High-grade myofibroblastic sarcoma of the mandible

Case report in a child and literature review

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

Research output: Contribution to journalArticle

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 - Jan 1 2015
Externally publishedYes

Fingerprint

Mandible
Sarcoma
Neoplasms
Mandibular Osteotomy
Mucin-1
Desmin
S100 Proteins
Vimentin
Keratins
Smooth Muscle
Actins
Tooth
Neck
Air
Head
Neoplasm Metastasis
Bone and Bones
Drug Therapy

Keywords

  • Differential diagnosis
  • Jaw bones
  • Myofibroblastic sarcoma

ASJC Scopus subject areas

  • Surgery
  • Oral Surgery

Cite this

de Albuquerque, R. L. C., Sousa Melo, S., Bastos, T. S., Rocha, R. S., Piva, M. R., & de Melo, M. F. B. (2015). High-grade myofibroblastic sarcoma of the mandible: Case report in a child and literature review. Oral Surgery, 8(2), 91-95. https://doi.org/10.1111/ors.12128

High-grade myofibroblastic sarcoma of the mandible : Case report in a child and literature review. / de Albuquerque, R. L.C.; Sousa Melo, Saulo; Bastos, T. S.; Rocha, R. S.; Piva, M. R.; de Melo, M. F.B.

In: Oral Surgery, Vol. 8, No. 2, 01.01.2015, p. 91-95.

Research output: Contribution to journalArticle

de Albuquerque, RLC, Sousa Melo, S, Bastos, TS, Rocha, RS, Piva, MR & de Melo, MFB 2015, 'High-grade myofibroblastic sarcoma of the mandible: Case report in a child and literature review', Oral Surgery, vol. 8, no. 2, pp. 91-95. https://doi.org/10.1111/ors.12128
de Albuquerque, R. L.C. ; Sousa Melo, Saulo ; Bastos, T. S. ; Rocha, R. S. ; Piva, M. R. ; de Melo, M. F.B. / High-grade myofibroblastic sarcoma of the mandible : Case report in a child and literature review. In: Oral Surgery. 2015 ; Vol. 8, No. 2. pp. 91-95.
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