Adrenocorticotropic hormone-producing pheochromocytoma: A case report

Krylov, Vadim; Dobreva, Ekaterina; Kharnas, Sergey S.; Kuzntesov, Nikolay; Nikolenko, V. N.; Марова, Е И; Моталов, В. Г.; Levkin, V.V.; Zharikov, Yu. O.; Sinelnikov, Mikhail Y.

doi:10.1016/j.ijscr.2020.01.053

Cited by 6 publications

(8 citation statements)

References 9 publications

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“…A review by Gabi et al described the characteristics of 58 previously reported cases of ACTH-producing PCC from 1977 to 2017 [18]. After 2017, additional seven additional reports [5,7,[19][20][21][22][23] described ACTH-producing PCC cases, and we have collected and analyzed the data of 65 cases in total.…”

Section: Discussionmentioning

confidence: 99%

“…Simultaneous production of catecholamine and ACTH is even rarer, ranging from 3 to 25% of cases of ectopic ACTH syndrome [2][3][4]. The major sources of ectopic ACTH production are bronchial carcinoid tumors (36-43%), lung cancers (18-20%), and medullary thyroid cancers (3-7%) [5]. Although the negative feedback regulation of ACTH/cortisol and the positive regulation of the catecholamine/cortisol axis are well understood, paradoxical ACTH upregulation under hypercortisolemia is observed in some ectopic ACTHproducing PCCs [6][7][8].…”

Section: Introductionmentioning

confidence: 99%

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Ectopic ACTH-producing neuroendocrine tumor occurring with large recurrent metastatic pheochromocytoma: a case report

Saishouji

Maeda²,

Hamada³

et al. 2022

BMC Endocr Disord

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Background Ectopic ACTH-dependent Cushing syndrome is rarely caused by pheochromocytoma (PCC). Glucocorticoid-regulated positive feedback loops in ACTH and catecholamines were proposed in some similar cases. Case presentation We present here an 80-year-old man who had previously undergone surgery for a left adrenal PCC and newly developed severe hypertension, hypokalemia, and typical Cushingoid manifestations. Investigations revealed hyperglycemia, hypokalemia, and extremely high catecholamines and their metabolites, ACTH and cortisol. Imaging modalities showed a recurrent large left adrenal mass positively visualized with 123I-metaiodobenzylguanidine as well as somatostatin receptor scintigraphy. Surgical interventions were not indicated; thus, metyrapone, phentolamine, and doxazocin were initiated, which successfully controlled his symptoms and biochemical conditions. With the evidence that metyrapone administration decreased ACTH and catecholamine levels, the existence of positive feedback loops was speculated. During the terminal stages of the disease, additional metyrosine treatment successfully stabilized his physiological and biochemical conditions. Upon the patient’s death, pathological autopsy was performed. Immunohistochemical analysis indicated that the tumor appeared to be co-positive with tyrosine hydroxylase (TH) as well as ACTH in most tumor cells in both PCC and liver metastasis. Most cells were clearly positive for somatostatin receptor 2 staining in the membrane compartment. The dense immunostaining of ACTH, TH, dopamine-β-hydroxylase and the large tumor size with positive feedback loops may be correlated with high levels of ACTH and catecholamines in the circulation. Conclusions We experienced a case of severe ectopic ACTH producing the largest reported recurrent malignant left PCC with liver metastases that presented positive feedback loops in the ACTH/cortisol and catecholamine/cortisol axes. Clinicians should be aware of the paradoxical response of ACTH on metyrapone treatment and possible steroid-induced catecholamine crisis.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Ectopic ACTH-producing neuroendocrine tumor occurring with large recurrent metastatic pheochromocytoma: a case report

Saishouji

Maeda²,

Hamada³

et al. 2022

BMC Endocr Disord

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“…3,7,8 Whilst hypertension appears to be the commonest presentation, occurring in 93% of cases, other patients have initially presented with hypokalaemia (83%), glucocorticoid-induced phaeochromocytoma crisis (18%) or rarely incidental adrenal adenoma. [2][3][4][5][6][7][8][9][10] Our patient presented with Cushing's syndrome and venous thromboembolism (VTE), with VTE occurring in 8% of patients with Cushing's syndrome pre-operatively. [4][5][6]8,11,12 She was referred to endocrinology, in Sweden, for suspected Cushing's syndrome.…”

Section: Introductionmentioning

confidence: 99%

“…[2][3][4][5][6][7][8][9][10] Our patient presented with Cushing's syndrome and venous thromboembolism (VTE), with VTE occurring in 8% of patients with Cushing's syndrome pre-operatively. [4][5][6]8,11,12 She was referred to endocrinology, in Sweden, for suspected Cushing's syndrome. She had central obesity with a body mass index of 41 kg/m 2 , moon face, 1-2 cm wide purple abdominal striae, proximal myopathy and blood pressure of 150/85 mmHg whilst taking four antihypertensive medications (enalapril 20 mg, hydrochlorothiazide 12.5 mg, felodipine 10 mg and doxazosin 4 mg daily).…”

Section: Introductionmentioning

confidence: 99%

“…4 –6 There are likely <100 cases of ACTH-secreting phaeochromocytoma reported worldwide. 3,7,8 Whilst hypertension appears to be the commonest presentation, occurring in 93% of cases, other patients have initially presented with hypokalaemia (83%), glucocorticoid-induced phaeochromocytoma crisis (18%) or rarely incidental adrenal adenoma. 2 –10 Our patient presented with Cushing’s syndrome and venous thromboembolism (VTE), with VTE occurring in 8% of patients with Cushing’s syndrome pre-operatively.…”

Section: Introductionmentioning

confidence: 99%

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Adrenocorticotropic hormone-secreting phaeochromocytoma as a cause of treatment-resistant hypertension and recurrent pulmonary emboli

Vollmer,

Borg

2023

Journal of the Royal College of Physicians of Edinburgh

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We report an unusual case of a patient presenting with Cushing’s syndrome caused by a phaeochromocytoma secreting adrenocorticotropic hormone (ACTH). The patient had a history of treatment-resistant hypertension, secondary amenorrhoea and tendency towards hypokalaemia. She had multiple signs of Cushing’s syndrome, such as swelling, bruising, abdominal striae and proximal myopathy. Hypokalaemia is more common in patients with ectopic ACTH-secretion than other causes of Cushing’s syndrome. Computed tomography, adrenal vein sampling and biochemistry could confirm an ACTH-secreting phaeochromocytoma. It is important to consider that hypersecretion of more than one hormone may exist in a unilateral adrenal adenoma. This patient also presented with recurrent pulmonary emboli, and there is an increased risk of venous thromboembolism in patients with ACTH-secreting phaeochromocytoma. Anticoagulation should be considered for as long as the disease is active. We demonstrate that unilateral adrenalectomy can be curative in patients with ACTH-secreting phaeochromocytoma.

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Introduction to Endocrinology

Al-Suhaimi¹

2022

Emerging Concepts in Endocrine Structure and Functions

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Adrenocorticotropic hormone-producing pheochromocytoma: A case report

Cited by 6 publications

References 9 publications

Ectopic ACTH-producing neuroendocrine tumor occurring with large recurrent metastatic pheochromocytoma: a case report

Ectopic ACTH-producing neuroendocrine tumor occurring with large recurrent metastatic pheochromocytoma: a case report

Adrenocorticotropic hormone-secreting phaeochromocytoma as a cause of treatment-resistant hypertension and recurrent pulmonary emboli

Introduction to Endocrinology

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