Adrenal vein sampling for ACTH-producing pheochromocytomas

Leader, Nicholas; Ushinsky, Alexander; Malone, Christopher

doi:10.1016/j.radcr.2021.06.055

Cited by 4 publications

(2 citation statements)

References 8 publications

(14 reference statements)

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“…A selective venous sampling from common hepatic and right internal jugular veins demonstrated an ACTH gradient of >2 suggesting the lesion as the source of EAS. Peripheral regional venous ACTH sampling has been successfully used previously in a few cases with EAS [20]. This is the first case of PHNET which was successfully evaluated with regional venous ACTH sampling, thus demonstrating its diagnostic utility in such a challenging scenario.…”

Section: Systematic Reviewmentioning

confidence: 84%

Pediatric Ectopic Cushing Syndrome Caused by Hepatic Neoplasms: A Case Report and Systematic Review

Jevalikar¹,

Ravindra²,

Reddy³

et al. 2023

Cureus

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Ectopic adrenocorticotropic hormone (ACTH) syndrome (EAS) is rare in children, and localizing the source of EAS is often challenging. Here, we report EAS in an adolescent boy who presented with Cushingoid features and had endogenous ACTH-dependent hypercortisolism on hormonal evaluation. Abdominal ultrasound and CT revealed a hepatic lesion with characteristics suggestive of hemangioma, whereas the lesion was tracer non-avid on 68 Ga-DOTANOC positron emission tomography/CT. A regional sampling of ACTH was done to confirm the hepatic lesion as the source of EAS, and a definitive ACTH gradient was observed between the hepatic vein and the right internal jugular vein. Further, a preoperative biopsy of the lesion revealed a small round cell tumor with positive immunostaining for ACTH and synaptophysin, suggestive of a neuroendocrine tumor. The patient was managed with partial hepatectomy, resulting in hormonal and clinical remission of Cushing syndrome. In a systematic review of pediatric EAS due to primary hepatic tumors (n = 11), calcifying nested stromal epithelial cell tumors were the most common. EAS-associated hepatic tumors were larger (≥10 cm) except benign primary hepatic neuroendocrine tumors (PHNET). The latter were misdiagnosed as hemangioma in two cases by anatomical imaging but correctly diagnosed by somatostatin receptor scintigraphy. Hepatic tumors causing EAS in children required extensive resection, except benign PHNET. Nevertheless, all benign tumors with an uncomplicated perioperative course demonstrated disease-free survival over a median follow-up period of two years.

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Section: Systematic Reviewmentioning

confidence: 84%

Pediatric Ectopic Cushing Syndrome Caused by Hepatic Neoplasms: A Case Report and Systematic Review

Jevalikar¹,

Ravindra²,

Reddy³

et al. 2023

Cureus

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“…AVS may be considered if ectopic ACTH secretion is suspected, and it has been used in other cases to localise and lateralise ACTH-producing phaeochromocytomas. [14][15][16] Pre-operative treatment with alpha blockade was initiated due to suspicion of a phaeochromocytoma and beta blockade was added due to palpitations. Beta blockers should not be commenced until adequate alpha blockade due to the risk of hypertensive crisis and pulmonary oedema.…”

Section: Discussionmentioning

confidence: 99%

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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