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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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dc.contributor.author Giraldo-Roldan, Daniela
dc.contributor.author Louredo, Brendo Vinicius Rodrigues
dc.contributor.author Penafort, Paulo Victor Mendes
dc.contributor.author Pontes, Hélder Antônio Rebelo
dc.contributor.author Alves, Aline Pinheiro
dc.contributor.author Lima, Fernando C. A.
dc.contributor.author Fonseca, Thamyres Campos
dc.contributor.author Abrahão, Aline Corrêa
dc.contributor.author Romañach, Mário José
dc.contributor.author Fonseca, Felipe Paiva
dc.contributor.author Delgado Azañero, Wilson Alejandro
dc.contributor.author Robinson, Liam
dc.contributor.author Van Heerden, Willie F. P.
dc.contributor.author de Almeida, Oslei Paes
dc.contributor.author Vargas, Pablo Agustin
dc.date.accessioned 2023-10-09T17:09:19Z
dc.date.available 2023-10-09T17:09:19Z
dc.date.issued 2023
dc.identifier.uri https://hdl.handle.net/20.500.12866/14232
dc.description.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. en_US
dc.language.iso eng
dc.publisher Springer
dc.relation.ispartofseries Head and Neck Pathology
dc.rights info:eu-repo/semantics/restrictedAccess
dc.rights.uri https://creativecommons.org/licenses/by-nc-nd/4.0/deed.es
dc.subject Low-grade myofibroblastic sarcoma en_US
dc.subject Oral pathology en_US
dc.subject Immunohistochemistry en_US
dc.subject Fusocellular neoplasia en_US
dc.subject Oral cavity en_US
dc.title Low-Grade Myofibroblastic Sarcoma of the Oral and Maxillofacial Region: An International Clinicopathologic Study of 13 Cases and Literature Review en_US
dc.type info:eu-repo/semantics/review
dc.identifier.doi https://doi.org/10.1007/s12105-023-01577-3
dc.relation.issn 1936-0568


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