Olnes Matthew J, Erlich Rodrigo
Department of Internal Medicine, Johns Hopkins Bayview Medical Center and Johns Hopkins School of Medicine, Baltimore, MD, USA.
Oncology. 2004;66(3):167-79. doi: 10.1159/000077991.
Cholangiocarcinoma is a malignant neoplasm arising from the biliary epithelium that was first described by Durand-Fardel in 1840. Today, it continues to defy diagnosis and treatment. It is difficult to diagnose in part because of its relative rarity, and because it is clinically silent until it becomes advanced disease with obstructive symptoms. The worldwide incidence of cholangiocarcinoma has risen over the past three decades. There is marked geographic variability in the prevalence of this disease, due in large part to regional environmental risk factors. Surgical resection remains the only curative treatment, and high priorities are improving diagnostic methods, and clinical staging for resection once the disease is suspected. A recent trend towards aggressive surgical management has improved outcomes. Chemotherapy, palliative stenting, and radiation are reserved for patients who are not resectable, those with recurrence after surgery, and those who decline surgical intervention. Recent trials using combination systemic chemotherapy and neoadjuvant chemoradiation are promising, but require further study. Over the past five years, several important studies have yielded new insights into the molecular mechanisms of cholangiocarcinoma tumorigenesis. In this review we discuss epidemiology, etiologic factors, molecular pathogenesis, diagnosis, staging, and treatment of cholangiocarcinoma. Particular focus is on recent studies into the cellular and molecular pathogenesis of the disease, recent chemotherapy trials, and newer methods of staging and screening for this devastating malignancy.
胆管癌是一种起源于胆管上皮的恶性肿瘤，1840年由杜兰德 - 法德尔首次描述。如今，它仍然难以诊断和治疗。部分原因在于其相对罕见，且在发展为具有梗阻症状的晚期疾病之前临床上并无明显症状，所以难以诊断。在过去三十年中，全球胆管癌的发病率有所上升。这种疾病的患病率存在显著的地域差异，这在很大程度上归因于区域环境风险因素。手术切除仍然是唯一的治愈性治疗方法，当怀疑患有该疾病时，提高诊断方法以及进行手术切除的临床分期是重中之重。最近积极的手术管理趋势改善了治疗效果。化疗、姑息性支架置入术和放疗适用于不可切除的患者、术后复发的患者以及拒绝手术干预的患者。近期使用联合全身化疗和新辅助放化疗的试验前景广阔，但仍需进一步研究。在过去五年中，几项重要研究对胆管癌肿瘤发生的分子机制有了新的见解。在本综述中，我们讨论胆管癌的流行病学、病因学因素、分子发病机制、诊断、分期和治疗。特别关注该疾病细胞和分子发病机制的最新研究、近期的化疗试验以及这种毁灭性恶性肿瘤分期和筛查的新方法。
