Cholangiocarcinoma is the most common biliary malignancy and the second most common hepatic malignancy following hepatocellular carcinoma. It can be classified anatomically as intrahepatic cholangiocarcinoma (iCCA), perihilar CCA, or distal CCA. The treatment of choice for iCCA is surgical resection, but only those with potentially resectable tumors can undergo surgery. The standard regimen for advanced stage iCCA is gemcitabine and cisplatin. We encountered two unusual cases of iCCA patients who were initially diagnosed as unresectable cases and underwent systemic chemotherapy, which showed great response and therefore enabled radical operation. The patients show that even though iCCA is a challenging disease, patients with good response to chemotherapy may have a chance to undergo radical surgery.
Carcinoma, Hepatocellular ; Cholangiocarcinoma ; Cisplatin ; Drug Therapy ; Humans

The prognosis for hilar cholangiocarcinoma is limited by tumor spread along the biliary tree leading to refractory obstructive cholestasis, cholangitis, and liver failure. Palliation with biliary endoprostheses results in median survival times of 4-6 months for advanced bile duct cancer. Photodynamic therapy (PDT) is a local photochemical tumor treatment consisting of a photosensitizing agent combined with laser irradiation of a distinct wavelength. Tumor ablation with PDT combined with biliary stenting reduces cholestasis and significantly improves median survival time. However, the treatment is not widely available, and the photosensitizers used for PDT cause prolonged photosensitivity. Optimum control of tumor spread along the bile ducts and control of cholestasis and cholangitis will prolong survival in one to two thirds of patients, and renders them suitable for other antitumor therapies.
Bile Duct Neoplasms/*drug therapy ; *Bile Ducts, Intrahepatic ; Cholangiocarcinoma/*drug therapy ; Humans ; *Photochemotherapy ; Treatment Outcome

Combined hepatocellular-cholangiocarcinoma (HCC-CC) is a primary liver cancer with histopathologic features of both hepatocelluar carcinoma and cholangiocarcinoma. As combined HCC-CC has been associated with poor outcomes, accurate diagnosis and proper treatment planning for patients are considered to be important for improving survival. Currently, surgery is known as the only treatment modality offering potential cure for localized disease. However, there are little published treatment options for advanced or recurrent disease. Furthermore, no published reports exist in respect to the applying successful curative resection after neoadjuvant therapy for advanced combined HCC-CC. Here, we report a case of combined HCC-CC subtype with stem cell feature, intermediate type who underwent curative surgical resection after neoadjuvant chemotherapy consisting of cisplatin and gemcitabine. Pathologic report revealed negative resection margin and follow-up imaging study shows no evidence of tumor recurrence.
Cholangiocarcinoma ; Cisplatin ; Diagnosis ; Drug Therapy* ; Follow-Up Studies ; Humans ; Liver Neoplasms ; Neoadjuvant Therapy ; Recurrence ; Stem Cells

BACKGROUND/AIMS: Standard treatments are not available for hilar nonresectable cholangiocarcinoma (NCC). It is unknown whether combination therapy of photodynamic therapy (PDT) plus systemic chemotherapy is superior to PDT alone. METHODS: We retrospectively reviewed 68 patients with hilar NCC treated with either PDT plus chemotherapy (PTD-C) or PDT monotherapy (PDT-M). The primary endpoint was the mean overall survival rate. Secondary endpoints included the 1-year survival rate, risk of cholangitic complications, and outcomes, which were evaluated according to the chemotherapy protocol. RESULTS: More than 90% of the study population had advanced hilar NCC Bismuth type III or IV. In the PDT-M group (n=35), the mean survival time was 374 days compared with 520 days in the PDT-C group (n=33, p=0.021). The 1-year survival rate was significantly higher in the PDT-C group compared with the PDT-M group (88% vs 58%, p=0.001) with a significant reduction of mortality (hazard ratio, 0.20; 95% confidence interval, 0.07 to 0.58; p=0.003). Gemcitabine monotherapy resulted in a shorter survival time compared with the gemcitabine combination therapy (mean, 395 days vs 566 days; p=0.09). Cholangitic complications were observed at a similar frequency in the PDT-C and PDT-M groups. CONCLUSIONS: Combining repeated PDT with a gemcitabine-based combination therapy might offer a significant survival benefit in patients with hilar NCC.
Bismuth ; Cholangiocarcinoma* ; Drug Therapy* ; Humans ; Mortality ; Photochemotherapy* ; Retrospective Studies ; Survival Rate

