Choledochal Cyst and Malignancy: A Plea for Lifelong Follow-Up

Abstract

Previous research has confirmed that patients with choledochal cyst have an elevated risk of cholangiocarcinoma and gallbladder carcinoma. Current data suggest a risk of malignancy of 6 to 30% in adults with choledochal cyst. Malignancy has also occasionally been identified in children and adolescents. Multiple factors, including the age of the patient, cyst type, histological findings, and localization, have an impact on the prognosis. Information on long-term outcomes after cyst excision is limited. However, recent data suggest a lifelong elevated risk of up to 4% of cancer development following operation. This paper presents a review of the literature on cancer in patients with choledochal cyst before and after excision. A postoperative follow-up concept that consists of annual controls of CA19–9 and abdominal ultrasound is introduced.

Keywords

choledochal cyst - cholangiocarcinoma - gallbladder carcinoma - cyst excision - follow-up

• References

• 1 Goral V. Cholangiocarcinoma: new insights. Asian Pac J Cancer Prev 2017; 18 (06) 1469-1473
• 2 Gupta A, Dixon E. Epidemiology and risk factors: intrahepatic cholangiocarcinoma. Hepatobiliary Surg Nutr 2017; 6 (02) 101-104
• 3 Doherty B, Nambudiri VE, Palmer WC. Update on the diagnosis and treatment of cholangiocarcinoma. Curr Gastroenterol Rep 2017; 19 (01) 2
• 4 Watanabe Y, Toki A, Todani T. Bile duct cancer developed after cyst excision for choledochal cyst. J Hepatobiliary Pancreat Surg 1999; 6 (03) 207-212
• 5 Lee SE, Jang J-Y, Lee Y-J. , et al; Korean Pancreas Surgery Club. Choledochal cyst and associated malignant tumors in adults: a multicenter survey in South Korea. Arch Surg 2011; 146 (10) 1178-1184
• 6 Hukkinen M, Koivusalo A, Lindahl H, Rintala R, Pakarinen MP. Increasing occurrence of choledochal malformations in children: a single-center 37-year experience from Finland. Scand J Gastroenterol 2014; 49 (10) 1255-1260
• 7 Sastry AV, Abbadessa B, Wayne MG, Steele JG, Cooperman AM. What is the incidence of biliary carcinoma in choledochal cysts, when do they develop, and how should it affect management?. World J Surg 2015; 39 (02) 487-492
• 8 Tyson GL, El-Serag HB. Risk factors for cholangiocarcinoma. Hepatology 2011; 54 (01) 173-184
• 9 Søreide K, Søreide JA. Bile duct cyst as precursor to biliary tract cancer. Ann Surg Oncol 2007; 14 (03) 1200-1211
• 10 Ronnekleiv-Kelly SM, Soares KC, Ejaz A, Pawlik TM. Management of choledochal cysts. Curr Opin Gastroenterol 2016; 32 (03) 225-231
• 11 Cerwenka H. Bile duct cyst in adults: interventional treatment, resection, or transplantation?. World J Gastroenterol 2013; 19 (32) 5207-5211
• 12 Bhavsar MS, Vora HB, Giriyappa VH. Choledochal cysts: a review of literature. Saudi J Gastroenterol 2012; 18 (04) 230-236
• 13 Soares KC, Arnaoutakis DJ, Kamel I. , et al. Choledochal cysts: presentation, clinical differentiation, and management. J Am Coll Surg 2014; 219 (06) 1167-1180
• 14 Jabłońska B. Biliary cysts: etiology, diagnosis and management. World J Gastroenterol 2012; 18 (35) 4801-4810
• 15 Xia H-T, Yang T, Liang B, Zeng JP, Dong JH. Treatment and outcomes of adults with remnant intrapancreatic choledochal cysts. Surgery 2016; 159 (02) 418-425
• 16 Liu Q-Y, Lai D-M, Gao M. , et al. MRI manifestations of adult choledochal cysts associated with biliary malignancy: a report of ten cases. Abdom Imaging 2013; 38 (05) 1061-1070
• 17 Chang J, Jang J-Y, Kang MJ, Jung W, Shin YC, Kim SW. Clinicopathologic differences in patients with gallbladder cancer according to the presence of anomalous biliopancreatic junction. World J Surg 2016; 40 (05) 1211-1217
• 18 Singham J, Yoshida EM, Scudamore CH. Choledochal cysts: part 1 of 3: classification and pathogenesis. Can J Surg 2009; 52 (05) 434-440
• 19 Todani T, Tabuchi K, Watanabe Y, Kobayashi T. Carcinoma arising in the wall of congenital bile duct cysts. Cancer 1979; 44 (03) 1134-1141
• 20 Dabbas N, Davenport M. Congenital choledochal malformation: not just a problem for children. Ann R Coll Surg Engl 2009; 91 (02) 100-105
• 21 La Pergola E, Zen Y, Davenport M. Congenital choledochal malformation: search for a marker of epithelial instability. J Pediatr Surg 2016; 51 (09) 1445-1449
