Connatal Intracranial Tumours

Abstract

Twelve cases of definitely connatal intracranial neoplasms present or producing symptoms at birth or within the first week of life are presented. They were selected from a series of 56 connatal tumors including probable or possible congenital intracranial neoplasms (SOLITARE and KRIGMAN) observed in a total of 730 intracranial tumours between ages 1 and 16 years, which corresponds to an incidence of 7,6%. Among the 12 reported cases, 8 females and 4 males, four (large teratoma, reticulum cell sarcoma of posterior fossa, osteoma of tentorium associated with cerebellar hypoplasia and facial cleft syndrome and cerebral astrocytoma) were recognized during the neonatal period, 3 tumours (2 cerebellar medulloblastomas, one cerebral medulloepithelioma) were diagnosed between 3 and 9 months of age, while in 5 children the diagnosis was made after age one year. The latter included frontal fibrolipoma, occipital dermoid, and hypothalamic hamartoma, while 2 tumours were radiologically confirmed. In 9 infants intra vitam diagnosis was possible, 3 underwent surgery, but surgical cure was only possible in 2 cases of superficial benign tumours. A short review of the principal morphological types of 132 connatal intracranial neoplasms collected from the literature in tabulated form is presented. The largest group form neuroectodermal tumours, particularly gliomas, followed by malformative neoplasms and mes-enchymal tumours, while metastases are rare. Almost three-quarters of connatal tumours are supratentorial. Since the majority of cases clinically presents as progressive hydrocephalus or increased intracranial pressure, the diagnosis is difficult. Recent experimental models of pre-natally induced nervous system tumours give no conclusions for the etiology of connatal intracranial neoplasms in man.

Keyword

Connatal Intracranial Neoplasms - Brain Tumours - Neonatal Period Congenital Hydrocephalus