Nervous System Hemangiopericytoma

The management of patients harboring central nervous system (CNS) hemangiopericytomas (HPCs) is a partially answered challenge. These are rare locally aggressive lesions, with potential for local recurrence, distal neural metastasis (DNM), and extraneural metastasis (ENM). Resection, when feasible, remains the initial treatment option, providing histological diagnosis and immediate relief of tumor-related mass effect. Patients receiving surgery alone or surgery and external beam radiotherapy (EBRT) show improved overall survival (OS) and progression-free survival as compared to those undergoing a biopsy alone (p = 0.01 and p = 0.02, respectively). Yet, in many instances, patient and tumor-related parameters preclude complete resection. EBRT or stereotactic radiosurgery (SRS) shares a significant role in achieving local tumor control, not shown to impact OS in HPC patients. The benefits of SRS/EBRT are clearly limited to improved local tumor volume control and neurologic function, not affecting DNM or ENM development. SRS provides acceptable rates of local tumor volume control coupled with treatment safety and a patient-friendly apparatus and procedure. Single-session SRS is most effective for lesions measuring <2 cm in their largest diameter (10 cm3 volume), with prescription doses of at >15 Gy. Systemic HPC disease is managed with various chemotherapeutic, immunotherapeutic, and anti-angiographic agents, with limited success. We present a short discussion on CNS HPCs, focusing our discussion on available evidence regarding the role of microsurgical resection, EBRT, SRS, chemotherapy, and immunotherapy for upfront, part of adoptive hybrid surgery approach or for recurrent HPCs.