NUMERO: #20231212

Título: ODONTOGENIC KERATOCYSTS ASSOCIATED WITH GORLIN GOLTZ-SYNDROME: A CASE REPORT

Nome do Apresentador: Ingrid Iara DAMAS

Categoria do Trabalho: Painel de caso clínico (PCC)

Área Temática: Patologia Oral

Resumo: A 19-year-old female patient presented at our clinic service with ocular proptosis, hypertelorism, palmoplantar pitting, melanocytic nevi on her face, a tongue with dark spots, and multiple skin lesions consistent with basal cell carcinoma. The patient reported social difficulties, intellectual deficit, and a history of numerous odontogenic cysts and ovarian fibromas/cysts in the past six years. A panoramic radiograph revealed multiple well-defined, unilocular radiolucent images in both the maxilla and mandible. The patient underwent several biopsies, which revealed fragments of a cystic capsule with a corrugated epithelium, presenting a uniform thickness and showing proliferation in some areas, consistent with odontogenic keratocyst. Based on the clinical, radiological, and histopathological findings, the diagnosis of Gorlin Goltz-syndrome was established, and the patient was referred to clinical genetics, where a PTCH1 gene mutation was confirmed. Currently, the patient is undergoing genetic counseling and follow-up.

Autor 1:  Ingrid Iara DAMAS

E-mail 1:  [email protected]

Autor 2:   João Figueira SCARINI

E-mail 2:  [email protected]

Autor 3 :  Reydson Alcides DE LIMA SOUZA

E-mail 3:  [email protected]

Autor 4:  Luccas LAVAREZE

E-mail 4:  [email protected]

Autor 5:  Érika Said Abu EGAL

E-mail 5:  [email protected]

Autor 6:  Albina ALTEMANI

E-mail 6:  [email protected]

Autor 7:  Fernanda Viviane MARIANO

E-mail 7:  [email protected]