Familial pancreatic cancer: Concept, management and issues

Hiroyuki Matsubayashi, Kyoichi Takaori, Chigusa Morizane, Hiroyuki Maguchi, Masamichi Mizuma, Hideaki Takahashi, Keita Wada, Hiroko Hosoi, Shinichi Yachida, Masami Suzuki, Risa Usui, Toru Furukawa, Junji Furuse, Takamitsu Sato, Makoto Ueno, Yoshimi Kiyozumi, Susumu Hijioka, Nobumasa Mizuno, Takeshi Terashima, Masaki MizumotoYuzo Kodama, Masako Torishima, Takahisa Kawaguchi, Reiko Ashida, Masayuki Kitano, Keiji Hanada, Masayuki Furukawa, Ken Kawabe, Yoshiyuki Majima, Toru Shimosegawa

Research output: Contribution to journalReview article

17 Citations (Scopus)

Abstract

Familial pancreatic cancer (FPC) is broadly defined as two first-degree-relatives with pancreatic cancer (PC) and accounts for 4%-10% of PC. Several genetic syndromes, including Peutz-Jeghers syndrome, hereditary pancreatitis, hereditary breast-ovarian cancer syndrome (HBOC), Lynch syndrome, and familial adenomatous polyposis (FAP), also have increased risks of PC, but the narrowest definition of FPC excludes these known syndromes. When compared with other familial tumors, proven genetic alterations are limited to a small proportion (< 20%) and the familial aggregation is usually modest. However, an ethnic deviation (Ashkenazi Jewish > Caucasian) and a younger onset are common also in FPC. In European countries, "anticipation" is reported in FPC families, as with other hereditary syndromes; a trend toward younger age and worse prognosis is recognized in the late years. The resected pancreases of FPC kindred often show multiple pancreatic intraepithelial neoplasia (PanIN) foci, with various K-ras mutations, similar to colorectal polyposis seen in the FAP patients. As with HBOC patients, a patient who is a BRCA mutation carrier with unresectable pancreatic cancer (accounting for 0%-19% of FPC patients) demonstrated better outcome following platinum and Poly (ADP-ribose) polymerase inhibitor treatment. Western countries have established FPC registries since the 1990s and several surveillance projects for highrisk individuals are now ongoing to detect early PCs. Improvement in lifestyle habits, including non-smoking, is recommended for individuals at risk. In Japan, the FPC study group was initiated in 2013 and the Japanese FPC registry was established in 2014 by the Japan Pancreas Society.

Original languageEnglish
Pages (from-to)935-948
Number of pages14
JournalWorld Journal of Gastroenterology
Volume23
Issue number6
DOIs
Publication statusPublished - Jan 1 2017

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Pancreatic Neoplasms
Hereditary Breast and Ovarian Cancer Syndrome
Adenomatous Polyposis Coli
Registries
Pancreas
Japan
Peutz-Jeghers Syndrome
Hereditary Nonpolyposis Colorectal Neoplasms
Mutation
Platinum
Habits
Life Style
Neoplasms

All Science Journal Classification (ASJC) codes

  • Gastroenterology

Cite this

Matsubayashi, H., Takaori, K., Morizane, C., Maguchi, H., Mizuma, M., Takahashi, H., ... Shimosegawa, T. (2017). Familial pancreatic cancer: Concept, management and issues. World Journal of Gastroenterology, 23(6), 935-948. https://doi.org/10.3748/wjg.v23.i6.935

Familial pancreatic cancer : Concept, management and issues. / Matsubayashi, Hiroyuki; Takaori, Kyoichi; Morizane, Chigusa; Maguchi, Hiroyuki; Mizuma, Masamichi; Takahashi, Hideaki; Wada, Keita; Hosoi, Hiroko; Yachida, Shinichi; Suzuki, Masami; Usui, Risa; Furukawa, Toru; Furuse, Junji; Sato, Takamitsu; Ueno, Makoto; Kiyozumi, Yoshimi; Hijioka, Susumu; Mizuno, Nobumasa; Terashima, Takeshi; Mizumoto, Masaki; Kodama, Yuzo; Torishima, Masako; Kawaguchi, Takahisa; Ashida, Reiko; Kitano, Masayuki; Hanada, Keiji; Furukawa, Masayuki; Kawabe, Ken; Majima, Yoshiyuki; Shimosegawa, Toru.

In: World Journal of Gastroenterology, Vol. 23, No. 6, 01.01.2017, p. 935-948.

