2022 Volume 59 Issue 3 Pages 304-308
Embryonal tumor with Multilayered Rosettes (ETMRs) has the worst prognosis among fetal brain tumors. There is no established standard of care, and multidisciplinary treatment that includes a combination of surgery, radiation therapy, and multidrug chemotherapy is often performed empirically. We encountered two cases of ETMR with different outcomes after multidisciplinary treatment, including surgery, radiation therapy, and chemotherapy for medulloblastoma. One patient developed local recurrence one month after treatment and underwent re-operation, re-irradiation, and bevacizumab (BEV) therapy and remained in remission for four years. The other patient developed three recurrences, including those during treatment, and underwent re-operation and additional radiotherapy with BEV but died 16 months after onset. Re-operation and re-irradiation for the recurrence of ETMR may be useful prolonging survival, and BEV therapy may be useful for preventing or suppressing the progression of radiation necrosis associated with an increased cumulative radiation dose.
