A prognostic systemic inflammation score (SIS) in patients with advanced intrahepatic cholangiocarcinoma.

PURPOSE: Systemic-inflammatory response parameters (SIR) are known prognostic markers in different tumour entities, but have not been evaluated in patients with iCCA treated with systemic chemotherapy. Therefore, we evaluated the impact of different SIR markers on the clinical course of patients with advanced iCCA treated at our center. METHODS: SIR markers were retrospectively evaluated in 219 patients with iCCA at the West-German-Cancer-Center Essen from 2014 to 2019. Markers included neutrophil/lymphocyte ratio (NLR), lymphocyte/monocyte ratio (LMR), CRP, and the modified Glasgow-Prognostic-Score (mGPS), which were correlated with clinico-pathological findings, response to chemotherapy (ORR), progression-free (PFS) and overall survival (OS) using Kaplan-Meier analyses, and Cox proportional models. RESULTS: Median overall survival (OS) of the entire cohort was 14.8 months (95% CI 11.2-24.4). Median disease-free survival (DFS) in 81 patients undergoing resection was 12.3 months (95% CI 9.7-23.1). The median OS from start of palliative CTX (OSpall) was 10.9 months (95% 9.4-14.6). A combined Systemic Inflammatory Score (SIS) comprising all evaluated SIR markers correlated significantly with ORR, PFS, and OSpall. Patients with a high SIS (≥ 2) vs. SIS 0 had a significantly inferior OSpall (HR 8.7 95% CI 3.71-20.38, p < 0.001). Multivariate analysis including known prognostic markers (ECOG, CA19-9, LDH, and N- and M-status) identified the SIS as an independent prognostic factor. CONCLUSIONS: Inflammatory markers associate with inferior survival outcomes in patients with iCCA. A simple SIS may guide treatment decisions in patients treated with systemic chemotherapy.

Contrast-Enhanced Ultrasound Patterns for the Non-invasive Diagnosis of Hepatocellular Carcinoma: A Prospective Multicenter Study in Histologically Proven Liver Lesions in a Real-Life Setting Demonstrating the Benefit of Extended Late Phase Observation.

The hallmark for the non-invasive diagnosis of hepatocellular carcinoma (HCC) with contrast-enhanced ultrasound (CEUS) in cirrhosis is arterial phase hyperenhancement (APHE), followed by late-onset (>60 s), mild washout. Large retrospective studies report this pattern of washout to occur in the vast majority of HCCs. However, a prospective multicenter validation of these findings is still missing. Thus, we initiated a prospective multicenter validation study assessing CEUS enhancement patterns in focal liver lesions of patients at risk for HCC. We analyzed lesions that were eventually histology proven in a real-life setting. CEUS patterns were assessed for subgroups of HCC, intrahepatic cholangiocellular carcinoma (iCCA) and non-HCC, non-iCCA lesions. The diagnosis was HCC in 316 lesions (median size: 40 mm), iCCA in 26 lesions (median size: 47.5 mm) and non-HCC, non-iCCA in 53 lesions (median size: 27 mm). Overall, 85.8% of HCCs exhibited APHE. APHE followed by washout occurred in 72.8% of HCCs and 50% of iCCAs and non-HCC, non-iCCA malignancies (p < 0.05). Early and marked washout was associated more commonly with iCCA; HCCs exhibited mostly late and mild washout (onset >4-6 min in 10% of cases). Our prospective data confirm that the typical pattern of APHE followed by late-onset, mild washout occurs in the majority of HCCs.

Quantitative analysis of gadoxetic acid-enhanced MRI for the differential diagnosis of focal liver lesions: Comparison between estimated intralesional gadoxetic acid retention by T1 mapping and conventional processing methods.

