Disorder Gene (Chromosome Location) Increased Risk of Pancreatic Cancer

Hereditary nonpolyposis colorectal cancer (Lynch II variant) hMSH2 (2p22), hMLH1 (3p21) ?

Hereditary breast and ovarian cancer BRCA2 (13q12-q13) 4–10X

Familial atypical multiple mole melanoma syndrome (FAMMM) p16 (9p21) 20–35X

Hereditary pancreatitis PRSS1 (7q35) 50–80X

Peutz-Jeghers syndrome STK11/LKB1 (19p13) 130X

through the head of the pancreas. As a consequence, there is marked distention of the biliary tree in about 50% of patients with carcinoma of the head of the pancreas, and most develop

jaundice. In marked contrast, carcinomas of the body and tail of the pancreas do not impinge on the biliary tract and hence remain silent for some time. They may be quite large

and widely disseminated by the time they are discovered. Pancreatic cancers often extend through the retroperitoneal space, entrapping adjacent nerves, and occasionally invade the

spleen, adrenals, vertebral column, transverse colon, and stomach. Peripancreatic, gastric, mesenteric, omental, and portahepatic lymph nodes are frequently involved, and the liver is often

enlarged owing to metastatic deposits. Distant metastases occur, principally to the lungs and bones.

Microscopically, there is no difference between carcinomas of the head of the pancreas and those of the body and tail of the pancreas. The appearance is usually that of a moderately to

poorly differentiated adenocarcinoma forming abortive tubular structures or cell clusters and exhibiting an aggressive, deeply infiltrative growth pattern( Fig. 19-14B ). Dense

stromal fibrosis accompanies tumor invasion, and there is a proclivity for perineural invasion within and beyond the organ. Lymphatic invasion is also commonly seen. The malignant

glands are atypical, irregular, small, and bizarre and are usually lined by

Figure 19-14Carcinoma of the pancreas. A, A cross-section through the head of the pancreas and adjacent common bile duct showing both an ill-defined mass in the pancreatic substance

(arrowheads) and the green discoloration of the duct resulting from total obstruction of bile flow. B, Poorly formed glands are present in densely fibrotic stroma within the pancreatic

substance; there are some inflammatory cells.

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