Diffuse Infantile Hepatic Hemangioendothelioma With Early Central Enhancement in an Adult: A Case Report of CT and MRI Findings

Abstract

Infantile hepatic hemangioendothelioma (IHH) is the most common vascular tumor of the liver in infancy. Adult with IHH is extremely rare. We presented a diffuse IHH in an adult patient with computed tomography (CT) and magnetic resonance image (MRI) findings.A 39-year-old man was admitted to our hospital because of a 2-year history of abnormal liver function tests and a 7-day history of jaundice. Physical examination revealed enlarged liver. Unenhanced abdominal CT showed enlargement of the liver with diffuse hypodensity. Enhanced CT on the arterial phase revealed multiple centrally enhanced lesions diffusely involved the enlarged liver. The enhanced areas of the lesions became larger on the portal phase and all the lesions became homogeneous enhanced on the delayed phase. These lesions showed heterogeneously hyperintense on T2-weighted image, hypointense on T1-weighted image, and early centrally enhanced on dynamic gadolinium-enhanced MRI, with complete tumor enhancement after 180 s. The patient underwent orthotopic liver transplantation. IHH type 2 was confirmed by pathology. The patient died of tumor recurrence in the liver 4 months after transplantation.Unlike the previously described imaging appearances of IHH, this case showed diffuse nodules with early central enhancement on CT and MRI. Considering the importance of the ability to differentiate IHH from other hepatic tumors, radiologists should be aware of these imaging appearances to establish knowledge of the entire spectrum of IHH.

FIGURE 1

Axial unenhanced CT (A) showed the enlarged liver with homogeneous hypodensity and the lesions were indistinct. Axial enhanced CT (B) in the arterial phase showed numerous enhanced nodules throughout the liver (arrows). In the portal phase (C), the sizes of the enhanced nodules became larger with increased extent of enhancement (arrows). In the delayed phase (D), the nodules became uniform enhancement and nearly indistinct. CT = computed tomography.

FIGURE 2

Unenhanced coronary (A) and axial (B) T2-weighted MR images showed diffuse nodules (ranging from a few millimeters to 4.4 cm in diameter) with heterogeneously high signal intensities. The portal branches were compressed (arrow). These nodules were hypointense on the axial unenhanced T1-weighted image (C). The axial enhanced T1-weighted image in the arterial phase (D) showed central enhancement of the nodules (arrow). In the portal phase (E), the hypervascular regions of the nodules became larger (arrow). In the delayed phase (F), the nodules became uniform enhancement (arrow) with reticular regions of hypointensity between the nodules. MR = magnetic resonance.

FIGURE 3

Histopathology (A, hematoxylin and eosin stain, ×200) showed twisting vascular channels lined with proliferating pleomorphic endothelial cells. Histopathology at higher (B, hematoxylin and eosin stain, ×400) magnification showed large hyperchromatic and pleomorphic endothelial cells with abundant mitoses, nuclear atypia, and prominent nucleoli. Immunohistochemically, the endothelial cells showed a strong CD-34 reaction (C, original magnification, ×200; D, original magnification, ×400).