NUMERO: #20260588

Título: NF1 CHILD WITH MANDIBULAR ODONTOGENIC FIBROMA AND PLEXIFORM NEUROFIBROMA RESECTED THREE YEARS APART

Nome do Apresentador: Ana Paula Abreu Vieira ALVES

Categoria do Trabalho: Pôster - Relato de Caso ou Série de Casos

Área Temática: Cirurgia e traumatologia bucomaxilofacial

Resumo: A three-year-and-nine-month-old child was brought to the outpatient clinic due to right mandibular swelling noticed by the parents. Clinical examination revealed underlying bony enlargement beneath a hyperpigmented macule, with additional smaller lesions distributed over the trunk and limbs. Computed tomography demonstrated an intraosseous lesion involving the mental region and mandibular body, characterized by cortical expansion, trabecular heterogeneity, irregular sclerosis, and well-defined lytic areas, measuring 40 mm. Surgical resection was performed, and histopathological analysis confirmed an odontogenic fibroma. The patient was referred for genetic evaluation, and a diagnosis of neurofibromatosis type 1 was established. During follow-up, a contiguous soft tissue lesion developed adjacent to the largest macule, extending into the ipsilateral submandibular space and measuring 64 mm. This lesion was surgically excised at seven years of age via an intraoral approach. The patient has currently received judicial authorization to initiate treatment with selumetinib, a MEK inhibitor.

Autor 1:  Ana Paula Abreu Vieira ALVES

E-mail 1:  [email protected]

Autor 2:   José Burgos PONCE

E-mail 2:  [email protected]

Autor 3 :  Daniel Henrique KOGA

E-mail 3:  [email protected]

Autor 4:  Marcos Curi MARTINS

E-mail 4:  [email protected]

Autor 5:  Camila Lopes CARDOSO

E-mail 5:  [email protected]

Autor 6:  Giuliano Saraceni Issa COSSOLIN

E-mail 6:  [email protected]