title: Calcitonin-secreting pancreatic endocrine tumors: systematic analysis of a rare tumor entity.
abstract: OBJECTIVE
  Pancreatic endocrine tumors (PETs) are characterized by the presence of hormone syndromes. Reports focusing on calcitonin-secreting PET (CTsPETs) are very rare. This study aimed to define a CTsPET-associated syndrome in regard to chemical, anatomical, and developmental aspects.
  
  
  METHODS
  A computerized MEDLINE search was conducted under the search items: "pancreatic endocrine tumor," "calcitonin," "neuroendocrine pancreatic tumor," and "pancreas." Results of clinical, histopathological, immunohistochemical, and biochemical assessments of all patients identified with CTsPET were registered and statistically analyzed.
  
  
  RESULTS
  Thirty-seven patients with CTsPET were identified. Mean serum calcitonin was elevated to the 89.2-fold of the upper reference value. Main symptoms were watery diarrhea (51.4%) and abdominal pain (35.1%). Most patients (59.5%) presented with metastatic spread at the time of diagnosis. Of all patients, 66.7% were alive after a mean follow-up of 28.9 months. Survival was higher in patients who underwent more aggressive surgical therapies independent from tumor sizes and in those with no metastases at the time of diagnosis.
  
  
  CONCLUSIONS
  High calcitonin levels should always raise suspicion of medullary thyroid carcinomas. However, when thyroid examination remains without pathological findings, a CTsPET should be excluded. An aggressive surgical approach even in cases with large primary tumor sizes may lead to a longer survival.
authors.affiliation: Department of Visceral, Thoracic and Vascular Surgery, Philipps University Marburg, Germany. Ralph.Schneider@med.uni-marburg.de
authors.forename: Ralph
authors.initials: R
authors.lastname: Schneider
authors.forename: Jens
authors.initials: J
authors.lastname: Waldmann
authors.forename: Zaher
authors.initials: Z
authors.lastname: Swaid
authors.forename: Annette
authors.initials: A
authors.lastname: Ramaswamy
authors.forename: Volker
authors.initials: V
authors.lastname: Fendrich
authors.forename: Detlef K
authors.initials: DK
authors.lastname: Bartsch
authors.forename: Katja
authors.initials: K
authors.lastname: Schlosser
mesh_terms: D015746:Abdominal Pain / Q000209:etiology; D000328:Adult; D000368:Aged; D002116:Calcitonin / Q000097:blood / Q000378:metabolism*; D018278:Carcinoma, Neuroendocrine; D017024:Chemotherapy, Adjuvant; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009361:Neoplasm Invasiveness; D010180:Pancreatectomy; D010190:Pancreatic Neoplasms / Q000150:complications / Q000378:metabolism* / Q000401:mortality / Q000473:pathology / Q000628:therapy; D016019:Survival Analysis; D015996:Survival Rate; D013964:Thyroid Neoplasms / Q000175:diagnosis; D013997:Time Factors; D016896:Treatment Outcome; D003969:Vipoma / Q000209:etiology
chemical_list: D002116:Calcitonin