Pancreatic Head Cancer and Intrahepatic Cholangiocarcinoma Occurring After Surgery for Congenital Biliary Dilatation Associated With Pancreaticobiliary Maljunction: A Case Report
Surgery, Japanese Red Cross Wakayama Medical Center, Wakayama, JPN
Abstract
Congenital biliary dilatation (CBD), frequently associated with pancreaticobiliary maljunction (PBM), predisposes patients to biliary tract malignancies due to chronic reflux of pancreatic juice into the biliary epithelium. Although biliary diversion reduces carcinogenic risk, malignancy may still arise many years after surgery. A 69-year-old woman underwent choledochal cyst excision and Roux-en-Y hepaticojejunostomy at age 52. Seventeen years later, she developed pancreatic head adenocarcinoma, treated with subtotal stomach-preserving pancreaticoduodenectomy (SSPPD) while preserving and reusing the original Roux limb. Six months later, she developed intrahepatic cholangiocarcinoma, requiring an extended right hepatectomy with reconstruction using the same preserved Roux limb. She remains disease-free 18 months after surgery. Patients with CBD and PBM remain at lifelong risk of malignancy, especially when surgery is performed in adulthood. Reuse of a preserved Roux limb is a feasible and effective strategy in complex reoperations.
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Keywords: biliary reconstruction, cholangiocarcinoma, congenital biliary dilatation, pancreatic cancer, pancreaticobiliary maljunction, reoperation
Article notes
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Accepted 2025 Dec 30; Collection date 2025 Dec.
Introduction
Pancreaticobiliary maljunction (PBM) is a congenital anomaly characterized by an abnormally long common channel located outside the duodenal wall, permitting reciprocal reflux of pancreatic and biliary secretions [1]. This abnormal reflux results in chronic epithelial injury, inflammation, and subsequent carcinogenesis within the pancreatobiliary system [2].
Congenital biliary dilatation (CBD), which frequently coexists with PBM, is therefore associated with a lifelong risk of malignancy [3]. Although excision of the extrahepatic bile duct with biliary diversion is the standard treatment, long-term follow-up studies have demonstrated that malignancies may continue to develop decades after surgery [3-7]. In particular, adult-onset CBD-defined as surgery performed in adulthood-may confer a higher lifetime carcinogenic risk due to prolonged exposure to pancreaticobiliary reflux prior to definitive correction.
The malignancies most commonly reported after CBD excision are extrahepatic cholangiocarcinoma and gallbladder cancer [3-7]. In contrast, pancreatic cancer is relatively rare in patients with PBM [8,9], and intrahepatic cholangiocarcinoma (IHCC) developing long after cyst excision has been reported in only a limited number of cases [4-7,10,11]. Sequential occurrence of pancreatic cancer followed by IHCC in a single patient is therefore exceptionally rare.
Here, we report a unique case of metachronous pancreatic head adenocarcinoma followed by IHCC occurring 17 years after CBD surgery associated with PBM. This case highlights the lifelong carcinogenic risk even decades after surgery and illustrates the feasibility of reusing a preserved Roux-en-Y limb during repeated major hepatopancreatobiliary procedures.
Case presentation
A 69-year-old woman presented with intermittent upper abdominal pain lasting several weeks. Laboratory evaluation revealed an elevated serum carbohydrate antigen 19-9 (CA19-9) level of 78.8 U/mL, while the carcinoembryonic antigen (CEA) level was within the normal range at 2.7 ng/mL. She had undergone excision of a congenital choledochal cyst with Roux-en-Y hepaticojejunostomy at 52 years of age for CBD associated with PBM. Her baseline performance status was good (ECOG 0), and she had no significant medical comorbidities.
Pancreatic head cancer
Contrast-enhanced computed tomography (CT) and magnetic resonance cholangiopancreatography (MRCP) revealed a pancreatic head mass without evidence of distant metastasis. Endoscopic ultrasound-guided biopsy confirmed adenocarcinoma. Subtotal stomach-preserving pancreaticoduodenectomy (SSPPD) was performed. The original Roux-en-Y limb and biliary-enteric anastomosis were carefully preserved and reused for reconstruction (Figure 1).
The postoperative course was uneventful, and serum tumor marker levels normalized.
Histopathological examination demonstrated moderately differentiated tubular adenocarcinoma with negative surgical margins (R0) and no lymph node metastasis (pT2N0M0). Based on pathological findings and patient preference, no adjuvant chemotherapy was administered.
Intrahepatic cholangiocarcinoma
Six months later, during routine postoperative surveillance, serum tumor markers were again elevated, with a CEA level of 5.6 ng/mL and a CA19-9 level of 518.0 U/mL. Contrast-enhanced CT, Positron emission tomography-CT (PET-CT) and magnetic resonance imaging revealed a hepatic hilar mass (Figure 2A-2C). Percutaneous biopsy confirmed IHCC.
Preoperative three-dimensional simulation using SYNAPCE VINCENT® was performed to visualize the vascular and biliary anatomy and to support operative planning (Figure 3). This simulation enabled a precise assessment of tumor location, its relationship to adjacent structures, and the anticipated resection line.
