Invasive Colloid Carcinoma Arising from Intraductal Papillary Neoplasm in a 50-Year-Old Woman with Primary Sclerosing Cholangitis

CASE REPORT

A 50-year-old woman with primary sclerosing cholangitis (PSC) came to our institution for transplant evaluation. She was diagnosed with ulcerative colitis at the age of 24, at which time she also underwent cholecystectomy for significant biliary colic. She did well over the years and required only intermittent treatment for ulcerative colitis. Three years prior to presentation, an elevated serum alkaline phosphatase activity was noted in preoperative laboratory tests for a gynecological procedure. She was referred for endoscopic retrograde cholangiopancreatography (ERCP), which revealed irregular strictures and dilatation of the intrahepatic bile ducts with a beadlike appearance, consistent with PSC. Initial bile duct brushing cytology found atypical cells consistent with acute inflammation. Over the next 2 years, she complained of intermittent fevers and right upper quadrant pain, both of which resolved with levofloxacin, but a percutaneous biliary drain was eventually needed. Thereafter, she had multiple episodes of acute cholangitis, despite frequent biliary tube evaluations and changes.

The decision was made to begin liver transplant evaluation. The patient's Model for End-Stage Liver Disease (MELD) score was 6. The carbohydrate antigen (CA) 19-9 level was 335 U/mL (n = 0-35 U/mL). Computed tomography (CT) at that time revealed changes consistent with PSC with an additional atypical finding of dilated ducts in the posterior right lobe, but no mass was appreciated (Fig. [1]). Portal lymphadenopathy, similar in appearance to that seen in a previous study 1 year earlier, was noted on CT scan. She underwent repeat ERCP with brushings that again revealed atypical cells without evidence of dysplasia. Both the elevated CA 19-9 (now 595 U/mL) and the cytology results were considered consistent with the clinical picture of recurrent bacterial cholangitis. An appeal was approved for a higher MELD score on the basis of recurrent cholangitis; within 2 months a liver became available and she underwent orthotopic liver transplantation.

Figure 1 CT scan of the liver performed 6 months prior to transplantation showing cystic dilatation of bile ducts in the posterior right lobe (arrow).

REFERENCES

• 1 Kim H J, Kim M H, Lee S K et al.. Mucin-hypersecreting bile duct tumor characterized by a striking homology with an intraductal papillary mucinous tumor (IPMT) of the pancreas.  Endoscopy. 2000;  32 389-393
• 2 Nakanuma Y, Sasaki M, Ishikawa A et al.. Biliary papillary neoplasm of the liver.  Histol Histopathol. 2002;  17 851-861
• 3 Shimonishi T, Zen Y, Chen T C et al.. Increasing expression of gastrointestinal phenotypes and p53 along with histologic progression of intraductal papillary neoplasia of the liver.  Hum Pathol. 2002;  33 503-511
• 4 Ishida M, Seki K, Honda K et al.. Intraductal mucinous tumors occurring simultaneously in the liver and pancreas.  J Gastroenterol. 2002;  37 1073-1078
• 5 Olsson R, Danielsson A, Jarnerot G et al.. Prevalence of primary sclerosing cholangitis in patients with ulcerative colitis.  Gastroenterology. 1991;  100 1319-1323
• 6 Bambha K, Kim W R, Talwalkar J et al.. Incidence, clinical spectrum, and outcomes of primary sclerosing cholangitis in a United States community.  Gastroenterology. 2003;  125 1364-1369
• 7 Haratake J, Kasai T, Makino H. Diffuse mucosal carcinoma of intrahepatic and extrahepatic bile ducts including gallbladder.  Pathol Int. 2002;  52 784-788
• 8 Hubens G, Delvaux G, Willems G et al.. Papillomatosis of the intra- and extrahepatic bile ducts with involvement of the pancreatic duct.  Hepatogastroenterology. 1991;  38 413-418
• 9 Colombari R, Tsui W M. Biliary tumors of the liver.  Semin Liver Dis. 1995;  15 402-413
• 10 Chae B W, Chung J P, Park Y N et al.. Villous adenoma of the bile ducts: a case report and a review of the reported cases in Korea.  Yonsei Med J. 1999;  40 84-89
• 11 Loh A, Kamar S, Dickson G H. Solitary benign papilloma (papillary adenoma) of the cystic duct: a rare cause of biliary colic.  Br J Clin Pract. 1994;  48 167-168
• 12 Bae J Y, Park Y N, Nakanuma Y et al.. Intestinal type cholangiocarcinoma of intrahepatic large bile duct associated with hepatolithiasis-a new histologic subtype for further investigation.  Hepatogastroenterology. 2002;  49 628-630
• 13 Yamamoto J, Kosuge T, Takayama T. Intrahepatic cholangiocarcinoma presenting intrabile duct extension: clinicopathologic study of five resected cases.  Jpn J Clin Oncol. 1997;  27 18-21
• 14 Suh K S, Roh H R, Koh Y T et al.. Clinicopathologic features of the intraductal growth type of peripheral cholangiocarcinoma.  Hepatology. 2000;  31 12-17
• 15 Batheja N, Suriawinata A, Saxena R et al.. Expression of p53 and PCNA in cholangiocarcinoma and primary sclerosing cholangitis.  Mod Pathol. 2000;  13 1265-1268
• 16 Abraham S C, Lee J-H, Hruban R H et al.. Molecular and immunohistochemical analysis of intraductal papillary neoplasms of the biliary tract.  Hum Pathol. 2003;  34 902-910
• 17 D'Angelica M, Brennan M F, Suriawinata A et al.. Intraductal papillary mucinous neoplasms of the pancreas: an analysis of clinicopathologic features and outcome.  Ann Surg. 2004;  239 400-408
• 18 Tajima Y, Kuroki T, Fukuda K et al.. An intraductal papillary component is associated with prolonged survival after hepatic resection for intrahepatic cholangiocarcinoma.  Br J Surg. 2004;  91 99-104
• 19 Jarnagin W R, Fong Y, DeMatteo R P et al.. Staging, resectability, and outcome in 225 patients with hilar cholangiocarcinoma.  Ann Surg. 2001;  234 507-517
• 20 Meyer C G, Penn I, James L. Liver transplantation for cholangiocarcinoma: results in 207 patients.  Transplantation. 2000;  69 1633-1637
• 21 Iwatsuki S, Todo S, Marsh J W et al.. Treatment of hilar cholangiocarcinoma (Klatskin tumors) with hepatic resection or transplantation.  J Am Coll Surg. 1998;  187 358-364
• 22 Seidel G, Zahurak M, Iacobuzio-Donahue C et al.. Almost all infiltrating colloid carcinomas of the pancreas and periampullary region arise from in situ papillary neoplasms: a study of 39 cases.  Am J Surg Pathol. 2002;  26 56-63