Rare ductal adenocarcinoma of the pancreas in patients younger than age 40 years

Cancer. 2004 Jan 1;100(1):173-82. doi: 10.1002/cncr.11860.


Background: Pancreatic ductal adenocarcinomas (PDACs) are extremely rare before age 40 years. The objective of the current study was to determine whether the features of PDACs in patients age < 40 years differ from those in older patients. The authors reviewed the literature and their own files.

Methods: The cases reported in the literature were evaluated to determine their precise diagnoses and characteristic features. In a series of 439 PDACs from the authors' files, tumors in patients age < 40 years were identified, and their clinicopathologic features and certain genetic features were compared with those in a selected group of patients age > 40 years.

Results: Of 71 pancreatic carcinomas reported in patients age < 40 years, only 20 fully qualified as PDACs. The remaining tumors represented malignancies other than PDACs, such as pancreatoblastoma, solid-pseudopapillary neoplasms, acinar cell carcinomas, and endocrine tumors. PDACs in patients age < 20 years were the absolute exception and commonly were associated with risk factors such as Peutz-Jeghers syndrome, hereditary pancreatic cancer syndrome, and preceding radiotherapy. In the authors' series of patients, there were 6 PDACs and 4 PDAC variants in patients age < 40 years (0.2%), all in male patients. These tumors compared well with the PDACs in patients age > 40 years in their pathologic and molecular findings. Three patients were age < or = 20 years, and 2 of those patients had a mucinous component with MUC2 positivity.

Conclusions: The incidence of PDACs in patients age < 40 years was approximately 0.3%, and the incidence in patients age < 20 years was 0.1%. Their clinicopathologic findings were comparable to those in patients age > 40 years, but they seemed to include more variants, particularly mucinous carcinomas. In addition, PDACs in younger patients frequently appeared to be associated with genetic factors.

Publication types

  • Review

MeSH terms

  • Adenocarcinoma / epidemiology
  • Adenocarcinoma / pathology*
  • Adolescent
  • Adult
  • Age of Onset
  • Carcinoma, Pancreatic Ductal / epidemiology
  • Carcinoma, Pancreatic Ductal / genetics
  • Carcinoma, Pancreatic Ductal / pathology*
  • Child
  • Female
  • Genetic Predisposition to Disease*
  • Humans
  • Incidence
  • Male
  • Pancreatic Neoplasms / epidemiology
  • Pancreatic Neoplasms / genetics
  • Pancreatic Neoplasms / pathology*
  • Risk Factors
  • Sex Factors