- Cutaneous lymphangiosarcoma that develops in lymphoedema following mastectomy with axillary node clearance or axillary radiation
- Term is also used wherever lymphangiosarcoma arises in primary congenital or secondary chronic lymphoedema
- Typically only arises in substantial and persistent lymphoedema
- Occurs in 1/200 pts post-mastectomy who survive post 5yrs with lymphoedema
