Department of Diagnostics and Public Health, Section of Pathology, University of Verona, University and Hospital Trust of Verona, Verona, Italy.
Division of General and Hepato-Biliary Surgery, Department of Surgery, Dentistry, Gynecology, and Pediatrics, University of Verona, University and Hospital Trust of Verona, Verona, Italy.
J Gastrointest Cancer. 2024 Dec;55(4):1634-1646. doi: 10.1007/s12029-024-01113-8. Epub 2024 Sep 16.
Intrahepatic cholangiocarcinoma is a biliary neoplasm usually showing a dismal prognosis. In early stages, surgical resection is the best treatment option, significantly increasing the overall survival. This approach is also recommended in the case of relapsing disease. In this study, we report the case of a patient affected by intrahepatic cholangiocarcinoma with multiple relapses and still alive for over 18 years. We also provide a systematic review regarding long-survivor (> 60 months) of intrahepatic cholangiocarcinoma.
A 41-year-old woman with no pathological history was diagnosed with localized intrahepatic cholangiocarcinoma and surgically treated with left hepatectomy. After the first intervention, the patients underwent three further surgical resections because of locoregional recurrences. Histologically, there were some significant similarities among all neoplasms, including the tubule-glandular architecture, but also morphological heterogeneity. The tumor immune microenvironment remained stable across the different lesions. The molecular analysis with next-generation sequencing demonstrated that all neoplasms shared the same genomic profile, including NBN and NOTCH3 mutations and chromosomes 1 and 3 alterations.
This case study highlights the essential role of a stringent follow-up after resection of intrahepatic cholangiocarcinoma for detecting early relapsing tumors. Moreover, it shows the importance of the molecular characterization of multiple tumors for understanding their real nature. The accurate study of long-surviving patients highlights the features that are critical for outcome improvement.
肝内胆管细胞癌是一种胆道肿瘤，通常预后较差。在早期阶段，手术切除是最佳治疗选择，可以显著提高总生存率。在疾病复发的情况下，也推荐这种方法。本研究报告了一例患有肝内胆管细胞癌且多次复发的患者，其存活时间超过 18 年。我们还对肝内胆管癌的长生存期（>60 个月）进行了系统回顾。
一名 41 岁女性，无病史，被诊断为局限性肝内胆管细胞癌，并接受左肝切除术治疗。第一次干预后，由于局部复发，患者又进行了三次手术切除。所有肿瘤在组织学上都有一些显著的相似之处，包括管状腺体结构，但也有形态异质性。肿瘤免疫微环境在不同病变中保持稳定。下一代测序的分子分析表明，所有肿瘤具有相同的基因组特征，包括 NBN 和 NOTCH3 突变以及染色体 1 和 3 的改变。
本病例研究强调了肝内胆管细胞癌切除后严格随访以检测早期复发肿瘤的重要性。此外，它还表明对多个肿瘤进行分子特征分析对于了解其真实性质的重要性。对长生存期患者的准确研究突出了改善预后的关键特征。