Research output: Contribution to journalReview article

Matsubayashi, H, Takaori, K, Morizane, C, Maguchi, H, Mizuma, M, Takahashi, H, Wada, K, Hosoi, H, Yachida, S, Suzuki, M, Usui, R, Furukawa, T, Furuse, J, Sato, T, Ueno, M, Kiyozumi, Y, Hijioka, S, Mizuno, N, Terashima, T, Mizumoto, M, Kodama, Y, Torishima, M, Kawaguchi, T, Ashida, R, Kitano, M, Hanada, K, Furukawa, M, Kawabe, K, Majima, Y & Shimosegawa, T 2017, 'Familial pancreatic cancer: Concept, management and issues', World Journal of Gastroenterology, vol. 23, no. 6, pp. 935-948. https://doi.org/10.3748/wjg.v23.i6.935
Matsubayashi H, Takaori K, Morizane C, Maguchi H, Mizuma M, Takahashi H et al. Familial pancreatic cancer: Concept, management and issues. World Journal of Gastroenterology. 2017 Jan 1;23(6):935-948. https://doi.org/10.3748/wjg.v23.i6.935
Matsubayashi, Hiroyuki ; Takaori, Kyoichi ; Morizane, Chigusa ; Maguchi, Hiroyuki ; Mizuma, Masamichi ; Takahashi, Hideaki ; Wada, Keita ; Hosoi, Hiroko ; Yachida, Shinichi ; Suzuki, Masami ; Usui, Risa ; Furukawa, Toru ; Furuse, Junji ; Sato, Takamitsu ; Ueno, Makoto ; Kiyozumi, Yoshimi ; Hijioka, Susumu ; Mizuno, Nobumasa ; Terashima, Takeshi ; Mizumoto, Masaki ; Kodama, Yuzo ; Torishima, Masako ; Kawaguchi, Takahisa ; Ashida, Reiko ; Kitano, Masayuki ; Hanada, Keiji ; Furukawa, Masayuki ; Kawabe, Ken ; Majima, Yoshiyuki ; Shimosegawa, Toru. / Familial pancreatic cancer : Concept, management and issues. In: World Journal of Gastroenterology. 2017 ; Vol. 23, No. 6. pp. 935-948.
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AU - Matsubayashi, Hiroyuki

AU - Takaori, Kyoichi

AU - Morizane, Chigusa

AU - Maguchi, Hiroyuki

AU - Mizuma, Masamichi

AU - Takahashi, Hideaki

AU - Wada, Keita

AU - Hosoi, Hiroko

AU - Yachida, Shinichi

AU - Suzuki, Masami

AU - Usui, Risa

AU - Furukawa, Toru

AU - Furuse, Junji

AU - Sato, Takamitsu

AU - Ueno, Makoto

AU - Kiyozumi, Yoshimi

AU - Hijioka, Susumu

AU - Mizuno, Nobumasa

AU - Terashima, Takeshi

AU - Mizumoto, Masaki

AU - Kodama, Yuzo

AU - Torishima, Masako

AU - Kawaguchi, Takahisa

AU - Ashida, Reiko

AU - Kitano, Masayuki

AU - Hanada, Keiji

AU - Furukawa, Masayuki

AU - Kawabe, Ken

AU - Majima, Yoshiyuki

AU - Shimosegawa, Toru

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N2 - Familial pancreatic cancer (FPC) is broadly defined as two first-degree-relatives with pancreatic cancer (PC) and accounts for 4%-10% of PC. Several genetic syndromes, including Peutz-Jeghers syndrome, hereditary pancreatitis, hereditary breast-ovarian cancer syndrome (HBOC), Lynch syndrome, and familial adenomatous polyposis (FAP), also have increased risks of PC, but the narrowest definition of FPC excludes these known syndromes. When compared with other familial tumors, proven genetic alterations are limited to a small proportion (< 20%) and the familial aggregation is usually modest. However, an ethnic deviation (Ashkenazi Jewish > Caucasian) and a younger onset are common also in FPC. In European countries, "anticipation" is reported in FPC families, as with other hereditary syndromes; a trend toward younger age and worse prognosis is recognized in the late years. The resected pancreases of FPC kindred often show multiple pancreatic intraepithelial neoplasia (PanIN) foci, with various K-ras mutations, similar to colorectal polyposis seen in the FAP patients. As with HBOC patients, a patient who is a BRCA mutation carrier with unresectable pancreatic cancer (accounting for 0%-19% of FPC patients) demonstrated better outcome following platinum and Poly (ADP-ribose) polymerase inhibitor treatment. Western countries have established FPC registries since the 1990s and several surveillance projects for highrisk individuals are now ongoing to detect early PCs. Improvement in lifestyle habits, including non-smoking, is recommended for individuals at risk. In Japan, the FPC study group was initiated in 2013 and the Japanese FPC registry was established in 2014 by the Japan Pancreas Society.

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