Usually the tumor is small and flat, but occasionally it is large. Itri JN, de Lange EE. A hilar-perihilar tumor is considered unresectable in the following cases 12: Differential diagnosis depends on whether the tumor is intrahepatic or extrahepatic and on the growth pattern. In a study by Sasaki et al. HPB (Oxford). The pattern of metastatic spread includes 1: An increase in margin-negative resection rates and survival can be achieved by resection of the ipsilateral hepatic lobe.

5. The outer margin of the thickened bile duct wall is smooth and clear. 6 In-vivo monitoring of development of cholangiocarcinoma induced with C. sinensis and N-nitrosodimethylamine in Syrian golen hamsters using ultrasonography and magnetic resonance imaging: a preliminary study Oncol.

MSKCC system for staging cholangiocarcinoma, hepatic duct involvement (unilateral vs. bilateral), portal vein involvement (ipsilateral vs contralateral vs. main), tumor extension into second order biliary radicles, tumor extension into surrounding adipose tissue or hepatic parenchyma, hepatic artery involvement (ipsilateral vs contralateral vs. common hepatic artery). Risk factors for intrahepatic and extrahepatic cholangiocarcinoma in the United States: a population-based case-control study. Clin. Nonunion of the right and left hepatic ducts with or without a visible thickened wall is a typical finding of infiltrating hilar cholangiocarcinoma [9, 27, 29]. 2007;5 (10): 1221-8. This classification is useful but has been modified over time. The bile ducts distal to the mass are typically dilated. Cholangiocarcinoma is a malignant tumor arising from the epithelium of the bile ducts. Most of these tumors are adenocarcinomas [ 1 ].

The Liver Cancer Study Group of Japan has proposed a new classification based on growth characteristics, with tumors being identified as mass-forming, periductal-infiltrating, and intraductal-growing types [10] (Figs. Irregular narrowing of the involved bile duct eventually results in obstruction. 2015;24 (1): 41-56. 7A, and 7B, 7C). In this type, the intraductal tumor may be polypoid, sessile, or superficially spreading along the lumen [17-20] (Figs. The tumor appears as a diffuse, firm, gray-white, annular thickening of the extrahepatic ducts with almost complete obstruction of the lumen [6]. Cai H, Kong WT, Chen CB, Shi GM, Huang C, Shen YH, Sun HC. 2010;14 (7): 1128-38. Robbins and Cotran pathologic basis of disease. Thus, the tumor grows longitudinally and extends along the axis of the bile duct like a branch of a tree [4]. Radiographics. Unable to process the form. 106 (5): 1011-5. In specimens of bile ducts from patients with hepatolithiasis, biliary intraepithelial neoplasia (BilIN) is a common finding and is considered to be a precursor lesion of cholangiocarcinoma. I thank Cheol Keun Park for his invaluable advice regarding the pathology of cholangiocarcinoma and Bonnie Hami and Young Joo Moon for copyediting the manuscript. These sizable tumors then float within the bile ducts and partially occlude the bile flow, or they may drain through the orifice of the papilla of Vater [17]. The incidence ranges from 0.3 to 6 per 100,000 inhabitants per year 14. The problem with traditional TNM staging for hilar cholangiocarcinoma is that a small, badly place tumor markedly worsens prognosis, and T staging was inadequate. According to the morphologic classification system proposed by the Liver Cancer Study Group of Japan (,5), cholangiocarcinoma is classified into mass-forming, periductal infiltrating, and intraductal growth types (,Fig 5). Classification. lymph nodes: enlarged regional (N1) or distant (N2) lymph nodes? W B Saunders Co. (2005) ISBN:0721601871. J. Gastrointest. Because the intraductal papillary tumor does not penetrate the bile duct wall, its outer margin is clear on sonography as well as on CT. Radiologic findings are basically the same as those for the intrahepatic intraductal form. Intraductal Papillary Mucinous Tumor of the Bile Duct as a Variant, Periductal-Infiltrating Cholangiocarcinoma, Review. Surgical resection should be tailored depending on the morphologic type and the stage of the tumors [10]. Varying appearances of cholangiocarcinoma: radiologic-pathologic correlation. However, some of the terminology and classifications are ambiguous and therefore confusing. Mucin is echo-free on sonography and water-attenuating on CT and therefore not visible. In specimens of bile ducts from patients with hepatolithiasis, biliary intraepithelial neoplasia (BilIN) is a common finding and is considered to be a precursor lesion of chola… 11. For intraductal-growing cholangiocarcinomas, tumor resection with a tumor-free margin is sufficient, and long-term patient survival can be expected [17-19]. 10. Address correspondence to J. H. Lim ([email protected]). Cholangiocarcinoma 2020: the next horizon in mechanisms and management.

