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Endocrine Abstracts (2016) 41 EP262 | DOI: 10.1530/endoabs.41.EP262

Vall d’Hebron, Barcelona, Spain.

Background: The Ectopic ACTH Syndromes (EAS) often associated with severe hypercortisolism are really a challenge.

Case presentation: We report the case of a 48-year-old woman, newly diagnosed with hypertension, hypokalemia despite renin-angiotensin blockers intake, and complaints of intense fatigue, muscular weakness, and easy bruising, which worse in four months. EAS was biochemically confirmed, but two urinary catecholamines collection were negatives despite a left adrenal topography in all image studies included MIBG was found. The patient dramatically worse after lanreotide treatment, we hypothesize, the lack of response to somatostatin analogues could be explained because of the extremely high hypercortisolism that downregulate somatostatin receptor type 2 expression, so after ketoconazole therapy and cortisol normalization, we repeat SSRS scan, but we do not find any change. Finally adrenal left gland and an associated extra adrenal ganglioneuroma were removed. Surprisingly only the adrenal gland had significant staining of ACTH. After removal, blood pressure was normalized without pills, hypercortisolemic parameters and the clinical symptoms were resolved.

Conclusion: We highlight surgery, if is feasible, in EAS, is ever the best choice and can be curative. Regarding pharmacological options we emphasized glucocorticoids can decrease the somatostatin receptor type 2, expression in tumor, with diagnostic and treatment implications. Finally we propose 68Gallium-SSTR-PET/CT, and pasireotide with somatostatin receptor type 5 affinity, could be better diagnostic and treatment alternatives in patients with very high cortisol levels.

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