Abstract
A 25-year-old woman was referred to our hospital with persistent upper abdominal pain. Preoperative imaging studies revealed a hilar bile duct stricture with portal venous encasement, and the patient underwent curative resection involving extended left hepatectomy and segmental portal vein resection. The pathological findings demonstrated a well-differentiated tubular adenocarcinoma of the bile duct with regional lymph node metastasis (stage IIIB according to the UICC TNM classification), as well as the overexpression of p53 proteins and the K-ras gene mutation in tumor cells. The patient has shown no evidence of recurrence in the 10 months since the operation. Although there are several reports of relatively young adults with cholangiocarcinoma, the majority of such patients demonstrate either an anomalous arrangement of the pancreaticobiliary duct system or primary sclerosing cholangitis. The absence of any morphological abnormalities in this patient’s biliary system implicates de novo carcinogenesis as the most likely cause of the cholangiocarcinoma.
References
Yamamoto M, Ariizumi S. Surgical outcomes of intrahepatic cholangiocarcinoma. Surg Today. 2011;41:896–902.
Nuzzo G, Giuliante F, Ardito F, Giovannini I, Aldrighetti L, Belli G, et al. Improvement in perioperative and long-term outcome after surgical treatment of hilar cholangiocarcinoma: results of an Italian multicenter analysis of 440 patients. Arch Surg. 2012;147:26–34.
Hasumi A, Matsui H, Sugioka A, Uyama I, Komori Y, Fujita J, et al. Precancerous conditions of biliary tract cancer in patients with pancreaticobiliary maljunction: reappraisal of nationwide survey in Japan. J Hepatobiliary Pancreat Surg. 2000;7:551–5.
Nakamura H, Katayose Y, Rikiyama T, Onogawa T, Yamamoto K, Yoshida H, et al. Advanced bile duct carcinoma in a 15-year-old patient with pancreaticobiliary maljunction and congenital biliary cystic disease. J Hepatobiliary Pancreat Surg. 2008;15:554–9.
Funabiki T, Matsubara T, Miyakawa S, Ishihara S. Pancreaticobiliary maljunction and carcinogenesis to biliary and pancreatic malignancy. Langenbecks Arch Surg. 2009;394:159–69.
Deneau M, Adler DG, Schwartz JJ, Hutson W, Sorensen J, Book L, et al. Cholangiocarcinoma in a 17-year-old boy with primary sclerosing cholangitis and inflammatory bowel disease. J Pediatr Gastroenterol Nutr. 2011;52:617–20.
Bjornsson E, Angulo P. Cholangiocarcinoma in young individuals with and without primary sclerosing cholangitis. Am J Gastroenterol. 2007;102:1677–82.
Saxena A, Chua TC, Chu FC, Morris DL. Improved outcomes after aggressive surgical resection of hilar cholangiocarcinoma: a critical analysis of recurrence and survival. Am J Surg. 2011;202:310–20.
Ebata T, Nagino M, Kamiya J, Uesaka K, Nagasaka T, Nimura Y. Hepatectomy with portal vein resection for hilar cholangiocarcinoma: audit of 52 consecutive cases. Ann Surg. 2003;238:720–7.
Murakami Y, Uemura K, Sudo T, Hashimoto Y, Nakashima A, Kondo N, et al. Prognostic factors after surgical resection for intrahepatic, hilar, and distal cholangiocarcinoma. Ann Surg Oncol. 2011;18:651–8.
Kayahara M, Nagakawa T. Recent trends of gallbladder cancer in Japan: an analysis of 4,770 patients. Cancer. 2007;110:572–80.
Yeh CN, Jan YY, Chen MF. Influence of age on surgical treatment of peripheral cholangiocarcinoma. Am J Surg. 2004;187:559–63.
Matsubara T, Funabiki T, Jinno O, Sakurai Y, Hasegawa S, Imazu H, et al. p53 gene mutations and overexpression of p53 product in cancerous and noncancerous biliary epithelium in patients with pancreaticobiliary maljunction. J Hepatobiliary Pancreat Surg. 1999;6:286–93.
