Epidemiology
An aggressive vascular sarcoma of adults that can arise in the skin, soft tissue, an irradiated breast, or chronic lymphedema. With a history of radiotherapy or chronic lymphedema, a persistent purplish plaque or mass must be investigated promptly.
Basic features
It can present as a bruise, a purplish plaque, a deep mass, or bleeding, and it often grows in an infiltrative way. Cases that arise over an irradiated area or with chronic lymphedema tend to show amplification of the MYC gene, a feature that helps confirm them.
Usual treatment
It requires multidisciplinary management with surgery, radiotherapy, and systemic treatment depending on the site and extent.
Outcomes and prognosis
It has a high risk of recurrence and metastasis and an unfavorable prognosis, so it needs prompt diagnosis and close follow-up.
