Congenital Tumors of the Central Nervous System: The MCH Experience

Abstract

Introduction: Congenital brain tumors in the younger pediatric population are rare lesions that are histologically distinct from those in the older pediatric population. Malignant histology is common, with persistently poor outcomes despite accessible neuroimaging and evolving adjuvant therapy. There remains scant literature about the natural history of these patients because of rarity and varied institutional experiences. Methods: A retrospective review was performed of congenital brain tumor patients surgically treated at the Montreal Children’s Hospital (MCH) over a 22-year period. Patients presenting in the first year of life were evaluated for demographic information, presenting symptoms, lesion location, and management. Analysis was by median rank test and χ² statistics. Results: 13 cases of congenital brain tumors were identified: 5 supratentorial and 8 infratentorial. Median age (p = 0.93) and gender (p = 0.57) did not differ by location, and predominant histologies were choroid plexus papilloma and primitive neuroectodermal tumor. Seizure activity was exclusive to supratentorial lesions (40%, p = 0.03), with hypotonia observed only among infratentorial lesions (50%, p = 0.02). There was equal incidence of hydrocephalus (69%, p = 0.57) and increasing head circumference (38%, p = 0.27) by lesion location. Supratentorial lesions were treated by total resection (n = 3), subtotal resection (n = 1), and biopsy (n = 1). Infratentorial lesions were treated by total resection (n = 1), subtotal resection (n = 2), biopsy (n = 1), no operation (n = 2), and decompressive laminectomy for two spinal lesions. Conclusions: Congenital brain tumor patients represent fewer than 2% of patients treated at MCH. An evolving understanding of management objectives for these lesions requires understanding institutional experiences. Patients with supratentorial lesions frequently present with seizures, hydrocephalus, and macrocrania, and more frequently underwent total resection at surgery.

Seizures in Children with Brain Tumours — Epidemiology, Significance, Management, and Outcomes

“…Although in some small series of patients, seizures have been found to occur in up to 50% of paediatric patients with a brain tumour [170], in most populations, the overall incidence of seizures in this patient population is considerably lower, in the 10-20% range [51,89,90]. This is largely due to the infratentorial location of the majority of paediatric tumours, where very few are epileptogenic [37].…”

“…Because children are more likely than adults to have infra-versus supratentorial lesions, the percentage of children presenting with seizures may be somewhat less than among adults, closer to the 10-20% than the 30-50% range [51,89,90], though 50% or more has been reported in some series [32,35]. As in adults, of these epileptic tumours, the vast majority are supratentorial.…”

“…Virtually every possible tumour type has been reported presenting as seizures, especially lowgrade gliomas [27, 30, 32, 34, 37, 39, 70-72, 75, 76, 78, 91-93] and glioneuronal tumours like ganglioglioma and dysembryoplastic neuroepithelial tumour [28,44,62,73,88,89,91,94,95]; but including oligodendroglioma [77,96,97], cortical ependymoma [98], medulloblastoma [47], subependymal giant cell astrocytoma (SEGA) [99], meningioma [10] 0 , thalamic and cerebellar glioma [38,46], and a variety of atypical, systemic and metastatic tumours, like primary meningeal osteosarcoma [84], acute lymphoblastic leukemia [101], anaplastic large cell lymphoma [102], neuroblastoma [103], melanoma [104], various sarcomas [105,106], Ewing's sarcoma [107], malignant germ cell tumours [108], and others [16,109].…”

“…However, these tumours are almost always associated with seizures. Consequently, they comprise a disproportionate percentage of tumour-associated epilepsy cases [28,44,62,73,88,89,91,94,95]. Moreover, DNETs tend to be extremely resistant to AED therapy [62,[182][183][184][185][186].…”

Fetal Growth and Childhood Cancer: A Population-Based Study

“…However, congenital brain tumors are rare, accounting for only 0.5% to 4% of all pediatric brain tumors. 34,35 …”