Noda Y, Fujita N, Kobayashi G, Ito K, Horaguchi J, Takasawa O, et al. Histological study of gallbladder and bile duct epithelia in patients with anomalous arrangement of the pancreaticobiliary ductal system: comparison between those with and without a dilated common bile duct. J Gastroenterol. 2007;42:211–8.
Nagai M, Watanabe M, Iwase T, Yamao K, Isaji S. Clinical and genetic analysis of noncancerous and cancerous biliary epithelium in patients with pancreaticobiliary maljunction. World J Surg. 2002;26:91–8.
Suto T, Habano W, Sugai T, Uesugi N, Kanno S, Saito K, et al. Infrequent microsatellite instability in biliary tract cancer. J Surg Oncol. 2001;76:121–6.
Rashid A, Ueki T, Gao YT, Houlihan PS, Wallace C, Wang BS, et al. K-ras mutation, p53 overexpression, and microsatellite instability in biliary tract cancers: a population-based study in China. Clin Cancer Res. 2002;8:3156–63.
Pandey M. Environmental pollutants in gallbladder carcinogenesis. J Surg Oncol. 2006;93:640–3.
Nagino M, Nimura Y, Nishio H, Ebata T, Igami T, Matsushita M, et al. Hepatectomy with simultaneous resection of the portal vein and hepatic artery for advanced perihilar cholangiocarcinoma: an audit of 50 consecutive cases. Ann Surg. 2010;252:115–23.
Skipworth JR, Olde Damink SW, Imber C, Bridgewater J, Pereira SP, Malago M. Review article: surgical, neo-adjuvant and adjuvant management strategies in biliary tract cancer. Aliment Pharmacol Ther. 2011;34:1063–78.
Murakami Y, Uemura K, Sudo T, Hayashidani Y, Hashimoto Y, Nakamura H, et al. Gemcitabine-based adjuvant chemotherapy improves survival after aggressive surgery for hilar cholangiocarcinoma. J Gastrointest Surg. 2009;13:1470–9.
Eudaly JA, Tizzano JP, Higdon GL, Todd GC. Developmental toxicity of gemcitabine, an antimetabolite oncolytic, administered during gestation to CD-1 mice. Teratology. 1993;48:365–81.
Meirow D. Reproduction post-chemotherapy in young cancer patients. Mol Cell Endocrinol. 2000;169:123–31.
Andersen CY, Kristensen SG, Greve T, Schmidt KT. Cryopreservation of ovarian tissue for fertility preservation in young female oncological patients. Future Oncol. 2012;8:595–608.
Chui AK, Island ER, Rao AR, Lau WY. The longest survivor and first potential cure of an advanced cholangiocarcinoma by ex vivo resection and autotransplantation: a case report and review of the literature. Am Surg. 2003;69:441–4.
Klein WM, Molmenti EP, Colombani PM, Grover DS, Schwarz KB, Boitnott J, et al. Primary liver carcinoma arising in people younger than 30 years. Am J Clin Pathol. 2005;124:512–8.
Fujiwara H, Kuroda D, Imano M, Nomura H, Kato M, Ohyanagi H. Early carcinoma of the gallbladder in a young female resected by laparoscopic cholecystectomy. Acta Med Kinki Univ. 1997;22:123–6.
Temizkan M, Ouwendijk JT, Middelkoop MP, Blankenstein M, ten Kate FJ, Tytgat GN. A young female with a cholangiocarcinoma in the past: is pregnancy allowed? Acta Gastroenterol Latinoam. 1996;26:31–4.
Suehiro T, Matsumata T, Iguchi T, Sanefuji K, Nomoto KI, Taketomi A, et al. Immunohistochemical examination of a resected advanced hilar cholangiocarcinoma arising in a 29-year-old male without primary sclerosing cholangitis. Case Rep Gastroenterol. 2010;4:144–52.
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Murakami, S., Ajiki, T., Ueno, K. et al. Curative resection of hilar cholangiocarcinoma in a 25-year-old woman: report of a case. Surg Today 44, 1350–1354 (2014). https://doi.org/10.1007/s00595-013-0574-9
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DOI: https://doi.org/10.1007/s00595-013-0574-9