Pancreatic cancer, an often fatal disorder affecting about 1% of the American population, is not usually considered a “Jewish” genetic disease, but researchers at the National Familial Pancreas Tumor Registry at Johns Hopkins University would beg to differ. The NFPTR operates a special sub-registry to specifically track pancreatic cancer patients and their relatives among the Ashkenazic Jewish community, and researchers at the registry are gradually making inroads in deciphering the disease’s genetic roots.
The NFPTR was founded in 1994 by Ralph Hruban, a professor of pathology and oncology at The Johns Hopkins University School of Medicine. “When we noticed that a lot of patients had a family history of pancreatic cancer, it was the natural thing to start a registry,” he told the Forward. Hruban now directs the Sol Goldman Pancreatic Cancer Research Center, which was endowed by the Sol Goldman Charitable Trust in 2002 and coordinates all the different pancreatic cancer research projects at Johns Hopkins.
According to Alison Klein, the NFPTR’s current director, the rationale behind using a registry to track cases of pancreatic cancer is twofold. “The first goal is obviously to identify pancreatic cancer genes,” she said. “We know from studies conducted in the registry over the last 10 to 15 years that pancreas cancer, like other cancers, does tend to cluster in families.” By tracing those familial patterns and identifying inherited genetic mutations, researchers can isolate specifically which genetic mutations are responsible for the development of pancreatic cancer.
The second goal, according to Klein, is to identify specific individuals predisposed to familial pancreatic cancer — usually defined as someone with two close relatives who have developed pancreatic cancer — and help them catch the disease as early as possible. Pancreatic cancer is fatal more than 95% of the time, but this is mostly because it is often caught very late. The symptoms of the cancer, which include jaundice and distended gall bladder, rarely manifest until the later stages of the disease, when treatment is all but impossible. By identifying people at risk for familial pancreatic cancer and tracking their pancreatic activity using endoscopic ultrasounds, the NFPTR hopes to save as many potential victims as possible.
While experts have known for years that certain groups, including African Americans, are more disposed to pancreatic cancer, interest in the Ashkenazic Jewish population did not develop until a few years ago. According to Klein, the sub-registry for Ashkenazim was created because of some early studies that have shown that pancreatic cancer may be more common among that population. As Klein also noted, the fact that Askhenazic Jews represent a relatively distinct population among Americans makes them especially useful in tracking genetic mutation. “Askhenazi Jews also have a unique population ancestry; they’re well suited for genetic studies,” she said.
As of now, the registry has enrolled 2,443 families, 17% of whom report Ashkenazic ancestry. This makes the NFPTR one of the largest pancreatic cancer registries in the country and the only one that specifically tracks Ashkenazic Jews. But according to Klein, there is still a lot of work to be done by NFPTR and the medical community as a whole. “While various genes for breast cancer, for example, have been discovered, we’re still at the early stages with pancreas cancer,” she said. “In order to better understand pancreatic cancer in the Ashkenazi Jewish population, the enrollment of additional families is necessary.”
For more information on the National Familial Pancreatic Tumor Registry at Johns Hopkins University, please contact coordinator Emily Palmisano by phone at (410) 955-3502 or by e-mail at firstname.lastname@example.org. You can also access NFPTR’s Web site at http://pathology.jhu.edu/pancreas/PartNFPTR.php