title: Familial and hereditary prostate cancer by definition in an italian surgical series: clinical features and outcome.
abstract: OBJECTIVE
  To evaluate the clinical impact of different definitions of hereditary prostate cancer (PCa), the relative risk (RR) of relatives of PCa cases and differences in clinical-pathological features and outcome as function of a family history in a surgical series exposed to Mediterranean diet.
  
  
  METHODS
  We classified as Sporadic (SPC), Familial (FPC) or Hereditary (HPC) 606 consecutive PCa cases, 65 years old or less at diagnosis, underwent radical retropubic prostatectomy between January 1, 1987 and December 31, 2002 (mean follow-up: 6.4 years). The disease-free, overall and PCa-specific survival were also compared between SPC and non-SPC (NSPC) cases.
  
  
  RESULTS
  Overall 12.5% of cases had a positive family history. We found 14 (2.3%) HPC cases versus 16 (2.6%) taking account of X-linked transmission. Relatives of early-onset PCa cases had a higher RR to PCa (4.3) compared to late-onset PCa cases. NSPC cases had a lower frequency of positive margins status (p=0.011), perineural infiltration (p=0.028) and positive lymph nodes (p=0.005) than SPC cases, but no differences were found in major prognostic factors (preoperative PSA, Gleason sum, pathological stage) and outcome endpoints as function of a family history.
  
  
  CONCLUSIONS
  A positive family history is an important risk factor to PCa. HPC frequency is probably underestimated because of exclusion of X-linked transmission. We support the similarity between SPC and NSPC with respect to biological aggressiveness.
authors.affiliation: Urologic Clinic, Department of Surgical and Oncological Sciences, University of Padua, Italy. emilio.sacco@unipd.it
authors.forename: Emilio
authors.initials: E
authors.lastname: Sacco
authors.forename: Tommaso
authors.initials: T
authors.lastname: Prayer-Galetti
authors.forename: Francesco
authors.initials: F
authors.lastname: Pinto
authors.forename: Matteo
authors.initials: M
authors.lastname: Ciaccia
authors.forename: Simonetta
authors.initials: S
authors.lastname: Fracalanza
authors.forename: Giovanni
authors.initials: G
authors.lastname: Betto
authors.forename: Francesco
authors.initials: F
authors.lastname: Pagano
mesh_terms: D000368:Aged; D018572:Disease-Free Survival; D005500:Follow-Up Studies; D020022:Genetic Predisposition to Disease; D006801:Humans; D007558:Italy / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local / Q000453:epidemiology; D010375:Pedigree; D011379:Prognosis; D011468:Prostatectomy; D011471:Prostatic Neoplasms / Q000235:genetics* / Q000401:mortality / Q000601:surgery; D012189:Retrospective Studies; D015996:Survival Rate