SEARCH

SEARCH BY CITATION

TO THE EDITORS:

Intraductal papillary neoplasm (IPN) is a rare condition characterized by noninvasive papillary neoplasms of the biliary tree for which treatment is still controversial. We present a case of orthotopic liver transplantation (OLT) for extensive IPN with carcinoma in situ (CIS) transformation.

In May 2010, a 42-year-old man was referred for acute cholangitis. His history was remarkable for recurrent cholangitis and a biliary cirrhosis of unknown etiology, for which he had been on a transplant waiting list for 2 years. During that time, follow-up magnetic resonance cholangiopancreatography (MRCP) showed progressive diffuse biliary dilation and stenosis.

On his arrival, MRCP showed known left intrahepatic biliary duct dilation with parenchymal atrophy and a new bilobular lesion of the main bile duct (Fig. 1). Endoscopic retrograde cholangiopancreatography and cholangioscopy showed excessive mucus discharge from the ampulla and a 26-mm polypoid lesion lying in the main bile duct, which biopsies revealed to be CIS. Because of the clinical presentation and the patient's history, the diagnosis of IPN was suspected. An extensive workup, including thoracic and abdominal computed tomography scans, a positron emission tomography scan, and biopsies of multiple enlarged hilar nodes showed no metastatic disease.

thumbnail image

Figure 1. MRCP image showing impressive left hepatic duct dilation with parenchymal atrophy and a polypoid lesion of the main bile duct.

Download figure to PowerPoint

Considering the patient's end-stage liver disease with a Model for End-Stage Liver Disease score of 28, the suspicion of extensive IPN of intrahepatic and extrahepatic ducts, and CIS with a completely negative metastatic workup, we decided that liver transplantation remained the better treatment option.

On June 26, 2010, the patient successfully underwent OLT with Roux-en-Y hepaticojejunostomy. The final pathology results showed biliary cirrhosis and diffuse IPN of extrahepatic and intrahepatic ducts with multiple high-grade dysplasia and CIS foci. No infiltrative cholangiocarcinoma was found. Unfortunately, the patient died 19 months later because of relapsing disease in the intrapancreatic portion of his native main bile duct with subsequent cholangiocarcinoma transformation.

IPN is a rare disease with only approximately 160 cases reported in the literature. Treatment for this condition is still very controversial: palliative stenting and drainage, radiotherapy,1 cholangioscopic laser ablation,2 and hepatectomy have all been tried with mixed results. However, the prognosis remains poor because of the high recurrence of the disease elsewhere in the biliary tree3 and the frequency of malignant transformation. The mean survival time ranges only from 28 to 61 months with hepatectomy and from 11 to 37 months with palliative care.4, 5 In our case, the extent of the disease and the associated cirrhosis led us to consider liver transplantation as a viable alternative, as already suggested by some authors.6 However, the presence of CIS was an initial source of concern for us.

To our knowledge, only 9 other cases of liver transplantation for IPN have been reported in the literature.7-15 With our case, there are now 2 reported recurrences of the disease in the native biliary tree left in place during surgery.15 Both patients died from that relapse. On the other hand, all patients who underwent pancreaticoduodenectomy concomitantly with OLT remained alive after follow-up periods of 22, 18, and 24 months, respectively.8, 10, 14

As for neoplasia, 2 of the 3 cases with frank invasive carcinoma according to the final pathology results died within a few months of OLT.13, 15 On the other hand, 3 of the 4 cases reported to have moderate- to high-grade dysplasia or CIS according to the final pathology results were well and alive 15 months, 18 months, and 16 years, respectively, after OLT.7, 10, 12 As for our case, the patient died from recurrent disease in his native biliary duct, but he had not shown any signs of metastasis up until that relapse. Therefore, we believe that his death cannot be attributed to the presence of the many high-grade dysplasia and CIS foci found in the explanted liver during OLT.

In conclusion, OLT for extensive high-grade IPN without any evidence of basement membrane transgression or metastasis on a full extension workup is a safe and possible treatment. However, the complete removal of the native biliary tree with concomitant pancreaticoduodenectomy should be mandatory, no matter what the extent of the disease is. We do not recommend transplantation for patients presenting with local or distal malignant invasion.

