Primary Cranial Angiosarcoma: Long-Term Survival Is Feasible with Multidisciplinary Management

• Published in: Journal of Neurological Surgery Part B: Skull Base Vol. 73; no. S 01
• Main Authors: Guthikonda, Bharat, Ahmed, Osama, Shorter, Cedric, Khan, Imad, Thakur, Jai, Jeroudi, Majed, Nanda, Anil
• Format: Conference Proceeding Journal Article
• Language: English
• Published: 17.04.2012
• ISSN: 2193-6331; 2193-634X
• DOI: 10.1055/s-0032-1312282

Introduction: Angiosarcomas are high-grade endothelial tumors remarkable for their rarity and malignant behavior. Central nervous system involvement with angiosarcoma has rarely been described and arises from mesenchymal elements of the cranium. Cranial angiosarcoma is extremely rare and appears to have an extremely poor overall prognosis. We describe a multidisciplinary approach to managing cranial angiosarcoma that has led to a good neurological outcome and survival greater than 3 years in a pediatric patient. We supplement this with a review of the existing literature regarding this rare and usually highly aggressive tumor. Methods: We present a 16-year-old male who was first noted to have a right-sided parietal subgaleal and cranial mass that was biopsied in 2008. Pathology was initially thought to be a 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 pathological diagnosis was revisited and, on further review and consultation, the biopsy from 2008 was upgraded to the more malignant diagnosis of angiosarcoma. The patient recovered from this hemorrhage and subsequently 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 with massive bilateral fronto-temporoparietal angiosarcoma. At this point, more definitive surgical intervention (which was previously thought to be too risky due to the extent and vascularity of the tumor) was reconsidered. Preoperative embolization of a large portion of the blood supply of the mass was performed. Total removal of bilateral subgaleal and cranial tumor was performed the day following embolization. The patient tolerated the extensive surgery well and was discharged on POD 6. The patient is currently in the process of completing radiation therapy to the entire tumor bed. This will be followed by a monoclonal antibody-based chemotherapy regimen to which the patient is naive. He has clinically done very well with no neurologic deterioration and long-term survival. Conclusion: We highlight the successful management of cranial angiosarcoma in the pediatric population using a multidisciplinary approach. With the combined efforts of pediatric oncology, radiation oncology, interventional neuroradiology, and neurosurgery, long-term survival (>3 years) is possible with this aggressive pathology.