A Case of Recurrent Pancreatic Head Neuroendocrine Carcinoma Presented with Ectopic Acth Syndrome

Abstract

A 69-year-old woman, who had been treated for type 2 diabetes mellitus elsewhere, was referred to our hospital because high blood glucose levels persisted and an abdominal MRI showed a 1-cm irregular-shaped tumor at the pancreas head. As a result of the examination in our hospital, the patient was diagnosed with pancreatic head neuroendocrine carcinoma (NEC) cT1N0M0 cStage I and underwent pancreaticoduodenectomy. The final diagnosis was NEC pT1cN0M0 fStage I of the head of the pancreas, and the resection was performed without any residual cancer (R0 resection).

Postoperatively hyperglycemic symptoms improved temporarily, but six months later, hyperglycemia, hypokalemia and generalized edema appeared. Furthermore, multiple liver metastases were found by an abdominal CT scan. Blood tests showed high levels of ACTH 216 pg/ml and cortisol 37.5 μg/dl. Additionally, dexamethasone suppression test revealed as ectopic ACTH syndrome resulting from recurrent liver metastases. Chemotherapy using etoposide in combination with cisplatin was performed. One year has passed since the surgery. Treatment is still ongoing with the symptoms controlled by metyrapone.

Since reports of ectopic ACTH syndrome of NEC are very rare, we report the case with some literature review.