Abstract / Summary
Angiomatoid Fibrous Histiocytoma (AFH) represents a rare and clinically deceptive mesenchymal neoplasm that primarily affects children and young adults. Currently, classified by the World Health Organisation (WHO) as a tumour of intermediate malignant potential, AFH is frequently characterised by an indolent clinical course; however, it presents a formidable diagnostic challenge due to its profound clinical and radiological heterogeneity. The Magnetic Resonance Imaging (MRI) features described are multiple internal blood-fluid levels, a T2-hypointense fibrous pseudocapsule, focal nodularity along the cyst wall, and the double rim sign. Definitive diagnosis rests upon histopathological confirmation and molecular testing for the pathognomonic EWSR1-CREB1 fusion. The primary management strategy is centred on wide local surgical excision. In this series, authors describe a heterogeneous group of clinical and radiological presentations of this entity within bone and muscle, ranging from superficial, well-circumscribed soft-tissue masses to aggressive, bone-eroding lesions. These varied cases highlight the absolute necessity of maintaining a high-index of clinical suspicion for AFH when evaluating any recurrent or atypical cystic musculoskeletal lesion.