title: ACTH-secreting neuroendocrine pancreatic tumor: a case report.
abstract: BACKGROUND
  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.
  
  
  RESULTS
  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.
  
  
  CONCLUSIONS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: University of Turin, Department of Oncology, School of Medicine, Teaching Hospital "San Luigi Gonzaga", Section of General Surgery, Regione Gonzole 10, 10043 Orbassano, Turin, Italy. Electronic address: alessandra.sur@gmail.com.
authors.forename: Alessandra
authors.initials: A
authors.lastname: Surace
authors.affiliation: University of Turin, Department of Oncology, School of Medicine, Teaching Hospital "San Luigi Gonzaga", Section of General Surgery, Regione Gonzole 10, 10043 Orbassano, Turin, Italy.
authors.forename: Alessia
authors.initials: A
authors.lastname: Ferrarese
authors.affiliation: University of Turin, Department of Oncology, School of Medicine, Teaching Hospital "San Luigi Gonzaga", Section of General Surgery, Regione Gonzole 10, 10043 Orbassano, Turin, Italy.
authors.forename: Rosa
authors.initials: R
authors.lastname: Benvenga
authors.affiliation: University of Turin, Department of Oncology, School of Medicine, Teaching Hospital "San Luigi Gonzaga", Section of General Surgery, Regione Gonzole 10, 10043 Orbassano, Turin, Italy.
authors.forename: Silvia
authors.initials: S
authors.lastname: Marola
authors.affiliation: University of Turin, Department of Oncology, School of Medicine, Teaching Hospital "San Luigi Gonzaga", Section of General Surgery, Regione Gonzole 10, 10043 Orbassano, Turin, Italy.
authors.forename: Jacopo
authors.initials: J
authors.lastname: Cumbo
authors.affiliation: University of Turin, Department of Oncology, School of Medicine, Teaching Hospital "San Luigi Gonzaga", Section of General Surgery, Regione Gonzole 10, 10043 Orbassano, Turin, Italy.
authors.forename: Matteo
authors.initials: M
authors.lastname: Rivelli
authors.affiliation: University of Turin, Department of Oncology, School of Medicine, Teaching Hospital "San Luigi Gonzaga", Section of General Surgery, Regione Gonzole 10, 10043 Orbassano, Turin, Italy.
authors.forename: Valter
authors.initials: V
authors.lastname: Martino
authors.affiliation: University of Turin, Department of Oncology, School of Medicine, Teaching Hospital "San Luigi Gonzaga", Section of General Surgery, Regione Gonzole 10, 10043 Orbassano, Turin, Italy.
authors.forename: Mario
authors.initials: M
authors.lastname: Solej
authors.affiliation: University of Turin, Department of Oncology, School of Medicine, Teaching Hospital "San Luigi Gonzaga", Section of General Surgery, Regione Gonzole 10, 10043 Orbassano, Turin, Italy.
authors.forename: Mario
authors.initials: M
authors.lastname: Nano
mesh_terms: D000324:Adrenocorticotropic Hormone / Q000378:metabolism*; D000328:Adult; D014408:Biomarkers, Tumor / Q000378:metabolism; D018278:Carcinoma, Neuroendocrine / Q000175:diagnosis* / Q000378:metabolism / Q000601:surgery; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D009682:Magnetic Resonance Spectroscopy; D010180:Pancreatectomy; D010190:Pancreatic Neoplasms / Q000175:diagnosis* / Q000378:metabolism / Q000601:surgery; D014057:Tomography, X-Ray Computed
chemical_list: D014408:Biomarkers, Tumor; D000324:Adrenocorticotropic Hormone