Massive abdominal and pelvic myxoma in Carney's syndrome

This report describes a massive abdominal and pelvic myxoma in a patient wi th Carney's syndrome. A 38 year old woman presented with abdominal distensi on and a palpable mass, and at operation a large pelvic and abdominal tumou r was identified and resected. The surgical specimen consisted of a lobulat ed mass, which on cut section had a uniform gelatinous consistency. The mas s surrounded both ovaries, the appendix, and the upper part of the uterus, but macroscopically did not appear to involve these organs. Histological ex amination showed plump stellate and spindle shaped cells set in an abundant myxoid stroma, in keeping with a myxoma. Immunohistochemical staining reve aled positivity of tumour cells for vimentin, but no reactivity to desmin, a-smooth muscle actin, S-100 protein, CD34, or AE1/AE3. This is the first d ocumented case of massive adominal and pelvic myxoma in a patient with Carn ey's syndrome. Clinicians and pathologists should be aware that myxomas in Carney's syndrome can rarely involve unusual sites other than the skin and heart.