Well-Differentiated Endocrine Carcinoma Originating From the Bile Duct in Association With a Congenital Choledochal Cyst

Abstract

We encountered a rare case of a well-differentiated endocrine carcinoma originating from the bile duct in association with a congenital choledochal cyst (CCC). The patient is a 28-year-old woman referred to our clinic for pruritus. Laboratory data showed mild elevation of serum hepatobiliary enzymes. Computed tomography and magnetic resonance imaging demonstrated pancreatobiliary maljunction and a Todani type IV-A CCC from the inferior bile duct to the bilateral intrahepatic bile ducts. A solid tumor was detected in the middle portion of the common bile duct. Pancreatoduodenectomy and total extrahepatic bile duct resection was performed. Based on pathologic and immunohistochemical examinations, a diagnosis of well-differentiated endocrine carcinoma was made according to the World Health Organization criteria. To our knowledge, this is the third report of a neuroendocrine tumor originating from the bile duct in association with a CCC.

Neuroendocrine tumor of distal bile duct

“…2,4 These tumors are known to occur in the background of chronic inflammation with consequent metaplasia 2,4 ; however, gallstones and choledochal cyst have been associated only in a minority of cases. 2,5 These tumors are known to occur in middle age (median age 47 years) with slight female preponderance, as in our case. 2 Most tumors are nonfunctional with only four reported functional cases -one case had diarrhea and other three had recurrent peptic ulceration due to secretion of serotonin and gastrin, respectively.…”

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

“…32,33 Additionally, Takahashi et al presented a 28-year-old woman with welldifferentiated endocrine carcinoma. 34 Li et al reported on a case with choledochal cyst and hepatobiliary cystadenoma resulting in a left hemihepatectomy with concomitant resection of the tumor and the cyst. 35…”

Non-functioning neuroendocrine tumors of the common hepatic duct: a case report and literature review

“…13 As for the management, there is no specific data concerning the extra pancreatic non GIT (ectopic pancreas) treatment, but surgery is still the only curative option combined to a multidisciplinary approach achieving a long post-operative disease free survival (DFS). 10,14,15,18 In the sporadic and locally advanced cases, no data support de-bulking surgery, but aggressive resection must be performed aiming for a R0 resection. 30 Circumferential invasion of either the portal vein with portal cavernoma or the superior mesenteric artery is considered a contraindication for surgery.…”

“…38 Some cases have been seen in the setting of choledochal cyst. 9,15 One case was associated with a Hodgkin Lymphoma of the duodenum and another case was described in the setting of Von Hippel Lindau syndrome. 18,20 Like all NET, surgery is the best treatment option and different strategies have been adopted according to the tumor location.…”