenaline levels in the present case were extremely high, compared to the reported levels of 589-2,870 pg/mL [8,14,22,29,33] and 5.8-302 μg/day [17,19,22,23,29,30,32,33], respectively, in ACTH-secreting pheochromocytoma. The extremely high levels of catecholamine suggested catecholamine-dominant ACTH-secreting pheochromocytoma, explaining the patient's weight loss, lack of typical Cushingoid appearances and low BMI, probably through enhanced lipolysis within the body.…”

Section: Discussioncontrasting

confidence: 70%

Metyrapone-responsive ectopic ACTH-secreting pheochromocytoma with a vicious cycle <i>via</i> a glucocorticoid-driven positive-feedback mechanism

et al. 2018

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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. F-FDG positron emission tomography showed uptake in the area of the adrenal tumor, whileI-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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Section: Discussioncontrasting

confidence: 70%

Metyrapone-responsive ectopic ACTH-secreting pheochromocytoma with a vicious cycle <i>via</i> a glucocorticoid-driven positive-feedback mechanism

et al. 2018

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“…Since 1977, there have been two likely cases of ASP; however, death occurred before fulfilment of the diagnostic criteria – myocardial infarction in a 67-year-old female and septic cerebral emboli in a 28-year-old female ( 13 , 16 ). The 29 surviving cases had multiple complications including cardiac arrest, pneumonia with respiratory failure, hypertensive encephalopathy, psychosis, cerebellar infarction, pulmonary embolus, haemorrhagic shock and sepsis ( 3 , 15 , 17 , 18 , 19 , 20 ). This highlights the morbidity associated with elevated cortisol and catecholamines and the need for prompt normalisation.…”

Section: Discussionmentioning

confidence: 99%

Bowel perforation complicating an ACTH-secreting phaeochromocytoma

Flynn

Baqar

Liu

et al. 2016

Endocrinology, Diabetes &Amp; Metabolism Case Reports

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SummaryACTH-secreting phaeochromocytoma (ASP) is a rare cause of ACTH-dependent Cushing’s syndrome (CS). We report the case of a 63-year-old female presenting with CS secondary to an ASP complicated by bowel perforation. This case report highlights ASP as an uncommon but important cause of ectopic ACTH secretion (EAS). There have been 29 cases of ASP, all of which were unilateral and benign, but associated with significant complications. Patients presenting with ASP have the potential for cure with unilateral adrenalectomy. Given this promising prognosis if recognised, ASP should be considered in the diagnostic workup of ACTH-dependent CS. As this case demonstrates, gastrointestinal complications can arise from severe hypercortisolaemia associated with CS. Early medical and surgical intervention is imperative as mortality approaches 50% once bowel perforation occurs.Learning pointsConsider phaeochromocytoma in the diagnostic workup of ACTH-dependent CS; screen with plasma metanephrines or urinary catecholamines.Serial screening may be required if ACTH-secreting phaeochromocytoma is suspected, as absolute levels can be misleading.Early catecholamine receptor blockade and adrenal synthesis blockade may avoid the need for rescue bilateral adrenalectomy in ACTH-secreting phaeochromocytoma.Consider early medical or surgical management when gastrointestinal features are present in patients with CS, as bowel perforation due to severe hypercortisolaemia can occur and is associated with significant mortality.

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Malignant paraganglioma presenting as cushing syndrome with virilism in childhood. Production of cortisol, androgens, and adrenocorticotrophic hormone by the tumor

Kitahara

Mori

Seki

et al. 1993

Cancer

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Background. A 12‐year‐old girl with intractable retroperitoneal paraganglioma experienced increased appetite, acne, obesity, “moon face,” and enlargement of the clitoris during the course of the tumor. Plasma cortisol, serum testosterone, and dehydroepiandrosterone sulfate (DHEA‐S) levels were increased to 34.1 μg/dl, 2.0 ng/ml, and 6.628 ng/ml, respectively. Adrenocorticotrophic hormone (ACTH) levels were not increased, and results of dexamethasone suppression tests were negative. Her condition was diagnosed as Cushing syndrome with virilism. Plasma cortisol levels were increased to a level of 107.1 μg/dl before death. Methods. Tumor samples were obtained at the time of autopsy. The concentrations of cortisol, androgens, ACTH, and catecholamines were assayed in the tumor extracts. The indirect immunoperoxidase procedure was performed on fixed tissues for cortisol, DHEA‐S, testosterone, and ACTH. Results. Extracts of the tumor masses contained steroid hormones: the amount of immunoreactive cortisol was 1.64 μg/g wet weight; the amount of immunoreactive testosterone was 25.60 ng/g wet weight; immunoreactive DHEA‐S, 579.00 ng/g wet weight: and immunoreactive ACTH, 891.00 pg/g wet weight in the metastatic mass of the lung. Immunohistochemically, immunoreactive cortisol, testosterone, and DHEA‐S were detectable in the tumor cells. The adrenal gland was atrophic. Conclusions. The patient is the first reported with malignant paraganglioma with the capacity to produce cortisol, androgens, and ACTH.

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Case Report: A Case of ACTH-Producing Pheochromocytoma

Cited by 7 publications

References 13 publications

Metyrapone-responsive ectopic ACTH-secreting pheochromocytoma with a vicious cycle <i>via</i> a glucocorticoid-driven positive-feedback mechanism

Metyrapone-responsive ectopic ACTH-secreting pheochromocytoma with a vicious cycle <i>via</i> a glucocorticoid-driven positive-feedback mechanism

Bowel perforation complicating an ACTH-secreting phaeochromocytoma

Malignant paraganglioma presenting as cushing syndrome with virilism in childhood. Production of cortisol, androgens, and adrenocorticotrophic hormone by the tumor

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