Primary Intracranial Angiomatoid Fibrous Histiocytoma: Two Case Reports and Literature Review

Background: Angiomatoid fibrous histiocytoma (AFH) is a rare, fibrohistiocytic, soft-tissue neoplasm. Intracranial AFH is extremely rare. Here we present 2 pediatric cases of intracranial AFH and perform a literature review on this disease entity.

Case description: We present 2 cases. The first case is a 10-year-old boy who presented with seizures and hemiparesis. The second case is an 11-year-old girl who presented with 2-year history of seizures. Radiologic images demonstrated right frontal lesions in both patients. Complete surgical resection was achieved. Histopathological findings established the diagnosis of intracranial AFH confirmed with fluorescence in situ hybridization and reverse transcriptase polymerase chain reaction testing that demonstrated EWSR1 gene rearrangement in both cases.

Conclusions: Twenty-two cases of intracranial AFH have been previously documented, with the majority of lesions located in the frontal lobe. Most cases occurred in adolescents and young adults, with a slight female predilection. Headaches and seizures constituted the most common clinical presentation. Complete surgical resection remains the standard of care in the management of this pathology.