PURPOSE: To compare the estimated quantity of intratumor gadoxetic acid retention using T1 mapping of gadoxetic acid-enhanced magnetic resonance imaging (MRI) versus conventional processing methods for the differential diagnosis of focal liver lesions. METHODS: Seventy patients with hepatic lesions (colorectal metastasis (CRM) [n = 28], hepatocellular carcinoma (HCC) [n = 20], hemangioma [n = 12], and intrahepatic cholangiocarcinoma (ICC) [n = 10]) underwent gadoxetic acid-enhanced MRI, including pre- and post-contrast T1-weighted imaging and T1 mapping. Quantitative analyses included the lesion-to-liver signal intensity ratio (SIR) on hepatobiliary phase images, the pre- and post-contrast lesion T1 value difference (ΔT1 [ms]), and the lesion retention index (LRI [%]), which was the estimated intralesional gadoxetic acid retention calculated on pre- and post-contrast T1 maps using a two-compartment pharmacokinetic model. Results were compared between the four subcategories of focal liver lesions using the Kruskal-Wallis test, followed by the post-hoc Dunn's test and receiver operating characteristic (ROC) analysis to distinguish between pairs of the four lesion subcategories. RESULTS: This study identified significant differences in the LRI of the four lesion subcategories (p <  0.01), without significant differences in ΔT1 or SIR. Post-hoc analysis demonstrated significant differences in CRM vs. hemangioma (p <  0.01), hemangioma vs. ICC (p <  0.01), and HCC vs. ICC (p =  0.047) for the LRI. CONCLUSIONS: The quantity of intratumor gadoxetic acid retention estimated using pre- and post- contrast T1 mapping could distinguish focal liver lesions, unlike conventional processing methods, and captured unique lesion characteristics.

[Long-term results of surgical treatment of intrahepatic cholangiocarcinoma]. / Otdalennye rezul'taty khirurgicheskogo lecheniya vnutripechenochnogo kholangiotsellyulyarnogo raka.

OBJECTIVE: To evaluate the long-term outcomes of surgical treatment of intrahepatic cholangiocarcinoma depending tumor dimensions, vascular invasion, lymph node metastases, cellular differentiation and quality of resection. MATERIAL AND METHODS: There were 46 patients with intrahepatic cholangiocellular cancer. Extended hemihepatectomy was made in 14 patients (30.4%), resection of two and three liver segments - in 17 cases (36.9%), standard hemihepatectomy - in 15 patients (32.6%). Liver resection was combined with extrahepatic bile duct resection in 5 (10.9%) patients. Liver resection was followed by biopsy of specimens. Dimension and number of tumors, differentiation grade, resection margin, liver capsule invasion, vascular invasion and regional lymph node metastases were analyzed. Forty-four (95.6%) patients were followed-up in long-term postoperative period. Statistical analysis was performed using Statistica 13.2 (Dell Inc., USA) and IBM SPSS Statistics v.25 (IBM Corp., USA) software package. Survival was analyzed using the Kaplan-Meier method. Overall 1-, 3- and 5-year survival rates with two-sided 95% confidence intervals (95% CI) were calculated using IBM SPSS Statistics v.25 software. RESULTS: Median survival was 37 months, 1-year - 75.9% (60.9-90.9%), 3-year - 57.6% (35.5-79.6%), 5-year - 36% (8.2-63.7%). Median survival after R1 resection was 37 months, R2 resection - 12 months. Median survival was not achieved in R0 group. We found significant differences in overall survival depending on quality of resection. Tumor dimension over 5 cm, low-grade adenocarcinoma, microvascular invasion and lymph node metastases were associated with impaired postoperative survival. However, differences were not significant. CONCLUSION: The main surgical strategy in patients with intrahepatic cholangiocarcinoma should be ensuring microscopically negative resection margin.

Treatment of Combined Hepatocellular and Cholangiocarcinoma.

Combined hepatocellular and cholangiocarcinoma (HCC-CC) is a rare primary liver cancer. It is constituted by neoplastic cells of both hepatocellular and cholangiocellular derivation. Different histology types of HCC-CC have been reported, hinting at heterogeneous carcinogenic pathways leading to the development of this cancer. Due to its rarity and complexity, mixed HCC-CC is a scantly investigated condition with unmet needs and unsatisfactory outcomes. Surgery remains the preferred treatment in resectable patients. The risk of recurrence, however, is high, especially in comparison with other primary liver cancers such as hepatocellular carcinoma. In unresectable or recurring patients, the therapeutic options are challenging due to the dual nature of the neoplastic cells. Consequently, the odds of survival of patients with HCC-CC remains poor. We analysed the literature systematically about the treatment of mixed HCC-CC, reviewing the main therapeutic options and their outcomes and analysing the most interesting developments in this topic with a focus on new potential therapeutic avenues.

Tumor-Infiltrating Lymphocytes and Macrophages in Intrahepatic Cholangiocellular Carcinoma. Impact on Prognosis after Complete Surgery.

