Palatal Soft Tissue Myxoma in a Patient with Carney Complex

Author(s)

Mariz, Bruno Augusto Linhares Almeida ; Tager, Elena Maria Jose Roman ; Fernandez, Carlos Cordon ; de Almeida, Oslei Paes ; Carlos, Roman

• Document type: Journal article
• Source: Head and Neck Pathology; v. 15, n. 3, p. 8-pg., 2020-10-21.

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. (AU)

• FAPESP's process: 18/24715-2 - FGF-2, FGFR-1, PI3K, Akt and COX-2 expression in Oral Leucoplakia, primary and metastatic Oral Squamous Cell Carcinoma
• Grantee: Bruno Augusto Linhares Almeida Mariz
• Support Opportunities: Scholarships in Brazil - Doctorate