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Das infantile Hämangioendotheliom der Leber - sonographische Diagnostik und Verlaufskontrolle

2001; Georg Thieme Verlag; Volume: 22; Issue: 06 Linguagem: Inglês

10.1055/s-2001-18922

1438-8782

R. Kardorff, Juri Fuchs, Matthias Peuster, Burkhard Rodeck,

Vascular Malformations and Hemangiomas

Hepatic haemangioendothelioma is the most frequently observed hepatic tumour of early infancy. Lesions may cause life-threatening disease due to av-shunt-related cardiac failure, Kasabach-Merritt syndrome or encroachment on surrounding tissue. In this paper, the value of ultrasonography at initial work-up as well as during follow-up under various management strategies is discussed.Retrospective analysis of sonographic and clinical data as well as outcome of 14 patients.The tumours may present initially with a typical sonographic pattern of a roundish solitary lesion consisting predominantly of massively perfused, tortuous cavities. In these cases, histological verification of the diagnosis is not mandatory, provided serological tumour markers are negative. Multifocal haemangioendotheliomata with a solid appearance, however, cannot be reliably distinguished from other entities sonographically. Tumour development - with or without therapy - can be followed up precisely using repeated ultrasound evaluations of tumour volume and sono-morphology as well as Doppler examination of tumour perfusion.Guidelines for the management of these patients are discussed, based on our experience and a review of the literature. Sonography proves to be of outstanding importance.