BACKGROUND: Immune infiltrate impacts prognosis of several tumors. To assess the prognostic impact of tumor-infiltrating lymphocytes and macrophages in patients undergoing resection for intrahepatic cholangiocellular carcinoma (ICC). METHODS: All consecutive patients undergoing surgery for ICC between 2008 and 2016 were considered. Inclusion criteria were complete resection and follow-up > 12 months. Tissue sections were immunostained for CD3+, CD4+, CD8+, Foxp3+, and CD68+. The number of positive cells was quantified using a computer-aided image analysis system. Different cut-off values were tested as predictors of overall survival (OS). RESULTS: Fifty-three patients were analyzed. ICC were T1 in 28 patients, multifocal in 11, and N+ in 13. After a median follow-up of 42 months, 5-year OS was 52.1%. The following immune infiltrate values were associated with better OS: CD3+ > 0.10% (5-year OS 63.3% vs. 13.6% if ≤ 0.10%, p = 0.001); CD8+ > 0.10% (56.2% vs. 28.6% if ≤ 0.10%, p = 0.051); Foxp3+ absent (59.4% vs. 16.0% if present, p = 0.049). CD4+ and CD68+ infiltrates were not associated with OS. Three-year OS rates in patients with 0, 1, and ≥ 2 negative prognostic factors were 73.6%, 47.3%, and 14.3%, respectively (p < 0.001). CD3+ infiltrate stratified prognosis in T1 tumors (3-year OS 71.7% if CD3+ > 0.10% vs. 14.3% if ≤ 0.10%, p < 0.001). CONCLUSIONS: Tumor-infiltrating lymphocytes are associated with prognosis of ICC patients after complete surgery. CD3+ and CD8+ infiltrate is associated with higher survival and lower recurrence risk, while Foxp3+ infiltrate is associated with worse prognosis. CD3+ infiltrate allows refining prediction of prognosis in early tumors.

Prognostic value of inflammation-based indexes for intrahepatic cholangiocarcinoma following curative resection.

It is widely acknowledged that inflammatory indices may serve as effective prognosis indicators for various malignancies. In the present study, the prognostic value of systemic inflammatory biomarkers for patients undergoing curative resection for intrahepatic cholangiocellular carcinoma (ICC) was investigated. Clinical data of ICC patients who underwent curative resection between September 2008 and July 2017 were collected. Inflammatory indictors were analyzed using the Area Under the Receiver Operating Characteristic Curve. Indictors that were significantly associated with the overall survival (OS) were used to establish a systemic inflammation-based score system and tested via nomogram using R software. The neutrophil To lymphocyte ratio (NLR) and lymphocyte to macrophages ratio (LMR) were significantly associated with the OS and disease-free survival of the patients. High NLR and low LMR were associated with worse clinicopathological and survival outcomes. The univariate and multivariate analyses indicated that tumor T stage, incisal margin, NLR and LMR were associated with the OS of the patients. The systemic inflammation-based scoring system based on LMR and NLR demonstrated a stronger discriminatory capacity and may serve as a useful prognostic parameter for patients undergoing curative resection for ICC. Low LMR and high NLR were observed to be associated with poor prognosis and worse clinical outcomes for patients with ICC undergoing curative surgery. A combined inflammation-based scoring system based on LMR and NLR may effectively predict the outcomes and serve as a novel prognostic predictor for these patients.

Microvessel density and angiogenesis in primary hepatic malignancies: Differential expression of CD31 and VEGFR-2 in hepatocellular carcinoma and intrahepatic cholangiocarcinoma.

