Medullocytoma (lipidized medulloblastoma). A cerebellar neoplasm of adults with favorable prognosis.

This study describes three cases of neuroectodermal cerebellar neoplasms occurring in adults, characterized by a monomorphic population of round cells with scanty cytoplasm and focal areas of lipid accumulation. Astrocytic and neuronal differentiation was confirmed in these cells by glial fibrillary acidic protein and synaptophysin immunoreactivity. Electron microscopy performed in two cases showed neuritic processes, synapses, and dense-core granules. Patients included two men and one woman, and the age at diagnosis was 36, 37, and 57 years, respectively. Two patients refused any postoperative treatment. One of these had two surgically removed recurrences after 10 and 11 years and died postoperatively from intracranial hemorrhage. The second had two recurrences after 10 and 15 years and is alive and in good health at the last follow-up. The third patient received postoperative radiotherapy and is alive and well after 2 years. Review of the literature revealed seven cases of cerebellar neoplasms with histological features similar to those observed in our series. These lesions have been considered a variant of medulloblastomas. The age of patients ranged from 42 to 77 years (mean age, 51 years); four were women, 3 men. Follow-up information available in two cases indicates a 5-year survival with surgery alone. These data indicate that these cerebellar neuroectodermal neoplasms have morphologically unique features and indolent biologic behavior that distinguish them from the highly aggressive medulloblastoma; the term medullocytoma for this form is suggested.

p53 protein expression in central nervous system neoplasms.

AIMS: To demonstrate, immunohistochemically, p53 protein expression in a selection of central nervous system tumours; to investigate the relation between p53 expression and that of the proliferation related antigen, PCNA. METHODS: Surgical specimens from 86 central nervous system tumours were routinely fixed, paraffin wax embedded, and immunostained with a monoclonal (PAb 1801) and a policlonal antibody (CM1) p53 protein and a monoclonal antibody against PCNA (PC10). Normal brain samples obtained at necropsy and 10 surgically obtained samples of gliotic brain parenchyma were also immunostained. RESULTS: p53 protein expression was observed in 35 of 86 brain tumours, suggesting frequent p53 gene mutation. p53 protein alterations were associated with all grades of malignancy in tumours displaying solely astrocytic differentiation, with the exception of pilocytic astrocytomas. In those showing oligodendroglial or ependymal differentiation they appeared to be restricted almost to only high grade lesions. No p53 immunoreactivity was observed in normal or gliotic brain tissue; p53 altered expression was not related to the percentage of PCNA labelled cells. CONCLUSIONS: The use of sophisticated gene amplification techniques or highly sensitive immunohistochemical methods might be useful in distinguishing between reactive and neoplastic astrocytic lesions, and in the identification of malignant progression in other non-astrocytic glial tumours. Tumours with very similar histogenetic differentiation features might actually be a genetically heterogeneous group with possible different clinical courses.

Cerebral germ cell tumor and XXY karyotype.

Metaphases from a cultured cerebral germ cell tumor (CGCT) in a boy with a 46,XY constitutional karyotype had 47 chromosomes with an additional X chromosome and a translocation (1;21)(q11;p11). CGCT appear to be nonrandomly associated with Klinefelter syndrome, and a supernumerary X chromosome and trisomy of the 1q21-->1qter region may be clonal abnormalities in these tumors. The predisposition of Klinefelter patients to develop CGCT may be due to the pathogenetic relevance of the extra X chromosome both as an acquired and a constitutional abnormality.

A peculiar case of malignant cerebral reticulosis: clinico-pathological study.

A man aged 56 years, previously healthy, developed asthenia, hypersonnia, apathy, later polydipsia and bulimia, headache and episodes of unconsciousness. There was temporary improvement with steriod therapy, but ever-deepening stupor appeared till death due to bronchopneumonia. All blood chemistry examinations were normal. The CSF IgG was elevated but no neoplastic cells were seen. At autopsy areas of grayish color in the basal gagnlia and granulomatous tissue in the floor of the third ventricle and in the mamillary bodies were seen, and the gonads were attrophied. Microscopically, in the floor of third ventricle, mamillary bodies and adjacent leptomeninges there was granulomatous tissue made up of more-or-less typical cells of the reticulum, polymorphonuclear cells, plasma cells and some phagocytes along with proliferation of small blood vessels and reticulin fibers. In addition, the white matter of the frontal lobes, pons, middle cerebellar peduncle and cerebellar white matter contained diffuse proliferation of pleomorphic histiocytic elements with questionable atypical mitoses. Notwithstanding, the morphology of our case suggests that it is a peculiar form of malignant reticulosis (or malignant histocytosis) related to histiocytosis X. The duplicity of the features of our case suggests it to be neoplastic, where the proliferative phase is followed by a granulomatous and sclerotic one.

Long-term follow-up of germinoma after stereotactic biopsy and brain radiotherapy: a cell kinetics study.

