NUMERO: #20260583

Título: INTRAOSSEOUS RHABDOMYOSARCOMA WITH TFCP2-REARRANGEMENT: A SERIES OF 4 CASES

Nome do Apresentador: Ana Luiza Oliveira Corrêa ROZA

Categoria do Trabalho: Apresentação Oral - Relato de Caso ou Série de Casos

Área Temática: Patologia Oral

Resumo: Rhabdomyosarcoma with TFCP2 rearrangement (RMS-TFCP2) is a high-grade malignancy characterized by fusion of TFCP2 with FUS or EWSR1 and a predilection for the gnathic bones. We report four additional cases of RMS-TFCP2 involving the mandible. The series included three males and one female, with a mean age of 37 years (range: 31-50 years), presenting with aggressive, ill-defined radiolucencies of the posterior mandible. Microscopically, all cases demonstrated infiltrative neoplastic cells with biphasic spindle and epithelioid morphology, variable cytologic atypia, and brisk mitotic activity. Notably, unusual features included osteoclast-type giant cells in one case and absence of desmin and MyoD1 expression in another. Nevertheless, FUS-TFCP2 gene fusion was confirmed by next-generation sequencing in all cases. Most patients underwent surgical resection; three developed local recurrence, and two died of disease. Although rare, RMS-TFCP2 is an aggressive neoplasm with poor prognosis and should be considered in the differential diagnosis of ill-defined radiolucent jaw lesions.

Autor 1:  Ana Luiza Oliveira Corrêa ROZA

E-mail 1:  [email protected]

Autor 2:   John FANTASIA

E-mail 2:  [email protected]

Autor 3 :  Daniel VINH

E-mail 3: 

Autor 4:  Jaden ERWIN

E-mail 4:  [email protected]

Autor 5:  Tori SEASOR

E-mail 5:  [email protected]

Autor 6:  Brian SHUMWAY

E-mail 6:  [email protected]

Autor 7:  Letícia Ferreira CABIDO

E-mail 7:  [email protected]