Hepatic Angiosarcoma HAS

HAS is an extremely rare neoplasm in pediatric subjects, with only a few dozen cases having been reported. HAS is frequently considered to be the malignant form of iHe [1]. In this regard, exposure to comparatively high levels of arsenic, both during pregnancy and in the postnatal period, has been shown to contribute to the onset of HAS in the presence of IHE [26].

The mean age of onset of HAS in children is four years. Histologically, this neoplasm is composed of malignant endothelial cells lining vascular channels of various dimensions, which tend to form sinusoids. Macroscopically, two forms of HAS can develop: a multifocal or multinodular form (Fig. 19,20), and a large, solitary mass. Both forms are associated with areas of necrosis and hemorrhage [10].

The clinical signs are non-specific, with progressive abdominal distension and a palpable mass in the right hypochondrium, often associated with abdominal pain, asthenia and weight loss. Hemor-rhagic ascites is a common sign and hemoperi-toneum is sometimes observed. This is a complication related to the vascular nature of the neoplasm on the one hand, and to the resulting thrombocy-topenia on the other. The neoplasm usually presents with normal serum AFP values [10,83].

Imaging findings for HAS in pediatric subjects on US, CT and MRI can be considered similar to those for the adult form of the lesion (see Chapt. 6, "Imaging of Malignant Focal Liver Lesions", section 6.1.4.1, "Angiosarcoma").

Focal Lesions The Spleen Mri

Fig. 20a-c. Angiosarcoma. The unenhanced CT scan (a) reveals enlargement of both the liver and the spleen with multiple hypodense nodules. Following contrast medium injection (b), the diffuse nodular tumors in the liver are much more evident and some hypervascularization (arrowheads) is apparent in the periphery. Multiple lesions can also be seen in the spleen. The coronal reconstruction (c) highlights the massive enlargement of the liver and spleen due to tumor infiltration

Fig. 20a-c. Angiosarcoma. The unenhanced CT scan (a) reveals enlargement of both the liver and the spleen with multiple hypodense nodules. Following contrast medium injection (b), the diffuse nodular tumors in the liver are much more evident and some hypervascularization (arrowheads) is apparent in the periphery. Multiple lesions can also be seen in the spleen. The coronal reconstruction (c) highlights the massive enlargement of the liver and spleen due to tumor infiltration

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