BACKGROUND: Microvessel density is an indicator of tumor-driven neoangiogenesis. Hepatocellular carcinoma (HCC) and intrahepatic cholangiocarcinoma (ICC) have distinct vascular patterns, which are also reflected in their imaging characteristics. Since a significant proportion of HCC are treated without biopsy confirmation, it is essential to discriminate HCC and ICC radiologically. The aim of our study was therefore to compare microvessel density and expression of VEGFR-2 in HCC and ICC, since these data may ultimately help us to better understand their imaging characteristics. Whereas CD31 documents vessel density, VEGFR-2 expression is an indicator of tumor-related neoangiogenesis. METHODS: CD31 and VEGFR-2 expressing microvessels were quantified on tissue microarrays of 95 resection specimens of HCC and 47 cases of ICC. Microvessel density was evaluated by counting immuno-reactive vascular structures both within the tumor and adjacent liver control tissue, respectively. Further 16 cases of ICC were immunostained for CD31 and VEGFR-2 on full sections. RESULTS: The frequency of VEGFR-2 (46.2/HPF; range 0-150) and CD31 (61.2/HPF; range 2.6-140) expressing vascular structures was significantly increased in HCC compared to adjacent liver parenchyma (VEGFR-2 33.3/HPF, range 0-87, CD31 21.4/HPF, range 0-78, both p < 0,001). ICC revealed significantly less VEGFR2-positive microvessels (15.4/HPF; range 2-77) compared to matched control tissue (42.3/HPF; range 4.6-109), whereas microvessel density with CD31 was comparable between ICC and adjacent liver (32.1/HPF; range 5.3-78 versus 28.0/HPF; range 5.3-57; p = 0.89). In ICC, the tumor-to-normal microvessel density ratio was 0.38 for VEGFR-2 and 1.24 for CD31. These ratios were nearly identical (VEGFR: 0.38; CD31: 0,97) for the 16 cases of ICC studied on whole sections, confirming the validity of the TMA approach. In contrast, ratios of VEGFR-2 and CD31 in HCC vs. adjacent liver were significantly higher (VEGFR: 2.23; CD31: 6.57). Expression of VEGFR-2 by tumor cells was not observed in any of the cases. CONCLUSIONS: HCC and ICC differ significantly in their microvessel density, confirming the hypovascular nature of ICC as compared to the hypervascularity of HCC. Of note, inverse tumor-to-normal ratios of microvascular VEGFR-2 expression between the two neoplasms indicate distinct features of neoangiogenesis. Whether these differences can be exploited for improvements in imaging of hepatic tumors and may play a role for anti-angiogenic treatment strategies requires further studies.

Research progress in the diagnosis and treatment of intrahepatic cholangiocellular carcinoma / 介入放射学杂志

Intrahepatic cholangiocellular carcinoma (ICC) is a primary tumor originating from the epithelial cells of bile duct. In recent years, incidence of ICC in the world is on the rise, and it has become the second common malignant tumors of the liver, with its incidence being next only to hepatocellular carcinoma (HCC). The onset of ICC is insidious, its clinical manifestations are lack of specificity, most of the patients are already in the advanced stage when the diagnosis is confirmed, thus, affecting the treatment and prognosis. Therefore, early diagnosis and treatment is essential. The radical treatment plan is mainly surgical excision, and other treatment options include systemic chemotherapy, local ablation, transcatheter arterial chemoembolization (TACE), selective intraarterial radiotherapy with yttrium-90 microspheres (SIRT-90Y), 125I seed implantation, etc. This article aims to make a comprehensive introduction about the recent advances in the diagnosis and treatment of ICC. (J Intervent Radiol, 2018, 27:285-289)

Differentiation of Intrahepatic Cholangiocellular Carcinoma from Hepatocellular Carcinoma in the Cirrhotic Liver Using Contrast-enhanced MR Imaging.

RATIONALE AND OBJECTIVES: This study aimed to investigate the potential of contrast-enhanced magnetic resonance imaging features to differentiate between mass-forming intrahepatic cholangiocellular carcinoma (ICC) and hepatocellular carcinoma (HCC) in cirrhotic livers. MATERIALS AND METHODS: This study, performed between 2001 and 2013, included 64 baseline magnetic resonance imaging examinations with pathohistologically proven liver cirrhosis, presenting with either ICC (n = 32) or HCC (n = 32) tumors. To distinguish ICC form HCC tumors, 20 qualitative single-lesion descriptors were evaluated by two readers, in consensus, and statistically classified using the chi-square automatic interaction detection (CHAID) methodology. Diagnostic performance was assessed by a receiver operating characteristic analysis. RESULTS: The CHAID algorithm identified three independent categorical lesion descriptors, including (1) liver capsular retraction; (2) progressive or persistent enhancement pattern or wash-out on the T1-weighted delayed phase; and (3) signal intensity appearance on T2-weighted images that could help to reliably differentiate ICC from HCC, which resulted in an AUC of 0.807, and a sensitivity and specificity of 68.8 and 90.6 (95% confidence interval 75.0-98.0), respectively. CONCLUSIONS: The proposed CHAID algorithm provides a simple and robust step-by-step classification tool for a reliable and solid differentiation between ICC and HCC tumors in cirrhotic livers.

Diagnostic accuracy and interobserver variability of Dynamic Vascular Pattern (DVP) in primary liver malignancies - A simple semiquantitative tool for the analysis of contrast enhancement patterns.

