Epidemiology
A rare vascular tumor of infants and children that can appear in the limbs or chest wall. If there is vascular swelling with bruising or low platelets, the evaluation must be urgent and coordinated.
Basic features
It is infiltrative and can be associated with pain, swelling, skin changes, or clotting abnormalities.
Usual treatment
Management is coordinated with the vascular and oncology teams; sirolimus (an mTOR inhibitor), often combined with corticosteroids, is today the preferred systemic treatment — especially when the Kasabach-Merritt phenomenon is present — while vincristine, embolization, or surgery serve as alternatives or add-ons.
Outcomes and prognosis
It does not usually metastasize, but it can cause significant local problems and requires close follow-up.