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Fulminant ectopic Cushing’s syndrome caused by metastatic small intestine neuroendocrine tumour – a case report and review of the literature

Journal Volume 87 - 2024
Issue Fasc.1 - Case reports
Author(s) B. Alliet 1, C. Severi 2, T. Veekmans 3, J. Cuypers 4, H. Topal 5, C. M. Deroose 6, T. Roskams 3, M. Bex 7, J. Dekervel 8
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PAGES 48-51
(1) Department of Gastroenterology, UZ Leuven, Leuven, Belgium
(2) Department of Gastroenterology, ZOL, Genk, Belgium
(3) Department of Pathology, UZ Leuven, Leuven, Belgium
(4) Department of Endocrinology, AZ Turnhout, Turnhout, Belgium
(5) Department of Abdominal Surgery, UZ Leuven, Leuven, Belgium
(6) Department of Nuclear Medicine, UZ Leuven, Leuven, Belgium
(7) Department of Endocrinology, UZ Leuven, Leuven, Belgium
(8) Department of Gastroenterology - Digestive Oncology, UZ Leuven, Leuven, Belgium

Cushing’s syndrome (CS) secondary to adrenocorticotropic hormone (ACTH) producing tumours is a severe condition with a challenging diagnosis. Ectopic ACTH-secretion often involves neuroendocrine tumours (NET) in the respiratory tract. ACTH-secreting small intestine neuro-endocrine tumours (siNET) are extremely rare entities barely reported in literature. This review is illustrated by the case of a 75-year old woman with fulminant ectopic CS caused by a ACTH-secreting metastatic siNET. Severe hypokalemia, fluid retention and refractory hypertension were the presenting symptoms. Basal and dynamic laboratory studies were diagnostic for ACTH-dependent CS. Extensive imaging studies of the pituitary and thorax-abdomen areas were normal, while [68Ga]Ga-DOTATATE PET-CT revealed increased small intestine uptake in the left iliac fossa. The hypercortisolism was well controlled with somatostatin analogues, after which a debulking resection of the tumour was performed. Pathological investigation confirmed a well-differentiated NET with sporadic ACTH immunostaining and post-operative treatment with somatostatin analogues was continued with favourable disease control.

Keywords: neuroendocrine tumour, Cushing’s syndrome, Paraneoplastic, ACTH, Somatostatin receptor.

The authors declare that they have no conflict of interest.
© Acta Gastro-Enterologica Belgica.
PMID 38431792