A case of ectopic ACTH secretion

Monica Cevenini, Elena Guidetti, Maria Camilla Fabbri, Eleonora Galassi, Paola Tomassetti, Roberto Corinaldesi

DOI: https://doi.org/10.7175/cmi.v6i1S.492


We report the case of a 48-year-old woman, with a rapidly progressing ACTH neuroendocrine tumour of the pancreas (PNET) and multiple liver metastases. The patient had previously suffered from a peptic ulcer which was responsive to PPI inhibitors and hypertension which was poorly controlled by therapy. Admitted to the hospital for severe asthenia and abdominal pain, she was diagnosed with poorly differentiated PNET with liver metastases, which were positive for synaptophysin, cytokeratin 7 and 9 and neuron specific enolase (NSE). Octreoscan scintigraphy was positive for somatostatin receptors in the pancreas and in two liver lesions. A rapidly progressive Cushing’s syndrome developed, presenting with the classical physical symptoms, hypokalemia and Lysteria monocytogenes meningitis. Ectopic ACTH production was confirmed and eventually the patient died from a septic shock within two months. The case reported focuses on the malignity and the rapid progression of an ACTH-producing PNET and calls attention to the possible fatal progression of these cases.


Ectopic adrenocorticotropic hormone syndrome; Cushing’s syndrome; NETs; Pancreatic neuroendocrine tumours

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