Abstract
AbstractMyofibroblastic sarcoma (MS) is a rare neoplasm with a predilection for the head–neck region. Most MSs are low‐grade tumours, but a rarer, less differentiated, high‐grade variant has been described. To date, only six cases of MS have been reported in children, all low‐grade tumours. We report a case of aggressive MS, presented as an ulcerated mass in the anterior region of the mandible of an 11‐year‐old girl. Radiographs showed extensive bone destruction and ‘floating‐in‐air’ teeth displacement. Histopathological analysis revealed proliferation of pleomorphic spindle‐shaped and round cells arranged in a storiform and fascicular pattern. Immunohistochemically, tumour cells showed intense positivity for α‐smooth muscle actin and vimentin but focal for desmin, CD99 and HHF‐35. Laminin, S‐100 protein, CD68, pan‐cytokeratin and epithelial membrane antigen were negative. These findings were interpreted as high‐grade MS. Chemotherapy and segmental mandibulectomy were used to achieve local control, but the patient died 6 months later as a result of multiple metastases.
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