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Low-Grade Myofibroblastic Sarcoma of the Oral and Maxillofacial Region: An International Clinicopathologic Study of 13 Cases and Literature Review

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Abstract

Low-grade myofibroblastic sarcoma (LGMS) represents an atypical tumor composed of myofibroblasts with a variety of histological patterns and with a high tendency to local recurrence and a low probability of distant metastases. LGMS has predilection for the head and neck regions, especially the oral cavity. This study aimed to report 13 new cases of LGMS arising in the oral and maxillofacial region. This study included LGMS cases from five oral and maxillofacial pathology laboratories in four different countries (Brazil, Peru, Guatemala, and South Africa). Their clinical, radiographic, histopathological, and immunohistochemical findings were evaluated. In this current international case series, most patients were females with a mean age of 38.7 years, and commonly presenting a nodular lesion in maxilla. Microscopically, all cases showed a neoplasm formed by oval to spindle cells in a fibrous stroma with myxoid and dense areas, some atypical mitoses, and prominent nucleoli. The immunohistochemical panel showed positivity for smooth muscle actin (12 of 13 cases), HHF35 (2 of 4 cases), β-catenin (3 of 5 cases), desmin (3 of 11 cases), and Ki-67 (range from 5 to 50%). H-caldesmon was negative for all cases. The diagnosis of LGMS was confirmed in all cases. LGMS shows predominance in young adults, with a slight predilection for the female sex, and maxillary region. LGMS should be a differential diagnosis of myofibroblastic lesions that show a proliferation of spindle cells in a fibrous stroma with myxoid and dense areas and some atypical mitoses, supporting the diagnosis with a complementary immunohistochemical study. Complete surgical excision with clear margins is the treatment of choice. However, long-term follow-up information is required before definitive conclusions can be drawn regarding the incidence of recurrence and the possibility of metastasis.

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Authors and Affiliations

  1. Department of Oral Diagnosis, Oral Pathology Area, Piracicaba Dental School, State University of Campinas, Piracicaba, SP, Brazil

    Daniela Giraldo-Roldan, Brendo Vinicius Rodrigues Louredo, Paulo Victor Mendes Penafort, Oslei Paes de Almeida & Pablo Agustin Vargas

  2. Oral Pathology Service, João de Barros Barreto University Hospital, Federal University of Pará, Belém, PA, Brazil

    Hélder Antônio Rebelo Pontes

  3. Oral & Maxilofacial Surgery, Hospital Federal dos Servidores do Estado, Rio de Janeiro, RJ, Brazil

    Aline Pinheiro Alves & Fernando C. A. Lima

  4. Department of Oral Diagnosis and Pathology, School of Dentistry, Federal University of Rio de Janeiro, Rio de Janeiro, RJ, Brazil

    Thamyres Campos Fonseca, Aline Corrêa Abrahão & Mário José Romañach

  5. Department of Oral Surgery and Pathology, School of Dentistry, Federal University of Minas Gerais, Belo Horizonte, MG, Brazil

    Felipe Paiva Fonseca

  6. Department of Oral and Maxillofacial Pathology and Medicine, Universidad Peruana Cayetano Heredia, Lima, Peru

    Wilson A. Delgado

  7. Department of Oral and Maxillofacial Pathology, Faculty of Health Sciences, School of Dentistry, University of Pretoria, Pretoria, South Africa

    Felipe Paiva Fonseca, Liam Robinson, Willie F. P. Van Heerden & Pablo Agustin Vargas

  8. Department of Oral Diagnosis, Oral Pathology Area Piracicaba Dental School, State University of Campinas (UNICAMP), Av. Limeira, 901, Piracicaba, São Paulo, 13414-903, Brazil

    Pablo Agustin Vargas

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  1. Daniela Giraldo-Roldan
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Contributions

All authors contributed substantially to the conception, draft, and design of this work, as well as participation in the acquisition, analysis, and interpretation of data for the work. The final version of this work was approved for publication by all parties included. If there is a need, all authors agree to be accountable for any aspects of the work, and we ensure that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. The authors also state that the material is original and has not been published elsewhere.

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Correspondence to Pablo Agustin Vargas.

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This study is in accordance with the ethical standards of the Research Ethics Committee of the Piracicaba Dental School and accepted with the following number: 72775717.8.3001.5418 and with the original 1964 Helsinki Declaration and its later amendments or comparable ethical standards.

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Giraldo-Roldan, D., Louredo, B.V.R., Penafort, P.V.M. et al. Low-Grade Myofibroblastic Sarcoma of the Oral and Maxillofacial Region: An International Clinicopathologic Study of 13 Cases and Literature Review. Head and Neck Pathol 17, 832–850 (2023). https://doi.org/10.1007/s12105-023-01577-3

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