PT - JOURNAL ARTICLE AU - W G McCluggage AU - M Y Walsh AU - C M Thornton AU - H R McClelland AU - S T Irwin TI - Massive abdominal and pelvic myxoma in Carney's syndrome AID - 10.1136/jcp.53.7.558 DP - 2000 Jul 01 TA - Journal of Clinical Pathology PG - 558--560 VI - 53 IP - 7 4099 - http://jcp.bmj.com/content/53/7/558.short 4100 - http://jcp.bmj.com/content/53/7/558.full SO - J Clin Pathol2000 Jul 01; 53 AB - This report describes a massive abdominal and pelvic myxoma in a patient with Carney's syndrome. A 38 year old woman presented with abdominal distension and a palpable mass, and at operation a large pelvic and abdominal tumour was identified and resected. The surgical specimen consisted of a lobulated mass, which on cut section had a uniform gelatinous consistency. The mass surrounded both ovaries, the appendix, and the upper part of the uterus, but macroscopically did not appear to involve these organs. Histological examination showed plump stellate and spindle shaped cells set in an abundant myxoid stroma, in keeping with a myxoma. Immunohistochemical staining revealed positivity of tumour cells for vimentin, but no reactivity to desmin, α-smooth muscle actin, S-100 protein, CD34, or AE1/AE3. This is the first documented case of massive adominal and pelvic myxoma in a patient with Carney'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.