• 22 Chu C-H, Chou W, Wang F, Yeh CN, Chen TC, Yeh TS. Expression profile of microRNA-200 family in cholangiocarcinoma arising from choledochal cyst. J Gastroenterol Hepatol 2016; 31 (05) 1052-1059
• 23 Katabi N, Pillarisetty VG, DeMatteo R, Klimstra DS. Choledochal cysts: a clinicopathologic study of 36 cases with emphasis on the morphologic and the immunohistochemical features of premalignant and malignant alterations. Hum Pathol 2014; 45 (10) 2107-2114
• 24 Kuyama H, Morine Y, Shimada M. , et al. Carcinogenic potential of biliary epithelium of congenital choledochal cyst model in rats: a special reference to HDAC expression. Hepatogastroenterology 2014; 61 (131) 795-801
• 25 Iwai N, Deguchi E, Yanagihara J. , et al. Cancer arising in a choledochal cyst in a 12-year-old girl. J Pediatr Surg 1990; 25 (12) 1261-1263
• 26 Fujiwara Y, Ohizumi T, Kakizaki G, Ishidate T. A case of congenital choledochal cyst associated with carcinoma. J Pediatr Surg 1976; 11 (04) 587-588
• 27 Kawamoto S, Hiraoka T, Maruta S, Watanabe E, Kanemitsu K, Tsuji T. A case of early cancer in cystic intrahepatic duct associated with congenital choledochal cyst. Hepatogastroenterology 1998; 45 (20) 428-432
• 28 Ono S, Sakai K, Kimura O, Iwai N. Development of bile duct cancer in a 26-year-old man after resection of infantile choledochal cyst. J Pediatr Surg 2008; 43 (06) E17-E19
• 29 He XD, Wang L, Liu W. , et al. The risk of carcinogenesis in congenital choledochal cyst patients: an analysis of 214 cases. Ann Hepatol 2014; 13 (06) 819-826
• 30 Price L, Kozarek R, Agoff N. Squamous cell carcinoma arising within a choledochal cyst. Dig Dis Sci 2008; 53 (10) 2822-2825
• 31 Terada T. Adenosquamous carcinoma in a congenital choledochal cyst associated with pancreatico-biliary maljunction. Pathol Int 2009; 59 (07) 482-485
• 32 Hua R, Zhang JF, Liu W, Huo YM, Sun YW. Signet-ring cell carcinoma coexisting with adenocarcinoma arising in a choledochal cyst: report of a case. Surg Today 2015; 45 (08) 1049-1052
• 33 Peng S, Cheng Y, Cheng N, Zhou Z. Neuroendocrine carcinoma developing in a choledochal cyst. Dig Liver Dis 2015; 47 (05) e7
• 34 Takahashi K, Sasaki R, Oshiro Y, Fukunaga K, Oda T, Ohkohchi N. Well-differentiated endocrine carcinoma originating from the bile duct in association with a congenital choledochal cyst. Int Surg 2012; 97 (04) 315-320
• 35 Li EL, Shi SD, Huang Y, Wu LQ. Arrangements of hepatobiliary cystadenoma complicated with congenital choledochal cyst: a case report and literature review. Medicine (Baltimore) 2015; 94 (03) e400
• 36 Ohashi T, Wakai T, Kubota M. , et al. Risk of subsequent biliary malignancy in patients undergoing cyst excision for congenital choledochal cysts. J Gastroenterol Hepatol 2013; 28 (02) 243-247
• 37 Ono S, Fumino S, Shimadera S, Iwai N. Long-term outcomes after hepaticojejunostomy for choledochal cyst: a 10- to 27-year follow-up. J Pediatr Surg 2010; 45 (02) 376-378
• 38 Kobayashi S, Asano T, Yamasaki M, Kenmochi T, Nakagohri T, Ochiai T. Risk of bile duct carcinogenesis after excision of extrahepatic bile ducts in pancreaticobiliary maljunction. Surgery 1999; 126 (05) 939-944
• 39 Kurokawa Y, Hasuike Y, Tsujinaka T. , et al. Carcinoma of the head of the pancreas after excision of a choledochal cyst. Hepatogastroenterology 2001; 48 (38) 578-580
• 40 Goto N, Yasuda I, Uematsu T. , et al. Intrahepatic cholangiocarcinoma arising 10 years after the excision of congenital extrahepatic biliary dilation. J Gastroenterol 2001; 36 (12) 856-862
• 41 Eriguchi N, Aoyagi S, Okuda K. , et al. Carcinoma arising in the pancreas 17 years after primary excision of a choledochal cysts: report of a case. Surg Today 2001; 31 (06) 534-537
• 42 Shimamura K, Kurosaki I, Sato D. , et al. Intrahepatic cholangiocarcinoma arising 34 years after excision of a type IV-A congenital choledochal cyst: report of a case. Surg Today 2009; 39 (03) 247-251
• 43 Kumamoto T, Tanaka K, Takeda K. , et al. Intrahepatic cholangiocarcinoma arising 28 years after excision of a type IV-A congenital choledochal cyst: report of a case. Surg Today 2014; 44 (02) 354-358
