Incidence, Etiologic Aspects and Clinicopathologic Features in Intrahepatic Cholangiocellular Carcinoma: A Study of 51 Cases From a Low-Endemicity Area (original) (raw)
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1996
The prevalence of primary liver cancer (PLC) varies throughout the world. It has been attributed to variations in incidence of the predominant histological type, hepatocellular carcinoma (HCC). The incidence of PLC types other than HCC such as cholangiocellular carcinoma (CCC) is far less known, especially in low-incidence areas. The aetiology of HCC and other PLC types is obscure, with the exception of the association between HCC and cirrhosis as well as chronic viral hepatitis. The present retrospective incidence and aetiology study concerns a well-defined population from a period with a high autopsy frequency. Preserved biopsy specimens were re-evaluated histopathologically and patient records were studied. Among 590 histologically verified cases of PLC, HCC constituted 90%, CCC 8% and a mixed form of these types 1%. At the end of the study period the annual age-standardised incidence rate of HCC was 3.6 cases per 100 000 inhabitants. Other PLC types were hepatoblastoma (n = 3), fibrolamellar carcinoma (n = 2), angiosarcoma (n = 1) and infantile haemangioendothelioma (n = 1), each constituting less than I % of the PLC cases. Comparing HCC with CCC we found that cirrhosis (70%) and alcoholism (21%) was significantly more frequent in HCC, and cholelithiasis was significantly more common (60%) in patients with CCC. In the majority of the PLC cases with liver cirrhosis this disorder was unknown before diagnosis of the tumour.
Nature Reviews Gastroenterology & Hepatology, 2016
Cholangiocarcinoma (CCA) is a heterogeneous group of malignancies that can emerge at every point of the biliary tree, from the canals of Hering to the main bile duct 1,2. According to their anatomical location, CCAs are classified as intrahepatic (iCCA), perihilar (pCCA) and distal CCA (dCCA), which have particular similarities but also important inter-tumour and intra-tumour differences that can affect the pathogenesis and outcome. CCAs, taken together, represent the second most frequent type of primary liver cancer and ~3% of all gastrointestinal neoplasias 1. However, the epidemiological profile of CCA and its subtypes (FIG. 1) displays enormous geographical variation, reflecting the exposure to different risk factors. Although in most countries CCA is a rare cancer (incidence <6 cases per 100,000 people), its incidence is exceptionally high in some countries and regions, including Chile, Bolivia, South Korea and North Thailand 1. In general, a progressive increase in iCCA incidence worldwide was reported up to the end of the past century, reaching a plateau in the past 10 years. By contrast, the incidences of both pCCA and dCCA seem to be decreasing 1. CCAs are generally asymptomatic in early stages and are usually diagnosed when the disease has already metastasized, by combining nonspecific biomarkers in serum and/or biopsy samples, as well as imaging methods 3,4. Late diagnosis compromises the effective therapeutic options, which are based on surgical resection and/or liver transplantation, whereas chemotherapies are virtually palliative given the marked chemoresistance of this cancer 4-6. Tumour size and other features such as anatomical location, vascular and lymph node invasion, and metastasis condition the potential surgical and/or radiological options but chances of recurrence are very high 4,5. Individual characterization (that is, genomic, epigenetic and molecu lar) of each tumour might provide valuable information on
Cancer of the Liver and Bile Ducts
Oncology, 2006
Hepatocellular carcinoma (HCC) is the fifth most common neoplasm in the world and the third most common cause of cancer death worldwide. 1 More than 500,000 deaths per year are attributed to HCC, representing 10% of all deaths from cancer. In select areas of Asia and Africa, HCC is the most common cause of death due to cancer. The incidence in Europe and the United States is relatively low but is increasing. In Europe, HCC is now the leading cause of death among patients with cirrhosis. 2 In the United States, epidemiologic studies have demonstrated a doubling of HCC incidence over the past two decades. 3 This increase, which has been attributed to the increasing prevalence of chronic hepatitis C virus (HCV) infection, is expected to continue over the next two decades, given the lag time between the onset of chronic hepatitis and development of HCC.
Pathobiology, 2016
Objectives: Analysis of a large local autopsy collective to gather epidemiological and histopathological data on hepatocellular carcinoma (HCC). Methods: We examined a large dataset of 44,104 autopsies performed at the Institute of Pathology, Basel, Switzerland, including 2 autopsy collectives (1969-1983 and 1988-2012) to gather current data on HCC in the advanced stage. A total of 398 HCC were diagnosed, accounting for around 1% of all autopsies. Results: As expected, most patients developing HCC had advanced stages of liver fibrosis or cirrhosis (F3/F4). However, in the more recent autopsy collective (1988-2012), our data also show an increase of HCC arising in livers without or with only mild to moderate fibrosis (F0-F2). Extrahepatic metastasis was found in 156 of 398 HCC (39.1%), with lung metastasis (74.5%) being the most common, followed by the bones (24.8%) and adrenal glands (19.1%). Conclusions: Our data therefore seem to suggest that, in the last 2 decades, despite the in...
