Congenital glioblastoma multiforme. Case report and review of the literature.

ABSTRACT

Congenital intracranial tumors are rare. If such a lesion is detected before birth, it is usually an incidental finding on fetal ultrasonography. The definition of a "congenital" tumor is controversial. The authors report the case of a "definite" congenital glioblastoma multiforme (GBM) diagnosed with the aid of ultrasonography and fetal magnetic resonance (MR) imaging in the 37th week of gestation. Postnatal MR imaging revealed a massive tumor occupying the patient's left temporoparietooccipital area. Angiography was performed to assess vascularity and embolize the main feeding arteries. Surgery was performed, and the tumor was successfully excised completely. The histopathological diagnosis of the tumor was GBM. An examination of the tumor cells revealed no p53 accumulation, a high MIB-1 index (87.5%), and no staining for epidermal growth factor receptor (EGFR). Adjuvant chemotherapy was administered, and the patient is doing well at 23 months of age. Congenital GBM should be considered in the differential diagnosis in cases in which a fetal ultrasonography study or fetal MR image reveals a tumor, especially in the presence of intratumoral hemorrhage. Radical tumor removal, administration of adjuvant therapy, and biological findings (such as a lack of the overexpression of p53 and EGFR in the tumor cells) all point to a longer survival time.