Treatment of cholangiocarcinoma complicating primary sclerosing cholangitis: The Mayo Clinic experience

OBJECTIVES: The aims of this retrospective study were to assess the frequen cy with which we used different treatment modalities for patients with prim ary sclerosing cholangitis (PSC) and cholangiocellular carcinoma (CCA). METHODS: A total of 41 patients with known CCA complicating PSC with a medi an age of 49 yr (range, 27-75 yr) were identified from a group of 1009 pati ents (4%) with PSC seen over 10 yr at the Mayo Clinic. RESULTS: These patients received mainly five forms of treatment: 10 patient s were treated with radiation therapy (RT) with or without 5-fluorouracil ( 5-FU) (seven with palliative and three with curative intent), nine with ste nt: placement for cholestasis, 12 with conservative treatment, four with su rgical resection (one of four received RT and 5-FU), and three patients wit h orthotopic liver transplantation and RT, with or without 5-FU. One patien t was treated with 5-FU alone, one with photodynamic therapy, and one patie nt with somatostatin analog. A total of 36 patients died, whereas four (10% ) patients survived (two with surgical resection, one with orthotopic liver transplantation and RT, and one with stent placement) during a median foll ow-up of 5.5 months (range, 1-75 months). One patient was lost to follow-up . CONCLUSIONS: In highly selective cases, resective surgery seems to be of be nefit in PSC patients with CCA. However, these therapies are rarely applied to these patients because of the advanced nature of the disease at the tim e of diagnosis. Efforts should be directed at earlier identification of pot ential surgical candidates. (Am J Gastroenterol 2001;96: 1164-1169. (C) 200 1 by Am. Coll. of Gastroenterology).