title: Unusual paraneoplastic neurological syndrome secondary to a well differentiated pancreatic neuroendocrine tumor: a case report and review of the literature.
abstract: BACKGROUND
  Paraneoplastic neurological syndrome (PNS) is a heterogeneous group of disorders affecting any part of the nervous system, in a patient affected by cancer. PNS is estimated to occur in 0.01 to 8% of cancer patients, with higher incidence in those with small cell lung cancer, gynecological tumours or hematological disease. Paraneoplastic cerebellar degeneration (PCD) is the most common PNS, but it has never been reported in patients with pancreatic well-differentiated neuroendocrine tumours.
  
  
  METHODS
  A 61-year-old man presented with an unusual PNS and absence of circulating neural auto-antibodies. Subsequently, contrast-enhanced computed tomography revealed a large pancreatic mass, together with multiple liver metastases, histologically diagnosed as a well-differentiated neuroendocrine tumor. Initial treatment with long-acting somatostatin analogue (octreotide LAR) and prednisone achieved a biochemical response (reduction of chromogranin A level) and a radiological disease control, but patient experienced only a brief improvement of neurological symptoms. Seven months after the onset of the symptoms, he died from neurological impairment.
  
  
  CONCLUSIONS
  PNS can be associated with metastatic non-functioning well-differentiated pancreatic neuroendocrine tumors. These tumors may be unresponsive to treatment with somatostatin analogues and an early neurological treatment should be considered for the optimal management of these uncommon cases.
authors.affiliation: Medical Oncology, Azienda Ospedaliera Universitaria San Luigi, Regione Gonzole 10, 10043, Orbassano, Torino, Italy. mariapia.brizzi@email.it.|University of Turin, Department of Oncology, Pathology, Azienda Ospedaliera Universitaria San Luigi, Regione Gonzole 10, 10043, Orbassano, Torino, Italy. mariapia.brizzi@email.it.|Department of Oncology, University of Turin, Azienda Ospedaliera Universitaria San Luigi, Regione Gonzole 10, 10043, Orbassano, Torino, Italy. mariapia.brizzi@email.it.
authors.forename: Maria Pia
authors.initials: MP
authors.lastname: Brizzi
authors.affiliation: Medical Oncology, Azienda Ospedaliera Universitaria San Luigi, Regione Gonzole 10, 10043, Orbassano, Torino, Italy. cristina.sonetto@gmail.com.|University of Turin, Department of Oncology, Pathology, Azienda Ospedaliera Universitaria San Luigi, Regione Gonzole 10, 10043, Orbassano, Torino, Italy. cristina.sonetto@gmail.com.|Department of Oncology, University of Turin, Azienda Ospedaliera Universitaria San Luigi, Regione Gonzole 10, 10043, Orbassano, Torino, Italy. cristina.sonetto@gmail.com.
authors.forename: Cristina
authors.initials: C
authors.lastname: Sonetto
authors.affiliation: Medical Oncology, Azienda Ospedaliera Universitaria San Luigi, Regione Gonzole 10, 10043, Orbassano, Torino, Italy. marco.tampellini@unito.it.|University of Turin, Department of Oncology, Pathology, Azienda Ospedaliera Universitaria San Luigi, Regione Gonzole 10, 10043, Orbassano, Torino, Italy. marco.tampellini@unito.it.|Department of Oncology, University of Turin, Azienda Ospedaliera Universitaria San Luigi, Regione Gonzole 10, 10043, Orbassano, Torino, Italy. marco.tampellini@unito.it.
authors.forename: Marco
authors.initials: M
authors.lastname: Tampellini
authors.affiliation: Medical Oncology, Azienda Ospedaliera Universitaria San Luigi, Regione Gonzole 10, 10043, Orbassano, Torino, Italy. massimo.dimaio@unito.it.|University of Turin, Department of Oncology, Pathology, Azienda Ospedaliera Universitaria San Luigi, Regione Gonzole 10, 10043, Orbassano, Torino, Italy. massimo.dimaio@unito.it.|Department of Oncology, University of Turin, Azienda Ospedaliera Universitaria San Luigi, Regione Gonzole 10, 10043, Orbassano, Torino, Italy. massimo.dimaio@unito.it.
authors.forename: Massimo
authors.initials: M
authors.lastname: Di Maio
authors.affiliation: University of Turin, Department of Oncology, Pathology, Azienda Ospedaliera Universitaria San Luigi, Regione Gonzole 10, 10043, Orbassano, Torino, Italy. marco.volante@unito.it.
authors.forename: Marco
authors.initials: M
authors.lastname: Volante
authors.affiliation: Medical Oncology, Azienda Ospedaliera Universitaria San Luigi, Regione Gonzole 10, 10043, Orbassano, Torino, Italy. giorgio.scagliotti@unito.it.|University of Turin, Department of Oncology, Pathology, Azienda Ospedaliera Universitaria San Luigi, Regione Gonzole 10, 10043, Orbassano, Torino, Italy. giorgio.scagliotti@unito.it.|Department of Oncology, University of Turin, Azienda Ospedaliera Universitaria San Luigi, Regione Gonzole 10, 10043, Orbassano, Torino, Italy. giorgio.scagliotti@unito.it.
authors.forename: Giorgio V
authors.initials: GV
authors.lastname: Scagliotti
mesh_terms: D017809:Fatal Outcome; D006801:Humans; D008113:Liver Neoplasms / Q000556:secondary; D008297:Male; D008875:Middle Aged; D018358:Neuroendocrine Tumors / Q000175:diagnosis*; D010190:Pancreatic Neoplasms / Q000175:diagnosis*; D020361:Paraneoplastic Syndromes, Nervous System / Q000175:diagnosis*