ACTH-secreting neuroendocrine pancreatic tumor: a case report

Incidence of neuroendocrine tumor (NET) is increased in the last thirty years from 1.1 to 5.2 cases per 100,000 people in the United States. They can originate from the pancreatic gland and for the majority of cases are not functioning (80%). A small percentage of functioning may produce adrenocorticotropic hormone (ACTH) and lead to ectopic ACTH Syndrome (EAS), responsible of Cushing-Syndrome.

We present a case of a 30 year-old woman suffering from EAS due to a neoformation of the pancreatic tail of the maximum diameter of 4 cm. The lesion was resectable at preoperatory imaging. The patient was subjected to distal splenopancreasectomy. Histological examination showed a well-differentiated neuroendocrine carcinoma pT3N0. The postoperative course was regular. At two years of follow-up patient is almost completely asymptomatic for Cushing's but she has developed multiple liver metastases, for which she began chemotherapy.

p-NET responsible for EAS is usually malignant and the radical treatment of excision of the lesion is not possible because they occur at the time of diagnosis with liver metastases or unresectable. Our patient had a mass at the time of diagnosis resectable but despite radical surgery, she has developed multiple liver metastases at two years and she was undergoing chemotherapy.

In agreement with previous literature we confirm the aggressive nature of pancreatic tumors secreting ACTH, despite radical surgery. Conversely, surgical treatment is effective on the resolution of clinical symptoms.