Gong Xue, Li Yanan, Yang Kaiying, Chen Siyuan, Ji Yi
Division of Oncology, Department of Pediatric Surgery, West China Hospital of Sichuan University, Chengdu 610041, China.
Pediatric Intensive Care Unit, Department of Critical Care Medicine, West China Hospital of Sichuan University, Chengdu 610041, China.
Precis Clin Med. 2022 Feb 11;5(1):pbac006. doi: 10.1093/pcmedi/pbac006. eCollection 2022 Mar.
Infantile hepatic hemangiomas (IHHs) are common benign tumors seen in the liver of infants. IHHs are true infantile hemangiomas (IHs) and have phases of proliferation and involution parallel to those of cutaneous IHs. The definition and classification of IHH are still confusing in the literature. The mechanisms during the pathogenesis of IHH have yet to be discovered. The clinical manifestations of IHH are heterogeneous. Although most IHH lesions are asymptomatic, some lesions can lead to severe complications, such as hypothyroidism, consumptive coagulopathy, and high-output congestive cardiac failure. Consequently, some patients can possibly encounter a fatal clinical condition. The heterogeneity of the lesions and the occurrence of disease-related comorbidities can make the treatment of IHH challenging. Oral propranolol is emerging as an effective systemic approach to IHH with obvious responses in tumor remission and symptom regression. However, the precise clinical characteristics and treatment strategies for patients with severe IHH have not yet been well established. Here, we summarize the epidemiology, pathogenic mechanism, clinical manifestations, diagnosis, and treatment of IHH. Recent updates and future perspectives for IHH will also be elaborated.
婴儿肝血管瘤(IHHs)是婴儿肝脏中常见的良性肿瘤。IHHs是真正的婴儿血管瘤(IHs),具有与皮肤IHs相似的增殖期和消退期。IHH的定义和分类在文献中仍存在混淆。IHH发病机制中的相关机制尚未被发现。IHH的临床表现具有异质性。虽然大多数IHH病变无症状,但一些病变可导致严重并发症,如甲状腺功能减退、消耗性凝血病和高输出量充血性心力衰竭。因此,一些患者可能会面临致命的临床状况。病变的异质性和疾病相关合并症的发生会使IHH的治疗具有挑战性。口服普萘洛尔正在成为一种有效的全身性治疗IHH的方法,在肿瘤消退和症状缓解方面有明显效果。然而,重度IHH患者的确切临床特征和治疗策略尚未完全确立。在此,我们总结了IHH的流行病学、致病机制、临床表现、诊断和治疗。还将阐述IHH的最新进展和未来展望。