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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References
Wassef M, Blei F, Adams D et al (2015) Vascular anomalies classification: recommendations from the international society for the study of vascular anomalies. Pediatrics 136:e203–e214
Merrow AC, Gupta A, Patel MN, Adams DM (2016) 2014 revised classification of vascular lesions from the International Society for the Study of vascular anomalies: radiologic-pathologic update. Radiographics 36:1494–1516
Mulliken JB, Enjolras O (2004) Congenital hemangiomas and infantile hemangioma: missing links. J Am Acad Dermatol 50:875–882
Gorincour G, Kokta V, Rypens F et al (2005) Imaging characteristics of two subtypes of congenital hemangiomas: rapidly involuting congenital hemangiomas and non-involuting congenital hemangiomas. Pediatr Radiol 35:1178–1185
George A, Mani V, Noufal A (2014) Update on the classification of hemangioma. J Oral Maxillofac Pathol 18:S117–S120
Nasseri E, Piram M, McCuaig CC et al (2014) Partially involuting congenital hemangiomas: a report of 8 cases and review of the literature. J Am Acad Dermatol 70:75–79
El Zein S, Boccara O, Soupre V et al (2020) The histopathology of congenital haemangioma and its clinical correlations: a long-term follow-up study of 55 cases. Histopathology 77:275–283
Kulungowski AM, Alomari AI, Chawla A et al (2012) Lessons from a liver hemangioma registry: subtype classification. J Pediatr Surg 47:165–170
Christison-Lagay ER, Burrows PE, Alomari A et al (2007) Hepatic hemangiomas: subtype classification and development of a clinical practice algorithm and registry. J Pediatr Surg 42:62–68
Roebuck D, Sebire N, Lehmann E, Barnacle A (2012) Rapidly involuting congenital haemangioma (RICH) of the liver. Pediatr Radiol 42:308–314
Mo JQ, Dimashkieh HH, Bove KE (2004) GLUT1 endothelial reactivity distinguishes hepatic infantile hemangioma from congenital hepatic vascular malformation with associated capillary proliferation. Hum Pathol 35:200–209
Hernández F, Navarro M, Encinas JL et al (2005) The role of GLUT1 immunostaining in the diagnosis and classification of liver vascular tumors in children. J Pediatr Surg 40:801–804
Franchi-Abella S, Gorincour G, Avni F et al (2012) Hepatic haemangioma-prenatal imaging findings, complications and perinatal outcome in a case series. Pediatr Radiol 42:298–307
Venables WN, Ripley BD (2002) Modern applied statistics with S, 4th edn. Springer
Ritz C, Baty F, Streibig JC, Gerhard D (2016) Dose-response analysis using R. PLoS One 10:1–13
Enjolras O, Mulliken JB, Boon LM et al (2001) Noninvoluting congenital hemangioma: a rare cutaneous vascular anomaly. Plast Reconstr Surg 107:1647–1654
Cossio ML, Dubois J, McCuaig CC et al (2019) Non-involuting congenital hemangiomas (NICH) with postnatal atypical growth: a case series. Pediatr Dermatol 36:466–470
Triana P, Rodríguez-Laguna L, Giacaman A et al (2020) Congenital hepatic hemangiomas: clinical, histologic, and genetic correlation. J Pediatr Surg 55:2170–2176
Gembruch U, Baschat AA, Gloeckner-Hoffmann K et al (2002) Prenatal diagnosis and management of fetuses with liver hemangiomata. Ultrasound Obstet Gynecol 19:454–460
Li K, Wang Z, Liu Y et al (2016) Fine clinical differences between patients with multifocal and diffuse hepatic hemangiomas. J Pediatr Surg 51:2086–2090
Jiao-Ling L, Xiu-Ping G, Kun-Shan C et al (2018) Huge fetal hepatic hemangioma: prenatal diagnosis on ultrasound and prognosis. BMC Pregnancy Childbirth 18:2
Choi YJ, Baek JH, Hong MJ, Lee JH (2015) Inter-observer variation in ultrasound measurement of the volume and diameter of thyroid nodules. Korean J Radiol 16:560–565
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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