LAUSR

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. OBJECTIVE To describe our own clinical case of cGBM and review the literature with prolonged survival. METHODS We report our 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. RESULTS Our 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 (WeeFIM) score of 109. Out of 160 articles screened, there were 10 articles included. A total of 15 patients, including our case, were analyzed qualitatively. The age at presentation ranged from 30 weeks age of gestation to 35 days. Most patients underwent surgical excision (86.7%) and adjuvant chemotherapy (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.