An unusual case of ectopic corticotrophin-releasing hormone syndrome caused by an adrenal noncatecholamine-secreting pheochromocytoma: a case report

Wang, Baoping; Yang, Leilei; Wang, Hao; He, Qing; Ma, Zhenzhen; Lin, Yi; Jiang, Chang-Xin; Sun, Hongzan; Liu, Ming

doi:10.1186/s12902-018-0269-8

Cited by 10 publications

(4 citation statements)

References 14 publications

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“…In many instances, one set of symptoms precedes the other by many years. Wang et al described a case of Cushing’s syndrome in which the resected adrenal immunostaining was positive for CRH and phaeochromocytoma 6. Clinically she did not have any symptoms of phaeochromocytoma and 24-hour urinary metanephrines and VMA was normal.…”

Section: Discussionmentioning

confidence: 99%

Ectopic adrenocorticotrophic hormone syndrome (EAS) with phaeochromocytoma: a challenging endocrine case with a happy ending

Hatta¹,

Lekkakou

Viswananth

et al. 2019

BMJ Case Rep

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Ectopic adrenocorticotropic hormone (ACTH) syndrome (EAS) is rarely caused by a phaeochromocytoma. We report a case of a 51-year-old woman with an 8-year history of severe constipation who underwent extensive investigations including gastroscopy, colonoscopy, ultrasonography, colonic transit studies and isotope defeacography, which did not reveal any pathology other than slow colonic transit time. The unifying diagnosis of ectopic ACTH and phaeochromocytoma was made after the case was initially investigated for an adrenal incidentaloma. Multiple challenges had to be overcome prior to surgery for the functioning adrenal adenoma including management of refractory hypokalaemia, poor nutritional status, persistent hyperglycaemia, labile blood pressure and florid hypercortisolaemia driving the metabolic derangements. She underwent an uneventful left-sided adrenalectomy and required no medication thereafter with normal blood pressure, blood glucose and serum potassium and resolution of constipation and abdominal symptoms. In conclusion, patients with EAS related to phaeochromocytoma are rare and present with distinctive diagnostic and management challenges but if diagnosed successfully and managed intensively, they are curable.

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Section: Discussionmentioning

confidence: 99%

Ectopic adrenocorticotrophic hormone syndrome (EAS) with phaeochromocytoma: a challenging endocrine case with a happy ending

Hatta¹,

Lekkakou

Viswananth

et al. 2019

BMJ Case Rep

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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%

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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“…Corticotrophin-releasing hormone (CRH) and cortisol are hormones associated with tension which regulate sebaceous activity [62]. When acne-affected skin is compared to unaffected skin, extremely high levels of CRH expression are detected in the sebaceous glands [63]. CRH suppresses sebocyte proliferation, activates sebum formation, and enhances the appearance of the enzyme 5hydroxysteroid dehydrogenase, which stimulates androgens [64].…”

Section: Corticotrophin-releasing Hormone (Crh) and Cortisolmentioning

confidence: 99%

Endocrine Disorders and Hormonal Therapy for Adolescent Acne

Assawaruangsin¹

2022

ijcsrr

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Acne vulgaris is a worldwide disease which is mostly found in teenagers. This has a crucial impact on their life’s quality, particularly when endocrine disorders have been implicated. Current consideration regarding antibiotic stewardship, failing with antibiotic utilization, and the improvement of antibiotic-resistant Propionibacterium acnes, there are many treatment alternatives for pimple therapy have been acknowledged. A following analysis investigates hormonal treatments for the therapy of acne vulgaris.

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An unusual case of ectopic corticotrophin-releasing hormone syndrome caused by an adrenal noncatecholamine-secreting pheochromocytoma: a case report

Cited by 10 publications

References 14 publications

Ectopic adrenocorticotrophic hormone syndrome (EAS) with phaeochromocytoma: a challenging endocrine case with a happy ending

Ectopic adrenocorticotrophic hormone syndrome (EAS) with phaeochromocytoma: a challenging endocrine case with a happy ending

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

Endocrine Disorders and Hormonal Therapy for Adolescent Acne

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