Epithelioid rhabdomyosarcoma: report of the first case in the jaw

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dc.creatorMaria Cássia Ferreira de Aguiar
dc.creatorMariana Saturnino de Noronha
dc.creatorRoger Lanes Silveira
dc.creatorJosé Augusto Dias Araújo
dc.creatorFlavio Sirihal Werkema
dc.creatorDiana Bell
dc.creatorPatrícia Carlos Caldeira
dc.date.accessioned2024-09-04T21:17:58Z
dc.date.accessioned2025-09-09T00:59:35Z
dc.date.available2024-09-04T21:17:58Z
dc.date.issued2020
dc.identifier.doihttps://doi.org/10.1016/j.oooo.2020.01.004
dc.identifier.issn2212-4411
dc.identifier.urihttps://hdl.handle.net/1843/75951
dc.languageeng
dc.publisherUniversidade Federal de Minas Gerais
dc.relation.ispartofOral Surgery Oral Medicine Oral Pathology Oral Radiology
dc.rightsAcesso Aberto
dc.subjectReview
dc.subjectMandible
dc.subjectMaxilla
dc.subjectEpithelium
dc.subjectRhabdomyosarcoma
dc.subjectImmunohistochemistry
dc.subjectExcision repair
dc.subjectDrug therapy
dc.subjectDiagnosis
dc.subject.otherd
dc.titleEpithelioid rhabdomyosarcoma: report of the first case in the jaw
dc.typeArtigo de periódico
local.citation.epagee315
local.citation.issue5
local.citation.spagee308
local.citation.volume130 Oral surgery oral medicine oral pathology oral radiology assunto Immunohistochemistry fao clínica
local.description.resumoObjectives: Epithelioid rhabdomyosarcoma (EpiRMS) is a novel morphologically distinct variant of rhabdomyosarcoma, with an unusually challenging microscopic diagnosis. The occurrence of rhabdomyosarcomas in the jaws is extremely rare. This study presents the first case of EpiRMS in the jaw (mandible) and a literature review of the previous 35 cases of EpiRMS. Study design: Here, we report a case of EpiRMS affecting an 18-year-old male patient. Clinical, imaging, microscopic, and immunohistochemical features are discussed and previously reported cases of EpiRMS are reviewed. Results: An 18-year-old male patient presented with an exophytic sessile growth on the buccal gingiva, and orthopantomography revealed irregular bone loss. Microscopic analysis showed a large number of cells with epithelioid appearance. Immunohistochemistry staining was positive for desmin, myogenin, MyoD1, smooth muscle actin, h-caldesmon, INI-1, and AE1-AE3. The patient's disease was staged as T4aN1M0 and was treated with surgical excision combined with chemotherapy. Conclusions: The occurrence of RMS in the mandible is rare, and this is the first case of EpiRMS in the jaw. EpiRMS is an unusual histologic subtype that mimics other sarcomas and epithelial malignancies, making diagnosis a challenge. A specific immunohistochemistry panel aids in the diagnosis. EpiRMS has an aggressive course and an unfavorable prognosis.
local.identifier.orcidhttps://orcid.org/0000-0001-9273-376X
local.publisher.countryBrasil
local.publisher.departmentFAO - DEPARTAMENTO DE CLÍNICA
local.publisher.initialsUFMG
local.url.externahttps://www.sciencedirect.com/science/article/pii/S2212440320300183?via%3Dihub

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