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Grade III meningiomas and haemangiopericytomas are a rare subgroup of aggressive tumours with poor associated overall survival. Data to guide optimal management is limited. This retrospective case series aims to document UK-based management approaches to this rare diagnosis. In particular, we hope to gain insight into the RT approaches being used across the UK and whether this is associated with a specific outcome. Data on response to systemic treatment may be useful for clinicians given the lack of evidence base in this area.

Patients with WHO Grade III malignant meningioma or haemangiopericytoma between over a 10-year period were identified. Data were collected on location of disease, primary management strategy and radiotherapy approach, with a focus on RT technique and dose/fractionation approach. Data was also collected on radiotherapy re-treatment, systemic management and survival.

9 centres participated in this case series of 79 patients; 86% of which were Grade III meningiomas and 14% haemangiopericytomas. Surgical resection was the initial management approach in 97.5% of patients with 44% judged as having an incomplete surgical resection. 64% had adjuvant radiotherapy. Of those that received adjuvant radiotherapy, 93.8% had external beam RT and 6.2% had SRS. Most frequently, patients received 60Gy in 30 fractions over a 6-week period. Mean OS with adjuvant RT was 33.9 months. Mean OS without adjuvant RT was 23.3 months.

68.4% experienced disease relapse following initial surgical and adjuvant management. 75% of those who progressed after treatment had relapse within the surgical bed/radiation field. 26% of patients who relapsed received salvage radiotherapy. Of those offered salvage radiotherapy, 3 were treated with IMRT and 9 were treated with SRS.

8 patients underwent radiotherapy re-treatment with a wide variety of dose-fractionation schedules utilised.

3.7% of patients were commenced on systemic treatment once local treatment options had been exhausted, none of whom had a favourable response to treatment.

Grade 3 meningiomas and haemangiopericytomas are rare tumours with poor outcomes. This case series reflects that surgical resection is the primary management strategy, but that an incomplete surgical resection was observed in almost half of cases.

Adjuvant RT remains an important part of the management paradigm in these patients with two thirds of patients completing this following surgery. The survival figures suggest adjuvant RT positively impacts on survival. Due to the frequent observation that anaplastic meningiomas often arise from previous lower grade disease, management decisions surrounding the use of radiotherapy re-treatment are often not straightforward, with radiosurgical approaches being increasingly utilised as confidence in these techniques advances.

Unfortunately, patients frequently exhausted local treatment options and a small number were commenced on systemic treatment, none of whom responded to treatment.