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Palatal Soft Tissue Myxoma in a Patient with Carney Complex

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Autor(es):
Mariz, Bruno Augusto Linhares Almeida ; Tager, Elena Maria Jose Roman ; Fernandez, Carlos Cordon ; de Almeida, Oslei Paes ; Carlos, Roman
Número total de Autores: 5
Tipo de documento: Artigo Científico
Fonte: Head and Neck Pathology; v. 15, n. 3, p. 8-pg., 2020-10-21.
Resumo

Carney complex (CNC) is a rare, autosomal dominant multiple neoplasia syndrome. Although cutaneous myxomas commonly occur in CNC patients, intraoral myxomas are extremely rare. We present a case of a palatal myxoma in a 21-year-old female patient with CNC, along with a review of the pertinent literature. She presented with a sessile nodule on the hard palate that microscopically showed a multilobulated and highly vascularized myxomatous tissue composed of loosely-arranged spindle, polygonal, and stellate cells, suggestive of myxoid neurofibroma. Six years after the oral lesion was removed, she presented with a growth hormone (GH)-producing pituitary adenoma, a cardiac myxoma, two cutaneous myxomas on the lower abdomen area, and one myxoma in the vaginal mucosa. Therefore, the final diagnosis of the palatal lesion was of a soft tissue myxoma related to CNC. The patient remains on close follow-up, with no recurrences of the palatal myxoma after 7 years. (AU)

Processo FAPESP: 18/24715-2 - Expressão de FGF-2, FGFR-1, PI3K, Akt e COX-2 em Leucoplasia Oral e Carcinoma Espinocelular Oral primário e metastático
Beneficiário:Bruno Augusto Linhares Almeida Mariz
Modalidade de apoio: Bolsas no Brasil - Doutorado