Malignant vascular tumors in young patients

Growing skull hemangioma: first and unique description in a patient with Klippel–Trénaunay–Weber syndrome

“…There are numerous reports about various types of tumors found in patients with KTW syndrome, including malignant peripheral nerve sheath tumor [6], angiosarcoma [3, 6], astrocytoma [2], hemangiopericytoma [4], and meningioma [8]. The fact that heterogeneous tumor types and localizations are still emerging reinforces the fact that this syndrome has a highly variable clinical expression.…”

Angiosarcoma of skull base in a 1-year-old child—A case report

“…They account for 2% of soft-tissue sarcomas [2], whilst sarcomas account for only 1% of all malignancies in adults. Angiosarcomas occur even more rarely in paediatric patients, representing 0.3% of paediatric sarcomas [3].…”

“…The disease was limited to the primary site in our case. Predisposing factors have been identified for extracranial disease and include radiation therapy, exposure to arsenic, thorium oxide (thorotrast) or vinyl chloride and previous trauma [2,4], however no risk factors have been identified for primary skull angiosarcomas [10,14]. In children, angiosarcoma has been reported in association with other pathologies including Aicardi syndrome [16], xeroderma pigmentosum [17], and neurofibromatosis 1 [18].…”

“…Histologically, haemangioendothelioma is characterized by anastamosing networks of irregular vascular channels lined by atypical, immature endothelial cells, often with prominent anaplasia [2,4]. Immunohistochemistry is useful in establishing the diagnosis (Fig.…”

“…Radiotherapy has been used as adjuvant therapy with surgery or as palliation for skull angiosarcoma [2,11], although its benefit remains uncertain [4,11]. Chemotherapy may be considered in the treatment of poorly differentiated tumours but at this stage too few patients have been treated to be able to establish firm treatment guidelines [6] There have been reports of successful chemotherapy in children for these tumours [20], thus warranting consideration of this treatment in paediatric patients [2].…”