Abstract
This report presents a case of Japanese familial pancreatic cancer (FPC) with multifocal pancreatic intraepithelial neoplasia (PanIN) lesions of the branch ducts probably associated with lobular parenchymal atrophy. The risk of pancreatic cancer is significantly increased in those associated with FPC, and this risk increases with increasing numbers of affected first-degree relatives, but there have been four Japanese cases reported. A 63-year-old Japanese male was referred to the hospital for evaluation and treatment of a pancreatic head tumor. His family history included pancreatic cancer in two-first-degree relatives and three-second-degree relatives. A pylorus-preserving pancreatoduodenectomy with a regional lymphadenectomy and intraoperative radiotherapy were performed. The histological findings of the main tumor showed a moderately differentiated tubular adenocarcinoma in the head of the pancreas without metastasis of the resected lymph nodes. Interestingly, multifocal PanIN lesions in the branch ducts were individually developed and some of these lesions were probably associated with small lesions of lobular parenchymal atrophy. He remained in good condition for 37 months after the operation. Although the concept of FPC has not been clearly established in Japan, nationwide registries of FPC are probably useful for management of FPC patients.
Similar content being viewed by others
References
Vitone LJ, Greenhalf W, Mcfaul CD, Ghaneh P, Neoptolemos JP. The inherited genetics of pancreatic cancer and prospects for secondary screening. Best Pract Res Clin Ganstroenterol. 2006;2:252–61.
Japan Pancreas Society. Report of national registration of pancreatic cancer. Suizo (J Jpn Panc Soc). 2007;22(1):e53.
Ghaneh P, Costello E, Neoptolemos JP. Biology and management of pancreatic cancer. Gut. 2007;56:1134–52.
Hruban RH, Pitman MB, Klimstra DS. Tumors of the pancreas. In: Silverg SG, Sobin LH, editors. Four series, fascicle 6. Washington D.C.: Armed Forces Institute of Pathology; 2007. p. 111–64.
Klein AP, Brune KA, Petersen GM, Goggings M, Tersmette AC, Offerhaus GJA, et al. Prospective risk of pancreatic cancer in familial pancreatic cancer kindreds. Cancer Res. 2004;64:2634–8.
Toyama K, Noto M, Sakaguchi T. Pancreatic cancer in father and daughter. Gan No Rinsho (Jpn J Cancer Clinics). 1984;30:1331–6.
Yamaguchi K, Kinoshita H, Hokazono K, Kawamoto M, Yamamoto H, Sugitani A, et al. Familial pancreatic cancer: report of one Japanese family. J Hepatobiliary Pancreat Surg. 2004;11:434–7.
Shouji H, Sekikawa K, Takakura Y, Matsuura H, Matsukawa H, Hashimoto N, et al. Autopsy case of familial pancreatic cancer in father and his daughter. Nippon Shokakibyo Gakkai Zasshi (abstract in Japanese) 2004;101:A867.
Kawaguchi K, Motoi F, Egawa S, Katayose Y, Rikiyama T, Fukuyama S, et al. A case of familial pancreatic cancer. Tohoku Igaku Zashi (abstract in Japanese) 2006;119:167.
Hurban RH, Aday NV, Albores-Saavedra J, Compton C, Garrett ES, Goodman SN, et al. Pancreatic intraepithelial neoplasia: a new nomenclature and classification system for pancreatic duct lesions. Am J Surg Pathol. 2001;25:579–86.
Hulst SPL. Zur kenntnis der Geneses des Adenokarzinomas und Karzinoms des Pankreas. Virchows Arch (B). 1905;180:288–316.
Hruban RH, Fukushima N. Pancreatic adenocarcinoma: update on the surgical pathology of carcinomas of ductal origin and PanINs. Mod Pathol. 2007;20:S61–70.
