13例肝先天性血管瘤的临床病理特征OA

Objective:Hepatic congenital hemangioma(HCH)is a rare vascular tumor of infancy with distinctive pathological features.It differs significantly from hepatic infantile hemangioma(HIH)in biological behavior and therapeutic strategies.This study aims to systematically summarize the clinical manifestations,imaging findings,pathological characteristics,and biological behavior of HCH in infants. Methods:A retrospective analysis was performed on the clinical,imaging,and histopathological data of 13 pediatric patients with pathologically confirmed HCH treated at Shanxi Children's Hospital between June 2019 and June 2025.Relevant literature was also reviewed. Results:Among the 13 patients,7 were male and 6 were female.The age at surgery ranged from 7 days to 20 months(median age,43 days).Prenatal ultrasonography detected hepatic space-occupying lesions in 10 patients.All patients presented with abdominal masses and hepatomegaly.All lesions were solitary,with 9 located in the right hepatic lobe and 4 in the left hepatic lobe.Contrast-enhanced CT/MRI demonstrated a typical centripetal enhancement pattern.Histologically,all 13 patients exhibited a characteristic zonal architecture consisting of 3 distinct regions from the center to the periphery:The central zone(map-like infarction accompanied by hemorrhage and calcification),the intermediate zone(fibrous tissue proliferation with slit-like small vessels),and the peripheral zone(a complex vascular network predominantly composed of capillary vessels).No cytological atypia was observed in vascular endothelial cells.Tumor tissues showed an infiltrative growth pattern intermixed with adjacent normal hepatic parenchyma without a clear boundary,and extramedullary hematopoiesis was identified in all cases.Tumor endothelial cells showed diffuse strong positivity for CD31,CD34,ETS-related gene(ERG),and Friend leukemia integration 1 transcription factor(Fli1).Glucose transporter-1(Glut-1)expression was negative in all cases.D2-40 staining was focally and weakly positive in a scattered pattern in 5 patients.The Ki-67 proliferation index ranged from 1%to 5%.During postoperative follow-up of 4-79 months,1 patient died 4 months after surgery due to pre-existing patent foramen ovale,neonatal respiratory failure,and hepatic failure.The remaining 12 patients recovered well,showed normal growth and development,and demonstrated no evidence of tumor recurrence on follow-up imaging. Conclusion:HCH predominantly occurs during infancy and commonly presents as an asymptomatic hepatic mass.The centripetal enhancement pattern on contrast-enhanced CT may provide diagnostic clues;however,definitive diagnosis still relies on histopathological examination.The key pathological features of HCH include characteristic zonal architecture and negative Glut-1 expression,with the latter serving as an important criterion for differentiation from HIH.HCH has a low risk of long-term recurrence and generally favorable prognosis after surgical resection.Nevertheless,giant tumors complicated by high-output heart failure may still carry a risk of mortality.