Hepatic angiosarcoma in an adult who had Wilms tumor treated in childhood: A case report

Abstract

Hepatic angiosarcoma is a rare, highly aggressive mesenchymal liver malignancy with poor prognosis that stems from the endothelial cells that line the walls of blood or lymphatic vessels. It is the third most common primary liver malignancy and is most prevalent among older males. It is difficult to diagnose due to various clinical presentations from asymptomatic to abdominal pain, pleural effusion, and liver failure. The diagnosis of liver angiosarcoma is suspected on imaging features and confirmed by histopathological assessment. Primary management is determined based on the stage of tumor from surgery to palliative care such as chemotherapy or tumor transarterial embolization. We report a 51-year-old female who presented with stage 4 liver angiosarcoma and a history of childhood Wilms tumor. We focus on tumor management using radiological modalities and pathological analysis and discuss secondary liver tumors in survivors of childhood Wilms tumor.

Keywords: CT scan; Hepatic angiosarcoma; Liver failure; Malignancy; Wilms tumor.

Fig. 1

(a, b). Contrast-enhanced venous phase axial computed tomography images show a 6.3 cm heterogeneous ill-defined hypodense mass (black arrow) located in the posterior inferior right hepatic lobe with extension to the heterogeneous subcapsular fluid collection (black asterisk) and multiple scattered hypoattenuating lesions throughout the liver representing intrahepatic metastases. Some liver cysts are also present. Heterogeneous right pleural effusion (white asterisk) is partially seen in (a). In (b) (inferior to a), the right hepatic lobe is small relative to the left hepatic lobe. The right kidney is surgically absent. An incidental left renal cyst is partially seen. Note hypoplastic right hemiabdomen with thinner subcutaneous fat and abdominal wall musculature secondary to childhood radiation therapy to the right abdomen.

Fig. 2

(a, b). Contrast-enhanced arterial phase (a) coronal and (b) sagittal reformatted computed tomography images show a heterogeneous ill-defined mass (black arrow) in the posterior inferior right hepatic lobe extending to the heterogeneous subcapsular fluid collection (black asterisk) and multiple scattered hypoattenuating lesions throughout the liver. Right pleural metastases with multiloculated heterogeneous malignant right pleural effusion (white asterisk) are also seen. Right hemidiaphragm (white arrowheads) is compressed between the right pleural metastases with loculated pleural lesion and subcapsular hepatic lesion.

Fig. 3

(a, b). Contrast-enhanced venous phase 3-D CT images of liver angiosarcoma with cinematic rendering (CR) in axial (a) and coronal (b) planes show heterogeneous nature of the dominant mass (black arrow) in the posterior right lobe of the liver extending to the hepatic subcapsular heterogeneous collection (black asterisk). Right hemidiaphragm (white arrowheads) is compressed between the right pleural metastases with loculated pleural effusion (white asterisk) and hepatic subcapsular hepatic lesion (black asterisk).