Standard systemic treatment has not been established for refractory meningioma. This retrospective study aimed to identify prognostic factors for overall survival and document outcomes of systemic therapies. We reviewed patients with meningioma followed at CHUM hospital between 2006 and 2022. Only patients with progression after first-line treatment were included. Among 750 patients, 107 (14%) experienced progression after first-line treatment. They were divided into two groups: Group 1 (n = 69, 64%) received salvage local treatments, and Group 2 (n = 38, 36%) received additional salvage systemic treatments. The median follow-up time from diagnosis was 7.5 years. 10-year OS was 88.3% (Group 1) vs. 67.2% (Group 2) (p = 0.009). Mean survival after stopping systemic treatment was 8.94 months. Key prognostic factors for poorer survival included age ≥ 65 (HR = 2.82; p = 0.009), WHO grade 2 or 3 (HR = 4.25; p = 0.004), and progression after second-line treatment (HR = 4.77; p = 0.004). Bevacizumab was associated with a mPFS of 12 months and 1-year OS of 64,6%, whereas non-Bevacizumab treatments—including Hydroxyurea, Somatostatin, and Sunitinib—were associated with a mPFS of 7 months and 1-year OS of 52,6%. This study highlights the fatal nature of recurrent meningiomas and the urgent need for systemic treatments that can improve their survival.

Primary urethral cancer is an extremely rare malignancy, accounting for less than 1% of all cancers. Due to its rarity, evidence-based treatment recommendations are lacking. We report the case of a 44-year-old woman with metastatic squamous cell urethral carcinoma and paraneoplastic Sweet syndrome. The tumor was p16-positive with strong PD-L1 expression (CPS > 50%). Following surgery and adjuvant chemoradiotherapy, the patient developed hepatic and lymph node metastases. Pembrolizumab was initiated as first-line systemic therapy because of prior hematologic toxicity with cisplatin. After four cycles, complete radiologic remission of metastases and full resolution of the Sweet syndrome were achieved. This case highlights the potential benefit of immune checkpoint inhibitors in metastatic urethral SCC, particularly in p16-positive and PD-L1-high tumors, suggesting an inflamed and immunogenic microenvironment. To our knowledge, this is the first reported case of paraneoplastic Sweet syndrome successfully treated with pembrolizumab. These findings underscore the need for further investigation of immunotherapy in this rare and challenging malignancy.