Abstract
Myofibroblastic 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.
Original language | English (US) |
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Pages (from-to) | 91-95 |
Number of pages | 5 |
Journal | Oral Surgery |
Volume | 8 |
Issue number | 2 |
DOIs | |
State | Published - May 1 2015 |
Externally published | Yes |
Keywords
- Differential diagnosis
- Jaw bones
- Myofibroblastic sarcoma
ASJC Scopus subject areas
- Surgery
- Oral Surgery