Abstract
Vascular anomalies (VAs) comprise a large variety of individual diagnoses that in
different phases of treatment require a diverse number of medical specialists to provide
optimal care. Medical therapies include agents usually associated with cancer chemotherapy,
such as vincristine, as well more immunomodulatory types of drugs, such as glucocorticoids
and sirolimus. These immunomodulating drugs are being successfully applied in cases
that are typically categorized as vascular tumors, including kaposiform hemangioendothelioma
(KHE) and tufted angioma (TA), as well as some of the more invasive types of vascular
malformations (i.e., microcystic lymphatic malformations and blue rubber bleb nevus
syndrome (BRBNS). These therapies need to be combined with good supportive care, which
often involves anticoagulation, antimicrobial prophylaxis, and comprehensive pain
and symptom-relief strategies, as well as appropriate drug monitoring and management
of side effects of medical treatment. The optimal care of these patients frequently
involves close collaboration between surgeons, interventional and conventional radiologists,
medical subspecialists, and nurses.
Keywords
vascular anomalies - chemotherapy - sirolimus - kaposiform hemangioendothelioma -
tufted angioma - blue rubber bleb nevus syndrome