The primary aim of this study is to report the long-term outcome of pineal and suprasellar germinoma after stereotactic biopsy and whole brain radiotherapy. The second purpose is to report an investigation of the biological features and cell kinetics of this peculiar and enigmatic brain tumour. Of 34 supratentorial germ cell tumours diagnosed and treated between 1980 and 1993, 20 patients were found to be affected by true germinoma localized in the pineal and/or suprasellar regions. The diagnosis was achieved by stereotactic biopsy in all cases. In 14 patients, the potential proliferative activity of the tumour was investigated by (3H)thymidine in vitro binding and labelling index determination. Chorionic gonadotropin, alpha-fetoprotein and embryonal carcinoma antigen were negative in the cerebrospinal fluid of these patients. All but 1 patient underwent whole brain radiotherapy. Clinical and neuroradiological follow-up ranged between 3 and 13 years (mean 8). Complete clinical and neuroradiological recovery was achieved in all patients after treatment. Fatal recurrences owing to neuraxis dissemination occurred in three cases. The labelling index in the whole series ranged between 0.1 and 5% (median 2.5). Only syncytiotrophoblastic cells had proliferative activity, while none of the lymphoid-like cells showed thymidine labelling.

A case of glioblastoma with multiple centers above and below the tentorium.

A case of multicentric glioblastoma with the clinical and histopathological findings is presented in which three lesions are located above and below the tnetorium. Multicentric glioblastomas are those which have no macroscopic or microscopic connection.

Chemotherapy as first treatment for primary malignant non-Hodgkin's lymphoma of the central nervous system preliminary data.

Non-Hodgkin's lymphoma of the central nervous system (NHL-CNS) is thought to account for about 1% of primary brain tumours. Radiation therapy has mainly been applied to treat cerebral lymphoma, but the low cure rate and the lack of enduring response have stimulated the search for alternatives. With the aim of postponing radiotherapy as long as possible, we tested the efficacy of a M-BACOD schedule administered immediately after histological diagnosis in 14 patients. After two M-BACOD courses 10 (71%) patients displayed an objective response (i.e. were apparently tumour-free when examined by CT). In 6 (60%) M-BACOD-responsive patients, radiotherapy was delayed for 5 months (without recurrences after a follow-up ranging from 9 to 18 months). Moreover, in 3 M-BACOD-responsive patients, no recurrence took place (even without radiotherapy) after a follow-up of 6-12 months. We conclude that radiation can be postponed after chemotherapy or delayed until tumor recurrence.

Oculomotor disturbances in Balint's syndrome: anatomoclinical findings and electrooculographic analysis in a case.

A 67-year-old man had repeated cerebral ischemic attacks, which resulted in a clinical picture combining paralysis of visual fixation, optic ataxia and impairment of visuospatial orientation, consistent with the definition of Balint's syndrome. Postmortem examination showed multiple lesions involving the occipital cortex of both sides and the white matter underlying the right insular cortex. EOG recording demonstrated a marked impairment of refixation saccades and saccades on verbal command. Smooth pursuit movements were completely abolished. Visual fixation was randomly achieved after many erratic exploratory movements and steadily maintained on the target (spasmodic fixation). During spasmodic fixation, EOG recording detected an ocular flicker resulting in a continuous instability of eye position. It is suggested that these findings may all be accounted for by the loss of panoramic vision due to a bilateral impairment of cortical areas 18 and 19.

Low-grade glial tumors in basal ganglia and thalamus: natural history and biological reappraisal.

The natural history of 70 patients affected by low-grade astrocytomas was recorded after the histological diagnosis was obtained by serial stereotactic biopsy. Forty-three percent of these patients died within 3 years. The value of cell kinetics assessment at the time of stereotactic biopsy was investigated, and the labeling index percent may be considered the most accurate prognostic factor in these histologically homogeneous astrocytomas. It has been confirmed that the young age of patients predicts a more favorable course, but the value of this also seems to be linked to and dependent on cell kinetics. These data are discussed in view of the opportunity to perform more aggressive "cytoreductive" treatments in deep brain tumors when these indices support an expected poor prognosis.

Cerebral multiple glio-vascular dysplasias--a case report.

We describe the clinical and neuropathologic features of a patient complaining of increased intracranial pressure lasting over two years. Brain CT showed three cystic lesions in the supratentorial regions. Autopsy disclosed multiple small glio vascular abnormalities, in some instances connected with a cystic cavity. The lesions were scattered throughout the white matter of both cerebral hemispheres and suggested a complex dysplasia.

Sialidosis type I: pathological study in an adult.

Histological and ultrastructural findings observed throughout the nervous system and the extranervous organs in a case of sialidosis type I, also known as normosomatic group, are reported. The patient was a 22-year-old male with non-familial progressive myoclonus, macular cherry-red spot, moderate cerebellar syndrome and normal intelligence. Biochemical study showed an alpha-N-acetylneuraminidase deficiency in cultured fibroblasts. A complete and early autopsy was performed. Neuropathological study showed two prominent lesions: the first one was a fine cytoplasmatic vacuolation in several neurons of the cortex, basal ganglia and thalamus and the second one was a diffuse neuronal intracytoplasmic storage of lipofuscin-like pigment (LLP). As for the extranervous organs the main light and electron microscope findings were observed in the hepatocytes and in the Kupffer's cells, which showed an enlarged cytoplasm and lipopigment granules in different amount. Vacuoles containing dense lamellar bodies were found in tubular epithelial cells of the kidney. To our knowledge this is the first complete autoptic study of a case of sialidosis type I.

