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Pathologic Classification and Biological Behavior of Pancreatic Neoplasia

  • Olca Basturk
  • Ipek Coban
  • N. Volkan Adsay
Reference work entry

Abstract:

Pancreatic neoplasms are classified according to which normal cell type of this organ they recapitulate, because the clinicopathologic and biologic characteristics of tumors are determined or manifested mostly by their cellular lineage. Most pancreatic neoplasms are of ductal lineage, characterized by tubular units, cysts, and papilla or mucin formation and expression of mucin-related glycoproteins and oncoproteins. There are also certain genes, molecules and mutations that are fairly specific to ductal neoplasms.

Invasive ductal adenocarcinoma constitutes the vast majority (>85%) of carcinomas of ductal lineage. Ductal adenocarcinoma is characterized by insidious infiltration and rapid dissemination, despite its relatively well-differentiated histologic appearance. The presumed precursors of ductal adenocarcinoma are microscopic intraductal proliferative changes that are now termed pancreatic intraepithelial neoplasia (PanIN). PanINs comprise a neoplastic transformation ranging from early mucinous change (PanIN-1A) to frank carcinoma in situ (PanIN-3). A similar neoplastic spectrum also characterizes intraductal papillary mucinous neoplasms and mucinous cystic neoplasms, which are cystic ductal-mucinous tumors with varying degrees of papilla formation, and may be associated with invasive carcinoma. Intraductal papillary mucinous neoplasms are associated with invasive carcinoma of the colloid type, which appears to be a clinicopathologically distinct tumor with indolent behavior. Conversely, mucinous cystic neoplasms are associated with invasive carcinoma of ordinary ductal adenocarcinoma type.

Although most ductal pancreatic neoplasia are characterized by some degree of mucin formation, serous tumors, of which serous cystadenoma is the sole example, lack mucin formation, presumably because they recapitulate centroacinar ducts. These are typically benign tumors.

Among non-ductal tumors of the pancreas, endocrine neoplasms are by far the most common. The vast majority of pancreatic endocrine neoplasia (PENs) is low-intermediate grade malignancies characterized by protracted clinical course. Those that are treated at an early stage are even considered “benign.” Poorly differentiated neuroendocrine (small cell) carcinomas are exceedingly uncommon and are highly aggressive tumors.

Pancreatic tumors of predominant acinar lineage, namely acinar cell carcinomas and pancreatoblastomas – the latter mostly a childhood malignancy – are uncommon, and are associated with aggressive clinical course, though not as dismal as that of ductal carcinomas. There is also a tumor in the pancreas which is of undetermined lineage: solid pseudopapillary neoplasm. It typically occurs in young females and follows a benign course in most instances.

Neoplasms may also arise from supportive tissue or may secondarily involve the pancreas. Although these are highly uncommon, they can be problematic in the differential diagnosis.

In conclusion, invasive ductal carcinoma is by far the most common tumor in the pancreas; however, occur in this organ are a plethora of neoplasms of other types with different clinicopathologic, molecular-genetic and biologic characteristics.

Keywords

Invasive Carcinoma Intraductal Papillary Mucinous Neoplasm Acinar Cell Carcinoma Pancreatic Endocrine Neoplasm Mucinous Cystic Neoplasm 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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Copyright information

© Springer Science+Business Media, LLC 2010

Authors and Affiliations

  • Olca Basturk
    • 1
  • Ipek Coban
    • 2
  • N. Volkan Adsay
    • 1
  1. 1.Department of PathologyEmory University School of Medicine and Winship Cancer InstituteAtlantaUSA
  2. 2.Department of PathologyNew York University School of MedicineNew YorkUSA

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