BACKGROUND: Contrast-enhanced ultrasound (CEUS) is a valuable tool in the diagnostic approach of focal liver lesions, but occasionally subjective and observer-dependent. Semiquantitative evaluation of dynamic CEUS (DCEUS) with standardised software programmes such as Dynamic Vascular Pattern (DVP) could help to improve diagnostic accuracy and objectivity in liver tumour assessment. OBJECTIVES: The present study aimed at evaluation of diagnostic accuracy of DVP in a clinical setting. MATERIALS AND METHODS: DVP images of 52 focal liver lesions [30 hepatocellular carcinomas (HCCs), 15 intrahepatic cholangiocellular carcinomas (ICCs), 7 focal nodular hyperplasias (FNHs)] were analysed by four blinded observers with different levels of CEUS-experience. Diagnostic accuracies for the assessment of dignity and entity were evaluated. RESULTS: Mean sensitivity, specificity, positive and negative predictive value for detection of malignancy with DVP were 48.4% /67.8% /92.7% and 29.3%, respectively. Total diagnostic accuracies for dignity/entity were 63.9% /38.5% (HCC: 58.3% /25.8%; ICC: 73.3% /50%; FNH: 67.9% /67.9%). Interreader-agreement was moderate (κ= 0.42-0.58). Differential diagnosis between ICC and HCC was most challenging. CONCLUSION: Although developed to improve diagnostic accuracy and objectivity in the assessment of focal liver lesions, DVP alone seems insufficient for differential diagnosis of HCC, ICC and FNH and cannot replace the skills of an experienced observer.

Current Technical Issues for Surgery of Primary Liver Cancer.

Primary liver cancer is the fifth most common cancer worldwide. Apart from liver transplantation, surgical resection has been accepted as the effective local treatment for hepatocellular carcinoma (HCC), one of the most common primary liver cancers. Recent technological innovations including navigation technology and intraoperative real-time fluorescence guidance have been utilized for liver resections in clinical practice. With respect to liver resection techniques, the laparoscopic approach has been increasingly gaining popularity as one of the minimally-invasive treatments of HCC. These technological innovations and technical advancements are expected to further improve the safety and efficacy of liver resections.

Combined hepatocellular and cholangiocarcinoma originating from the same clone: a pathomolecular evidence-based study.

BACKGROUND: Combined hepatocellular and cholangiocarcinoma (CHC) is a unique subtype of liver cancer comprising both hepatocellular carcinoma (HCC) and intrahepatic cholangiocarcinoma (ICC); however, its cellular origin remains unclear. The purpose of this study was to investigate the clinicopathologic features and the clonal relationship between HCC and ICC in 34 patients with CHC. METHODS: The clinicopathologic features and prognosis of the 34 CHC patients were compared with those of 29 patients with separated HCC and ICC (SHC). Loss of heterozygosity (LOH) at 10 highly polymorphic microsatellite markers was detected in 16 CHC and 10 SHC tissues for determination of the clonal origin of CHC. Expression of hepatocyte markers [hepatocyte paraffin 1 (Hep Par 1) and glypican 3 (GPC3)] and cholangiocyte markers [cytokeratin (CK)7 and 19] in tumor tissues was examined by immuno histochemical analysis. RESULTS: In the 16 CHC specimens, the difference in LOH patterns between HCC and ICC was less than 30%, suggesting the same clonal origin of HCC and ICC. Consistent with this finding, immunohistochemical analysis revealed that hepatocyte markers (Hep Par 1 and GPC3) and cholangiocyte markers (CK7 and CK19) were simultaneously expressed in both the HCC and ICC components in 52.9% of CHC specimens, suggesting that the two components shared a similar phenotype with hepatic progenitor cells (HPCs). On the contrary, in all 10 SHC cases, the difference in LOH patterns between the HCC and ICC components was greater than 30%, suggesting different clonal origins of HCC and ICC. Overall survival and disease-free survival were shorter for patients with CHC than for patients with SHC (P < 0.05). CONCLUSIONS: Our results suggest that the HCC and ICC components of CHC may originate from the same clone, having the potential for dual-directional differentiation similar to HPCs. CHC tended to exhibit the biological behaviors of both HCC and ICC, which may enhance the infiltrative capacity of tumor cells, leading to poor clinical outcomes for patients with CHC.

Radioembolization with (90)Y resin microspheres for intrahepatic cholangiocellular carcinoma: prognostic factors.

