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

doi:10.1111/ors.12128

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 journal › Article › peer-review

2 Scopus citations

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)
Pages (from-to)	91-95
Number of pages	5
Journal	Oral Surgery
Volume	8
Issue number	2
DOIs	https://doi.org/10.1111/ors.12128
State	Published - May 1 2015
Externally published	Yes

Keywords

Differential diagnosis
Jaw bones
Myofibroblastic sarcoma

ASJC Scopus subject areas

Surgery
Oral Surgery

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10.1111/ors.12128

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@article{c2aacb12d4954470960fbefe1305443f,

title = "High-grade myofibroblastic sarcoma of the mandible: Case report in a child and literature 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.",

keywords = "Differential diagnosis, Jaw bones, Myofibroblastic sarcoma",

author = "{de Albuquerque}, {R. L.C.} and Melo, {S. L.S.} and Bastos, {T. S.} and Rocha, {R. S.} and Piva, {M. R.} and {de Melo}, {M. F.B.}",

note = "Publisher Copyright: {\textcopyright} 2014 The British Association of Oral Surgeons and John Wiley & Sons Ltd.",

year = "2015",

month = may,

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doi = "10.1111/ors.12128",

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T2 - Case report in a child and literature review

AU - de Albuquerque, R. L.C.

AU - Melo, S. L.S.

AU - Bastos, T. S.

AU - Rocha, R. S.

AU - Piva, M. R.

AU - de Melo, M. F.B.

PY - 2015/5/1

Y1 - 2015/5/1

N2 - 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.

AB - 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.

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KW - Jaw bones

KW - Myofibroblastic sarcoma

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

Abstract

Keywords

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