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.
© Acta Gastro-Enterologica Belgica.
ABOUT THE CONTRIBUTORS
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B Alliet
Department of Gastroenterology, UZ Leuven, Leuven, Belgium.
C Severi
Department of Gastroenterology, ZOL, Genk, Belgium.
T Veekmans
Department of Pathology, UZ Leuven, Leuven, Belgium.
J Cuypers
Department of Endocrinology, AZ Turnhout, Turnhout, Belgium.
H Topal
Department of Abdominal Surgery, UZ Leuven, Leuven, Belgium.
C M Deroose
Department of Nuclear Medicine, UZ Leuven, Leuven, Belgium.
T Roskams
Department of Pathology, UZ Leuven, Leuven, Belgium.
M Bex
Department of Endocrinology, UZ Leuven, Leuven, Belgium.
J Dekervel
Department of Gastroenterology – Digestive Oncology, UZ Leuven, Leuven, Belgium.
