Abstract Benign fibrous histiocytoma (BFH) of bone is an exceptional entity, representing less than one per cent of benign osseous tumours. Its rarity and histological overlap with other fibro-histiocytic lesions often lead to diagnostic uncertainty. We report a rare case of BFH involving the sacrum in a paediatric patient, emphasising the diagnostic pitfalls and the importance of clinicoradiological correlation. Histopathological examination revealed a storiform spindle-cell proliferation with foamy histiocytes and multinucleated giant cells, consistent with BFH. This case underscores the need to recognize BFH as part of the morphologic continuum with non-ossifying fibroma and giant cell tumour, as defined by the 2020 WHO classification. Awareness of its distinctive features is crucial to prevent misdiagnosis and ensure appropriate management of this rare benign bone lesion.
Tazi et al. (Wed,) studied this question.