Scientific article
Case report
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English

Low-grade myofibroblastic sarcoma of the oral cavity: a report of three cases illustrating an emerging disease in children

Published inDermatopathology, vol. 8, no. 1, p. 1-9
Publication date2021
Abstract

Low-grade myofibroblastic sarcoma (LGMS) is a mesenchymal tumor of myofibroblasts that occurs more frequently in adults. A series of three cases is presented to illustrate that LGMS may also occur within the oral cavity in children and adolescents. The first case (Case 1) occurred intra-osseously in the mandible, while the remaining two presented as gingival swellings and were purely restricted to soft tissue (cases 2 and 3). The intra-osseous lesion arose in a 7-year-old girl, whereas the gingival lesions were observed in a 12-year-old girl (Case 2) and a 13-year-old boy (Case 3). Histopathologically, all cases were composed of spindle shaped cells arranged into long fascicles showing mild to moderate degree of nuclear atypia. Ki-67 (MIB-1) proliferation activity was relatively low, amounting to 3-5% in all cases. Immunohistochemically, all cases showed smooth muscle actin (SMA) positivity in spindle cells, while desmin, beta catenin, cytokeratin, and CD34 were negative, resulting in a diagnosis of LGMS. In conclusion, current series of three cases of LGMSs that occurred in the oral cavity in a child and two adolescent patients is presented to highlight an emerging disease that requires additional data for further characterization.

Keywords
  • Low-grade myofibroblastic sarcoma
  • Oral cavity
  • Mandible
  • Gingiva
  • Malignancy
  • Immunohistochemistry
Citation (ISO format)
JAYASOORIYA, Primali Rukmal et al. Low-grade myofibroblastic sarcoma of the oral cavity: a report of three cases illustrating an emerging disease in children. In: Dermatopathology, 2021, vol. 8, n° 1, p. 1–9. doi: 10.3390/dermatopathology8010001
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Identifiers
Journal ISSN2296-3529
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