Type:
Educational Exhibit
Keywords:
Abdomen, Liver, Paediatric, CT, MR, Ultrasound, Education, Congenital, Haemangioma, Neoplasia
Authors:
C. M. Rutten, S. Franchi-Abella; 021/FR
DOI:
10.26044/ecr2021/C-10459
Background
Hemangiomas are the most common benign neonatal vascular tumor. They are caused by abnormal proliferation of vascular endothelial cells. They most commonly affect the skin, but can occur in nearly any organ.
Cutaneous hemangiomas have been categorized into two types based on their clinical and histological features: infantile and congenital. Infantile hemangiomas appear within the first few weeks of life, progressively enlarge over months and then involute. They stain positive for GLUT1. In contrast, congenital hemangiomas are fully grown at birth and do not usually exhibit postnatal growth. They stain negative for GLUT1. Three subtypes of congenital hemangioma have been described based on their evolution pattern: rapidly involuting (RICH), partially involuting (PICH), and non involuting (NICH) (Fig. 3,4) [1, 2].
The liver is the second most common site of hemangiomas after the skin, and hepatic hemangiomas (HHs) are the most common benign neonatal liver tumor. At pathologic analysis, HHs demonstrate multiple endothelium-lined vascular channels of varying size surrounded by fibroproliferate tissue (Fig. 5). Although histologically benign, they present with a spectrum of clinical manifestations, which can range from asymptomatic to life-threatening. Common presenting signs of HHs include: hepatomegaly, abdominal mass or distension, and congestive heart failure. Multiple cutaneous hemangiomas should also raise suspicion for HHs. Some HHs may be diagnosed prenatally (Fig. 6, 7). HHs are classified into three morphologic subtypes: focal, multifocal and diffuse (Fig. 8) [3]. Diagnostic imaging plays a critical role in identifying and assessing the progression of this disease. Ultrasound is usually sufficient to establish the diagnosis, but a multimodality imaging approach may be helpful. Imaging features are variable, especially on ultrasound. However, detailed descriptions of imaging findings are scarce in the literature.
We present a pictorial review of HHs illustrating their varying presentations and evolution patterns based on a retrospective review of 127 infants with HH over an 18-year period at a tertiary referral center for pediatric liver disease.