Ann Hepatobiliary Pancreat Surg.  2019 May;23(2):192-196. 10.14701/ahbps.2019.23.2.192.

Development of adenocarcinoma at the remnant intrapancreatic cyst 16 years after resection of the choledochal cyst

• Oh SY
• Kwon JH
• Hwang S

• KMID: 2448776
• DOI: http://doi.org/10.14701/ahbps.2019.23.2.192

Abstract

One of the main reasons to resect choledochal cyst (CC) is to prevent malignant transformation. After resection of CC, the remnant intrapancreatic bile duct is no longer exposed to the enzymatic insult from pancreatic juice activated by the bile. Thus theoretically, the risk of malignant transformation disappears. We present a case showing development of adenocarcinoma from the remnant CC 16 years after resection of CC. A 43 year-old female patient underwent resection of type I CC 16 years ago, leaving a small remnant portion of CC as it was located deep within the pancreas close to the anomalous union of the pancreatobiliary duct. Four years later, intrahepatic ducts were dilated due to anastomotic stricture of the hepaticojejunostomy, which was resolved after repeated balloon dilatation. On follow-up examination at postoperative 16 years, a mass within the pancreas head was identified. As the mass was identified as adenocarcinoma on biopsy, pylorus-preserving pancreatoduodenectomy was performed. Pathologic examination showed a 3.5 cm-sized moderately differentiated adenocarcinoma arising from the remnant CC, with no lymph node metastasis. The patient recovered uneventfully, and is currently undergoing adjuvant chemotherapy. This case indicates that remnant CC can undergo malignant transformation. Therefore, complete resection of CC coupled with life-long follow-up is emphasized.

Keyword

Malignant transformation; Incomplete resection; Anomalous union of pancreatobiliary duct

MeSH Terms

Adenocarcinoma*
Bile
Bile Ducts
Biopsy
Chemotherapy, Adjuvant
Choledochal Cyst*
Constriction, Pathologic
Dilatation
Female
Follow-Up Studies
Head
Humans
Lymph Nodes
Neoplasm Metastasis
Pancreas
Pancreatic Juice
Pancreaticoduodenectomy

Figure

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  Fig. 1 Preoperative radiologic findings. (A) Fusiform dilatation of the intra- and extrahepatic ducts is identified. (B) Magnetic resonance cholangiopancreatography finding suggests choledochal cyst with anomalous union of pancreatobiliary duct and stone formation in the dilated extrahepatic duct. (C) Endoscopic retrograde cholangiopancreatography finding shows choledochal cyst with anomalous union of pancreatobiliary duct of a long common channel.

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  Fig. 2 Computed tomography taken 2 years after choledochal cyst excision. (A) A polypoid lesion (arrow) less than 1cm was found in the distal common bile duct stump. (B) An endoscopic retrograde cholangiopancreatography biopsy confirmed chronic inflammation with fibrosis.

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  Fig. 3 Radiologic findings taken 10 years after choledochal cyst excision. (A) Percutaneous transhepatic biliary drainage (PTBD) tubogram shows nearly complete occlusion of the hepaticojejunostomy. (B) Balloon dilatation was performed with crossover of a PTBD tube. (C) Hepaticojejunostomy stricture was resolved after stent insertion.

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  Fig. 4 Computed tomography taken 16 years after choledochal cyst excision reveals a 3.0 cm-sized mass (arrow) arising from the remnant intrapancreatic choledochal cyst without significant pancreatic duct dilatation.

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  Fig. 5 Gross photographs of the resected specimen after pancreatoduodenectomy. (A) A mass is located at the remnant choledochal cyst portion. (B) A moderately differentiated nodular-type cholangiocarcinoma of 3.5 cm×2.7 cm×1.9 cm, was diagnosed with no metastasis in 15 lymph nodes.

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  Fig. 6 Follow-up computed tomography at 2 weeks after pancreatoduodenectomy with extended pancreatic transection shows no unusual findings.

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