Estefanía-Fernández Karla, Triana Paloma, Ramírez-Amorós Carla, Gaspar-Pérez Mireia, Muñoz-Serrano Antonio Jesús, Velayos María, San Basilio María, Buitrago Nelson M, Parrón Manuel, Andrés Ane, Hernández-Oliveros Francisco, López Gutiérrez Juan Carlos
Department of Pediatric Surgery, La Paz Children's University Hospital, 28046 Madrid, Spain.
Department of Pediatric Surgery, University Hospital Parc Taulí, 08208 Sabadell, Spain.
Children (Basel). 2025 Jul 7;12(7):893. doi: 10.3390/children12070893.
: Congenital hepatic hemangiomas (CHHs) are typically considered rapidly involuting tumors, similar to their cutaneous counterparts (RICHs). However, non-involuting tumors remain poorly characterized. This study examines the evolutionary patterns and management strategies for non-involuting congenital hepatic hemangiomas (NICHHs). : We conducted a retrospective review of clinical, imaging, histological, and genetic data of children diagnosed with NICHH-defined as showing no signs of involution for at least 18 months-between 1991 and 2022. : Seven patients (five females, two males) were identified. The median age at diagnosis was 42 days (range: 0-1440). Five patients had asymptomatic lesions, predominantly located in the right hepatic lobe. Histologic confirmation was available in three cases, and a gene mutation was identified in one. The median follow-up period was 75 months (range: 35-191). Three patients with giant NICHH were treated with sirolimus, resulting in partial response in two cases and lesion stabilization in one. The four untreated patients showed diverse evolutionary patterns, including delayed involution and tardive growth. : NICHH lesions demonstrate distinct long-term evolution. Accurate diagnosis and regular monitoring are essential to avoid unnecessary interventions. Sirolimus may offer a promising non-surgical treatment for select patients, particularly those with giant lesions.
先天性肝血管瘤（CHHs）通常被认为是快速消退的肿瘤，与其皮肤对应物（快速消退型先天性血管瘤，RICHs）相似。然而，非消退型肿瘤的特征仍不清楚。本研究探讨非消退型先天性肝血管瘤（NICHHs）的演变模式和管理策略。
我们对1991年至2022年间被诊断为NICHH（定义为至少18个月无消退迹象）的儿童的临床、影像、组织学和基因数据进行了回顾性分析。
共纳入7例患者（5例女性，2例男性）。诊断时的中位年龄为42天（范围：0 - 1440天）。5例患者有无症状病变，主要位于右肝叶。3例有组织学确诊，1例发现基因突变。中位随访期为75个月（范围：35 - 191个月）。3例巨大NICHH患者接受了西罗莫司治疗，2例部分缓解，1例病变稳定。4例未治疗的患者表现出不同的演变模式，包括延迟消退和迟发性生长。
NICHH病变显示出独特的长期演变。准确诊断和定期监测对于避免不必要的干预至关重要。西罗莫司可能为特定患者，特别是那些有巨大病变的患者提供一种有前景的非手术治疗方法。
