Background: Cholangiocarcinomas are relatively rare tumors most frequently arising from the epithelium of the hilar bile ducts. The diagnosis is often made in advanced (symptomatic) stages, which accounts for the infavorable prognosis with a 5-year survival of less than 10%. Identification of perihilar cholangiocarcinoma (pCCA) is often challenging because there is no single method offering sufficient diagnostic accuracy. Summary: Most tumors arise in patients without specific risk factors. Clinical symptoms of pCCA are nonspecific and reliable biomarkers are lacking, with carbohydrate antigen 19-9 being the most commonly used tumor marker (but with a low accuracy). Cross-sectional imaging (CT and MRI) is used to identify and map hilar strictures and determine resectability by showing vascular involvement. Endoscopic ultrasound offers additional information on locoregional tumor spread and lymph node involvement. Endoscopic retrograde cholangiography in combination with cholangioscopy gives direct access to and imaging of hilar strictures but it does not always distinguish between pCCA and benign hilar strictures. Tissue acquisition for histological diagnosis is challenging, with frequent sampling errors regardless of the method of biopsy procurement because of the cellular paucity of tumor tissue. Key Messages: In suspected perihilar malignancy, a mosaic of clinical data has to be taken into account. Histological evaluation of (endoscopically harvested) specimens is pivotal to differential diagnosis. Several new techniques to increase diagnostic accuracy are under investigation (biomarkers and genetic testing among others).
Visc Med

中文翻译：

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疑似肺门周围恶性肿瘤的诊断方法

背景：胆管癌是相对罕见的肿瘤，最常见于肝门胆管的上皮细胞。诊断通常是在晚期（有症状）阶段进行的，这是预后不良的原因，5 年生存率低于 10%。肺门周围胆管癌 (pCCA) 的鉴定通常具有挑战性，因为没有一种方法可以提供足够的诊断准确性。概括：大多数肿瘤发生在没有特定危险因素的患者身上。pCCA 的临床症状是非特异性的，缺乏可靠的生物标志物，碳水化合物抗原 19-9 是最常用的肿瘤标志物（但准确性低）。横截面成像（CT 和 MRI）用于识别和映射肺门狭窄，并通过显示血管受累来确定可切除性。内窥镜超声提供了有关局部区域肿瘤扩散和淋巴结受累的更多信息。内镜逆行胆管造影与胆管镜检查相结合，可以直接进入肺门狭窄并进行成像，但它并不总是能区分 pCCA 和良性肺门狭窄。用于组织学诊断的组织采集具有挑战性，关键信息：在疑似肺门周围恶性肿瘤中，必须考虑一系列临床数据。（内窥镜采集的）标本的组织学评估是鉴别诊断的关键。一些提高诊断准确性的新技术正在研究中（生物标志物和基因检测等）。
粘性医学