NUMERO: #20250291

Título: AGGRESSIVE FIBROMATOSIS AFFECTING THE MANDIBLE IN A PEDIATRIC PATIENT: AN IMMUNOHISTOCHEMICAL STUDY

Nome do Apresentador: Gabriela Esperanza Maradiaga POSANTES

Categoria do Trabalho: Painel de caso clínico (PCC)

Área Temática: Patologia Oral

Resumo: Aggressive fibromatosis (AF), or desmoid tumor, is a rare, benign, yet locally invasive fibroblastic proliferation, with high recurrence rates. It originates from connective tissue, including fascial sheaths and musculoaponeurotic structures. AF has an estimated incidence of 5-6 cases per million individuals per year. Approximately 15% of AF cases affect the head and neck, notably jaws. The etiology of AF is multifactorial, including trauma, endocrine disorders, and chromosomal abnormalities. Some AF cases are linked to Gardner syndrome (GS). Recognizing AF as a GS manifestation is crucial for early diagnosis, genetic counseling, and multidisciplinary management. Here, we report a 2-year-old girl, without GS features, who presented AF affecting the left side of the mandible several months ago. Immunohistochemistry showed positivity for vimentin, α-SMA, calponin, CD10, and b-catenin. Ki-67 highlighted was low (<3%). Complete surgical removal of the tumor was performed. Currently, the patient is being monitored by a multidisciplinary team, without recurrences.

Autor 1:  Gabriela Esperanza Maradiaga POSANTES

E-mail 1:  [email protected]

Autor 2:   Karina Helen MARTINS

E-mail 2:  [email protected]

Autor 3 :  Luana Stefanie Silvino GONÇALVES

E-mail 3:  [email protected]

Autor 4:  Brenda Carolina Pattigno FORERO

E-mail 4:  [email protected]

Autor 5:  Ana Paula Teizen Peres

E-mail 5:  [email protected]

Autor 6:  Mariangela Ottoboni BRUNALDI

E-mail 6:  [email protected]

Autor 7:  Jorge Esquiche LEÓN

E-mail 7:  [email protected]