LAUSR

BACKGROUND Intracranial angiomatoid fibrous histiocytomas (AFHs) are very rare tumors. Histologically, classical cases have been reported exclusively in adults with only myxoid variants identified in children. Here, we report the clinical presentation, treatment, biopsy and molecular test results for two children with classical intracranial AFH and combine this with a literature review of published intracranial AFH and AFH-like cases. CASE DESCRIPTION Two pediatric females, presenting with abnormal neurological signs, were diagnosed with intracranial AFHs from histopathological analysis. No myxoid features were identified. Fluorescence in situ hybridization (FISH) and Reverse Transcriptase Polymerase Chain Reaction (RT-PCR) testing demonstrated EWS1-ATF1 and EWS1-CREM gene fusions respectively, verified by Sanger sequencing. Both patients underwent surgery only. The first child developed local recurrence five years from initial surgery. Following a further complete resection, this patient has remained recurrence-free over a subsequent six year follow-up period. The second patient has recently developed local multi-nodular recurrence 28 months after initial surgery and is awaiting surgical re-excision. No additional chemotherapy/radiotherapy has been administered to either patient. CONCLUSIONS This paper describes the first two cases of non-myxoid intracranial AFH in children; confirmed by molecular analysis. Our results suggest that a tumor spectrum incorporating classical and myxoid intracranial AFHs can occur in children and that gross total resection represents the treatment strategy of choice at diagnosis or following recurrence.