Cyto-histological diagnosis on wet smears of neurosurgical biopsies.

Since 1930 the supravital staining of thin smears of small specimens from brain tumors taken at operation has been considered useful. In more recent time this technique has become mandatory in multiple serial stereotactic biopsies because it give consistent support in "real time" to this procedure. Many important features can be considered in the bioptic fragment, such as: morphology, cellularity, specific microscopical structures, regressive processes, necrotic tissues, neoangiogenesis and mixed cellular population. But, for the correct interpretation of these data, a close collaboration among the neurosurgeon, the neuroradiologist and the cytopathologist must be guaranteed.

Predictive accuracy of cell kinetics data in glial tumors investigated by serial stereotactic biopsy.

The prognostic accuracy of cell kinetics investigations in patients affected from glial tumors, submitted to serial stereotactic biopsy is reported. The methodology is described and the results obtained in 103 patients are discussed with particular regard to mature and anaplastic astrocytoma series. In conclusion the value of the procedure has been clearly demonstrated in glial tumors different form glioblastoma.

Familial frontal lobe oligodendroglioma. Case report.

Two cases of frontal bilateral oligodendroglioma invading the corpus callosum occurred in a 56-year old man and his 32-year old son. CT images of both patients are presented.

Meningo-cortical calcifying angiomatosis and celiac disease.

A woman with ophthalmic migraine was found to have bilateral cerebellar and cerebral calcifications. She progressively developed severe intracranial hypertension, with swelling of the brain and downward transtentorial and tonsillar herniation. Because steroid treatment was ineffective, the right occipital pole was resected. Histological study demonstrated meningo-cortical calcifying angiomatosis. Within 2 months, brain swelling and papilledema disappeared. Subtle signs of malabsorption led to the hypothesis of celiac disease, confirmed by jejunal biopsy. Similar cerebral histological findings have been reported in the brain of two young patients affected by epilepsy and celiac disease. The association between cerebral calcifications and celiac disease is peculiar; the pathogenetic relationship is unknown.

[Generalised triglyceride lipidosis (triglyceridosis). 1st anatomo-clinical report]. / Lipidose généralisée a triglycérides (triglycéridose). Première contribution anatomo-clinique

The case reported, known under the name of Lipid Storage Myopathy, occurred in a twenty year old woman. The first symptoms occurred between the age of 14 and 16 years. A complete autopsy was carried out. The entire musculature was involved. There was fatty infiltration of the myocardium as well as marked fatty degeneration of the liver and kidneys. Histological and histochemical examination revealed an accumulation of triglycerides, distributed throughout almost all the organs, this being confirmed by chemical examinations. The latter did not, however, show any changes in phospho-glyco-sulpho-lipid, cholesterol or cholesterol content. Gas chromatography of the total free fatty acids revealed an increase in short chain fatty acids. The authors stress the generalized nature of the pathological process leading to the accumulation of triglycerides, in a disease which up to present time had been considered to be a myopathy and therefore propose the name Generalised Triglyceridosis of Generalised Triglyceride Lipidosis.

The hypodense meningioma: report of two cases.

Cystic or necrotic hypodense areas are occasionally seen within meningiomas on CT scans. Two diffusely hypodense endotheliomatous meningiomas are reported. The low density was due to a microcystic structure, which caused a spongy appearance and consistency.

Cerebromeningeal pleomorphic xanthoastrocytoma. Report on four cases: clinical, radiologic and pathological features. (Including a case with malignant evolution).

Three cases of cerebromeningeal pleomorphic xanthoastrocytoma are presented and the recurrence of a fourth case, previously described in 1980, is examined because of its evolution towards malignancy. Clinical, radiologic and pathological aspects are discussed and the general features of the previously reported cases of xanthoastrocytoma are reviewed.

Intracranial dissemination of pituitary adenoma. Case report and review of the literature.

Leptomeningeal dissemination of pituitary adenoma is a very rare occurrence. The present report describes the case of a 28 year old man with a nonfunctioning pituitary adenoma which was operated on and irradiated. Eight years later, the patient developed Cushing's syndrome and multiple leptomeningeal masses were revealed by brain CT and MNR. The diagnosis was ACTH-cell adenoma, without malignant histological signs. The growth fraction of the tumor, detected by means of the immunohistochemical demonstration of proliferating cell nuclear antigen (PCNA), was 5.45% of cells; this figure is higher than that reported for non-recurrent pituitary adenomas. From a review of the reported cases, the possibility of predicting late malignant behaviour is discussed. The microscopic aspect has no prognostic value, since metastasizing cases are not overtly malignant in a histological or cytological sense. The application of methods aimed at detecting the growth fraction of the tumor may prove useful in the early identification of aggressive pituitary tumors.