Akasbi Imad, Chaouche Ismail, Bouardi Nizar El, Akammar Amal, Chahdi Hajar O, Zerrari Ismail, Lahlali Maria, Abid Hakima, Alami Badr, Lahmidani Nada, Boubbou Meryem, Sidi Adil Ibrahimi, Maaroufi Mustapha, Lamrani My Youssef Alaoui
Adult Radiology Department, CHU Hassan II, Sidi Mohammed Ben Abdellah University, Fez, Morocco.
Radiology Department, Mother and Child Hospital, CHU Hassan II, Sidi Mohammed Ben Abdellah University, Fez, Morocco.
Radiol Case Rep. 2025 May 20;20(8):3879-3885. doi: 10.1016/j.radcr.2025.04.125. eCollection 2025 Aug.
Alagille syndrome is a rare genetic disorder causing multisystem complications, including biliary abnormalities, chronic liver disease, and rarely, hepatocellular carcinoma (HCC), which is difficult to manage due to liver dysfunction and vascular abnormalities. We report a 29-year-old female, diagnosed with Alagille syndrome in infancy, who developed infiltrative HCC at age 20 and underwent lipiodol-based transarterial chemoembolization (TACE) in 2015, with a second session in 2016 for recurrence. Remarkably, she has achieved an exceptional 9-year survival post-treatment with stable imaging and no recurrence, a highly uncommon outcome for HCC in Alagille syndrome, highlighting TACE's potential as an effective palliative treatment for HCC in Alagille syndrome, particularly for patient's ineligible for liver transplantation, and underscoring the need for further studies on locoregional therapies in this rare population.
阿拉吉尔综合征是一种罕见的遗传性疾病，可导致多系统并发症，包括胆道异常、慢性肝病，以及罕见的肝细胞癌（HCC），由于肝功能障碍和血管异常，这种疾病难以治疗。我们报告了一名29岁女性，婴儿期被诊断为阿拉吉尔综合征，20岁时发展为浸润性HCC，并于2015年接受了基于碘油的经动脉化疗栓塞术（TACE），2016年因复发进行了第二次治疗。值得注意的是，她在治疗后已存活9年，影像稳定且无复发，这在阿拉吉尔综合征相关的HCC中是非常罕见的结果，凸显了TACE作为阿拉吉尔综合征相关HCC有效姑息治疗方法的潜力，特别是对于不适合肝移植的患者，并强调了对这一罕见人群局部区域治疗进行进一步研究的必要性。
