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http://hdl.handle.net/1843/75951| Type: | Artigo de Periódico |
| Title: | Epithelioid rhabdomyosarcoma: report of the first case in the jaw |
| Authors: | Maria Cássia Ferreira de Aguiar Mariana Saturnino de Noronha Roger Lanes Silveira José Augusto Dias Araújo Flavio Sirihal Werkema Diana Bell Patrícia Carlos Caldeira |
| Abstract: | Objectives: 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. |
| Subject: | Review Mandible Maxilla Epithelium Rhabdomyosarcoma Immunohistochemistry Excision repair Drug therapy Diagnosis |
| language: | eng |
| metadata.dc.publisher.country: | Brasil |
| Publisher: | Universidade Federal de Minas Gerais |
| Publisher Initials: | UFMG |
| metadata.dc.publisher.department: | FAO - DEPARTAMENTO DE CLÍNICA |
| Rights: | Acesso Aberto |
| metadata.dc.identifier.doi: | https://doi.org/10.1016/j.oooo.2020.01.004 |
| URI: | http://hdl.handle.net/1843/75951 |
| Issue Date: | 2020 |
| metadata.dc.url.externa: | https://www.sciencedirect.com/science/article/pii/S2212440320300183?via%3Dihub |
| metadata.dc.relation.ispartof: | Oral Surgery Oral Medicine Oral Pathology Oral Radiology |
| Appears in Collections: | Artigo de Periódico |
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