Primary intracranial neoplasms of infancy and early childhood.

We investigated the age-related location, gender distribution, and histology of 107 brain tumors in children under 4 years of age seen in our department between 1984 and 1997. The male-to-female ratio was 1.4 (62/45 cases) with a prevalence of supratentorial tumors (60/47 = 1.3); the main histological entity was astrocytoma (33.6%), followed by ependymoma (14.0%). In the 1st year of life 22 cerebral neoplasms became clinically apparent. A higher ratio for supratentorial tumors was revealed (17/5 = 3.4), but without gender preference, and primitive neuroectodermal tumors (PNET) were the most frequent (5/22). In the 2nd year 25 tumors were found. The male-to-female ratio was 1.5 (15/10) and the supratentorial-to-infratentorial ratio, 1.1 (13/12). The two most common entities were astrocytoma and ependymoma (6 cases each). In addition, a survey of previously published investigations into this subject was performed and a compilation of data on 1960, 545 and 1084 tumors in children below the age of 1, 2 and 4 years, respectively, was prepared, which makes it the most extensive review of brain tumors of infancy and early childhood yet undertaken.

Intracranial tumours in the first year of life. A report on 51 cases.

The authors report on 51 infants with intracranial tumours treated in an eleven-year period; these infants represent 13% of the total population of children with intracranial tumours who have been operated on in the same institution during the same period of time. Males (28 cases) were slightly more frequent. Astrocytomas (17 cases), medulloblastomas (12 cases), and ependymal tumours (5 cases) were the commonest histologic types. Signs and symptoms of increased intracranial pressure were by far the most frequent clinical manifestations, followed by seizure disorders. Thirty tumours were localized within the supratentorial, and 21 within the subtentorial compartment. The parasellar region (10 cases) and the lateral cerebral ventricles (8 cases) for the supratentorial tumours, the inferior cerebellar vermis and fourth ventricle (13 cases) for the infratentorial tumours appeared to be the preferred topographic locations. Craniotomies were carried out in 44 infants, with a total or radical removal of the tumour in 19 cases, a subtotal removal in 6 cases, and a partial removal in 17 cases. In 3 cases only a biopsy procedure was performed. Twenty-nine of these patients required an ancillary procedure such as CSF shunting. Three subjects underwent a biopsy procedure and 1 infant the insertion of a CSF shunting device only. Surgery was not performed in 5 cases. Overall, there were two surgical deaths. Two infants died before any surgical treatment could be performed. Radiation therapy was administered to 9 patients when they had reached three years of age. Chemotherapy was given to 21 infants, according to various chemotherapeutic protocols. During the postoperative period 20 deaths (39%) were recorded. Two patients were lost to follow-up. From 1 to 10 years after the operation, 29 patients are still alive, 14 of them (28%) with a normal psychomotor development, 10 (20%) with some neurological or mental deficits, and 5 (10%) with severe psychomotor retardation. There was no apparent correlation in this series between late outcomes and the histological type of the tumour.

Metastasising congenital subcutaneous sacrococcygeal ependymoma.

A case of myxopapillary ependymoma located subcutaneously in sacrococcygeal area of a 18 months male child with metastasis to right inguinal lymph nodes is described; the tumour was present since birth. Histological examination of the tumour besides myxopapillary structures, also revealed an ependyma lined cystic space, resembling ventricular cavity. So far only two cases of congenital metastasising subcutaneous sacrococcygeal ependymoma have been described. However, the present case had a very aggressive post-operative biological behaviour.

[Congenital ependymoma. Case report and immunohistochemical studies]. / Kongenitales Ependymom. Fallmitteilung sowie immunhistochemische Untersuchungen.

Reported in this paper is a congenital ependymoma in an 23-week old foetus. The neoplasm was well vascularised and contained typical ependymal rosettes. The tumour cells did not react with GFAP-antiserum. They reacted weakly with neuron-specific enolase and vimentin and exhibited strong antigenicity with S-100-protein-antiserum. Cytokeratin antigen was recordable from some tumour cells. The tumour was sufficiently mature for classification as ependymoma. Immunohistochemical findings suggested possible ectodermal origin of the tumour cells.

Congenital ependymoblastoma presenting as a sacrococcygeal mass in a newborn: an immunohistochemical, light and electron microscopic study.

We describe a case of congenital ependymoblastoma presenting as a subcutaneous mass in the sacrococcygeal area of a newborn male. The tumor was composed of primitive cells disposed in compact sheets and cords and exhibiting focal ependymal differentiation. No other line of cellular differentiation was identified by either immunohistochemistry or ultrastructural study. Elevated serum alpha-fetoprotein was found, which decreased following surgical extirpation of the tumor. We propose that this tumor had its origin in the ependymal medullary vestige, similar to other sacrococcygeal ependymomas.

Congenital anaplastic ependymoma: a case report of familial glioma.

A congenital anaplastic infratentorial ependymoma, occurring in a female infant whose sister also died of brain tumor, is described. The tumor, located in the cerebellum and the fourth ventricle, is characterized by predominantly undifferentiated neuroepithelial cells associated with numerous mitoses, and partially of the cells demonstrating differentiation into ependymal cells and astrocytes. Of special interest is the fact that the elder sister of the patient had also died of a congenital glioma located in the cerebellum and the fourth ventricle, which leads to the discussion about the influences of genetic factors in the development of the familial gliomas as well as about the histogenesis of the tumor examined.

[Congenital brain tumors in children under 1 year old]. / Vrozhdennye opukholi golovnogo mozga u detei pervogo goda zhizni.

Secondary, paraventricular and, less frequently, primary tumors of the lateral and third ventricles usually of supratentorial localization prevailed among congenital tumors of the brain. They impair the drainage of cerebrospinal fluid and cause internal hydrocephalus. The clinical picture of congenital cerebral tumors is characterized by a manifest hypertensive-hydrocephalic syndrome and a severe general condition. The neurological symptomatics, the EEG and the Echo-EG usually yielded exact information on internal hydrocephalus but rarely helped in determining the localization and character of the tumor. Pneumoventriculography, angiography, computer tomography, and radioisotope diagnosis were methods which provided the most information. Pneumoventriculography, however, causes many complications and the method of isotope diagnosis and computer tomography is therefore preferable because it is sparing and safe for the use in children of the first year of life.

Congenital ependymoblastoma.

An ependymoblastoma with widespread dissemination within the central nervous system of a stillborn, premature male infant is reported.