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Three cases of primary rhabdoid tumour of the CNS (RT-CNS) are presented. In case 1 a hemispheric tumour developed in a 10.5 months old girl, who survived for 6 months after incomplete resection, radio- and polychemotherapy. Case 2 was a 4 years and 8 months old boy with a large IIIrd ventricle tumour, who died of leptomeningeal tumour dissemination 7 months after diagnosis despite radiotherapy. In case 3 a pineal mass occurring in a 14 month old female was radioresistant and totally exstirpated. The child died due to tumour recurrence two months later. Autopsy examination revealed widespread leptomeningeal dissemination. All three cases fulfilled light and electron microscopic criteria of RT-CNS including abundant eosinophilic cytoplasm, vesicular nuclei with large nucleoli and conspicuous anti-vimentin positive filaments. Extensive immunohistochemical studies showed expression of epithelial (EMA, KL1), macrophage (alpha-1 antichymotrypsin), neuro-ectodermal (GFAP, NSE, β-tubulin III) and myogenic markers (desmin, actin). Different stress proteins (alpha-B crystallin, HSP70) were also expressed. Tumour cells showed a proliferation (MIB1) index of 28.4% (case 1) and 33.4% (case 2). From our study it can be concluded that RT-CNS reveals significant immuno-morphological heterogeneity thus supporting the view that it is not a specific pathological entity but merely a phenotypic appearance of different neoplasms, some of which are linked to primitive neuro-ectodermal tumours. [ABSTRACT FROM AUTHOR]

Eighteen granular cell tumors from various sites were examined with antisera directed against protein S-100, neuron specific enolase (NSE), alpha-1-antichymotrypsin, and alpha-1-antitrypsin, glial fibrillary acidic protein (GFAP), lysozyme, factor VIII-related antigen, myoglobin and vimentin, as well as with a monoclonal antibody (lu-5) directed against a panepithelial marker. The immunocytochemical reaction pattern of the tumors was heterogeneous. The brain and pituitary tumors and one thyroid tumor reacted for alpha-1-antichymotrypsin and alpha-1-antitrypsin, but not for S-100 protein and NSE. However, tumors from other sites showed immunoreactions for S-100 protein and NSE and some also for vimentin. Reactions for alpha-1-antichymotrypsin and alpha-1-antitrypsin were not observed. All other reactions were similarly negative. We conclude that the morphologically homogeneous group of granular cell tumors is biologically heterogeneous. [ABSTRACT FROM AUTHOR]

Investigation of GFAP in 50 medulloblastomas showed a few GFAP-positive tumour cells in 5 cases only; 17 tumours were negative, and 28 showed a 'pseudopositivity', i.e. GFAP-bearing cells were identified as reactive or degenerating astrocytes, intermingled with tumour elements. A high GFAP content was seen in 2 small-cell gliomas of the cerebellum, whereas 3 pineoblastomas, 2 neuroblastomas of CNS, and one medulloepithelioma were negative. GFAP is a very good method for identificating astrocytes, but does not seem to be reliable for identifying the origin of undifferentiated tumours such as medulloblastomas. In these neoplasms glial differentiation is lacking or extremely rare, GFAP-positivity being mostly an artifact. The investigation of small tumour samples or the positivity of a single cell are inadequate data for a correct evaluation of the findings, especially taking in mind that GFAP of degenerated astrocytes can be phagocytised by cells other than glial (e.g., macrophages, epithelial and meningioma cells). The importance of carefully checking the whole structure of the tumour is stressed, GFAP positivity or negativity being not a sufficient criterion for its nosological classification. [ABSTRACT FROM AUTHOR]

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The authors describe radiologic and pathoanatomic findings in the very rare Mafucci-Kast syndrome. In a 39-year-old Libyan patient multiple enchondromas of the fifth ray of the left hand were found with signs of malignant transformation. Furthermore, there were angiomas of the skin, of the soft meninges, and the bone. In addition, there was a low-grade malignant astrocytoma of the frontal lobe of the brain. Chromosome analysis revealed a normal male chromosome set. The tendency to develop malignant tumors which is repeatedly emphasized in the literature was also shown in the present case. The reason for this, especially for the high spontaneous rate of malignant transformation of multiple enchondromas, is unknown. The occurrence of angiomas and multiple enchondromas in cartilaginous performed bone suggests the presence of mesodermodysplasia. [ABSTRACT FROM AUTHOR]