Hruban RH, Takaori K, Klimstra DS, Adsay NV, Albores-Saavedra J, Biank AV, et al. An illustrated consensus on the classification of pancreatic intraepithelial neoplasia and intraductal mucinous neoplasms. Am J Surg Pathol. 2004;28:977–87.
Hisa T, Suda K, Nobukawa B, Ohkubo H, Shiozawa S, Ishigame H, et al. Distribution of intraductal lesions in small invasive ductal carcinoma of the pancreas. Pancreatology. 2007;7:341–6.
Brune K, Abe T, Canto M, O’Malley L, Klein AP, Maitra A, et al. Multifocal neoplastic precursor lesions associated with lobular atrophy of the pancreas in patients having a strong family history of pancreatic cancer. Am J Surg Pathol. 2006;30:1067–76.
Murphy KM, Brune KA, Griffin C. Evaluation of candidate genes MAP2K4, MADH4, ACVR1B, in familial pancreatic cancer: deleterious BRCA2 mutation in 17%. Cancer Res. 2002;62:3789–93.
Goggins M, Schutte M, Lu J, Moskaluk CA, Weinstein CL, Yeo CJ, et al. Germline BRCA2 gene mutations in patients with apparently sporadic pancreatic carcinoma. Cancer Res. 1996;56:5360–4.
Bartsch DK, Sina-Frey M, Lang S, Wild A, Gerdes B, Barth P, et al. CDKN2A germline mutations in familial pancreatic cancer. Ann Surg. 2002;235:730–7.
Su GH, Hruban RH, Bova GS, Bova GS, Tang DJ, Shekher MC, et al. Germline and somatic mutations of the STK11/LKB1 Peutz-Jeghers gene in pancreatic and biliary cancers. Am J Pathol. 1999;154:1835–40.
Whitcom DC, Gorry MC, Preston RA, Furey W, Sossenheimer MJ, Ulrich CD, et al. Hereditary pancreatitis is caused by a mutation in the cationic trypsinogen gene. Nature Genet. 1996;14:141–5.
Witt H, Luck W, Hennies HC, Claβen M, Kage A, Laβ U, et al. Mutations in the gene encoding the serine protease inhibitor, Kazal type 1 are associated with chronic pancreatitis. Nature Genet. 2000;25:213–6.
Lynch HT, Voorhees GJ, Lanspa SJ, McGreevy PS, Lynch J. Pancreatic carcinoma and hereditary nonpolyposis colorectal cancer: a family study. Br J Cancer. 1985;52:271–3.
Pogue-Geile KL, Chen R, Bronner MP, Crnogorac-Jurcevic T, Moyes KW, Dowen S, et al. Palladin mutation causes familial pancreatic cancer and suggests a new cancer mechanism. PLoS Medicine. 2006;3:1–13.
Rubenstein JH, Scheiman JM, Anderson MA. A clinical and economic evaluation of endoscopic ultrasound for patients at risk for familial pancreatic adenicarcinoma. Pancreatology. 2007;5:514–25.
Brand RE, Lerch MM, Rubinstein WS, Neoptolemous JP, Whitcomb DC, Hruban RH, et al. Advances in counseling and surveillance of patients at risk for pancreatic cancer. Gut. 2007;56:1460–9.
Brentall TA. Management strategies for the patients with hereditary pancreatic cancer. Curr Treat Options Oncol. 2005;6:437–45.
Muller MW, Friess H, Kleeff J, Dahmen R, Wagner M, Hinz U, et al. Is there still a role for total pancreatectomy? Ann Surg. 2007;246:966–75.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Inagaki, M., Goto, J., Tokusashi, Y. et al. Multifocal pancreatic intraepithelial neoplasia (PanIN) lesions of the branch ducts associated with lobular parenchymal atrophy in a Japanese patient diagnosed to have familial pancreatic cancer. Clin J Gastroenterol 2, 103–108 (2009). https://doi.org/10.1007/s12328-008-0047-7
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s12328-008-0047-7