An acute and severe immunodeficiency syndrome due to a pancreatic ACTH-producing tumor

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