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Spontaneous evolution patterns of focal congenital hepatic hemangiomas: a case series of 25 patients

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Abstract

Background

Hepatic hemangiomas are the most common benign liver tumors of infancy. They are termed congenital if fully developed at birth or infantile if they appear in the first weeks of life. Previous studies suggested that most focal hepatic hemangiomas are congenital in nature, exhibit no postnatal growth and have an evolution that parallels their cutaneous counterparts. They are subdivided by pattern of involution, whether rapidly involuting (RICH), partially involuting (PICH) or non-involuting (NICH) congenital hemangiomas. In our experience, some focal hepatic hemangiomas show postnatal growth, behaving like infantile forms.

Objectives

To analyze the spontaneous evolution of focal congenital hepatic hemangiomas with quantification of tumor volume changes over time and to identify initial postnatal ultrasound (US) imaging biomarkers predictive of their evolution pattern.

Materials and methods

A retrospective review of clinical, imaging and pathology data of children diagnosed with focal congenital hepatic hemangioma (prenatal diagnosis or age at diagnosis <7 days and/or glucose transporter protein 1 [GLUT1]-negative tumor) diagnosed between 2000 and 2018 was performed with analysis of tumor volume changes over time. Exclusion criteria were treatment inducing a tumor volume change (hepatic artery embolization, propranolol, or corticosteroids), imaging follow-up less than 1 month or fewer than two US examinations. Volumetric analysis was based on US and cross-sectional imaging. Lesion volumes were estimated using the standard ellipsoid formula. A 35% margin of error was assumed for tumor volume variation to account for variability in measurements. Imaging studies, including US, computed tomography, and magnetic resonance imaging, were reviewed and initial postnatal US features were correlated with evolution pattern.

Results

Twenty-five patients with focal congenital hepatic hemangiomas were included. The median follow-up time was 46.5 months (range: 4–144 months). Eight (32%) lesions showed postnatal growth before involuting, without signs of intralesional hemorrhage, as do cutaneous infantile hemangiomas. The other 17 (68%) lesions exhibited a strict decrease in volume with age, of which 15 underwent complete involution (8 before age 18 months and 7 after age 18 months) and 2 underwent partial involution. The different evolution patterns of focal congenital hepatic hemangiomas showed overlapping imaging features and we found no initial US feature to be significantly associated with postnatal growth. However, large vascular spaces with marked vascularity at US were noted in three of the eight rapidly involuting lesions.

Conclusion

Focal congenital hepatic hemangiomas are not the equivalent of cutaneous RICH, as some may increase in size and tumor regression may be rapid or slow. The different evolution patterns of focal congenital hepatic hemangiomas show overlapping US features.

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Acknowledgements

The images shown in Fig. 2a–d, Fig. 3a–d, Fig. 4a, b and Fig. 5e, g were previously published in a poster presentation: Rutten C, Franchi-Abella S (2021) The many faces, facets and phases of pediatric hepatic hemangiomas [European Congress of Radiology 202 /C-10459]. 10.26044/ecr2021/C-10459

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Authors and Affiliations

  1. Department of Pediatric Radiology, Bicêtre Hospital, 78 Rue du Général Leclerc, 94270, Le Kremlin-Bicêtre, France

    Caroline Rutten & Stéphanie Franchi-Abella

  2. Department of Pediatric Hepatology and Liver Transplantation, Bicêtre Hospital, AP-HP, Le Kremlin-Bicêtre, France

    Delphine Ladarre, Oanez Ackermann & Emmanuel Gonzales

  3. Reference Centre for Vascular Diseases of the Liver, FSMR FILFOIE, ERN RARE LIVER, Bicêtre Hospital, AP-HP, FHU Hépatinov, Paris-Saclay University, Le Kremlin-Bicêtre, France

    Delphine Ladarre, Oanez Ackermann, Emmanuel Gonzales, Catherine Guettier & Stéphanie Franchi-Abella

  4. INSERM UMRS 1193, Paris-Saclay University, FHU Hépatinov, Orsay, France

    Emmanuel Gonzales & Catherine Guettier

  5. Department of Pathology, Bicêtre Hospital, AP-HP, Le Kremlin-Bicêtre, France

    Catherine Guettier

  6. DMU Smart Imaging, BIOMAPS UMR 9011 CNRS – INSERM – CEA, Le Kremlin-Bicêtre, France

    Stéphanie Franchi-Abella

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  1. Caroline Rutten
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  2. Delphine Ladarre
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  6. Stéphanie Franchi-Abella
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Correspondence to Stéphanie Franchi-Abella.

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Rutten, C., Ladarre, D., Ackermann, O. et al. Spontaneous evolution patterns of focal congenital hepatic hemangiomas: a case series of 25 patients. Pediatr Radiol 52, 1048–1060 (2022). https://doi.org/10.1007/s00247-021-05277-4

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  • DOI: https://doi.org/10.1007/s00247-021-05277-4

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