Aggressive surgical treatment of intrahepatic cholangiocarcinoma: predictors of outcomes

Roayaie S, Guarrera JV, Ye MQ, et al. (1998) Aggressive surgical treatment of intrahepatic cholangiocarcinoma: predictors of outcomes. J Am Coll Surg, 187(4):365-372
CODE: ICCSR98

Abstract: Median actuarial survivals were 42.9 6 8.9 months for resectable and 6.7 6 3.6 months for unresectable patients (p 5 0.005). Noncurative resection offers a survival advantage over no resection.

Introduction: Lack of etiologic factors such as intrahepatic lithiasis and liver flukes make this tumor 10 times less prevalent in Western countries.3,4

Liver Resection for Hilar and Peripheral Cholangiocarcinomas: A Study of 62 Cases

Madariaga JR, Iwatsuki S, Todo S, Lee RG, Irish W, Starzl TE (1998) Liver resection for hilar and peripheral cholangiocarcinomas: a study of 62 cases. Ann Surg, 227(1):70-79
CODE: ICCJRM98

Abstract: The survival rates for HCCA and PCCA were 79% (+8%) and 67% (+8%) at 1 year; 39% (+10%) and 40% (+9%) at 3 years; and 8% (+7%) and 35% (+10%) at 5 years, respectively.The median survival was 24 (±4) months for HCCA and 19 (+8) months for PCCA.

Surgical management of intrahepatic cholangiocarcinoma: a 31-year experience

Lieser MJ, Barry MK, Rowland C, Ilstrup DM, Nagorney DM (1998) Surgical management of intrahepatic cholangiocarcinoma: a 31-year experience. J Hepatobiliary Pancreat Surg, 5(1):41-47

Abstract: Intrahepatic cholangiocarcinoma (ICC) is the second most common malignant primary tumor of the liver. It is, though, a rare tumor and little is known regarding its natural history, clinicopathologic characteristics, or the outcomes of surgical therapy.

Hepatic Resection and Transplantation for Peripheral Cholangiocarcinoma

Casavilla FA, Marsh JW, Iwatsuki S, et al. (1997) Hepatic resection and transplantation for peripheral cholangiocarcinoma. J Am Coll Surg, 185(5):429-436

Abstract: Recent publications have questioned the role of orthotopic liver transplantation (OLT) in treating advanced or unresectable peripheral cholangiocarcinoma (Ch-Ca). We reviewed our experience with Ch-Ca to determine survival rates, recurrence patterns, and risk factors. Table 3

Cholangiocarcinoma: A compact review of the literature

Ustundag Y, Bayraktar Y (2008) Cholangiocarcinoma: a compact review of the literature. World J Gastroenterol, 14(42):6458-6466
CODE: ICCYU08

Abstract: These tumors progress insidiously, and liver failure, biliary sepsis, malnutrition and cancer cachexia are general modes of death associated with this disease. CC is resectable for cure in only a small percentage of patients.

The Epidemiology of Cholangiocarcinoma

Shaib Y, El-Serag HB (2004) The epidemiology of cholangiocarcinoma. Semin Liver Dis, 24(2):115-125

Introduction
Because ICC originates in small biliary ducts or ductules, it usually presents as an intrahepatic mass and is therefore grouped in some studies with hepatocellular carcinoma (HCC) as a primary liver tumor. However, Klatskin tumors, which occur at the bifurcation of the right and left hepatic ducts, have traditionally been classified as extrahepatic.10,11

Diagnosis and management of cholangiocarcinoma

Sandhu DS, Roberts LR (2008) Diagnosis and management of cholangiocarcinoma. Curr Gastroenterol Rep, 10(1):43-52

Cholangiocarcinoma (CC) is a rare yet frequently fatal tumor that causes significant morbidity and mortality due to late presentation. Radiology is the mainstay of CC diagnosis; however, advances in understanding the pathogenesis of CC, including the role of oncogenes, inflammation-mediated genomic instability, and interleukin-6/STAT-3 signaling pathways, may allow development of new diagnostic and prognostic markers and targets for CC therapy.

Natural History and Prognostic Factors of Advanced Cholangiocarcinoma without Surgery, Chemotherapy, or Radiotherapy: A Large-Scale Observational Study

Park J, Kim MH, Kim KP, et al. (2009) Natural History and Prognostic Factors of Advanced Cholangiocarcinoma without Surgery, Chemotherapy, or Radiotherapy: A Large-Scale Observational Study. Gut Liver, 3(4):298-305

Abstract
Result: The overall survival time of the entire cohort (n=330) was median 3.9 months (range; 0.2 to 67.1). The survival time was significantly shorter in the intrahepatic cholangiocarcinoma group (3.0±5.3 months) than in the hilar cholangiocarcinoma group (5.9±10.1 months; Kaplan-Meier survival analysis).

A new staging system for mass-forming intrahepatic cholangiocarcinoma: analysis of preoperative and postoperative variables

Okabayashi T, Yamamoto J, Kosuge T, et al. (2001) A new staging system for mass-forming intrahepatic cholangiocarcinoma: analysis of preoperative and postoperative variables. Cancer, 92(9):2374-2383


Abstract
RESULTS: The rate of operative mortality in this patient cohort was 5%, and the overall 1-year, 3-year, and 5-year survival rates were 68%, 35%, and 29%, respectively, with a median survival of 19.6 months. A multivariate analysis revealed that independent negative prognostic factors were 1) hepatic regional lymph node metastasis, 2) multiple tumor presentation, 3) symptomatic tumor, and 4) the presence of vascular invasion.

