pralsetinib in the treatment of pleomorphic xanthoastrocytoma (PXA) with RET-activated: a case report
RET-activated gliomas are exceptionally rare and associated with a poor prognosis, and there are no established effective therapies. Clinical data on RET inhibitors are predominantly derived from solid tumors such as non-small cell lung cancer and medullary thyroid carcinoma, with data for central nervous system tumors being scarce. The present report describes a case of pleomorphic xanthoastrocytoma with RET activation that progressed following the STUPP regimen but subsequently exhibited a favorable response to pralsetinib, teniposide and bevacizumab. The tumor area decreased from 4.84 cm2 before treatment to 1.92 cm2 (calculated based on the longest and maximum perpendicular diameters). A comprehensive literature review revealed no comparable reported cases.