'Proximal-type' epithelioid sarcoma: is Agent Orange still at large?

Abstract

BACKGROUND AND AIMS:

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.

PATIENT AND 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.

CONCLUSION:

Unusual soft tissue swellings in a background of chemical exposure should be investigated thoroughly with a view to early, appropriate treatment.

• Authors: A Chaudhuri, M D Harris
• Journal: Annals of the Royal College of Surgeons of England (Ann R Coll Surg Engl) Vol. 85 Issue 6 Pg. 410-2 (Nov 2003) ISSN: 0035-8843 [Print] England
• PMID: 14629885 (Publication Type: Case Reports, Journal Article)

Chemical References

• Defoliants, Chemical
• Environmental Pollutants
• Polychlorinated Dibenzodioxins
• 2,4-Dichlorophenoxyacetic Acid
• Agent Orange
• 2,4,5-Trichlorophenoxyacetic Acid

Topics

• 2,4,5-Trichlorophenoxyacetic Acid (poisoning)
• 2,4-Dichlorophenoxyacetic Acid (poisoning)
• Abdominal Neoplasms (chemically induced)
• Agent Orange
• Defoliants, Chemical (poisoning)
• Environmental Pollutants (poisoning)
• Humans
• Magnetic Resonance Imaging
• Male
• Middle Aged
• Military Personnel
• Perineum
• Polychlorinated Dibenzodioxins (poisoning)
• Sarcoma (chemically induced)
• Vietnam