Intraductal papillary mucinous carcinoma of the pancreas associated with pancreas divisum: a case report and review of the literature

BMC Gastroenterology

Yoshitsugu Tajima¹  Riruke Maruyama²  Seiji Yano¹  Asuka Araki²  Noriyoshi Ishikawa²  Yasunari Kawabata¹  Takeshi Nishi¹ 

[1] Department of Digestive and General Surgery, Shimane University Faculty of Medicine, 89-1 Enyacho, Izumo 693-8501, Shimane, Japan;Department of Organ Pathology, Shimane University Faculty of Medicine, 89-1 Enyacho, Izumo 693-8501, Shimane, Japan

关键词: Ventral pancreas;    Pancreas divisum;    Pancreas;    Intraductal papillary mucinous carcinoma;   

Others  :  1223122
DOI  :  10.1186/s12876-015-0313-3

 received in 2015-02-19, accepted in 2015-06-30,  发布年份 2015

【 摘 要 】

Background

Pancreas divisum, the most common congenital anomaly of the pancreas, is caused by failure of the fusion of the ventral and dorsal pancreatic duct systems during embryological development. Although various pancreatic tumors can occur in patients with pancreas divisum, intraductal papillary mucinous neoplasm is rare.

Case presentation

A 77-year-old woman was referred to our hospital because she was incidentally found to have a cystic tumor in her pancreas at a regular health checkup. Contrast-enhanced abdominal computed tomography images demonstrated a cystic tumor in the head of the pancreas measuring 40 mm in diameter with slightly enhancing mural nodules within the cyst. Endoscopic retrograde pancreatography via the major duodenal papilla revealed a cystic tumor and a slightly dilated main pancreatic duct with an abrupt interruption at the head of the pancreas. The orifice of the major duodenal papilla was remarkably dilated and filled with an abundant extrusion of mucin, and the diagnosis based on pancreatic juice cytology was “highly suspicious for adenocarcinoma”. Magnetic resonance cholangiopancreatography depicted a normal, non-dilated dorsal pancreatic duct throughout the pancreas. The patient underwent a pylorus-preserving pancreaticoduodenectomy under the diagnosis of intraductal papillary mucinous neoplasm with suspicion of malignancy arising in the ventral part of the pancreas divisum. A pancreatography via the major and minor duodenal papillae on the surgical specimen revealed that the ventral and dorsal pancreatic ducts were not connected, and the tumor originated in the ventral duct, i.e., the Wirsung’s duct. Microscopically, the tumor was diagnosed as intraductal papillary mucinous carcinoma with microinvasion. In addition, marked fibrosis with acinar cell depletion was evident in the ventral pancreas, whereas no fibrotic change was noted in the dorsal pancreas.

Conclusion

Invasive ductal carcinomas of the pancreas associated with pancreas divisum usually arise from the dorsal pancreas, in which the occurrence of pancreatic cancer may link to underlying longstanding chronic pancreatitis in the dorsal pancreas; however, the histopathogenesis of intraductal papillary mucinous neoplasm in this anomaly is a critical issue that warrants further investigation in future.

【 授权许可】

   
2015 Nishi et al.

【 图 表 】

Fig. 1.

Fig. 2.

Fig. 3.

Fig. 4.

Fig. 5.

Fig. 6.

Fig. 7.