REFERENCES

  1. Top of page
  • 1
    Gunvén P, Gorsetman J, Ohlsén H, Rudén BI, Lundell G, Skoog L. Six-year recurrence free survival after intraluminal iridium-192 therapy of human bilobar biliary papillomatosis. A case report. Cancer 2000; 89: 69-73.
  • 2
    Lai R, Freeman ML, Mallery S. EUS in multiple biliary papillomatosis. Gastrointest Endosc 2002; 55: 121-125.
  • 3
    Lam CM, Yuen ST, Yuen WK, Fan ST. Biliary papillomatosis. Br J Surg 1996; 83: 1715-1716.
  • 4
    Lee SS, Kim MH, Lee SK, Jang SJ, Song MH, Kim KP, et al. Clinicopathologic review of 58 patients with biliary papillomatosis. Cancer 2004; 100: 783-793.
  • 5
    Yeung YP, AhChong K, Chung CK, Chun AY. Biliary papillomatosis: report of seven cases and review of English literature. J Hepatobiliary Pancreat Surg 2003; 10: 390-395.
  • 6
    Rambaud S, Nores JM, Paolaggi JA. Papillomatosis of the biliary tract [in French]. Presse Med 1989; 18: 1329-1332.
  • 7
    Imvrios G, Papanikolaou V, Lalountas M, Patsiaoura K, Giakoustidis D, Fouzas I, et al. Papillomatosis of intra- and extrahepatic biliary tree: successful treatment with liver transplantation. Liver Transpl 2007; 13: 1045-1048.
  • 8
    Dumortier J, Scoazec JY, Valette PJ, Ponchon T, Boillot O. Successful liver transplantation for diffuse biliary papillomatosis. J Hepatol 2001; 35: 542-543.
  • 9
    Beavers KL, Fried MW, Johnson MW, Zacks SL, Gerber DA, Weeks SM, et al. Orthotopic liver transplantation for biliary papillomatosis. Liver Transpl 2001; 7: 264-266.
  • 10
    Marion-Audibert AM, Guillet M, Rode A, Barnoud R, Mesnil A, Ducerf C, et al. Diffuse biliary papillomatosis: a rare indication for liver transplantation [in French]. Gastroenterol Clin Biol 2009; 33( pt 1): 82-85.
  • 11
    Ciardullo MA, Pekolj J, Acuña Barrios JE, Gadano A, Mullen E, Castaing D, de Santiñañes E. Multifocal biliary papillomatosis: an indication for liver transplantation [in French]. Ann Chir 2003; 128: 188-190.
  • 12
    Charre L, Boillot O, Goffette P, Geubel A, Gigot JF, Sempoux C, Lerut J. Long-term survival after isolated liver transplantation for intrahepatic biliary papillomatosis. Transpl Int 2006; 19: 249-252.
  • 13
    Böttger T, Sorger K, Jenny E, Junginger T. Progressive papillomatosis of the intrahepatic and extrahepatic bile ducts. Case report. Acta Chir Scand 1989; 155: 125-129.
  • 14
    Dhupar R, de Vera ME, Marsh JW, Fontes PA, Steel JL, Zeh HJ, Gamblin TC. Simultaneous pancreatectomy and liver transplantation: a single-institution experience. HPB (Oxford) 2009; 11: 242-246.
  • 15
    Gigot JF, Geubel A, Haot J, Pringot J, Mainguet P, Descamps C, et al. Papillomatosis of the biliary tract [in French]. Acta Endosc 1989; 19: 345-366.

Simon Lessard M.D.*, Geneviève Soucy M.D.†, Michel Dagenais M.D.‡, Catherine Vincent M.D.*, * Departments of Hepatology, Saint-Luc Hospital, University of Montreal Hospital Center Montreal, Quebec, Canada, † Pathology, Saint-Luc Hospital, University of Montreal Hospital Center Montreal, Quebec, Canada, ‡ Hepatobiliary and Pancreatic Surgery, Saint-Luc Hospital, University of Montreal Hospital Center Montreal, Quebec, Canada.