High-grade myofibroblastic sarcoma of the mandible: Case report in a child and literature review

R. L.C. de Albuquerque, S. L.S. Melo, T. S. Bastos, R. S. Rocha, M. R. Piva, M. F.B. de Melo

Research output: Contribution to journalArticlepeer-review

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 languageEnglish (US)
Pages (from-to)91-95
Number of pages5
JournalOral Surgery
Volume8
Issue number2
DOIs
StatePublished - May 1 2015
Externally publishedYes

Keywords

  • Differential diagnosis
  • Jaw bones
  • Myofibroblastic sarcoma

ASJC Scopus subject areas

  • Surgery
  • Oral Surgery

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