Non-Involuting Congenital Hepatic Hemangioma: Natural History and Management

Background: Focal liver hemangiomas have traditionally been considered as rapidly involuting tumors, akin to their cutaneous counterparts (RICH). However, non-involuting tumors remain poorly investigated and inadequately understood. This study explores the evolution patterns and management strategies for non-involuting congenital hepatic hemangiomas (NICHH). Methods: We conducted a retrospective review of clinical, imaging, histological, and genetic data of children diagnosed with NICHH (defined as having no signs of involution for at least 18 months) from 1991 to 2022. Results: We included 7 patients (5 females and 2 males) diagnosed with NICHH. The median age at diagnosis was 42 days (range: 0-1440). Seventy-one percent of lesions were asymptomatic, with the right hepatic lobe being the most common location (71.4%). Histological analysis was available in 3 patients confirming the diagnosis of CHH. A genetic study conducted in one patient identified a mutation in GNAQ gene. The median follow-up duration was of 75 months (range: 35-191). During follow-up, 3 patients with giant NICHH received sirolimus treatment, resulting in a partial response in 2 patients, while the lesion in the other patient remained stable. The 4 untreated patients exhibited various evolutionary patterns, some of which had not been previously described. Conclusions: This is the first case series to examine the long -term evolution of NICHH. Accurate diagnosis and periodic follow-up of these lesions can prevent unnecessary aggressive treatments. Sirolimus may offer a promising option for reducing the size or accelerate the involution of NICHH.