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The influence of Laser radiation on neuroma formation was investigated in rat sciatic nerves. Peripheral nerve trunks were divided by focussed Neodym Yag Laser and compared with those transected by microscissors. The nerves were re-exposed at different time intervals up to 240 days after initial operation. True neuroma formation could not be observed after laser transection. Sharp division resulted in widespread amputation neuromas consisting of regenerating axons and connective tissue. Neodym Yag laser radiation by its scattering effect may cause deep coagulation necrosis and thrombosis of vasa nervorum thus sealing the dome of the divided nerve trunk. The formation of amputation neuromas may be suppressed by laser application. [ABSTRACT FROM AUTHOR]

Giant cell tumours of the brain are commonly a) monstrocellular sarcomas, b) giant cell glioblastomas or gliosarcomas, or c) 'giant cell gliomas', i.e. gliomas characterized by many atypical, giant-sized cells with monstrous nuclei. This last group is commonly found in the temporal region and should not be mistaken for a) and b), because, in spite of their 'malignant' appearance, these tumours can present benign biological behaviour. Three such cases are described. In two cases the patients received postoperatively neither radio- nor chemotherapy: seven and three years respectively after operation no signs of recurrences were present. A third patient died within two years after operation. However, his tumour had histological signs of malignancy. The importance of exact histological diagnosis is stressed. This prevents misinterpretations in estimating the efficacy of postoperative therapy. [ABSTRACT FROM AUTHOR]

Immunoelectrophoresis of extracts of 200 intracranial tumours against rabbit anti-glioblastoma serum gave positive results (= precipitation) in all cases of tumours of neuroectodermal origin such as glioblastoma, astrocytoma, oligodendroglioma, ependymoma, neurinoma, and spongioblastoma. No immunoelectrophoretic precipitation was seen for any of the tumours of mesenchymal origin, for instance meningioma and metastases of cancer. On the basis of these findings, immunoelectrophoresis is considered to be a reliable method for differentiation between tumour tissue of neuroectodermal and non-neuroectodermal origin. Among the 41 posterior fossa tumours some unusual observations were made. Cerebellar angioblastoma (Lindau tumour) showed an atypically located precipitation line, which for the present is interpreted as an immunological reaction to vascular wall tissue. Furthermore, among the group of so-called medulloblastomas, two subgroups were distinguished on the basis of three parameters. The first of these subgroups comprises tumours whose immunoelectrophoretic pattern resembles that of gliomas, which are histologically characterized by neuroectodermal structures and which occur in younger children (5-10 years). The tumours of the second subgroup, which do not show this neuroectodermal immunoelectrophoretic pattern, have a sarcomatous character histologically, and occur in patients aged between 10 and 50 years. The view that medulloblastoma comprises a number of different types of tumour seems to be confirmed by this finding. [ABSTRACT FROM AUTHOR]

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Copyright of Der Pathologe is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Morphological investigations were carried out on brains of 274 foetuses and children with a verified chromosomal anomaly. In more than two thirds of these cases pathological changes were not detectable. In the other cases the most frequent findings were: (1) holoprosencephaly/arhinencephaly, mainly observed in trisomy 13, but also in one case each of trisomy 18 and trisomy 21; (2) corpus callosum defects, mainly found in trisomy 18, but also in some cases of trisomies 13 and 21; (3) cerebellar nerve cell heterotopias and cerebral microdysplasias, present mainly in trisomy 21, but also in trisomies 13 and 18 as well as in cases with different chromosomal syndromes; (4) small hamartomas of the brain stem were found in two cases of trisomy 18. In a control group of 85 cases with 'multiple malformations', but without a verified chromosomal anomaly, similar observations were recorded. In particular, in a subgroup of cases with ascertained normal karyotype (31 out of 85 cases) seven holoprosencephalies, four corpus callosum defects, eight cerebellar heterotopias as well as three cerebral cortical microdysplasias and three stenoses of the aqueduct were found. It thus seems reasonable to assume that structural alterations of the central nervous system (CNS) are neither constant nor obligatorily accompanying a specific chromosomal syndrome. However, their appearance follows patterns of semi-specificity. On the other hand, one has to realize that in about one third of holoprosencephalies no chromosome anomaly is present. Moreover, cerebellar nerve cell heterotopias cannot be considered as 'true' malformations, since they are very frequently found in normal newborns (mostly prematures), and disappear progressively with increasing age ('transitional microdysplasias'). [ABSTRACT FROM AUTHOR]

