Congenital Glioblastoma Multiforme with Long-Term Childhood Survival: A Case Report and Systematic Review

Background: Congenital glioblastoma multiforme (cGBM) is an infrequent primary central nervous system tumor occurring within the first few months of life with a reported poor overall prognosis. Our objective was to describe our own clinical case of cGBM and review the literature of cGBM cases with prolonged survival.

Case description: We report a case of cGBM with prolonged survival at 4 years. A systematic review was conducted on cases of cGBM with long-term childhood survival. We searched online databases until August 2019 for relevant articles. The patient underwent an emergency right hemicraniectomy with excision and biopsy of the right cerebral hemisphere mass and insertion of a ventriculoperitoneal shunt. At present, she is a 52-month-old child with good speech and minimal left hemiparesis and able to ambulate, with a Functional Independence Measure for Children score of 109. Out of 160 articles screened, there were 10 articles included. A total of 15 patients, including the present case, were analyzed qualitatively. The age at presentation ranged from 30 weeks' gestation to 35 days. Most patients underwent surgical excision (n = 13, 86.7%) and adjuvant chemotherapy (n = 10, 66.7%). The reported range of survival of these patients was from 27 to 110 months.

Conclusions: Limited evidence from 15 cases of cGBM suggests that surgical excision and/or chemotherapy may prolong the survival of patients. Therefore, these interventions may be offered and performed to patients with cGBM on a case-by-case basis. Larger clinical studies or registry-based information are necessary to substantiate the implications of our review.