Total resection of an intracerebral hemangioendothelioma in an infant - Case report and review of the literature

Hemangioendotheliomas (HEs) are vasoformative tumors rarely seen in the CNS , Histopathological features determining aggressive phenotypes have not bee n well defined. Potential cytogenetic alterations in these tumors have not been previously reported. We present a 4-month-old male infant with a tempo ral robe HE. Fluorescence in situ hybridization and spectral karyotype anal ysis revealed translocation of chromosome 11q23. This case constitutes the first report of a gross total resection of an intracerebral HE in the pedia tric age group, and of potential cytogenetic alterations involved in its pa thogenesis. Based on our report and review of the literature, we note a dis crepancy between histopathological criteria of aggressive tumor biology and clinical behavior. Further study of chromosomal abnormalities may be helpf ul in defining factors associated with a higher risk of malignant behavior. We conclude that gross total resection is currently the best available tre atment option for these tumors, regardless of histology, Copyright (C) 2001 S. Karger AG. Basel.