Liver transplantation for treating cholangiocarcinoma (CCA) has potential benefits in terms of accomplishing radical resection even in patients with unresectable tumor and treating underlying disease such as primary sclerosing cholangitis (PSC). Despite this ideal rationale, the initial results of liver transplantation alone for CCA have been uniformly poor. Because of a high recurrence rate and a poor survival rate, CCA has been viewed as an absolute contraindication for transplantation. However, from the 1990s, liver transplantation following high dose neoadjuvant radiotherapy with chemotherapy has achieved excellent results, in a few medical centers, for selected patients with unresectable hilar CCA arising in the setting of PSC. Especially, the Mayo Clinic reported a 5-year survival rate of 73% for 120 patients in 2010 by performing neoadjuvant therapy and liver transplantation. We reviewed the results of liver transplantation in patients with hilar CCA and the protocol of the Mayo Clinic to consider the feasibility of liver transplantation for Korean patients with hilar CCA. Although liver transplantation with neoadjuvant therapy cannot be applied to most Korean patients due to the different disease entities and the good results with performing radical surgical resection, it can be considered as an alternative curative treatment option for selected patients with anatomically unresectable hilar CCA or CCA arising in the setting of PSC.
Cholangiocarcinoma ; Cholangitis, Sclerosing ; Humans ; Liver ; Liver Transplantation ; Neoadjuvant Therapy ; Recurrence ; Survival Rate ; Transplants

Alopecia due to metastatic skin cancer is a rare form of cutaneous metastasis and it should be considered as a diagnosis of localized alopecia. It also signifies the failure of treatment of the primary cancer. A 51-year-old Korean woman presented with multiple pea-sized smooth hairless nodules and an ulcerated nodule on the parietal scalp regions. About two and a half years ago, she underwent an operation and chemotherapy for cholangiocarcinoma. The tumor cells seen in the scalp nodule were the same as those seen in the primary tumor. To the best of our knowledge, this is the first reported case of secondary alopecia due to cutaneous metastasis of cholangiocarcinoma in Korean dermatological literature.
Alopecia* ; Cholangiocarcinoma* ; Diagnosis ; Drug Therapy ; Female ; Humans ; Middle Aged ; Neoplasm Metastasis ; Rabeprazole ; Scalp* ; Skin Neoplasms ; Ulcer

No abstract available
Bile Duct Neoplasms/*drug therapy/mortality ; *Bile Ducts, Intrahepatic ; Cholangiocarcinoma/*drug therapy/mortality ; Humans ; *Photochemotherapy ; Survival Rate

OBJECTIVETo explore the diagnosis and treatment of the cholangiocarcinoma.

METHODSForty one patients with cholangiocarcinoma who were enrolled in our hospital from January 1970 to January 2005 were retrospectively analyzed.

RESULTSAmong these 41 patients, the 1, 3, and 5-year survival rate was 82.3%, 45.8%, 45.8%, respectively, with radical operation, and was 11.0%, 0, 0 with non-radical operation (chi2 = 21.38, P < 0.01). The 1-year and 3-year survival rate was 11.0% and 0 in 9 patients treated with laparatomy, which was not significantly different from those treated with non-radical operation (chi2 = 0.02, P = 0.89). Four patients did not receive operation and all died within one year. Among 25 patients who did not experience lymph node metastasis, the 1, 3, and 5-year survival rate was 58.4%, 27.3%, and 27.3%. Among 16 patients who were found lymph node metastasis, the 1-year and 3-year survival rate was 61.8% and 0 (chi2 = 13.85, P < 0.01).

CONCLUSIONOperation is the most effective treatment for cholangiocarcinoma. Radical operation is the only curative treatment.

