Management of recurrent giant hemangiopericytoma: illustrative cases

Abstract

BACKGROUND Hemangiopericytoma (HPC) is a rare malignancy accounting for 0.4% of intracranial tumors. HPCs are characterized by local aggressiveness, high rates of recurrence, and a tendency to metastasize to extracranial sites. These features make management of HPCs challenging, often requiring a combination of radical resection and radiation. Given their rarity, optimal treatment algorithms remain undefined. OBSERVATIONS The authors report a series of four patients who underwent resection of intracranial HPC. Mean age at presentation was 49.3 years. Three patients had reoperation for progression of residual tumor, and one patient was surgically retreated for recurrence. One patient received adjuvant radiotherapy following initial resection, and three patients received adjuvant radiotherapy following resection of recurrent or residual disease. There was one death in the series. Average progression-free survival and overall survival following the index procedure were 32.8 and 82 months, respectively. Progression occurred locally in all patients, with metastatic recurrence in one patient. LESSONS The current gold-standard treatment for intracranial HPC consists of gross-total resection followed by radiation therapy. This approach allows satisfactory local control; however, given the tendency for these tumors to recur either locally or distally within or outside of the central nervous system, there is a need for salvage therapies to improve long-term outcomes for patients.