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
