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

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

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

Orbital lesions may be the presenting sign of a systemic disease, such as a metastatic cancer. A metastatic cancer of the orbit, which has been reported to account for 2~3% of all orbital tumor, is a malignant neoplasm that had spread by hematogenous routes to the orbit from a distant primary site. Although metastatic orbital tumors are mostly from breast, lung, prostate, rarely, they can be originated from gastrointestinal tract such as large intestine, ileum and pancreas. However, there has been no report of metastatic orbital tumor from biliary system, until now. Herein, we report a unique case of metastatic orbital tumor from the intrahepatic cholangiocarcinoma, which has been successfully controlled with systemic chemotherapy and radiotherapy.
Biliary Tract ; Breast ; Cholangiocarcinoma* ; Drug Therapy ; Gastrointestinal Tract ; Ileum ; Intestine, Large ; Lung ; Neoplasm Metastasis* ; Orbit* ; Pancreas ; Prostate ; Radiotherapy

Primary hepatic tuberculoma is a rare malady that is not accompanied by local symptoms, so the diagnosis can frequently be delayed or misconceived as other disease. We report here on an unusual case of primary hepatic tuberculoma that was misconceived as a cholangiocarcinoma on the imaging study. A 54-year-old man presented with dyspepsia and weight loss for 1 month. Abdominal computerized tomography demonstrated a solitary space-occupying lesion on the left lobe of the liver that suggested there was a cholangiocarcinoma accompanied with gastric outlet obstruction. The lesion was diagnosed by the ultrasonographic guided liver biopsy as a chronic granulomatous inflammation with necrosis, and the patient was treated via surgical resection with gastro-jejunostomy followed by antituberculosis chemotherapy. Twelve months later, no evidence of recurrence was noted when examining the patient's symptoms and the imaging studies.
Biopsy ; Cholangiocarcinoma* ; Diagnosis ; Drug Therapy ; Dyspepsia ; Gastric Outlet Obstruction ; Humans ; Inflammation ; Liver ; Middle Aged ; Necrosis ; Recurrence ; Tuberculoma* ; Weight Loss