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Journal of Clinical Pathology 2000;53:558-560; doi:10.1136/jcp.53.7.558
Copyright © 2000 by the BMJ Publishing Group Ltd & Association of Clinical Pathologists.
J Clin Pathol 2000; 53:558-560
© 2000 Journal of Clinical Pathology

Short report

Massive abdominal and pelvic myxoma in Carney's syndrome

W G McCluggage1, M Y Walsh1, C M Thornton1, H R McClelland3, S T Irwin2

1 Department of Pathology, Royal Group of Hospitals Trust, Grosvenor Road, Belfast BT12 6BL, Northern Ireland
2 Department of Colorectal Surgery, Royal Group of Hospitals Trust, Belfast BT12 6BL, Northern Ireland
3 Department of Obstetrics and Gynaecology, Belfast City Hospital, Belfast BT9 7AB, Northern Ireland

Correspondence to:
Dr McCluggage

Note in proof: since the writing of this report, the patient has had a massive recurrence of abdominal myxoma (confirmed histologically).

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, {alpha}-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.

Key Words: abdomen • pelvis • myxoma • Carney's syndrome • immunohistochemistry


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