NUMERO: #20260359

Título: Head and Neck Low-Grade Fibromyxoid Sarcoma in adults - a systematic review

Nome do Apresentador: Letícia Maria Motta de ANDRADE

Categoria do Trabalho: Pôster - Investigação Original (IO)

Área Temática: Estomatologia

Resumo: Introduction
The aim of the present study was to systematically review the clinical features, anatomical distribution, recurrence patterns, surgical management, and genetic and immunohistochemical profiles of head and neck LGFMS (HNLGFMS).
Study Design
A systematic review was performed according to PRISMA guidelines and registered in PROSPERO (CRD42024579089). Case reports and series published in english were retrieved from multiple electronic databases. After eligibility screening, 30 studies were included, and demographic, clinicopathological, therapeutic, and molecular data were extracted and analyzed.
Results
HNLGFMS predominantly affected the oral and maxillofacial region (26.7%) and neck, followed by sinonasal and maxillary sites. Surgical excision or resection was the mainstay of definitive treatment, with biopsy representing the most common diagnostic procedure. Local recurrence was observed in 16.3% of cases, whereas 51.2% of patients remained recurrence-free during a mean follow-up of 42.5 months. Metastasis were uncommon. Immunohistochemical analysis demonstrated consistent vimentin expression and strong MUC4 positivity, while S100, CD34, cytokeratins, and desmin were generally negative. Molecular testing identified FUS and EWSR1 rearrangements in a subset of cases, including FUS-CREB3L2 and EWSR1-CREB3L1/2 fusions.
Conclusion
HNLGFMS presents distinct features, immunophenotypic and molecular characteristics and low recurrence, with surgery as the cornerstone of management.

Autor 1:  Letícia Maria Motta de ANDRADE

E-mail 1:  [email protected]

Autor 2:   Alexia Antunes DELUCA

E-mail 2:  [email protected]

Autor 3 :  Eduarda OLIVEIRA

E-mail 3:  [email protected]

Autor 4:  Gabriela Sauer LLANTADA

E-mail 4:  [email protected]

Autor 5:  Lauren Frenzel SCHUCH

E-mail 5:  [email protected]

Autor 6:  Manoela Domingues MARTINS

E-mail 6:  [email protected]

Autor 7:  Emily Ferreira Salles PILAR

E-mail 7:  [email protected]