IS Case 142: Klatskin Tumor

Anthony Conde, MD, MPH

Imaging Sciences URMC 2008
Publication Date: 2009-05-21

History

A 67-year-old man presented to a local hospital with sepsis. At that time, he was slightly jaundiced and had slightly elevated transaminases and was diagnosed with primary biliary cirrhosis. He had three further hospitalizations within three months for recurrent infections. He was subsequently referred to the our hospital where a CA19-9 level was drawn and was markedly elevated. An MRI/MRCP was performed. Pathology from brushings performed on an ERCP on the day admission confirmed the MRI suspicions of malignant cells derived from adenocarcinoma (Klatskin's Tumor).

Findings

A 0.8 cm x 0.9 cm enhancing lesion/focus at the confluence of the right and left hepatic ducts that may represent tumor (Klatskin tumor) vs. scar. There is extensive intrahepatic ductal dilatation proximal to this lesion. The common bile duct is normal in caliber.

Diagnosis

Klatskin Tumor

Discussion

Klatskin tumor (hilar cholangiocarcinoma) is a primary malignancy arising from bile duct epithelium, at the confluence of the right and the left hepatic duct. It is the most common primary malignancy of the bile ducts. Although they are typically small, slow-growing, locally invasive tumors that rarely metastasize, they carry a poor prognosis if left untreated, with a mean survival time of approximately 3 months from presentation. Obstructive jaundice, episodes of cholangitis, and progressive liver failure are responsible not only for the poor outcome but also for a poor quality of life. Sonography, CT and MRI may be used to suggest the diagnosis. The major issue of imaging with this tumor is to determine whether the tumor is resectable. Sonography is usually the initial imaging study in patients with jaundice. Sonography is accurate in visualization the level of biliary obstruction, but has problems in demonstrating the obstructing lesion in this type of tumor. Conventional CT has problems in visualizing the extension of these tumors in the biliary tree. MRI including MRCP is the imaging modality of choice to visualize Klatskin tumor. Because of its higher contrast resolution and multiplanar capability, MRI and MRCP are able to more accurately detect and preoperatively assess patients with hilar cholangiocarcinoma, interrogating all involved structures. Nevertheless, hilar tumors are often small at presentation, which adds to the difficulties of demonstration and characterization. They usually present as short stricture or small well-defined mass with inhomogeneous and progressive enhancement, prominent ductal dilatation lobar atrophy, and segmental portal venous occlusion. Satellite lesions are rare.

References

  1. Alden ME, Waterman FM, Topham AK, Barbot DJ, Shapiro MJ, Mohiuddin M. Cholangiocarcinoma: clinical significance of tumor location along the extrahepatic bile duct. Radiology. 1995 Nov;197(2):511-6. [PMID: 7480704]
  2. Guthrie JA, Ward J, Robinson PJ. Hilar cholangiocarcinomas: T2-weighted spin-echo and gadolinium-enhanced FLASH MR imaging. Radiology. 1996 Nov;201(2):347-51. [PMID: 8888221]
  3. Wallner BK, Schumacher KA, Weidenmaier W, Friedrich JM. Dilated biliary tract: evaluation with MR cholangiography with a T2-weighted contrast-enhanced fast sequence. Radiology. 1991 Dec;181(3):805-8. [PMID: 1947101]

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