AIM: To investigate the prognostic factors that predict overall survival after radioembolization in patients with cholangiocellular carcinoma. METHODS: The study comprised 16 patients who received radioembolization with Y(90) resin microspheres for cholangiocarcinoma. The statistical relationships between overall survival after radioembolization and age, number, dimension and fluorodeoxyglucose (FDG) avidity of liver lesions, liver tumor load, presence of extrahepatic metastases, and radiological response were analyzed. RESULTS: Mean 1.7 ± 0.1 GBq(90)Y microspheres were administered to a total of 16 patients (mean age: 55.37 ± 17.7; 8 males, 8 females). Mean AST, ALT, and total bilirubin levels were calculated as 35 ± 15, 40 ± 37 IU/L, and 0.77 ± 0.37 mG/dL, respectively. In 6 patients, 1 liver lesion was determined, in 2 patients ≤ 5, and in 8 patients >5, with dimensions varying between 12 and 120 mm. The liver lesions of 13 patients were FDG avid (mean SUVmax: 7.4 ± 2.2). Extrahepatic metastases were demonstrated in 5 patients. Tumor load of 4, 8, and 4 patients was calculated as <25, 25-50, and >50%, respectively. Five patients were responsive to treatment. During the follow-up period of 243 (range 98-839) days, 12 patients died. In Cox-regression analysis, FDG avidity (p = 0.02), the dimensions (p = 0.03) of the liver lesion, tumor load (p = 0.02), and radiological response (p = 0.01) were found to be statistically significant parameters predictive of overall survival after radioembolization (p = 0.006). CONCLUSION: FDG avidity and the dimension of the largest liver lesion, tumor load, and radiological response are prognostic factors in patients receiving radioembolization for cholangiocellular carcinoma. Patients with lower tumor load, FDG-negative tumors, and smaller tumors seem to survive longer after radioembolization.

Intrahepatic cholangiocellular carcinoma and hepatocellular carcinoma developed after a 6-year sustained virological response to interferon therapy for chronic hepatitis C.

A 67-year-old male patient presenting with chronic hepatitis C (CHC) achieved a sustained virological response (SVR) following 6 months of treatment with 6 million units of beta-interferon (IFN). The SVR state continued for 6 years. Hepatocellular carcinoma (HCC) developed in liver segments 4 and 5, and was treated with transcatheter arterial chemoembolization, followed by radiofrequency ablation of the tumors. A recurrence of HCC occurred in segment 4 one and a half years after the initial treatment for HCC and a new tumor also developed in segment 8. These tumors were diagnosed to be recurrent HCC, and the three hepatic segments were resected. The pathological examination and immunostaining of the tumors revealed the tumor in segment 4 to be a well to moderately differentiated typical HCC. On the other hand, the tumor in segment 8 was a moderately to poorly differentiated adenocarcinoma and was diagnosed as an intrahepatic cholangiocellular carcinoma (ICC). HCC developed from CHC in a patient who achieved a 6-year SVR after IFN therapy, followed one and a half years later by the development of a heterochronous ICC at a different site, thus indicating the presence of HCC-ICC double cancer. This was an exceedingly rare and clinically important case in terms of the carcinogenic mechanism of HCC and ICC from a post-SVR CHC patient. We have to be aware of the possible development not only of HCC but of ICC after SVR in CHC patients.

Mucin-Producing Intrahepatic Cholangiocelluar Carcinoma Presenting as a Focal Dilatation of the Intrahepatic Bile Duct

A case of a mucin-producing intrahepatic cholangiocellular carcinoma (MPCC) is reported. A 58-year old female presented with epigastric discomfort of several years duration. The physical examination and laboratory findings were normal. Abdominal ultrasonography (US) and computed tomography (CT) showed a focal dilatation of the right posterior intrahepatic bile duct. There was no abnormal mass in the liver parenchyma. Endoscopic retrograde cholangiopancreaticography (ERCP) showed a filling defect in the right posterior hepatic duct. There was no anatomical abnormality and abnormal staining on the heaptic angiography. At the operation, the right posterior hepatic duct was filled with mucin. The patient had a right posterior segmentectomy. Histologically, a 2.5 X 0.6 X 0.6 cm sized mucin-producing intrahepatic cholangiocellular carcinoma was found in segment 6 of the liver. The postoperative recovery was good, and the patient has had a good social life for the last 3 years, with no evidence of tumor recurrence. In patients with a focal dilatation of the intrahepatic bile duct on CT or US with no underlying cause, an intrahepatic malignancy has to be suspected.