【 参考文献 】

• [1]DiMagno MJ, Wamsteker EJ: Pancreas divisum. Curr Gastroenterol Rep. 2011, 13:150-6.
• [2]Cotton PB: Congenital anomaly of pancreas divisum as cause of obstructive pain and pancreatitis. Gut. 1980, 21:105-14.
• [3]Liao Z, Gao R, Wang W, Ye Z, Lai XW, Wang XT, et al.: A systematic review on endoscopic detection rate, endotherapy, and surgery for pancreas divisum. Endoscopy. 2009, 41:439-44.
• [4]Kim MH, Lee SS, Kim CD, Lee SK, Kim HJ, Park HJ, et al.: Incomplete pancreas divisum: is it merely a normal anatomic variant without clinical implications? Endoscopy. 2001, 33:778-85.
• [5]Kamisawa T, Tu Y, Egawa N, Tsuruta K, Okamoto A: Clinical implications of incomplete pancreas divisum. JOP. 2006, 7:625-30.
• [6]Varshney S, Johnson CD: Pancreas divisum. Int J Pancreatol. 1999, 25:135-41.
• [7]Klein SD, Affronti JP: Pancreas divisum, an evidence-based review: part I, pathophysiology. Gastrointest Endosc. 2004, 60:419-25.
• [8]Kamisawa T, Yoshiike M, Egawa N, Tsuruta K, Okamoto A, Funata N: Pancreatic tumor associated with pancreas divisum. J Gastroenterol Hepatol. 2005, 20:915-8.
• [9]Takuma K, Kamisawa T, Tabata T, Egawa N, Igarashi Y: Pancreatic diseases associated with pancreas divisum. Dig Surg. 2010, 27:144-8.
• [10]Bassi C, Dervenis C, Butturini G, Fingerhut A, Yeo C, Izbicki J, et al.: Postoperative pancreatic fistula: an international study group (ISGPF) definition. Surgery. 2005, 138:8-13.
• [11]Michael L: Exocrine Pancreas: Congenital Anomalies: Pancreas Divisum. In Sabiston Textbook of Surgery. 18th edition. Edited by Townsend CM, Beauchamp RD, Evers BM, Mattox KL. Saunders, Philadelphia; 2008:1591.
• [12]Warshaw AL, Simeone JF, Schapiro RH, Flavin-Warshaw B: Evaluation and treatment of the dominant dorsal duct syndrome (pancreas divisum redefined). Am J Surg. 1990, 159:59-66.
• [13]Zippi M, De Quarto A: Intraductal papillary mucinous neoplasm associated to pancreas divisum. J Gastoint Dig Syst. 2014, 4:171.
• [14]Origuchi N, Kimura W, Muto T, Esaki Y: Mucinproducingadenoma associated with pancreas divisum and hepatic hilar carcinoma: an autopsy case. J Gastroenterol. 1996, 31:455-9.
• [15]Thayer SP, Fernández-del Castillo C, Balcom JH, Warshaw AL: Complete dorsal pancreatectomy with preservation of the ventral pancreas: a new surgical technique. Surgery. 2002, 131:577-80.
• [16]Yarze JC, Chase MP, Herlihy KJ, Nawras A: Pancreas divisum and intraductal papillary mucinous tumor occurring simultaneously in a patient presenting with recurrent acute pancreatitis. Dig Dis Sci. 2003, 48:915.
• [17]Sakurai Y, Matsubara T, Imazu H, Hasegawa S, Miyakawa S, Ochiai M, et al.: Intraductal papillary-mucinous tumor of the pancreas head with complete absence of the ventral pancreatic duct of Wirsung. J Hepatobiliary Pancreat Surg. 2004, 11:293-8.
• [18]Sakate Y, Ohira M, Maeda K, Yamada N, Nishihara T, Nakata B, et al.: Intraductal papillary-mucinous adenoma developed in the ventral pancreas in a patient with pancreas divisum. J Hepatobiliary Pancreat Surg. 2004, 11:366-70.
• [19]Talbot ML, Foulis AK, Imrie CW: Total dorsal pancreatectomy for intraductal papillary mucinous neoplasm in a patient with pancreas divisum. Pancreatology. 2005, 5:285-8.
• [20]Akizuki E, Kimura Y, Mukaiya M, Honnma T, Koito K, Katsuramaki T, et al.: A case of intraductal papillary mucinous tumor associated with pancreas divisum. Pancreas. 2006, 32:117-8.
• [21]Scatton O, Sauvanet A, Cazals-Hatem D, Vullierme MP, Ruszniewski P, Belghiti J: Dorsal pancreatectomy: an embryology-based resection. J Gastrointest Surg. 2006, 10:434-8.
• [22]Sterling MJ, Giordano SN, Sedarat A, Belitsis K: Intraductal papillary mucinous neoplasm associated with incomplete pancreas divisum as a cause of acute recurrent pancreatitis. Dig Dis Sci. 2007, 52:262-6.
• [23]Santi L, Renzulli M, Patti C, Cappelli A, Morieri ML: First case of 2 intraductal papillary mucinous tumors of both ventral and dorsal ducts in pancreas divisum. Pancreas. 2010, 39:110-1.
• [24]Ringold DA, Yen RD, Chen YK: Direct dorsal pancreatoscopy with narrow-band imaging for the diagnosis of intraductal papillary mucinous neoplasm and pancreas divisum (with video). Gastrointest Endosc. 2010, 72:1263-4.
• [25]Nakagawa Y, Yamauchi M, Ogawa R, Watada M, Mizukami K, Okimoto T, et al.: Complete pancreas divisum with patulous minor papilla complicated by multifocal branch-duct intraductal papillary mucinous neoplasms. Endoscopy. 2013, 45:E199-200.
• [26]KC, Czapla A, Thakkar S. Acute pancreatitis: pancreas divisum with ventral duct intraductal papillary mucinous neoplasms. BMJ Case Rep. 2014;Oct 7; published online.
• [27]Tanaka M, Fernández-del Castillo C, Adsay V, Chari S, Falconi M, Jang JY, et al.: International consensus guidelines 2012 for the management of IPMN and MCN of the pancreas. Pancreatology. 2012, 12:183-97.
• [28]Wada K, Kozarek RA, Traverso LW: Outcomes following resection of invasive and noninvasive intraductal papillary mucinous neoplasms of the pancreas. Am J Surg. 2005, 189:632-7.
• [29]Suzuki Y, Atomi Y, Sugiyama M, Isaji S, Innui K, Kimura W, et al.: Japanese multiinstitutional study of intraductal papillary mucinous tumor and mucinous cystic tumor, cystic neoplasm of the pancreas: a Japanese multiinstitutional study of intraductal papillary mucinous tumor and mucinous cystic tumor. Pancreas. 2004, 28:241-6.
• [30]Traverso LW, Kozarek RA, Simpson T, Galagan KA: Pancreatic duct obstruction as a potential etiology of pancreatic adenocarcinoma: a clue from pancreas divisum. Am J Gastroenterol. 1993, 88:117-9.
• [31]Saltzberg DM, Schreiber JB, Smith K, Cameron JL: Isolated ventral pancreatitis in a patient with pancreas divisum. Am J Gastroenterol. 1990, 85:1407-10.
• [32]Sanada Y, Yoshizawa Y, Chiba M, Nemoto H, Midorikawa T, Kumada K: Ventral pancreatitis in a patient with pancreas divisum. J Pediatr Surg. 1995, 30:665-7.
• [33]Talamini G, Zamboni G, Salvia R, Capelli P, Sartori N, Casetti L, et al.: Intraductal papillary mucinous neoplasms and chronic pancreatitis. Pancreatology. 2006, 6:626-34.