title: Varying features of early age-of-onset "sporadic" and hereditary nonpolyposis colorectal cancer patients.
abstract: OBJECTIVE
  Although the criteria for clinical diagnosis of hereditary nonpolyposis colorectal cancer are not fully agreed on, young age seems to be a common trait. The purpose of this study is to identify clinicopathologic features of hereditary nonpolyposis colorectal cancer in early age-of-onset colorectal cancer patients stratified as a function of family cancer history.
  
  
  METHODS
  Two hundred thirty consecutive colorectal cancer patients 40 years or older at time of diagnosis were registered into an ongoing database during a ten-year period. Accurate family history was obtained via medical records, telephone calls, and questionnaires on 146 patients. According to extent of family history of cancer, patients were stratified into seven groups: 1) fulfilling Amsterdam criteria, 2) fulfilling less strict criteria, 3) having at least one first-degree relative with colorectal cancer, 4) having at least one distant relative with colorectal cancer, 5) having at least one first-degree relative with any cancer, 6) having at least one distant relative with any cancer, 7) having no family history of cancer.
  
  
  RESULTS
  Twenty-two of 146 patients fulfilled Amsterdam and less strict hereditary nonpolyposis colorectal cancer criteria (15 percent). These hereditary nonpolyposis colorectal cancer patients were significantly younger (31 vs. 35 years; P = 0.0003) and had more metachronous colorectal cancer (27 percent vs. 2 percent; P = 0.007) and less colorectal cancer with nodal or metastatic spread than the non-hereditary nonpolyposis colorectal cancer patients (35 percent vs. 65 percent; P = 0.01).
  
  
  CONCLUSIONS
  Precise familial cancer assessment in early age-of-onset colorectal cancer increases the yield of hereditary nonpolyposis colorectal cancer diagnosis. Because of the frequent development of metachronous colorectal cancer and favorable prognosis, extensive rather than segmental surgery should be considered in early age-of-onset colorectal cancer patients belonging to hereditary nonpolyposis colorectal cancer families.
authors.affiliation: Department of Surgery, Memorial Sloan-Kettering Cancer Center, New York, New York 10021, USA.
authors.forename: J G
authors.initials: JG
authors.lastname: Guillem
authors.forename: J
authors.initials: J
authors.lastname: Puig-La Calle
authors.forename: C
authors.initials: C
authors.lastname: Cellini
authors.forename: M
authors.initials: M
authors.lastname: Murray
authors.forename: J
authors.initials: J
authors.lastname: Ng
authors.forename: M
authors.initials: M
authors.lastname: Fazzari
authors.forename: P B
authors.initials: PB
authors.lastname: Paty
authors.forename: S H
authors.initials: SH
authors.lastname: Quan
authors.forename: W D
authors.initials: WD
authors.lastname: Wong
authors.forename: A M
authors.initials: AM
authors.lastname: Cohen
mesh_terms: D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D015179:Colorectal Neoplasms / Q000175:diagnosis* / Q000401:mortality; D003123:Colorectal Neoplasms, Hereditary Nonpolyposis / Q000175:diagnosis* / Q000401:mortality / Q000473:pathology; D003937:Diagnosis, Differential; D005190:Family; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D016609:Neoplasms, Second Primary / Q000175:diagnosis; D011379:Prognosis; D015996:Survival Rate