no abstract available.

Biliary tract cancer (BTC) is a rare malignancy with increasing incidence and mortality over the past two decades. Despite its unique anatomical and molecular features, BTC is typically diagnosed at advanced stages, thereby limiting treatment options and resulting in poor prognosis. Recent advances in molecular profiling and immunotherapy have revolutionized the BTC management, illustrating its molecular and immunologic underpinnings. Immune checkpoint inhibitors such as durvalumab and pembrolizumab, when combined with gemcitabine/ cisplatin, provide considerable survival benefits, with phase 3 trials such as TOPAZ-1 and KEYNOTE-966 demonstrating improved overall and progression-free survivals with immune checkpoint inhibitors compared to the conventional gemcitabine/cisplatin therapy. Therapies targeting FGFR2 fusions, IDH1 mutations, and HER2 overexpression have further expanded the therapeutic landscape, offering precision medicine opportunities for patients with actionable mutations. However, challenges persist owing to the rarity of BTC and inherent limitations of small cohort studies. Future research should focus on large, multicenter, prospective trials, establish new treatment paradigms, and expand therapeutic applications. In addition, integrating next-generation sequencing into routine practice will enhance molecular-based treatment approaches. This review explores the latest advancements in BTC immunotherapy and targeted therapies, emphasizing their clinical applicability in improving patient outcomes.

Biliary tract cancer (BTC) is a rare malignancy with increasing incidence and mortality over the past two decades. Despite its unique anatomical and molecular features, BTC is typically diagnosed at advanced stages, thereby limiting treatment options and resulting in poor prognosis. Recent advances in molecular profiling and immunotherapy have revolutionized the BTC management, illustrating its molecular and immunologic underpinnings. Immune checkpoint inhibitors such as durvalumab and pembrolizumab, when combined with gemcitabine/ cisplatin, provide considerable survival benefits, with phase 3 trials such as TOPAZ-1 and KEYNOTE-966 demonstrating improved overall and progression-free survivals with immune checkpoint inhibitors compared to the conventional gemcitabine/cisplatin therapy. Therapies targeting FGFR2 fusions, IDH1 mutations, and HER2 overexpression have further expanded the therapeutic landscape, offering precision medicine opportunities for patients with actionable mutations. However, challenges persist owing to the rarity of BTC and inherent limitations of small cohort studies. Future research should focus on large, multicenter, prospective trials, establish new treatment paradigms, and expand therapeutic applications. In addition, integrating next-generation sequencing into routine practice will enhance molecular-based treatment approaches. This review explores the latest advancements in BTC immunotherapy and targeted therapies, emphasizing their clinical applicability in improving patient outcomes.

Biliary tract cancer (BTC) is a rare malignancy with increasing incidence and mortality over the past two decades. Despite its unique anatomical and molecular features, BTC is typically diagnosed at advanced stages, thereby limiting treatment options and resulting in poor prognosis. Recent advances in molecular profiling and immunotherapy have revolutionized the BTC management, illustrating its molecular and immunologic underpinnings. Immune checkpoint inhibitors such as durvalumab and pembrolizumab, when combined with gemcitabine/ cisplatin, provide considerable survival benefits, with phase 3 trials such as TOPAZ-1 and KEYNOTE-966 demonstrating improved overall and progression-free survivals with immune checkpoint inhibitors compared to the conventional gemcitabine/cisplatin therapy. Therapies targeting FGFR2 fusions, IDH1 mutations, and HER2 overexpression have further expanded the therapeutic landscape, offering precision medicine opportunities for patients with actionable mutations. However, challenges persist owing to the rarity of BTC and inherent limitations of small cohort studies. Future research should focus on large, multicenter, prospective trials, establish new treatment paradigms, and expand therapeutic applications. In addition, integrating next-generation sequencing into routine practice will enhance molecular-based treatment approaches. This review explores the latest advancements in BTC immunotherapy and targeted therapies, emphasizing their clinical applicability in improving patient outcomes.

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Primary sclerosing cholangitis (PSC) is a slowly progressive cholestatic liver disease. In cases of PSC, liver transplantation is the only effective treatment that can delay the disease's natural course. We report a case of rapidly progressive PSC requiring liver transplantation. A 52-year-old woman visited our hospital with abdominal pain. There was no evidence of PSC, as there was no elevation in cholestatic liver enzymes at her first visit. Although her total bilirubin was in a normal range at the initial visit, liver dysfunction progressed rapidly. Despite endoscopic procedures and ursodeoxycholic acid intake, total bilirubin levels rose to 18.9 mg/dL, and liver transplantation was performed 17 months after her first visit. PSC was pathologically confirmed after liver transplantation.
Abdominal Pain ; Bilirubin ; Cholangiopancreatography, Endoscopic Retrograde ; Cholangiopancreatography, Magnetic Resonance ; Cholangitis, Sclerosing ; Female ; Humans ; Liver ; Liver Diseases ; Liver Transplantation ; Reference Values ; Ursodeoxycholic Acid