title: Efficacy and Long-Term Safety of Everolimus in Pancreatic Neuroendocrine Tumor Associated with Multiple Endocrine Neoplasia Type I: Case Report.
abstract: BACKGROUND
  Approximately 10% of pancreatic neuroendocrine tumors (NET) are associated with familial syndromes, with the most common type being multiple endocrine neoplasia type 1 (MEN-1). However, the available evidence on how to treat NET comes from studies in sporadic NET.
  
  
  METHODS
  Here we report the case of a 51-year-old male patient with a metastatic MEN-1-associated pancreatic NET and hypercalcemia related to primary hyperparathyroidism and tumor-secreted parathyroid-related protein. The patient was treated with everolimus, and showed complete resolution of hypercalcemia and tumor control for 3 years when he presented with pulmonary cryptococcosis and disease progression.
  
  
  CONCLUSIONS
  This case report describes the activity of everolimus in a patient with MEN-1-associated pancreatic NET, its efficacy in treating malignant hypercalcemia associated with NET and the risk of opportunistic infections with prolonged use of this agent.
authors.affiliation: Department of Clinical Oncology, Instituto de Ensino e Pesquisa São Lucas, São Paulo, Brazil.
authors.forename: Manuel C D F
authors.initials: MC
authors.lastname: Maia
authors.forename: Delmar
authors.initials: D
authors.lastname: Muniz Lourenço
authors.forename: Rachel
authors.initials: R
authors.lastname: Riechelmann
mesh_terms: D000970:Antineoplastic Agents / Q000008:administration & dosage / Q000009:adverse effects; D000068338:Everolimus / Q000008:administration & dosage* / Q000009:adverse effects*; D006801:Humans; D008137:Longitudinal Studies; D008297:Male; D008875:Middle Aged; D009377:Multiple Endocrine Neoplasia / Q000188:drug therapy* / Q000473:pathology; D018358:Neuroendocrine Tumors / Q000188:drug therapy* / Q000473:pathology; D009894:Opportunistic Infections / Q000139:chemically induced* / Q000473:pathology / Q000517:prevention & control; D010190:Pancreatic Neoplasms / Q000188:drug therapy* / Q000473:pathology; D016896:Treatment Outcome
chemical_list: D000970:Antineoplastic Agents; D000068338:Everolimus