Congenital Glioblastoma Multiforme: Complete Resection with Long-Term Survival and a Novel Technique of Contralateral Cystoventriculostomy

Abstract

Congenital glioblastomas are rare primary tumours of the central nervous system with poor prognosis if left untreated. We report the case of a 4-week-old infant with such a tumour treated by surgical excision and a course of postoperative chemotherapy. After the chemotherapy, the patient displayed a neurological deterioration and follow-up magnetic resonance imaging (MRI) scans revealed no signs of recurrence, but showed entrapment and significant distension of the right lateral ventricle. A novel technique of contralateral cystoventriculostomy was performed to attempt to decompress the cyst and improve the infant's neurological status. After surgery there was a significant neurological improvement, and 30 months after resection the child is alive, with mild hemiparesis and no signs of recurrence on follow-up MRI scan. The cystoventriculostomy is patent and continues to decompress the encapsulated ventricle.

Congenital Glioblastoma Multiforme: An Unusual and Challenging Tumor

“…To our knowledge, there are 83 cases in the literature considered as congenital by the authors. [1][2][3][4][5][6][7][8][9][10][11] We, however, accept the definition of Solitare and Krigman as modified by Wakai et al Given the fact that GBM is a primitive, rapidly growing neoplasm and in agreement with previous reports, we also consider a tumor of this type diagnosed at 1 year of life unlikely to be congenital. 3,43 We believe that to understand the special features of congenital GBM, it is important to use strict definition criteria, even if this results in "filtering out" some publications involving cases where the congenital nature of the tumor is unclear or debatable.…”

“…In cases where follow-up imaging is available, significant change in the size of the intracranial mass might be described, not only due to the tumor's reported tendency to increase rapidly, but sometimes due to the presence of hemorrhage as well (►Table 1). 2,4,9,29,35 It should be noted that there are no specific diagnostic criteria for congenital GBM. Other congenital brain tumors, as well as vascular malformations, infarctions, and primary hemorrhage should be included in the differential diagnosis since a definite diagnosis cannot be made only by prenatal sonography.…”

“…4 As a result, once an intracranial lesion is detected, subjection to fetal MRI scan should be the next step. 2,3 Most congenital GBMs are described as heterogeneous, typically solid masses, with varied signal intensity and with areas of hemorrhage and necrosis (►Table 2). 3,29,35,45 Necrosis with ringlike enhancement has been proposed as the major MRI criterion differentiating GBM from other intracranial tumors (especially anaplastic astrocytomas).…”

“…1 Although first described by Holt almost a century ago, the designation of a central nervous system (CNS) tumor as "congenital" still remains controversial. [2][3][4] Reportedly, congenital tumors of the CNS are limited to 1.1 to 3.6 incidents per 100,000 newborns, [5][6][7] representing less than 2% of all childhood brain tumors. 3,8 Teratomas are the commonest, but numerous cases of astrocytomas among other types of tumors have also been described in the literature.…”

Perinatal (fetal and neonatal) astrocytoma: a review

“…Nevertheless, cure was not always out of the question. All six patients who had surgical resection and chemotherapy [10, 11, 46, 50], one with surgical resection, embolization, and chemotherapy [74], and two of six who received surgery as the only treatment, survived [93] (Table 2). Neonatal survival of 50 % was higher than fetal, which was only 6.5 %.…”