Angiosarcoma is a rare malignant tumor with an aggressive course and poor prognosis. It is typically seen in adults but very rarely seen in children. Angiosarcoma of the skull with brain metastasis is exceptionally rare. Due to the rare nature of these tumors, much is left unknown about clinical progression and treatment guidelines are not well established.
A 14-year-old male patient presented with an enlarging mass on the parietal region of the head. Further investigations revealed a mass lesion involving scalp and skull tissue. Biopsy result showed angiosarcoma and the patient underwent multiple surgical interventions including scalp excision, craniectomy on tumor site, and excision of brain metastases. He also received chemotherapy and radiation therapy. Despite aggressive treatment, disease progression could not be controlled.
Here we report a pediatric patient with intracranially invasive angiosarcoma of the scalp and skull, with recurrent hemorrhagic metastases to the brain. This is a very rare case in pediatric age group with very poor prognosis. Our patient had impressively longer survival than those reported in the literature despite multiple hemorrhagic brain metastasis and this is most likely related to our aggressive treatment strategy that includes multiple craniotomies for metastatic tumor resection in addition to neoadjuvant chemotherapy and radiation therapy. We believe optimal treatment of skull angiosarcoma in children should aim gross total resection of the skull tumor including involving scalp and dura as well as neoadjuvant chemotherapy and radiation therapy, and the patient should be followed closely with repeated brain MRI studies to pursue additional surgeries to remove brain metastasis if feasible.