'Proximal-type' epithelioid sarcoma: is Agent Orange still at large?
OBJECTIVE
Proximal-type epithelioid sarcomas of the perineum are extremely rare. The authors provide an overview of this condition in relation to the history of exposure to Agent Orange.


METHODS
A 54-year-old man presented with a rapidly growing perineal subcutaneous mass that was shown to be a proximal-type epithelioid sarcoma. The case is discussed. An Internet Medline search was performed and the current literature reviewed.


RESULTS
Only 4 primary perineal sarcomas have been described in the literature. Epithelioid sarcomas are uncommon, aggressive tumours with a propensity for locoregional recurrence. They are recognised by the US Veterans Affairs department as linked to exposure to Agent Orange, an organochlorine defoliant containing the contaminant 2,3,7,8-tetrachlorodibenzo-p-dioxin (TCDD). However, the role of Agent Orange in sarcomagenesis is still controversial.


CONCLUSIONS
Unusual soft tissue swellings in a background of chemical exposure should be investigated thoroughly with a view to early, appropriate treatment.
Chaudhuri
A
A
Department of Surgery, Peterborough District Hospital, Peterborough, UK. arindam@chaudhuri.freeserve.co.uk
Harris
M D
MD
D015085:2,4,5-Trichlorophenoxyacetic Acid / Q000506:poisoning*; D015084:2,4-Dichlorophenoxyacetic Acid / Q000506:poisoning*; D000008:Abdominal Neoplasms / Q000139:chemically induced*; D000075182:Agent Orange; D003678:Defoliants, Chemical / Q000506:poisoning*; D004785:Environmental Pollutants / Q000506:poisoning*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D008889:Military Personnel; D010502:Perineum; D000072317:Polychlorinated Dibenzodioxins / Q000506:poisoning*; D012509:Sarcoma / Q000139:chemically induced*; D014744:Vietnam
D003678:Defoliants, Chemical; D004785:Environmental Pollutants; D000072317:Polychlorinated Dibenzodioxins; D015084:2,4-Dichlorophenoxyacetic Acid; D000075182:Agent Orange; D015085:2,4,5-Trichlorophenoxyacetic Acid