Infantile Brain Tumors: A Review of Literature and Future Perspectives

Abstract

Brain tumors in infants including those diagnosed in fetal age, newborns and under a year old represent less than 10% of pediatric nervous system tumors and present differently when compared with older children in terms of clinical traits, location and histology. The most frequent clinical finding is a macrocephaly but non-specific symptoms can also be associated. The prognosis is usually poor and depends on several factors. Surgery continues to be the main option in terms of therapeutic strategies whereas the role of chemotherapy is not yet well defined and radiotherapy is exceptionally undertaken. In view of this situation, a molecular characterization could assist in providing therapeutic options for these tumors. This review highlights the recent advances in the diagnosis and treatment of brain tumors in infants with a particular focus on the molecular landscape and future clinical applications.

Central nervous system tumours in neonates: what should the neonatologist know?

“…The gestational age can orient toward a specific histological diagnosis; for example, teratomas and hamartomas generally develop before the 22nd week, germ cell tumours between the 22 nd and the 32 nd weeks and astrocytomas and glioblastomas after the 32 nd weeks [ 31 ].…”

Brain tumors in the first two years of life

“…Di Rocco et al had previously listed the ten most common brain tumors in infancy that were, in a descending order, astrocytoma, medulloblastoma, ependymoma, choroid plexus papilloma, primitive neuroectodermal tumor (PNET), teratoma, sarcoma, meningioma, ganglioglioma and neuroblastoma [32]. While in a recent review, teratoma was found to be the most common congenital tumor followed by gliomas and choroid plexus papillomas, while embryonal tumors including medulloblastomas and atypical teratoid/rhabdoid tumors (ATRTs) are less frequent [33]. Ependymoma is another subtype that is diagnosed also in infants [34] although its incidence is higher under the age of four years [33].…”

“…While in a recent review, teratoma was found to be the most common congenital tumor followed by gliomas and choroid plexus papillomas, while embryonal tumors including medulloblastomas and atypical teratoid/rhabdoid tumors (ATRTs) are less frequent [33]. Ependymoma is another subtype that is diagnosed also in infants [34] although its incidence is higher under the age of four years [33]. Pinealoblastomas and craniopharyngiomas are other tumors that are encountered more rarely in this age group [25,35].…”

“…Large head and bulging fontanelles are the main presenting symptoms and signs secondary to increased intracranial pressure secondary to large tumors or associated hydrocephalus [6,13,27,36]. The manifestations sometimes are non-specific owing to open fontanelles and non-fused sutures which permits skull expansion which makes detection of positive signs in the new born and young children is and many infants with manifestations of failure to thrive, vomiting, irritability, and delayed milestones are subjected to delayed diagnosis [16,33,47]. Some cases especially in the second year of life may present with focal neurological manifestations as weakness, cranial nerve affections or seizures [16,30].…”

“…In a review done by Wakai et al [49], 11.5% of neonatal brain tumors had associated anomalies of various nonspecific types. Teratomas was reported to be associated with hydrocephalus, cleft lip and palate, proptosis, and anencephaly [29,33]. Some tumors are parts of familial syndromes as optic pathway tumors in neurofibromatosis type 1, meningiomas in neurofibromatosis type 2, PNET in Li-Fraumeni syndrome, and subependymal giant astrocytoma occurs with tuberous sclerosis [30].…”