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Congenital intracranial tumors are very rare and only account for 0.5–1.5% of all childhood brain tumors. Even rarer are those with prenatal manifestation. The most common of these present at birth are teratomas, which show divergent differentiation with 90% of them containing tissues from all three germ layers. We report a rare case of an intrauterine congenital immature teratoma in a female fetus at 23 weeks of gestation, which was sonographically diagnosed in vivo by detection of the tumor and associated craniomegaly. Because of the poor prognosis, termination of the pregnancy was induced by Rivanol instillation. The cerebral tumor was confirmed at autopsy and was not associated with any other malformations. Histological and immunohistochemical features of this tumor are presented. [ABSTRACT FROM AUTHOR]

The neuropathological findings at autopsy in four cases of type I and three of type II lissencephaly are presented. Type I lissencephaly is characterized by agyriapachygyria with a markedly thickened cerebral cortex with four coarse histological layers. The normally myelinated white matter, often with neuronal heterotopias, is very narrow, and the gray-to-white matter ratio is inverted (about 4:1); there are no white-gray interdigitations. Claustrum and capsula extrema are absent. Ventricular dilatation is present, especially of the occipital horns. In the hypoplastic brain stem large olivary heterotopias can often be observed. Severe cerebellar malformations, obstructive hydrocephalus, severe eye abnormalities, and congenital muscular dystrophy are not seen. Clinically, type I lissencephaly presents as 'isolated lissencephaly sequence' or as 'Miller-Dieker syndrome' with characteristic facial dysmorphism. The long survival of 20 years achieved by one of our patients is very uncommon. Type II lissencephaly is characterized by widespread agyria. Usually, obstructive hydrocephalus is present with a thin cerebral mantle showing a slightly thickened cortex and a narrow, hypomyelinated white matter often with neuronal heterotopias (gray-to-white matter ratio about 1:1). The border between gray and white matter is blurred. Claustrum and capsula extrema are absent. Histologically, the cortex appears disorganized without layering; widespread leptomeningeal gliomesenchymal proliferations and glioneuronal heterotopias are present. Hydrocephalus is caused by obstruction of the subarachnoid space, aqueductal stenosis, and Dandy-Walker malformation. The latter is a typical finding in type II lissencephaly together with severe cerebellar cortical dysplasias. Aplasia of the olfactory nerves, gliotic fusion of the frontal lobes, and an occipital cephalocele may be found. Type II lissencephaly occurs in clinical syndromes with severe eye abnormalities and congenital muscular dystrophy (the so-called 'Walker-Warburg syndrome'). The congenital muscular dystrophies have no dystrophin deficiency, as we were able to show immunohistochemically in the muscle biopsies of two further patients with this syndrome. [ABSTRACT FROM AUTHOR]

In the past, contradictory results have been reported concerning the specificity of neuronal or glial cell markers. However, we have investigated this aspect in a large group of more than 550 brain tumors (among them 60 medulloblastomas). These contradictions can easily be explained by considering two basic facts. First, every neoplastic cell population, especially in embryonic tumors, diffusely infiltrates the brain tissue: non-neoplastic cells, intermingled with tumor cells, can therefore give rise to immunohistochemical and histogenetic misinterpretations. Second, different cell markers can be expressed by one and the same cell (e.g., GFAP, NSE, vimentin), making nosological interpretation of the tumor difficult, impossible, or at best rather subjective. Clear-cut marker positivity is mostly found in the differentiated tumors for which the nosological classification is already clear by the usual histological methods. Only synaptophysin seems to be a reliable marker for neurogenic cells. In embryonic brain tumors (so-called PNET), no correlations between the presence of a given cell marker and the biological behavior of the tumor have so far been detected. [ABSTRACT FROM AUTHOR]

