title: Appendiceal neoplasms and pseudomyxoma peritonei: a population based study.
abstract: BACKGROUND
  Pseudomyxoma peritonei (PMP) is a rare disease with an estimated incidence of 1 per million per year, and is thought to originate usually from an appendiceal mucinous epithelial neoplasm. However it is not known exactly how often these neoplasms lead to PMP. The aim of this study is to investigate the incidence of both lesions and their relation.
  
  
  METHODS
  The nationwide pathology database of the Netherlands (PALGA) was searched for the incidence of all appendectomies, the incidence of primary epithelial appendiceal lesions and the incidence and pathology history of patients with PMP. All regarded the 10-year period of 1995-2005.
  
  
  RESULTS
  In the 10-year period 167,744 appendectomies were performed in the Netherlands. An appendiceal lesion was found in 1482 appendiceal specimens (0.9%). Nine percent of these patients developed PMP. Coincidentally, an additional epithelial colonic neoplasm was found in 13% of patients with an appendiceal epithelial lesion. A mucinous epithelial neoplasm was identified in 0.3% (73% benign, 27% malignant) of appendiceal specimens and 20% of these patients developed PMP. For mucocele and non-mucinous neoplasm the association with PMP was only 2% and 3%, respectively. From the nationwide database 267 patients (62 men and 205 women) with PMP were identified, which demonstrates an incidence of PMP in the Netherlands approaching 2 per million per year. The primary site was identified in 68% and dominated by the appendix (82%).
  
  
  CONCLUSIONS
  Primary epithelial lesions of the appendix are rare. One third of these lesions are mucinous epithelial neoplasms and especially these tumours may progress into PMP. The incidence of PMP seems to be higher than thought before. Furthermore there is a considerable risk of an additional colonic epithelial neoplasm in patients with an epithelial neoplasm at appendectomy.
authors.affiliation: Department of Surgery, The Netherlands Cancer Institute, Antoni van Leeuwenhoek Hospital, Plesmanlaan 121, 1066 CX Amsterdam, The Netherlands. r.m.smeenk@amc.uva.nl
authors.forename: R M
authors.initials: RM
authors.lastname: Smeenk
authors.forename: M L F
authors.initials: ML
authors.lastname: van Velthuysen
authors.forename: V J
authors.initials: VJ
authors.lastname: Verwaal
authors.forename: F A N
authors.initials: FA
authors.lastname: Zoetmulder
mesh_terms: D002288:Adenocarcinoma, Mucinous / Q000453:epidemiology* / Q000473:pathology / Q000601:surgery; D000293:Adolescent; D000328:Adult; D017677:Age Distribution; D000368:Aged; D000369:Aged, 80 and over; D001062:Appendectomy / Q000379:methods; D001063:Appendiceal Neoplasms / Q000453:epidemiology* / Q000473:pathology / Q000601:surgery; D016009:Chi-Square Distribution; D002648:Child; D015331:Cohort Studies; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D015994:Incidence; D007813:Laparotomy / Q000379:methods; D008297:Male; D008875:Middle Aged; D009378:Neoplasms, Multiple Primary / Q000453:epidemiology* / Q000473:pathology / Q000601:surgery; D009426:Netherlands / Q000453:epidemiology; D010534:Peritoneal Neoplasms / Q000453:epidemiology* / Q000473:pathology / Q000601:surgery; D011336:Probability; D011553:Pseudomyxoma Peritonei / Q000453:epidemiology* / Q000473:pathology / Q000601:surgery; D012042:Registries; D012189:Retrospective Studies; D018570:Risk Assessment; D017678:Sex Distribution; D016019:Survival Analysis