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INTRODUCTION
Biliary papillomatosis is a rare disease of the biliary tract with over 200 reported cases in literature.1,2 This disease entity is characterized by multiple papillary tumors of both intrahepatic and extrahepatic bile ducts, with clinical features such as abdominal pain, jaundice, and relapsing episodes of cholangitis, due to biliary tract occlusion by biliary stones, mucous plugs, tumor detritus, and blood clots.3 Recurrent superinfections of the biliary tract can lead to secondary biliary cirrhosis, which further leads to liver failure.4-7
Biliary papillomatosis has two unique features: the widespread pattern along the biliary tract, which is responsible for high rates of recurrence after resection; and the likelihood of malignant transformation.8 Accordingly, complete resection of the whole biliary tree is needed to cure this disease.
We herein present a case of a male patient with biliary papillomatosis with malignant change who underwent living donor liver transplantation (LDLT) and is still alive for 10 years despite lung metastasis.
CASE
A 49-year-old man was admitted with intrahepatic ductal mass suggesting intrahepatic cholangiocarcinoma. His liver function was normal without jaundice. Tests for hepatitis B and C virus were negative. Dynamic computed tomography (CT) scan and magnetic resonance pancreatocholangiography showed radiological findings comparable to diffuse intraductal papillomatosis (Fig. 1).
Percutaneous transhepatic biliary drainage (PTBD) catheter was inserted (Fig. 2A) and percutaneous transhepatic cholangioscopy (PTCS) was performed for tissue biopsy (Fig. 2B). The lumens of the whole intrahepatic ducts were covered with villotubular adenomas with focal high-grade dysplasia (Fig. 3A). Some areas showed atypical villous proliferation, which was consistent with well-differentiated adenocarcinoma (Fig. 3B).
To treat biliary papillomatosis, photodynamic therapy had been performed, however, unsatisfactory outcomes were anticipated due to diffuse involvement of the whole intrahepatic bile ducts (Fig. 4). Thus, LDLT using his son’s liver graft was planned.
At 10 days after photodynamic therapy, LDLT operation was performed using a modified right liver graft. After examination of the whole abdomen, the distal bile duct was dissected and transected at the upper margin of the pancreas. Intraoperative frozen-section biopsy of the distal common bile duct resection margin was free from disease. The standardized surgical procedures were applied to LDLT operation using a modified right liver graft with a graft-recipient weight ratio of 1.2. Since the common bile duct was resected, Roux-en-Y hepaticojejunostomy was performed for biliary reconstruction. The patient recovered uneventfully and was discharged 3 weeks after the LDLT operation (Fig. 5).
The pathology report of the explant liver specimens revealed the following: intraductal papillary neoplasms with an associated invasive carcinoma that invaded to the subepithelial connective tissue; no involvement of the liver parenchyma; positive lymphovascular invasion; no perineural invasion; and disease-free bile duct resection margin (Fig. 6).
Although the distal bile duct resection margin was free of tumor, the risk of tumor recurrence at the remnant intrahepatic bile duct still remained. Therefore, staged pylorus-preserving pancreatoduodenectomy was planned.
However, at 3 month posttransplant, the patient was readmitted due to extensive retropancreatic abscess of unknown cause (Fig. 7A). The retropancreatic abscess was barely resolved for 2 months despite percutaneous pigtail drainage (Fig. 7B). Since we did not identify the cause of retropancreatic abscess and acute appendicitis was suspected on follow-up CT scan, exploratory laparotomy was performed (Fig. 7C). We could not find any abnormality causing persistent abscess as the appendix remained normal, thus multiple abdominal drains were inserted at the abscess site. Thereafter, the patient improved gradually and was discharged at 8 months posttransplant. Due to late development of retropancreatic abscess and its slow recovery, we did not consider staged pylorus-preserving pancreatoduodenectomy any longer. The patient recovered complete at 1 year posttransplant without any evidence of disease recurrence (Fig. 8).
However, at 5 years posttransplant, a lung nodule was identified at the left upper lobe during routine follow-up chest CT scan, which was proven to be metastatic carcinoma on CT-guided core biopsy (Fig. 9A). Pulmonary metastasectomy was performed, in which the nodule was a 1.5 cm-sized well differentiated adenocarcinoma, compatible to metastatic lesion. One year later, a local recurrence was detected at the left upper lobe (Fig. 9B), which was also resected and turned to be a 1.8 cm-sized metastatic adenocarcinoma. The patient underwent adjuvant chemotherapy. However, one year later, another lung nodule was detected at the left upper lobe (Fig. 9C), which was resected again. It was proven to be a 2.5 cm-sized metastatic adenocarcinoma (Fig. 9D). No further recurrence has developed during the recent 3 years to date. Currently, this patient is alive to date for 10 years after LDLT, despite repeated episodes of pulmonary metastasis.
DISCUSSION
Biliary papillomatosis is a rare disease of the biliary tract characterized by the presence of multiple papillary tumors in the biliary tree. According to the World Health Organization, considering the similar characteristics of biliary papillomatosis with its pancreatic counterpart, this entity has been renamed biliary intraepithelial papillary neoplasia.9
Lee et al.10 reviewed 58 cases of biliary papillomatosis and reported a malignant transformation rate of 83% in the setting of biliary adenomas. These results suggested that biliary papillomatosis should be considered as a premalignant condition with high malignant potential. The pathogenesis of progression from benign to malignant disease may follow the adenoma-carcinoma sequences. Yeung et al.8 also reported a malignant transformation rate of 42% in 78 patients, and only 55% of the patients could undergo curative resection.
The unique spreading pattern of biliary papillomatosis and the high rates of malignant transformation accounts for the high recurrence rates after resection.4,5,10 Considering these features, the only curative treatment is removal of the whole biliary tree, which can be achieved through liver transplantation and pancreatoduodenectomy. In the present case, some of the distal bile duct was involved and the extrapancreatic bile duct was resected during the LDLT operation. In addition, staged pancreatoduodenectomy was planned to remove the remnant intrapancreatic duct completely, however, it was not performed due to late development of retropancreatic abscess. To our knowledge, the present case was the first case of LDLT for biliary papillomatosis.
There was a case report of deceased donor liver transplantation performed to a patient with biliary papillomatosis who survived for 10 years.11 In this patient, there was no sign of involvement of the distal part of the common bile duct, hence pancreatoduodenectomy was not performed. During follow-up, at 3 years posttransplant, CT scan showed enlargement of the pancreatic head due to suspected local recurrence. Although suspicious tumor recurrence developed, the patient did not undergo pancreatoduodenectomy because the liver graft had recurrent fibrosis. During follow-up, the lesion of the pancreatic head had slightly grown, however, there was no definite evidence of recurrent disease for 10 years.11 In contrast, it was also reported that one patient underwent total pancreatectomy and liver transplantation sequentially without evidence of malignant transformation, but after 2 years biliary adenocarcinoma developed at the liver graft.12 Vibert et al.13 reported a series of three cases and concluded that in the absence of invasive carcinoma and positive lymph nodes, LT can be successfully performed.
In the present case, the underlying cause of retropancreatic abscess was unknown. We only presumed that late development of retropancreatic abscess might be associated with tissue damage from the precedent photodynamic therapy. So far, photodynamic therapy has a limited role in treating biliary papillomatosis and biliary cancers.14,15
Experience on our case and literature review suggest that patients with biliary papillomatosis with or without malignant transformation are selectively indicated for liver transplantation. Concurrent pancreatoduodenectomy may be also indicated if the distal bile duct is involved.
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