Association of a choledochocele and pancreatic carcinoma

Introduction and importance Pancreaticobiliary maljunction (PBM) is a rare congenital anomaly that is frequently associated with carcinoma of the biliary tract. However, there is still no clear evidence that PBM is associated with pancreatic tumors. Here we describe a case of gallbladder cancer and intraductal papillary mucinous neoplasm (IPMN) that is associated with PBM. Case presentation A 72-year-old man underwent a cholecystectomy with hepatectomy (S4a + S5) and regional lymph node dissection for gallbladder adenocarcinoma invading the front lobe branch of the hepatic artery. A pylorus-preserving pancreaticodudenectomy was also performed for pancreatic IPMN. Clinical discussion Presence of mucin type 6 (MUC6) -positive pyloric gland metaplasia in both the dilated pancreatic duct and the gallbladder background mucosa suggests that pancreatic IPMN and gallbladder cancer may have a common phenotypic origin. Additionally, analysis of 41 reported cases of pancreatic cancer associated with PBM revealed that in all metachronous multiple cancer cases, biliary tract cancer preceded the pancreatic cancer with congenital biliary dilatation accompanied by PBM. The analysis also revealed an increased proportion of pancreatic cancer cases with PBM in patients who had not undergone a flow diversion procedure located in pancreatic head. Conclusion We show an interesting relationship between pancreatic/gallbladder cancer and PBM. More comprehensive evaluations of the whole pancreaticobiliary system in follow-up of patients with PBM is required to understand the full extent of this relationship.

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“…adenoca GB (syn.) Extended cholecystectomy+PD Unk 16 Silas, et al [34] ### 75F A III Head and uncinate Adenoca. None Endoscopic drainage 4mo.…”

Section: Discussionmentioning

confidence: 99%

A case report of gallbladder cancer and pancreas cystic neoplasm associated with pancreaticobiliary maljunction

Sato

Ito

Masaki

et al. 2021

International Journal of Surgery Case Reports

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“…There are only 12 reported cases of pancreatic cancer occurring in association with a choledochal cyst or PBM (Table 1). [5][6][7][8][9][10][11][12][13][14][15] These patients consisted of seven women and five men ranging in age from 15 to 75 years (mean 44.3 years). They were younger in age and the frequency was higher in women than in patients with the more common type of pancreatic cancer because choledochal cyst is most often encountered in young women.…”

Section: Discussionmentioning

confidence: 99%

Carcinoma Arising in the Pancreas 17 Years After Primary Excision of a Choledochal Cyst: Report of a Case

Eriguchi¹,

Aoyagi²,

Okuda³

et al. 2001

Surgery Today

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We report herein the case of a 42-year-old woman in whom a pancreatic carcinoma was found 17 years after excision of a choledochal cyst with Roux-en-Y hepaticojejunostomy. Although the incidence of biliary carcinoma associated with choledochal cyst is high, reports of cancer developing in the remaining intrapancreatic biliary tract or pancreas in patients who have previously undergone primary excision of a choledochal cyst are extremely rare. Long-term follow-up is recommended even for patients who have undergone excisional surgery for a choledochal cyst, in consideration of the possibility that cancer associated with the intrapancreatic biliary tract or pancreas may develop.

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“…üf particular interest were the only two published cases of malignant degeneration involving choledochoceles (8,21). Silas (29) re ports on a 75-year old woman with the rare combination of ade nocarcinoma of the pancreas with a choledochocele; Benhidjeb (4) describes a 69-year old patient with an associated gallbladder carcinoma. The duodenallining of the cele suggests a congenital duodenal abnormality (duplication, congenital diverticulum) (23,24), removing choledochoceles from the accepted c1assifications of congenital biliary dilatations.…”

Section: Aetiology and Pathologymentioning

confidence: 99%

Choledochocele - A Rare Cause of Necrotising Pancreatitis in Childhood

Weißer¹,

Bennek²,

Hörmann³

2000

Eur J Pediatr Surg

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Recurring complaints of unknown origin in the upper abdomen during childhood are not frequent. Choledochoceles, or type III choledochal cysts, are rare congenital abnormalities of unclear aetiology. They are rarely considered in the differential diagnosis of upper abdominal symptoms in childhood. Only 130 cases of choledochoceles have been reported in world literature to date. Only 20% of these patients were children or adolescents. The symptoms do not specifically suggest a choledochocele, and are usually attributed to other disorders of the upper intestinal tract, frequently resulting in a delayed diagnosis. We present the case of a 10-year old girl in whom a congenital choledochocele was diagnosed by gastro-intestinal studies and endoscopic retrograde cholangiopancreatography (ERCP) following a 2-years history of recurrent pancreatitis. Various types of choledochoceles are presented in the literature, classified according to morphology and histology. Most authors consider them to be a form of choledochal cyst. Their embryonic development is related to that of congenital duodenal diverticula. Although congenital intraluminal duodenal dilatations are of known oncogenic significance, only two cases of adenocarcinoma arising from a choledochocele have been described to date. Established treatment of large choledochoceles in childhood is transduodenal marsupialization.

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Association of a choledochocele and pancreatic carcinoma

Cited by 15 publications

References 17 publications

A case report of gallbladder cancer and pancreas cystic neoplasm associated with pancreaticobiliary maljunction

A case report of gallbladder cancer and pancreas cystic neoplasm associated with pancreaticobiliary maljunction

Carcinoma Arising in the Pancreas 17 Years After Primary Excision of a Choledochal Cyst: Report of a Case

Choledochocele - A Rare Cause of Necrotising Pancreatitis in Childhood

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