RESUMO
BACKGROUND: Angiosarcomas are high-grade endothelial tumors remarkable for their rarity and malignant behavior. Primary calvarial angiosarcoma is an extremely rare entity and its behavior usually sets it apart from other angiosarcoma types. We highlight the successful management of cranial angiosarcoma using a multidisciplinary approach. CASE DESCRIPTION: We present a 16-year-old male who was first noted to have a right-sided parietal cranial mass that was biopsied in 2008. Pathology was initially thought to be Kaposiform hemangioendothelioma. The patient subsequently underwent chemotherapy with vincristine. The patient did well until early 2010, when he suffered a right-sided intraparenchymal intratumoral hemorrhage. At this time, the original pathologic diagnosis was revisited and the diagnosis was upgraded to an angiosarcoma. The patient underwent a second round of chemotherapy using vincristine, cyclophosphamide, and actinomycin. The tumor continued to progress despite this treatment and he developed extensive skull deformity. At this point more definitive surgical intervention was reconsidered. Preoperative embolization of the mass was performed followed by aggressive surgical resection of the bony disease. The patient tolerated the procedure well and was discharged 6 days postoperatively without any new deficits. The patient is currently in the process of completing radiation therapy to entire tumor bed. He has clinically done well with no neurologic deterioration and has demonstrated long-term survival (>3 years). CONCLUSION: With the combined efforts of pediatric oncology, radiation oncology, interventional neuroradiology, and neurosurgery, a survival of greater than 3 years is possible with this aggressive pathology.