A case of severe ectopic ACTH syndrome from an occult primary – diagnostic and management dilemmas

Endocrinology, Diabetes & Metabolism Case Reports

article

Harish Venugopal¹  Katherine Griffin¹  Saima Amer¹ 

[1] Department of Diabetes and Endocrinology, Gold Coast University Hospital, 1 Hospital Boulevard

关键词: Adult;    Male;    White;    Australia;    Adrenal;    Pituitary;    Endocrine-related cancer;    ACTH;    Cortisol;    Glucocorticoids;    Hyperparathyroidism (primary);    Parathyroid adenoma;    Cushing's syndrome;    Ectopic Cushing syndrome;    Hypercortisolaemia;    Muscle atrophy;    Myasthaenia;    Haematoma;    Dexamethasone suppression (high dose);    Inferior petrosal sinus sampling;    Dexamethasone suppression (low dose);    Cortisol;    free (24-hour urine);    Cortisol (serum);    ACTH;    Potassium;    Bicarbonate;    CRH stimulation;    Adrenalectomy;    Percutaneous adrenal ablation;    Ketoconazole;    Hydrocortisone;    Glucocorticoids;    Metyrapone;    Radiology/Rheumatology;    Surgery;    Novel treatment;    December;    2015;   

DOI  :  10.1530/EDM-15-0099

学科分类：血液学

来源: Bioscientifica Ltd.

【 摘 要 】

Resection of primary tumour is the management of choice in patients with ectopic ACTH syndrome. However, tumours mayremain unidentified or occult in spite of extensive efforts at trying to locate them. This can, therefore, pose a majormanagement issue as uncontrolled hypercortisolaemia can lead to life-threatening infections. We present the case of a66-year-old gentleman with ectopic ACTH syndrome from an occult primary tumour with multiple significant complicationsfrom hypercortisolaemia. Ectopic nature of his ACTH-dependent Cushing’s syndrome was confirmed by non-suppression withhigh-dose dexamethasone suppression test and bilateral inferior petrosal sinus sampling. The primary ectopic sourceremained unidentified in spite of extensive anatomical and functional imaging studies, including CT scans and Dotatate-PETscan. Medical adrenolytic treatment at maximum tolerated doses failed to control his hypercortisolaemia, which led torecurrent intra-abdominal and pelvic abscesses, requiring multiple surgical interventions. Laparoscopic bilateraladrenalectomy was considered but decided against given concerns of technical difficulties due to recurrent intra-abdominalinfections and his moribund state. Eventually, alcohol ablation of adrenal glands by retrograde adrenal vein approach wasattempted, which resulted in biochemical remission of Cushing’s syndrome. Our case emphasizes the importance ofaggressive management of hypercortisolaemia in order to reduce the associated morbidity and mortality and alsodemonstrates that techniques like percutaneous adrenal ablation using a retrograde venous approach may be extremelyhelpful in patients who are otherwise unable to undergo bilateral adrenalectomy.

【 授权许可】

CC BY-NC-ND