An anaplastic ependymoma with chondroid metaplasia after stereotactic radiosurgery: illustrative case

BACKGROUND Anaplastic ependymomas are malignant CNS tumors with poor prognosis. Chondroid metaplasia in ependymomas is extremely rare, and its clinical significance remains unclear. OBSERVATIONS A 21-year-old male underwent gross-total resection and adjuvant radiotherapy for an anaplastic ependymoma. Over the following 6 years, he experienced multiple recurrences treated with endoscopic resections, Gamma Knife radiosurgery, temozolomide, and bevacizumab. Seven years after initial surgery, a previous irradiated cerebellar lesion showed aggressive regrowth and was resected. Histopathology revealed chondroid metaplasia within the recurrent tumor. Although temporary control of the tumor within the irradiated field with proton therapy was achieved, the rapid progression of disseminated lesions proved fatal. LESSONS This is the fifth reported case of chondroid metaplasia in an ependymoma following radiotherapy. Once metaplasia occurs, the prognosis is highly unfavorable, particularly in anaplastic cases. Although proton therapy was administered under suboptimal conditions, the initial tumor control suggests it may be more effective if applied earlier. In the absence of effective chemotherapy, repeated minimally invasive surgeries and radiosurgeries may help preserve cognitive function and control disease progression. https://thejns.org/doi/10.3171/CASE25308