Bile Duct Neoplasms ; diagnosis ; pathology ; therapy ; Bile Ducts, Intrahepatic ; Cholangiocarcinoma ; diagnosis ; secondary ; therapy ; Female ; Humans ; Lymphatic Metastasis ; Male

Evidence suggests that combined gemcitabine-cisplatin chemotherapy extends survival in patients with advanced biliary tract cancer (BTC). We conducted a systematic review in order to collate this evidence and assess whether gemcitabine-cisplatin efficacy is influenced by primary tumor site, disease stage, or geographic region, and whether associated toxicities are related to regimen. MEDLINE (1946-search date), EMBASE (1966-search date), ClinicalTrials. gov (2008-search date), and abstracts from major oncology conferences (2009- search date) were searched (5 Dec 2013) using terms for BTC, gemcitabine, and cisplatin. All study types reporting efficacy (survival, response rates) or safety (toxicities) outcomes of gemcitabine-cisplatin in BTC were eligible for inclusion; efficacy data were extracted from prospective studies only. Evidence retrieved from one meta-analysis (abstract), four randomized controlled trials, 12 nonrandomized prospective studies, and three retrospective studies supported the efficacy and safety of gemcitabine-cisplatin for BTC. Median overall survival ranged from 4.6 to 11.7 months, and response rate ranged from 17.1% to 36.6%. Toxicities were generally acceptable and manageable. Heterogeneity in study designs and data collected prevented formal meta-analysis, however exploratory assessments suggested that efficacy did not vary with primary tumor site (gallbladder vs. others), disease stage (metastatic vs. locally advanced), or geographic origin (Asia vs. other). Incidence of grade 3/4 toxicities was not related to gemcitabine dose or cisplatin frequency. Despite individual variation in study designs, the evidence presented suggests that gemcitabine-cisplatin is effective in patients from a diverse range of countries and with heterogeneous disease characteristics. No substantial differences in toxicity were observed among the different dosing schedules of gemcitabine and cisplatin.
Appointments and Schedules ; Biliary Tract Neoplasms* ; Cholangiocarcinoma ; Cisplatin* ; Congresses as Topic ; Drug Therapy ; Gallbladder Neoplasms ; Humans ; Incidence ; Population Characteristics

BACKGROUNDS/AIMS: Little is known about clinical features and survival outcome in locally advanced unresectable extrahepatic cholangiocarcinoma (EHC). The aim was to investigate the clinical features and the survival outcome in these patients, and to evaluate the role of palliative resections in locally advanced unresectable EHC. METHODS: Between 1995 and 2007, 280 patients with locally advanced unresectable EHC were identified. Clinical, pathologic, and survival data were investigated. A comparative analysis was done between those who received palliative resection (PR) and those who were not operated on (NR). RESULTS: The overall median survival of the study population was 10+/-1 months, and the 3- and 5-year survival rates (YSR) were 8.5% and 2.5%, respectively. The median survival, 3- and 5-YSR of PR were 23 months, 32.1% and 13.1%, respectively. For NR, they were 9 months, 3.9% and 0%, which were significantly worse than PR (p<0.001). In univariate analysis, T classification, N classification, tumor location, palliative resection, adjuvant treatment, chemotherapy, and radiation therapy were factors that showed survival difference between PR and NR. Regional lymph node metastasis (RR, 2.084; 95% CI, 1.491-2.914; p<0.001), non-resections (RR, 2.270; 95% CI, 1.497-3.443; p<0.001), and no chemotherapy (RR, 1.604; 95% CI, 1.095-2.349; p=0.015) were identified as risk factors for poor outcome on multivariate analysis. CONCLUSIONS: Without evidence of systemic disease, palliative resection may provide some survival benefit in selected locally advanced unresectable EHCs and adjuvant treatment may further improve survival outcome.
Cholangiocarcinoma* ; Classification ; Drug Therapy ; Humans ; Lymph Nodes ; Multivariate Analysis ; Neoplasm Metastasis ; Palliative Care ; Risk Factors ; Survival Rate