| Literature DB >> 33089457 |
Bruno Augusto Linhares Almeida Mariz1, Elena María José Román Tager2, Carlos Cordón Fernandez3, Oslei Paes de Almeida2, Roman Carlos4.
Abstract
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.Entities:
Keywords: Carney complex; Mouth; Myxoma; Oral myxoma; Palatal myxoma; Palate
Mesh:
Year: 2020 PMID: 33089457 PMCID: PMC8385087 DOI: 10.1007/s12105-020-01241-0
Source DB: PubMed Journal: Head Neck Pathol ISSN: 1936-055X