Chief Cushie ~MaryO~ Posted March 4 Chief Cushie Report Share Posted March 4 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 B alliet, c severi, t veekmans, j cuypers, h topal, c m deroose, t roskams, m bex, j dekervel 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. From https://www.physiciansweekly.com/fulminant-ectopic-cushings-syndrome-caused-by-metastatic-small-intestine-neuroendocrine-tumour-a-case-report-and-review-of-the-literature/ Quote Link to comment Share on other sites More sharing options...
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.
Recommended Posts
Join the conversation
You can post now and register later. If you have an account, sign in now to post with your account.