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Copyright of Acta Neurochirurgica is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Archiv für Psychiatrie und Nervenkrankheiten is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

A retrograde study was performed in 105 patients operated upon between 1950 and 1972 for a cerebellar astrocytoma. Complete histories were available for 89 patients. Forty-five patients died within the first 3 months after operation. Of the 32 patients who were still alive, 14 patients had been operated upon 20-30 years previously and 18 patients 10-19 years previously. Twelve patients died over 3 months after the operation; in 6 cases, recurrence of tumour was the cause of death. Of the 7 patients who were irradiated postoperatively, 5 died. In 26 patients the tumor had infiltrated the brain-stem and only 7 patients survived the operation. However, 2 patients are still alive after 25 and 10 years, respectively. The findings indicate that patients operated upon for a localized cerebellar astrocytoms can be considered cured and irradiation and chemotherapy are not warranted. When the tumour has infiltrated the brain-stem, a survival period of more than 20 years is possible after partial resection. [ABSTRACT FROM AUTHOR]

The subject of this paper is a 2-year-old child with progressive paraparesis. MRI showed a large lumbosacral intradural-extramedullary mass and the histological diagnosis was Langerhans cell histiocytosis. The histopathological and neuroradiological findings are discussed. [ABSTRACT FROM AUTHOR]

Ocular symptoms are frequently observed in Walker-Warburg (WWS) and associated syndromes. The majority of patients present with malformations of the anterior segment and severe retinal dysplasia. We report on the findings in a female patient with WWS who died at the age of 9 months. Major ocular findings were: severe iridocorneal malformation, a membran-like structure of the lens and funnel-shaped retinal dysplasia. The retina presented various grades of differentiation with rosettes and atypical sequences of cells, e.g. ganglion cells intermingled between granular layers. The anterior part of the retina presented as a primitive homogeneous layer with a cell-free space that might be interpreted as the primary optic ventricle. This finding suggests that we are dealing with a primary dysplastic non-attachment rather than a real detachment of the retina in WWS. The malformation of the anterior segment was not typical of the Peters' anomaly, as usually described in WWS, but of Rieger's syndrome. [ABSTRACT FROM AUTHOR]

In 11 cases of intracranial, temporal arachnoid cysts the etiology, clinical and radiographic findings, surgical treatment, and outcome are reviewed in respect of post-traumatic subdural hematoma. Cysts of the middle cranial fossa are susceptible to trauma, which may cause bleeding either into the cyst or into the subdural space. Signs and symptoms of increasing intracranial pressure (ICP), local neurological deficits, and sometimes epileptic seizures may lead to hospitalization. CT or MRI scans are diagnostic in these cases. In cases of intracranial mass lesion with displacement of the midline structures and increasing ICP, osteoplastic craniotomy is performed and the lateral wall of the cyst is resected down to the tentorial notch by a microsurgical procedure, with opening into the basal cisterns. There were no operative or postoperative complications in 11 consecutive cases. However, one boy required a cystoperitoneal shunt 3 months later as a result of hydrocephalus following subdural hematoma. Asymptomatic arachnoid cysts are discussed with respect to brain function and social behavior. [ABSTRACT FROM AUTHOR]

Myxopapillary ependymomas are benign tumours which occasionally metastasize along cerebrospinal fluid pathways. Extraneural metastases of spinal ependymomas, however, are very rare, even more so when situated in the pleura. We report the case of a 67 year old woman presenting with shortness of breath after recurrent myxopapillary ependymomas of the cauda equina. Chest X-ray showed multiple pleural lesions diagnosed as metastases of a myxopapillary ependymoma. The MIB-1 proliferation index was 3.1% for the initial spinal tumour, 14.2% for the first and 11.2% for the second recurrence while 12.0% for the pleural metastasis. [ABSTRACT FROM AUTHOR]