Tumor resection is currently the most optimal treatment and the ability of the tumor to infiltrate longitudinally and radially along the biliary tree necessitates aggressive resection strategies such as ipsilateral hepatectomy. Again, capsular retraction may be evident. 12. It should be noted that when attempting resection, tumor size in itself is unimportant. Precise characterization of these tumors in terms of their growth pattern and staging is mandatory for optimal treatment planning and for determining a prognosis. Malhi H, Grant EG, Duddalwar V. Contrast-Enhanced Ultrasound of the Liver and Kidney. BMC cancer. Sometimes, the detached tumor implants at the lumen of the adjacent bile duct, resulting in multiple tumors (papillomatosis) (Figs. Periductal infiltrating intrahepatic tumors are most common at the hilum (comprise over 70% of hilar-perihilar cholangiocarcinomas), where they are known as Klatskin tumors 3, but can also be seen in combination with mass-forming tumors within the liver. Vilgrain V. Staging cholangiocarcinoma by imaging studies. It can also slough spontaneously and, simulating bile duct stones, occlude the bile ducts [17].

7B). Clin. intrahepatic vascular involvement with numerous local metastases, atrophy of a liver lobe with contralateral portal vein or hepatic artery encasement, atrophy of a liver lobe with contralateral secondary biliary radicle involvement, central necrosis (high T2 signal) is more common, regular margin, but there are exceptions to this, intratumoral cysts do not communicate with the biliary tree. Surg. Saxena A, Chua TC, Sarkar A et-al. 17 (9): 557. Extrahepatic Cholangiocarcinoma: What the Surgeon Needs to Know RadioGraphics Fundamentals | Online Presentation. At other times, they may not be dilated, even if the tumor arises from one of the intrahepatic ducts [23]. Some of the involved bile ducts dilate cystically, whereas others dilate diffusely and proportionally. Hepatol. Traditionally, the Bismuth-Corlette classification (developed in 1975) was an anatomic description used to assess resectability of hilar/perihilar cholangiocarcinoma 2.

Appearances on MR are similar to those described above for CT, except that MR is more sensitive to contrast enhancement 3 and bile duct visualization. This classification is considered to be the most reasonable because it describes the gross appearance, growing characteristics, biologic behavior, and prognostic implication for patients [11, 12] and because it is helpful for radiologic interpretation. Cholangiocarcinoma can develop in any part of the extrahepatic duct, occurring in 50-75% of reported cases in the upper third of the duct including the hepatic hilum, in 10-25% in the middle third, and in 10-20% in the lower third [30-32]. According to this new classification, which I use for this review article, the exophytic or nodular type matches the mass-forming type, the infiltrating or sclerosing type matches the periductal-infiltrating, and the polypoid or papillary type matches the intraductal-growing type. On CT or MRI, the thickened bile ducts can be visualized as an enhancing ring or spot (Figs. Rosai [5] classified cholangiocarcinoma as polypoid and sclerosing. {"url":"/signup-modal-props.json?lang=us\u0026email="}. 1. In contrast to mass-forming cholangiocarcinoma, periductal-infiltrating cholangiocarcinoma tends to spread along the bile duct wall via the nerve and perineural tissue of Glisson's capsule toward the porta hepatis [1, 6]. 4. There may be fine irregularities, a velvety or serrated contour along the bile ducts, representing the papillary surface of the tumor [17]. There is usually some proximal (i.e. ADVERTISEMENT: Radiopaedia is free thanks to our supporters and advertisers. Although overall cholangiocarcinoma is rare, there are significant regional variations in incidence with much higher rates seen in south-east Asia and the Middle East 2. Cholangiocarcinoma: classification, diagnosis, staging, imaging features, and management 7 March 2017 | Abdominal Radiology, Vol. Lymph node metastases and extrahepatic metastases are much more likely than with hepatocellular carcinoma. Sonography, CT, and MR cholangiopancreatography show severe dilatation of the intraand extrahepatic ducts. Most intraductal-growing cholangiocarcinomas are papillary adenocarcinomas [17-20], comprising innumerable frondlike infoldings of proliferated columnar epithelial cells and slender fibrovascular cores [17]. Periductal infiltrating intrahepatic: tumors typically are associated with altered caliber bile duct (narrowed or dilated) without a well-defined mass.

42, No. Various terminology and classifications have been used to describe the pathologic and radiologic appearance of cholangiocarcinoma, and each describes a specific aspect of the tumor. On sonography and CT, the tumor may appear as a small mass. Macroscopically cholangiocarcinomas have a number of different growth patterns (see below), and their macroscopic appearance will reflect this. The surgical treatment should be tailored according to the gross morphology of the cholangiocarcinoma.

In this review, I describe the gross appearance of intrahepatic and extrahepatic cholangiocarcinomas, correlate the pathologic and imaging findings, consider the mode of spread of these tumors, and discuss the clinical significance of the various growth patterns of cholangiocarcinoma. Vilgrain V. Staging cholangiocarcinoma by imaging studies. It is typically a microscopic lesion with a flat or micropapillary dysplastic epithelium. 2008;10 (2): 106-9. Lobar or segmental hepatic atrophy is usually associated with vascular invasion 6. Morphologic classification of cholangiocarcinoma is useful for understanding the biological behavior of this tumor as well as for planning and choosing the appropriate treatment and for predicting prognosis [13, 14]. An intraductal mass can appear as an echogenic mass on sonography and as an enhancing soft-tissue mass on CT (Fig. 8A, 8B, and 8C).

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