Cholangiocarcinoma A Spectrum of Intrahepatic, Perihilar, and Distal Tumors

Nakeeb A, Pitt HA, Sohn TA, et al. (1996) Cholangiocarcinoma. A spectrum of intrahepatic, perihilar, and distal tumors. Ann Surg, 224(4):463-473; discussion 473-465

Statistical Analysis

All data are presented as percentage of patients or mean ± standard error of the mean. Percentages were compared by chi square analysis, and means were analyzed by analysis of variance. Survival curves were constructed by the Kaplan-Meier technique and were compared by the log-rank test. Cox's proportional hazard survival analysis was used to determine which parameters affected survival. A p value < 0.05 was considered significant.

Cholangiocarcinoma: Modern Advances in Understanding a Deadly Old Disease

Malhi H, Gores GJ (2006) Cholangiocarcinoma: modern advances in understanding a deadly old disease. J Hepatol, 45(6):856-867

Introduction

Most cholangiocarcinomas arise in the absence of any known predisposition (5)

Classification

Cholangiocarcinomas can be classified into 3 distinct categories by anatomic location. These are intrahepatic cholangiocarcinoma, hilar cholangiocarcinoma and distal extrahepatic bile duct cancers (Fig. 1 v Imp).

Cholangiocarcinoma: Morphologic Classification According to Growth Pattern and Imaging Findings

Lim JH (2003) Cholangiocarcinoma: morphologic classification according to growth pattern and imaging findings. AJR Am J Roentgenol, 181(3):819-827


IntroductionThe prognosis for mass-forming and periductal-infiltrating cholangiocarcinomas is generally unfavorable, whereas the prognosis for intraductal-growing cholangiocarcinoma is much better (or excellent) after surgical resection  [11–14].

Guidelines for the diagnosis and treatment of cholangiocarcinoma: consensus document

Khan SA, Davidson BR, Goldin R, et al. (2002) Guidelines for the diagnosis and treatment of cholangiocarcinoma: consensus document. Gut, 51 Suppl 6:VI1-9

DIAGNOSIS
Clinical Features: Cholangiocarcinoma usually presents after the disease is advanced. This is particularly true with more proximal intrahepatic and perihilar tumours obstructing one duct, which often present with systemic manifestations of malignancy, such as malaise, fatigue, and weight loss. Some cases are detected incidentally as a result of deranged liver function tests, or ultrasound scans performed for other indications.

New insights on cholangiocarcinoma

Gatto M, Alvaro D (2010) New insights on cholangiocarcinoma. World J Gastrointest Oncol, 2(3):136-145

Introduction

CCA is characterized by a bad prognosis, with a median survival of less than 24 mo[2-4] and a scarce response to chemotherapy[5-9] The only curative therapy is surgical resection or liver transplantation but, unfortunately, the majority of patients are diagnosed at advanced stage, when surgical therapies are excluded. This should stimulate research on the identification of effective surveillance strategies that would permit detection of early CCA or, better yet, premalignant lesions in patients at increased risk, particularly patients with primary sclerosing cholangitis (PSC). Serum and bile tumor markers, non invasive and endoscopic-based imaging modalities, and histology and cytology have been attempted with varying success[11].

Cholangiocarcinoma – current treatment options

Friman S (2011) Cholangiocarcinoma--current treatment options. Scand J Surg, 100(1):30-34

Summary

In intrahepatic CCA, HR remains the treatment of choice whereas with concomitant liver disease such as cirrhosis or primary sclerosing cholangitis (PSC), LT is the only option.

Cholangiocarcinoma (CCA) is a malignant disease of the epithelial cells in the intra- and extrahepatic bile ducts. While still a rare malignant disease, CCA is the second most common primary malignancy of the liver. The incidence is increasing; especially the incidence of intrahepatic CCA (1).

Cholangiocarcinoma: Advances in Pathogenesis, Diagnosis, and Treatment

Blechacz B, Gores GJ (2008) Cholangiocarcinoma: advances in pathogenesis, diagnosis, and treatment. Hepatology, 48(1):308-321

Introduction

Cholangiocarcinoma (CCA) is an epithelial cancer originating from the bile ducts with features of cholangiocyte differentiation.1 CCA is the second most common primary hepatic malignancy, and epidemiologic studies suggest its incidence is increasing in Western countries. 2 Advanced CCA has a devastating prognosis, with a median survival of <24 months.3 Three different growth patterns of extrahepatic CCA can be observed: (1) periductal infiltrating, (2) papillary or intraductal, and (3) mass forming.4 Intrahepatic CCA typically presents as an intrahepatic mass. An additional goal is to stimulate further interest in this disease with the hope of improving outcomes for this still highly lethal malignancy.

Etiologic and Clinical Characteristics of Peripheral and Hilar Cholangiocarcinoma

Altaee MY, Johnson PJ, Farrant JM, Williams R (1991) Etiologic and clinical characteristics of peripheral and hilar cholangiocarcinoma. Cancer, 68(9):2051-2055

Abstract: Prolonged high alcohol consumption was a prominent feature in both categories (45% and 37%, respectively). Among the women, 35% of those with the peripheral tumor had used oral contraceptive preparations.