Long-term therapy with Bevacizumab in a young patient affected by NF2. Stop or continue treatment? An update of a case report and review of the literature

Abstract

Neurofibromatosis type 2 (NF2) is an autosomal dominant condition caused by pathogenic variants in the NF2 gene. To date, cytotoxic chemotherapy has no established role in the treatment of NF-2. Historical case reports of malignant schwannomas have documented responses to chemotherapies with cyclophosphamide, vincristine and doxorubicin, in patients who develop pulmonary metastases. Recently, several studies proposed the use of anti-HER2, anti-EGFR, anti-platelet-derived growth factor receptors. As reported in our previous review of the literature, vascular endothelial growth factor (VEGF) and its receptor VEGFR-1 have been detected in schwannomas with the best results. We described the case of a young patient with NF2 treated for long time with Bevacizumab. Here, we report the update of the previous case report.

Qu-Du-San-Jie decoction induces growth inhibition and vascular normalization in NF2-associated vestibular schwannoma

“…Several preclinical and clinical trials have demonstrated that antiangiogenic therapies can inhibit NF2-associated VS tumor growth and postpone the related hearing loss (Wong et al, 2010;Gao et al, 2015;Killeen et al, 2019;Renzi et al, 2020). However, responses to these drugs, such as Bevacizumab, are not durable, suggesting that either longer maintenance therapy or new strategies are required (Nigro et al, 2020;Renzi et al, 2020). Our bioinformatic analysis showed that tumor angiogenesis-related proteins, including EGFR, FGF1, FGF2, VEGRR2, and PDGFRA, were in the top 15 key targets in the PPI network of QDSJ decoction treating NF2-associated VS. Our immunofluorescence staining results confirmed that the tumor vessel densities and the fractions of collapsed vessels were decreased, while the fractions of pericyte-covered vessels were increased after QDSJ treatment, suggesting that QDSJ could reduce angiogenesis and improve tumor vascular normalization in NF2-associated VS xenografts.…”