title: Goblet cell carcinoids: characteristics of a Danish cohort of 83 patients.
abstract: BACKGROUND
  Appendiceal goblet cell carcinoids (GCCs) exhibit neuroendocrine and adenocarcinoma features.
  
  
  METHODS
  Analysis of demography, pathology, prognostic markers, treatment and survival in 83 GCC patients (f/m: 56/27) diagnosed 1992-2013.
  
  
  RESULTS
  Median age for f/m was 59/58 years, respectively, and similar for localized and disseminated disease. At diagnosis 54 patients had localized appendiceal disease (f/m: 29/25). According to TNM 24% had Stage I, 70% had Stage II and 6% had Stage III. Twenty-nine patients had disseminated disease (f/m: 27/2). Chromogranin A, synaptophysin and p53 were positive in >90%. Serotonin was positive in 70%. Median Ki67 index was 32% (6-75%) and higher in Tang group C (50%) compared to group A (30%; p<0.0001), and group B (30%; p<0.004). All patients had surgery. Sixty-three (76%) had radical resections including all patients with localized disease. Median OS was 83 months. The 1-, 5- and 10-year survival rates were 90%, 58%, and 38%, respectively. For localized disease OS was 164 months and 1-, 5- and 10-year survival rates were 100%, 80%, and 55%, respectively. For disseminated disease OS was 19 months and 1-, 5- and 10-year survival rates were 73%, 18% and 6%, respectively. The 1-, 5- and 10 year-survival rates for f/m were 87%/96%, 49%/76% and 31%/57%, respectively (p = 0.02). According to the Tang classification group A, B, and C OS was 118, 83 and 20 months, respectively (p = 0.0002).
  
  
  CONCLUSIONS
  The Tang classification was found to be a significant prognostic factor, while the Ki67 index was not. Localized GCCs occurred equally in males and females, while disseminated GCCs were mostly seen in females. Median age of patients with localized disease and disseminated disease was identical. Cox regression analysis found Stage IV, focally positive synaptophysin and non-radical surgery as strongest negative prognostic factors.
authors: Department of Surgical Gastroenterology, European NET Centre of Excellence, Rigshospitalet, Faculty of Health Sciences, University of Copenhagen, Copenhagen, Denmark; Department of Oncology, European NET Centre of Excellence, Rigshospitalet, Faculty of Health Sciences, University of Copenhagen, Copenhagen, Denmark; Department of Pathology, European NET Centre of Excellence, Rigshospitalet, Faculty of Health Sciences, University of Copenhagen, Copenhagen, Denmark; Department of Endocrinology, European NET Centre of Excellence, Rigshospitalet, Faculty of Health Sciences, University of Copenhagen, Copenhagen, Denmark; Department of Clinical Physiology, Nuclear Medicine and PET and Cluster for Molecular Imaging, European NET Centre of Excellence, Rigshospitalet, Faculty of Health Sciences, University of Copenhagen, Copenhagen, Denmark.
authors: Ingrid Holst
authors: IH
authors: Olsen
authors: Department of Hepatology and Gastroenterology, European NET Centre of Excellence, Aarhus University Hospital, Aarhus, Denmark.
authors: Nanna
authors: N
authors: Holt
authors: Department of Oncology, European NET Centre of Excellence, Rigshospitalet, Faculty of Health Sciences, University of Copenhagen, Copenhagen, Denmark.
authors: Seppo W
authors: SW
authors: Langer
authors: Department of Pathology, European NET Centre of Excellence, Rigshospitalet, Faculty of Health Sciences, University of Copenhagen, Copenhagen, Denmark.
authors: Jane P
authors: JP
authors: Hasselby
authors: Department of Hepatology and Gastroenterology, European NET Centre of Excellence, Aarhus University Hospital, Aarhus, Denmark.
authors: Henning
authors: H
authors: Grønbæk
authors: Department of Surgical Gastroenterology, European NET Centre of Excellence, Rigshospitalet, Faculty of Health Sciences, University of Copenhagen, Copenhagen, Denmark.
authors: Jens
authors: J
authors: Hillingsø
authors: Department of Surgical Gastroenterology, European NET Centre of Excellence, Rigshospitalet, Faculty of Health Sciences, University of Copenhagen, Copenhagen, Denmark.
authors: Masti
authors: M
authors: Mahmoud
authors: Department of Oncology, European NET Centre of Excellence, Aarhus University Hospital, Aarhus, Denmark.
authors: Morten
authors: M
authors: Ladekarl
authors: Department of Surgery, European NET Centre of Excellence, Aarhus University Hospital, Aarhus, Denmark.
authors: Lene H
authors: LH
authors: Iversen
authors: Department of Clinical Physiology, Nuclear Medicine and PET and Cluster for Molecular Imaging, European NET Centre of Excellence, Rigshospitalet, Faculty of Health Sciences, University of Copenhagen, Copenhagen, Denmark.
authors: Andreas
authors: A
authors: Kjær
authors: Department of Pathology, European NET Centre of Excellence, Rigshospitalet, Faculty of Health Sciences, University of Copenhagen, Copenhagen, Denmark.
authors: Birgitte H
authors: BH
authors: Federspiel
authors: Department of Surgical Gastroenterology, European NET Centre of Excellence, Rigshospitalet, Faculty of Health Sciences, University of Copenhagen, Copenhagen, Denmark; Department of Endocrinology, European NET Centre of Excellence, Rigshospitalet, Faculty of Health Sciences, University of Copenhagen, Copenhagen, Denmark.
authors: Ulrich
authors: U
authors: Knigge
mesh_terms: D000328:Adult; D000368:Aged; D002276:Carcinoid Tumor / Q000000981:diagnostic imaging / Q000453:epidemiology* / Q000473:pathology / Q000628:therapy*; D015331:Cohort Studies; D016208:Databases, Factual; D003718:Denmark / Q000453:epidemiology; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009367:Neoplasm Staging; D011184:Postoperative Period; D011379:Prognosis; D011877:Radionuclide Imaging; D017481:Receptors, Somatostatin / Q000378:metabolism; D012189:Retrospective Studies; D016019:Survival Analysis
chemical_list: D017481:Receptors, Somatostatin