A Case of Congenital Glioblastoma

Abstract

  Congenital glioblastoma is a very rare disease for which no standard treatment has been established. The prognosis for survival in untreated patients is approximately 2 months. We present a case of congenital glioblastoma in a male infant, which was identified during the fetal period. The first biopsy failed to provide a diagnosis. A second operation was performed 3 months later, which revealed the tumor to be glioblastoma. Although oral temozolomide (TMZ) was temporarily effective, tumor regrowth necessitated a third tumor resection, local irradiation to residual tumor, and administration of bevacizumab in combination with TMZ. He died 15 months after his birth. Histological examination revealed that the histological and molecular-biological characteristics of the second specimen resembled those of adult primary glioblastoma. The expression levels of p53 and MGMT (O⁶-methylguanine DNA methyltransferase) and mutational analyses of histone H3.3, and BRAF were similar even after TMZ treatment.