NUMERO: #20250193

Título: Aggressive mandibular rhabdomyosarcoma with TFCP2 rearrangement: A case report

Nome do Apresentador: Farah Essgui Orellana MARTINEZ

Categoria do Trabalho: Painel de caso clínico (PCC)

Área Temática: Estomatologia

Resumo: The WHO recently classified a new aggressive rhabdomyosarcoma (RMS) subtype characterized by TFCP2 rearrangement. A 38-year-old female presented with a 3-month left mandibular swelling, dental mobility, and persistent pain. Imaging showed an extensive osteolytic lesion involving teeth 34-36. Incisional biopsy immunoprofile indicated an intraosseous RMS. Consequently, a left segmental pelvimandibulectomy with facial reconstruction plate placement was performed. Eight months post-surgery, the patient developed painful swelling extending to the right mandible and enlarged submental lymph nodes, suggesting metastasis. A subsequent biopsy and molecular analysis confirmed an RMS with TFCP2 rearrangement. Due to renal dysfunction, chemotherapy was started at a reduced dose. The patient subsequently developed neutropenia, worsening pain, and mucositis. Due to adverse effects, chemotherapy was suspended. The patient declined a new chemotherapy cycle and passed away one month later. TFCP2-rearranged RMS are aggressive lesions with high metastatic potential and poor prognosis. Follow-up data for this subtype remains limited.

Autor 1:  Farah Essgui Orellana MARTINEZ

E-mail 1:  [email protected]

Autor 2:   Douglas Matheus Lima FARIAS

E-mail 2:  [email protected]

Autor 3 :  Liova Amaru CHABOT-DÍAZ

E-mail 3:  [email protected]

Autor 4:  Ana Paula Negreiros Nunes ALVES

E-mail 4:  [email protected]

Autor 5:  Ciro Dantas SOARES

E-mail 5:  [email protected]

Autor 6:  Roque Soares Martins NETO

E-mail 6:  [email protected]

Autor 7:  Mário Rogério Lima MOTA

E-mail 7:  [email protected]