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Low-Grade Myofibroblastic Sarcoma of the Oral Cavity: A Report of Three Cases Illustrating an Emerging Disease in Children

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...

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Autores principales: Jayasooriya, Primali Rukmal, Athukorala, Chamara, Attygalla, Manjula, Mendis, Balapuwaduge Ranjit Rigobert Nihal, Lombardi, Tommaso
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7838777/
https://www.ncbi.nlm.nih.gov/pubmed/33401376
http://dx.doi.org/10.3390/dermatopathology8010001
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author Jayasooriya, Primali Rukmal
Athukorala, Chamara
Attygalla, Manjula
Mendis, Balapuwaduge Ranjit Rigobert Nihal
Lombardi, Tommaso
author_facet Jayasooriya, Primali Rukmal
Athukorala, Chamara
Attygalla, Manjula
Mendis, Balapuwaduge Ranjit Rigobert Nihal
Lombardi, Tommaso
author_sort Jayasooriya, Primali Rukmal
collection PubMed
description 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.
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spelling pubmed-78387772021-01-28 Low-Grade Myofibroblastic Sarcoma of the Oral Cavity: A Report of Three Cases Illustrating an Emerging Disease in Children Jayasooriya, Primali Rukmal Athukorala, Chamara Attygalla, Manjula Mendis, Balapuwaduge Ranjit Rigobert Nihal Lombardi, Tommaso Dermatopathology (Basel) Case Report 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. MDPI 2021-01-01 /pmc/articles/PMC7838777/ /pubmed/33401376 http://dx.doi.org/10.3390/dermatopathology8010001 Text en © 2021 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/).
spellingShingle Case Report
Jayasooriya, Primali Rukmal
Athukorala, Chamara
Attygalla, Manjula
Mendis, Balapuwaduge Ranjit Rigobert Nihal
Lombardi, Tommaso
Low-Grade Myofibroblastic Sarcoma of the Oral Cavity: A Report of Three Cases Illustrating an Emerging Disease in Children
title Low-Grade Myofibroblastic Sarcoma of the Oral Cavity: A Report of Three Cases Illustrating an Emerging Disease in Children
title_full Low-Grade Myofibroblastic Sarcoma of the Oral Cavity: A Report of Three Cases Illustrating an Emerging Disease in Children
title_fullStr Low-Grade Myofibroblastic Sarcoma of the Oral Cavity: A Report of Three Cases Illustrating an Emerging Disease in Children
title_full_unstemmed Low-Grade Myofibroblastic Sarcoma of the Oral Cavity: A Report of Three Cases Illustrating an Emerging Disease in Children
title_short Low-Grade Myofibroblastic Sarcoma of the Oral Cavity: A Report of Three Cases Illustrating an Emerging Disease in Children
title_sort low-grade myofibroblastic sarcoma of the oral cavity: a report of three cases illustrating an emerging disease in children
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7838777/
https://www.ncbi.nlm.nih.gov/pubmed/33401376
http://dx.doi.org/10.3390/dermatopathology8010001
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