title: A histomorphologic comparison of familial and sporadic pancreatic cancers.
abstract: BACKGROUND
  It is estimated that approximately 10% of pancreatic cancers have a familial component. Many inheritable genetic syndromes are associated with increased risk of pancreatic cancer, such as Peutz-Jeghers syndrome, hereditary breast-ovarian cancer and familial atypical multiple mole melanoma, but these conditions account for only a minority of familial pancreatic cancers. Previous studies have identified an increased prevalence of noninvasive precursor lesions, including pancreatic intraepithelial neoplasia, in the pancreata of patients with a strong family history of pancreatic cancer. A detailed investigation of the histopathology of invasive familial pancreatic cancer could provide insights into the mechanisms responsible for familial pancreatic cancer, as well as aid early detection and treatment strategies.
  
  
  METHODS
  We have conducted a blinded review of the pathology of 519 familial and 651 sporadic pancreatic cancers within the National Familial Pancreas Tumor Registry. Patients with familial pancreatic cancer were defined as individuals from families in which at least a pair of first-degree relatives have been diagnosed with pancreatic cancer.
  
  
  RESULTS
  Overall, there were no statistically significant differences in histologic subtypes between familial and sporadic pancreatic cancers (p > 0.05). In addition, among surgical resection specimens within the study cohort, no statistically significant differences in mean tumor size, location, perineural invasion, angiolymphatic invasion, lymph node metastasis and pathologic stage were identified (p > 0.05).
  
  
  CONCLUSIONS
  Similar to sporadic pancreatic cancer, familial pancreatic cancer is morphologically and prognostically a heterogeneous disease.
authors.affiliation: The Sol Goldman Pancreatic Cancer Research Center and Department of Pathology, Johns Hopkins University School of Medicine.
authors.forename: Aatur D
authors.initials: AD
authors.lastname: Singhi
authors.affiliation: The Sol Goldman Pancreatic Cancer Research Center and Department of Pathology, Johns Hopkins University School of Medicine.
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Ishida
authors.affiliation: The Sol Goldman Pancreatic Cancer Research Center and Department of Pathology, Johns Hopkins University School of Medicine.
authors.forename: Syed Z
authors.initials: SZ
authors.lastname: Ali
authors.affiliation: The Sol Goldman Pancreatic Cancer Research Center and Department of Pathology, Johns Hopkins University School of Medicine.|Department of Medicine, Johns Hopkins University School of Medicine.
authors.forename: Michael
authors.initials: M
authors.lastname: Goggins
authors.affiliation: Department of Medicine, Johns Hopkins University School of Medicine.
authors.forename: Marcia
authors.initials: M
authors.lastname: Canto
authors.affiliation: The Sol Goldman Pancreatic Cancer Research Center and Department of Pathology, Johns Hopkins University School of Medicine.|Department of Oncology, Johns Hopkins University School of Medicine.|Department of Surgery, Johns Hopkins University School of Medicine.
authors.forename: Christopher
authors.initials: C
authors.lastname: Wolfgang
authors.affiliation: The Sol Goldman Pancreatic Cancer Research Center and Department of Pathology, Johns Hopkins University School of Medicine.
authors.forename: Zina
authors.initials: Z
authors.lastname: Meriden
authors.affiliation: The Sol Goldman Pancreatic Cancer Research Center and Department of Pathology, Johns Hopkins University School of Medicine.
authors.forename: Nicholas
authors.initials: N
authors.lastname: Roberts
authors.affiliation: The Sol Goldman Pancreatic Cancer Research Center and Department of Pathology, Johns Hopkins University School of Medicine.|Department of Oncology, Johns Hopkins University School of Medicine.|Department of Epidemiology, Johns Hopkins Bloomberg School of Public Health, Baltimore, MD.
authors.forename: Alison P
authors.initials: AP
authors.lastname: Klein
authors.affiliation: The Sol Goldman Pancreatic Cancer Research Center and Department of Pathology, Johns Hopkins University School of Medicine.|Department of Oncology, Johns Hopkins University School of Medicine.
authors.forename: Ralph H
authors.initials: RH
authors.lastname: Hruban
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001706:Biopsy; D002277:Carcinoma / Q000235:genetics* / Q000473:pathology* / Q000601:surgery; D015331:Cohort Studies; D042241:Early Diagnosis; D005260:Female; D020022:Genetic Predisposition to Disease; D006801:Humans; D008297:Male; D008875:Middle Aged; D009361:Neoplasm Invasiveness / Q000473:pathology; D010180:Pancreatectomy; D010190:Pancreatic Neoplasms / Q000235:genetics* / Q000473:pathology* / Q000601:surgery; D011379:Prognosis; D012042:Registries; D016896:Treatment Outcome
keywords: Familial; Hereditary; Histology; Morphology; Pancreatic cancer; Pathology