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Orofacial Manifestations Assisting the Diagnosis of Cowden Syndrome in a Middle-Aged Patient: Case Report and Literature Overview.
Sousa-Neto, Sebastião Silvério; de Arruda, José Alcides Almeida; Martins, Allisson Filipe Lopes; Abreu, Lucas Guimarães; Mesquita, Ricardo Alves; Mendonça, Elismauro Francisco.
Afiliação
  • Sousa-Neto SS; Department of Stomatology (Oral Pathology), School of Dentistry, Universidade Federal de Goiás, Goiânia, Goiás, Brazil.
  • de Arruda JAA; Departament of Oral Surgery, Pathology and Clinical Dentistry, School of Dentistry, Universidade Federal de Minas Gerais, Av. Pres. Antônio Carlos, 6627, room 3202 D. Pampulha, Belo Horizonte, Minas Gerais, CEP: 31270-901, Brazil. alcides_almeida@hotmail.com.
  • Martins AFL; School of Dentistry, Centro Universitário Goyazes, Trindade, Goiás, Brazil.
  • Abreu LG; Department of Child's and Adolescent's Oral Health, School of Dentistry, Universidade Federal de Minas Gerais, Belo Horizonte, Minas Gerais, Brazil.
  • Mesquita RA; Departament of Oral Surgery, Pathology and Clinical Dentistry, School of Dentistry, Universidade Federal de Minas Gerais, Av. Pres. Antônio Carlos, 6627, room 3202 D. Pampulha, Belo Horizonte, Minas Gerais, CEP: 31270-901, Brazil.
  • Mendonça EF; Department of Stomatology (Oral Pathology), School of Dentistry, Universidade Federal de Goiás, Goiânia, Goiás, Brazil.
Head Neck Pathol ; 16(1): 304-313, 2022 Mar.
Article em En | MEDLINE | ID: mdl-34106409
Middle-aged and old adults (≥ 50 years) diagnosed with Cowden syndrome (CS) with orofacial manifestations are uncommon. We describe a case of CS in a 53-year-old female showing "narrow bird-like" face, macrocephaly, acral keratoses, oral candidiasis, burning in the mouth, and multiple asymptomatic papillomatous lesions with a cobblestone pattern distributed on the alveolar ridge, tongue, buccal mucosa, and commissure. The histopathological features of lesions of the oral mucosa were those of papillary fibroepithelial hyperplasia. Immunohistochemistry revealed strong positivity for PTEN and p53 in most epithelial cells, while the expression of Bcl-2, S-100, and Ki-67 was weak/negative. According to a review conducted in PubMed, Web of Science, Embase, and Scopus for the analysis of reports of CS individuals ≥ 50 years with orofacial manifestations, 56 cases have been described in literature. Predilection for women was observed, with a female:male ratio of 2.3:1. Thirty-five (62.5%) individuals developed some malignant neoplasms. Oral health providers should be aware of the orofacial aspects of CS, including multiple papillomatosis, which can be an important criterion for diagnosis. Since malignancies may occur in older adults with CS, the need for strict surveillance is necessary. The present case has been under follow-up for 7 years without evidence of other manifestations.
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Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Papiloma / Síndrome do Hamartoma Múltiplo Tipo de estudo: Diagnostic_studies / Systematic_reviews Limite: Aged / Female / Humans / Male / Middle aged Idioma: En Revista: Head Neck Pathol Assunto da revista: NEOPLASIAS / PATOLOGIA Ano de publicação: 2022 Tipo de documento: Article País de afiliação: Brasil País de publicação: Estados Unidos
Texto completo
Adicionar na Minha BVS
Imprimir
XML
PubMed Links
Buscar no Google

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Papiloma / Síndrome do Hamartoma Múltiplo Tipo de estudo: Diagnostic_studies / Systematic_reviews Limite: Aged / Female / Humans / Male / Middle aged Idioma: En Revista: Head Neck Pathol Assunto da revista: NEOPLASIAS / PATOLOGIA Ano de publicação: 2022 Tipo de documento: Article País de afiliação: Brasil País de publicação: Estados Unidos