The patient subsequently underwent an extended right hepatectomy with en bloc resection of the previous hepaticojejunostomy. The preserved Roux-en-Y limb was reused for left hepatic duct-jejunal anastomosis (Figure 4A-4C). The postoperative course was uneventful, and no adjuvant chemotherapy or radiotherapy was administered. She remains alive without evidence of recurrence 18 months after the second surgery and continues regular surveillance with cross-sectional imaging and tumor marker assessment.
Pathology
Histopathological examination of the pancreatic lesion demonstrated moderately differentiated tubular adenocarcinoma (Figure 5A). The hepatic tumor was diagnosed as moderately differentiated IHCC arising from the right intrahepatic bile duct (Figure 5B).
There was no histologic continuity between the two tumors, confirming the diagnosis of metachronous double primary cancers rather than recurrence or metastatic disease.
Discussion
PBM results in continuous exposure of the biliary tract to pancreatic enzymes and bile acids, leading to chronic epithelial injury, inflammation, and carcinogenesis [1,2]. Although cyst excision significantly reduces this risk, long-term studies have shown that the cumulative incidence of malignancy continues to increase, particularly beyond 15-30 years after surgery [3-7,10,11]. Adult-onset CBD may be associated with an even higher lifetime risk due to prolonged preoperative exposure to pancreaticobiliary reflux.
In the present case, CBD excision was performed at 52 years of age, suggesting that carcinogenic processes may have already been initiated. This prolonged exposure may explain the subsequent development of two distinct primary malignancies-pancreatic head adenocarcinoma followed by IHCC. While pancreatic cancer after CBD excision is rare [8,9], IHCC has been reported in several long-term follow-up studies [4-7,10,11]. We reviewed the literature, and previous cases of malignancy after cyst excision are summarized in Table 1. To our knowledge, this is the first English-language report describing sequential pancreatic head adenocarcinoma followed by IHCC after CBD excision associated with PBM.
| No. | Citation | Patient (age/sex) | Age at initial surgery | Interval to malignancy | Tumor site / histology | Key notes |
| 1 | Eriguchi N et al., 2001 [9] | 42, F | 25 years | 17 years | PDAC | Pancreatic carcinoma after choledochal cyst excision. |
| 2 | Ohashi T et al., 2013 [3] | Series | Various | 13–32 years | Biliary malignancies | Large series with long intervals. |
| 3 | Nishiyama R et al., 2011 [4] | 61, F | 28 years | 33 years | IHCC | IHCC with chronic inflammation. |
| 4 | Shimamura K et al., 2009 [10] | 44, M | 10 years | 34 years | IHCC | Giant hepatic tumor decades later. |
| 5 | Kumamoto T et al., 2014 [5] | 40, F | 12 years | 28 years | IHCC | Long-term carcinogenesis. |
| 6 | Kato H et al., 2015 [6] | 46, F | 14 years | 32 years | CC | Arising from remnant intrapancreatic duct. |
| 7 | Chen Y et al., 2016 [7] | 59, F | 46 years | 13 years | Hilar CC | Metachronous hilar CC. |
| 8 | Park SW et al., 2012 [11] | 42, F | 33 years | 9 years | CBD cancer | Cancer from remnant intrapancreatic duct. |
| 9 | Present case | 69, F | 52 years | 17 years + 6 months | PDAC & IHCC | First case of both cancers after excision. |
The occurrence of metachronous tumors at different anatomical sites can be explained by a field carcinogenesis phenomenon. Chronic pancreaticobiliary reflux creates a diffuse carcinogenic environment throughout the pancreatobiliary epithelium, allowing independent tumors to arise over time without histologic continuity.
Despite a shared carcinogenic background, pancreatic ductal adenocarcinoma and IHCC likely arise through different biological pathways. Pancreatic cancer is thought to develop via pancreatic intraepithelial neoplasia driven by chronic inflammation and genetic instability, whereas IHCC is associated with prolonged biliary epithelial injury, cholestasis, and inflammatory changes within the intrahepatic bile ducts.
This case also underscores the importance of lifelong surveillance, even decades after cyst excision. Although evidence-based surveillance protocols are lacking, long-term follow-up incorporating periodic imaging and tumor marker assessment may be reasonable. The delayed emergence of malignancy in this patient highlights the persistence of carcinogenic risk despite definitive surgery.
From a surgical standpoint, this case offers important educational value for reoperative planning. Reuse of a preserved Roux-en-Y limb during repeated hepatopancreatobiliary operations was safe and effective, avoided additional bowel resection, and simplified reconstruction in a complex operative field.
The limitations of this report include its single-case design and the absence of genetic or molecular analyses, which preclude causal inference or generalization. Nevertheless, the long latency, clear pathological distinction between tumors, and successful surgical management provide valuable insight into long-term oncologic risk and operative strategy in patients with PBM after CBD excision.
Conclusions
CBD associated with PBM carries a lifelong risk of malignancy, even decades after cyst excision, particularly when surgery is performed in adulthood. Sequential development of pancreatic head adenocarcinoma and intrahepatic cholangiocarcinoma, as demonstrated in this case, underscores the need for sustained vigilance and lifelong surveillance. In addition, the reuse of a preserved Roux-en-Y limb represents a feasible and safe reconstructive option in complex hepatopancreatobiliary reoperations.
Disclosures
Footnote Group
References
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