• 44 Baik H, Park Y-H, Seo SH. , et al. Cholangiocarcinoma in choledochal cyst after cystoenterostomy: how a mistreated choledochal cyst can progress to malignancy. Ann Hepatobiliary Pancreat Surg 2016; 20 (04) 201-203
• 45 Rush E, Podesta L, Norris M, Lugo D, Makowka L, Hiatt JR. Late surgical complications of choledochal cystoenterostomy. Am Surg 1994; 60 (08) 620-624
• 46 Todani T, Watanabe Y, Narusue M, Tabuchi K, Okajima K. Congenital bile duct cysts: classification, operative procedures, and review of thirty-seven cases including cancer arising from choledochal cyst. Am J Surg 1977; 134 (02) 263-269
• 47 Yamaguchi M. Congenital choledochal cyst. Analysis of 1,433 patients in the Japanese literature. Am J Surg 1980; 140 (05) 653-657
• 48 Lilly JR. Surgery of coexisting biliary malformations in choledochal cyst. J Pediatr Surg 1979; 14 (06) 643-647
• 49 Filler RM, Stringel G. Treatment of choledochal cyst by excision. J Pediatr Surg 1980; 15 (04) 437-442
• 50 Todani T, Watanabe Y, Toki A, Urushihara N. Carcinoma related to choledochal cysts with internal drainage operations. Surg Gynecol Obstet 1987; 164 (01) 61-64
• 51 Kaneko K, Ando H, Watanabe Y. , et al. Secondary excision of choledochal cysts after previous cyst-enterostomies. Hepatogastroenterology 1999; 46 (29) 2772-2775
• 52 Makin E, Davenport M. Understanding choledochal malformation. Arch Dis Child 2012; 97 (01) 69-72
• 53 Schmidt AI, Engelmann C, Till H, Kellnar S, Ure BM. Minimally-invasive pediatric surgery in 2004: a survey including 50 German institutions. J Pediatr Surg 2007; 42 (09) 1491-1494
• 54 Ure BM, Schier F, Schmidt AI, Nustede R, Petersen C, Jesch NK. Laparoscopic resection of congenital choledochal cyst, choledochojejunostomy, and extraabdominal Roux-en-Y anastomosis. Surg Endosc 2005; 19 (08) 1055-1057
• 55 Watanabe Y, Sato M, Tokui K, Koga S, Yukumi S, Kawachi K. Laparoscope-assisted minimally invasive treatment for choledochal cyst. J Laparoendosc Adv Surg Tech A 1999; 9 (05) 415-418
• 56 Ng WT. Bile duct carcinogenesis after excision of extrahepatic bile ducts in pancreaticobiliary maljunction. Surgery 2000; 128 (03) 492-494
• 57 Ng DWJ, Chiow AKH, Poh WT, Tan SS. Metachronous cholangiocarcinoma 13 years post resection of choledochal cyst-is long-term follow-up useful?: a case study and review of the literature. Surg Case Rep 2016; 2 (01) 60
• 58 Nishiyama R, Shinoda M, Tanabe M. , et al. Intrahepatic cholangiocarcinoma arising 33 years after excision of a choledochal cyst: report of a case. Int Surg 2011; 96 (04) 320-325
• 59 Yamataka A, Ohshiro K, Okada Y. , et al. Complications after cyst excision with hepaticoenterostomy for choledochal cysts and their surgical management in children versus adults. J Pediatr Surg 1997; 32 (07) 1097-1102
• 60 Tajiri T, Tate G, Inagaki T. , et al. Mucinous cystadenoma of the pancreas 17 years after excision of gallbladder because of a choledochal cyst. J Gastroenterol 2004; 39 (02) 181-187
• 61 Otani T, Shioiri T, Mishima H. , et al. Bile duct schwannoma developed in the remnant choledochal cyst-a case associated with total agenesis of the dorsal pancreas. Dig Liver Dis 2005; 37 (09) 705-708
• 62 Ishibashi T, Kasahara K, Yasuda Y, Nagai H, Makino S, Kanazawa K. Malignant change in the biliary tract after excision of choledochal cyst. Br J Surg 1997; 84 (12) 1687-1691
• 63 Fujisaki S, Akiyama T, Miyake H. , et al. A case of carcinoma associated with the remained intrapancreatic biliary tract 17 years after the primary excision of a choledochal cyst. Hepatogastroenterology 1999; 46 (27) 1655-1659
• 64 Lee SE, Choi YS, Kim MK, Oh HC, Do JH. Bilateral ovarian metastasis from distal common bile duct carcinoma developing after choledochal cyst excision. Korean J Hepatobiliary Pancreat Surg 2015; 19 (02) 75-77
• 65 Todani T, Watanabe Y, Mizuguchi T, Fujii T, Toki A. Hepaticoduodenostomy at the hepatic hilum after excision of choledochal cyst. Am J Surg 1981; 142 (05) 584-587
• 66 Song G, Jiang X, Wang J, Li A. Comparative clinical study of laparoscopic and open surgery in children with choledochal cysts. Saudi Med J 2017; 38 (05) 476-481
• 67 Wirth TC, Vogel A. Surveillance in cholangiocellular carcinoma. Best Pract Res Clin Gastroenterol 2016; 30 (06) 987-999