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BACKGROUND: Choledochal cysts (CC) are congenital bile duct dilatations, intra-and/or extrahepatic, that can be associated with an anomalous pancreaticobiliary junction (APBJ). CCs are thought to predispose patients to an increased risk of cholangiocarcinoma. We hypothesized that the presence of APBJ with CC may lead to other biliary tree malignancies. METHODS: This is a retrospective cohort study including all patients managed at our institution for CC and/or APBJ from 1998 to 2018. CC and APBJ was confirmed with either magnetic resonance cholangiopancreatography or endoscopic retrograde cholangiopancreatography and classified according to the Todani classification. APBJ were classified according to the Komi classification. RESULTS: A total of 103 patients with CCs were identified with a mean age was 44.53 [18–74] and follow-up for a mean 2.9 [0–16] years. Patients were most commonly identified as Caucasian (n=62), followed by Asian (n=16) and Hispanic (n=10). Sixty-two (60%) of the patients had an APBJ: 46 (45%) P-C type, 13 (13%) C-P type and 3 (3%) complex type. Type I CC were the most common (n=73), followed by type IV CC (n=18), type III (n=5), type V (n=4) and type II (n=3). CC had a mean size of 23 [6–64] mm. Seventy-two (70%) of patients underwent surgery for CCs [53 (51%) with APBJ and 19 (18%) without]. Malignancy was noted in 7 (7%) patients consisting of: 2 cholangiocarcinomas, 2 gallbladder adenocarcinomas, 1 gallbladder neuroendocrine carcinosarcoma, 1 squamous cell carcinoma of bile ducts and 1 intraductal papillary neoplasm ‘biliary papillomatosis’ with malignant transformation. Six of these patients had type I CCs and APBJ, and type IV without APBJ was found in the remaining patient. Additionally, 2 patients had metaplasia and 4 had hyperplasia, but no malignancy in the surgical specimen. Two patients who presented with advanced malignancy died within a year of diagnosis. CONCLUSIONS: Our results suggest that in patients with CC and APBJ there is an increased risk of cholangiocarcinoma, but other biliary tract malignancies should be considered as well. Long-term follow-up in a larger cohort is necessary to confirm these findings.

BACKGROUND: Intraductal tubulopapillary neoplasms (ITPN) of the bile duct are a newly described entity in the family of biliary malignant lesions. They are characterized histologically by almost exclusive tubular growth within the bile duct. Given their rarity, information on biologic behavior and clinical features is limited. We describe the natural history of two consecutive patients diagnosed with ITPN of the bile duct. METHODS: Cases were identified by dedicated pathologic review of biliary resection specimens. Patient demographic data including medical history, presenting symptoms, tumor markers, imaging features, pathologic findings, and survival were obtained from the medical record. RESULTS: Both patients were male, diagnosed at age 64 and 50. Both presented with pruritus, jaundice, and increased liver function tests. CA19-9 levels were elevated at 256 and 137. CT scan showed intrahepatic biliary ductal dilatation from intrabiliary masses. The 64-year-old patient had an obstructing mass visualized in the common hepatic duct (CHD) abutting the confluence of the right and left hepatic duct without evidence of vascular involvement. The 50-year-old also had a large obstructing mass in the proximal CHD with abutment of the right hepatic artery and portal vein. MRCP images were similar, demonstrating soft tissue masses in the proper hepatic duct with polypoid intraductal extension into the left hepatic duct. Tumors measured 4.0 cm × 2.5 cm and 2.5 cm × 1.9 cm. Following complete staging both patients underwent a liver resection [(I) left hepatectomy, caudate, and bile duct resection; (II) extended right hepatectomy, caudate, and bile duct resection). Both patients had portal lymphadenectomies and hepaticojejunostomy reconstruction. Final pathology of both patients revealed pT1N0 invasive adenocarcinoma arising in association with an ITPN and high-grade dysplasia. Neither patient received adjuvant chemotherapy. The patients are alive at 23 and 1.5 months without evidence of disease. CONCLUSIONS: Patients with ITPN of the bile duct present with biliary obstruction with tumors showing significant intraductal tubular growth. Despite the presence of invasive carcinoma, no LN metastases were seen and both patients had early stage disease. Further study is necessary to determine whether malignancy arising in the setting of ITPN is associated with more favorable survival compared to conventional hilar cholangiocarcinoma.