Journal of Clinical Oncology, 2011
To identify factors associated with outcome after surgical management of intrahepatic cholangiocarcinoma (ICC) and examine the impact of lymph node (LN) assessment on survival. Patients and Methods From an international multi-institutional database, 449 patients who underwent surgery for ICC between 1973 and 2010 were identified. Clinical and pathologic data were evaluated using uni-and multivariate analyses. Results Median tumor size was 6.5 cm. Most patients had a solitary tumor (73%) and no vascular invasion (69%). Median survival was 27 months, and 5-year survival was 31%. Factors associated with adverse prognosis included positive margin status (hazard ratio [HR], 2.20; P Ͻ .001), multiple lesions (HR, 1.80; P ϭ .001), and vascular invasion (HR, 1.59; P ϭ .015). Tumor size was not a prognostic factor (HR, 1.03; P ϭ .23). Patients were stratified using the American Joint Committee on Cancer/International Union Against Cancer T1, T2a, and T2b categories (seventh edition) in a discrete step-wise fashion (P Ͻ .001). Lymphadenectomy was performed in 248 patients (55%); 74 of these (30%) had LN metastasis. LN metastasis was associated with worse outcome (median survival: N0, 30 months v N1, 24 months; P ϭ .03). Although patients with no LN metastasis were able to be stratified by tumor number and vascular invasion (N0; P Ͻ .001), among patients with N1 disease, multiple tumors and vascular invasion, either alone or together, failed to discriminate patients into discrete prognostic groups (P ϭ .34). Conclusion Although tumor size provides no prognostic information, tumor number, vascular invasion, and LN metastasis were associated with survival. N1 status adversely affected overall survival and also influenced the relative effect of tumor number and vascular invasion on prognosis. Lymphadenectomy should be strongly considered for ICC, because up to 30% of patients will have LN metastasis.
Clinicoradiologic and sonographic patterns of metastasis in hepatocellular carcinoma
Journal of the National Medical Association
Hepatocellular carcinoma (HCC) is usually diagnosed at an advanced stage, when little remedy could be offered. There is a need for relatively affordable, available and noninvasive tests for diagnosis, staging and detection of metastasis among individuals at risk. A clinical, chest radiographic (CXR) and abdominal ultrasonographic examination was carried out to detect and evaluate the pattern of metastasis among 53 untreated patients. One patient had clinical paraparesis with no outward evidence of metastasis. CXR revealed lung metastasis in 11 (20.8%), with multifocal deposits and bilateral involvement in 10 (18.8%), and unilateral single deposit in one. Two (3.8%) patients had perihilar lymphadenopathy and consolidation, respectively, while 18 (34%) patients had elevated right hemidiaphragm and four (7.5%) had pleural effusion.
Annals of Surgery, 2007
Objective: To assess long-term survival and prognostic factors in a large series of patients with bile duct cancer. Summary Background Data: The incidence of bile duct cancer is low but increasing. Determinants of survival vary in the literature, due to a lack of sufficient numbers of patients in most series. Methods: We studied 564 consecutive patients with bile duct cancer operated upon between 1973 and 2004. Patients were divided into intrahepatic, perihilar, and distal groups. Principle outcome measures were complications, 30-day mortality, and survival. Results: Of the 564 patients, 44 (8%) had intrahepatic, 281 (50%) had perihilar, and 239 (42%) had distal tumors. Approximately half (294, 52%) were treated before 1995, while 270 (48%) were treated thereafter. The perioperative mortality rate was 4%. In log-rank analyses, survival was higher in the later time period (P ϭ 0.002), in patients with intrahepatic disease (P ϭ 0.001), with negative resection margins (P Ͻ 0.001), with well/moderately differentiated tumors (P Ͻ 0.001), and those with negative lymph nodal status (P Ͻ 0.001). In multivariate analysis, negative margins (P Ͻ 0.001), tumor differentiation (P Ͻ 0.001), and negative nodal status (P Ͻ 0.001), but not tumor diameter, were significant independent prognostic factors. In R0-resected patients, lymph node status (P Ͻ 0.001), but not tumor diameter, histology, or differentiation, further predicted survival. The median survivals for R0-resected intrahepatic, perihilar, and distal tumors were 80, 30, and 25 months, respectively, and the 5-year survivals were 63%, 30%, and 27%, respectively. Conclusion: R0 resection remains the best chance for long-term survival, and lymph node status is the most important prognostic factor following R0 resection.
Digestive and Liver Disease, 2008
Intrahepatic cholangiocarcinoma and colorectal cancer liver metastasis are the most primary and secondary adenocarcinoma of the liver, respectively. A large-scale long-term comparative study of these two cohort patient is lacking. A total of 166 colorectal cancer liver metastasis patients and 206 intrahepatic cholangiocarcinoma patients who had undergone curative liver resection were retrospectively analysed. Among 206 intrahepatic cholangiocarcinoma, there were 47 intraductal growth type-intrahepatic cholangiocarcinoma and 159 non-intraductal growth type-intrahepatic cholangiocarcinoma. The demographics, clinicopathological data, immunohistochemical study and survival were analysed. The intrahepatic cholangiocarcinoma patients were more female-predominated, associated with hepatolithiasis, symptomatic, jaundiced, and with larger tumour size compared with those of colorectal cancer liver metastasis. Prognostic factors of intrahepatic cholangiocarcinoma were pathologic staging, histologic pattern and section margin; whereas prognostic factors of colorectal cancer liver metastasis were rectal origin, differentiation, section margin and bilobar distribution. CK7 and CK20 differentiated majority of intrahepatic cholangiocarcinoma from colorectal cancer liver metastasis, while CDX2 and MUC5AC helped to differentiate inconclusive cases. The 1-, 3-, 5- and 10-year survival rates of colorectal cancer liver metastasis were 77%, 31%, 20% and 14%, compared to 53%, 21%, 13% and 7% of intrahepatic cholangiocarcinoma (p=.0001). Furthermore, the survival of colorectal cancer liver metastasis was comparable to staged II intrahepatic cholangiocarcinoma (p=.8866) and intraductal growth type-intrahepatic cholangiocarcinoma (p=.1915). Demographics, precipitating factor, clinical manifestations, and prognostic factors of colorectal cancer liver metastasis and intrahepatic cholangiocarcinoma differed remarkably. High incidence of CDX2 and MUC2 expression in colorectal cancer liver metastasis and intraductal growth type-intrahepatic cholangiocarcinoma might explain their similar cytoarchitecture and survival.