Serifis Nikolaos, Tsilimigras Diamantis I, Cloonan Daniel J, Pawlik Timothy M
Center for Transplantation Sciences, Massachusetts General Hospital, Boston, MA, USA.
Department of Surgery, The Ohio State University Wexner Medical Center, Columbus, OH, USA.
Hepat Med. 2021 Nov 2;13:93-104. doi: 10.2147/HMER.S278136. eCollection 2021.
Intrahepatic cholangiocarcinoma (ICC) is one of the rarest and most aggressive types of cancer. The symptoms of ICC patients can be vague, leading to late diagnosis and dismal prognosis. In this review, we investigated the treatment options for ICC, as well as ways to overcome challenges in identifying and treating this disease. Imaging remains the gold standard to diagnose ICC. Patients are staged based on the tumor, nodes and metastases (TNM) staging system. Patients eligible for surgical resection should undergo surgery with curative intent with the goal of microscopically disease-free margins (R0 resection) along with lymphadenectomy. Minimal invasive surgery (MIS) and liver transplantation have recently been offered as possible ways to improve disease outcomes. ICC recurrence is relatively common and, thus, most patients will need to be treated with systemic therapy. Several clinical trials have recently investigated the use of neoadjuvant (NT) and adjuvant therapies for ICC. NT may offer an opportunity to downsize larger tumors and provide patients, initially ineligible for surgery, with an opportunity for resection. NT may also treat occult micro-metastatic disease, as well as define tumor biology prior to surgical resection, thereby decreasing the risk for early postoperative recurrence. Adjuvant systemic therapy may improve outcomes of patients with ICC following surgery. Ongoing clinical trials are investigating new targeted therapies that hold the hope of improving long-term outcomes of patients with ICC.
肝内胆管癌（ICC）是最罕见且侵袭性最强的癌症类型之一。ICC患者的症状可能不明确，导致诊断延迟和预后不佳。在本综述中，我们研究了ICC的治疗选择，以及克服在识别和治疗该疾病过程中所面临挑战的方法。影像学检查仍然是诊断ICC的金标准。患者根据肿瘤、淋巴结和转移（TNM）分期系统进行分期。符合手术切除条件的患者应接受具有治愈意图的手术，目标是实现显微镜下切缘无癌（R0切除）并进行淋巴结清扫。近年来，微创外科手术（MIS）和肝移植已成为改善疾病治疗效果的可能方法。ICC复发相对常见，因此，大多数患者需要接受全身治疗。最近有几项临床试验研究了ICC新辅助（NT）和辅助治疗的应用。新辅助治疗可能为缩小较大肿瘤提供机会，并为最初不符合手术条件的患者提供切除机会。新辅助治疗还可能治疗隐匿性微转移疾病，并在手术切除前明确肿瘤生物学特性，从而降低术后早期复发风险。辅助全身治疗可能改善ICC患者术后的治疗效果。正在进行的临床试验正在研究有望改善ICC患者长期治疗效果的新靶向治疗方法。
