[On the small hyperchromatic nuclei of medulloblastomas].
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Biomedical subjects
Publications and source records attributed to D Schiffer.
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A case of acute muscle necrosis with probable myoglobinuria is presented. The patient had been taking excessive doses of phenformin and fenfluramine, and a toxic etiology is suggested. Muscle necrosis is tentatively attributed either to the combined effect of these drugs on some underlying biochemical abnormality of muscle or to a defect of oxygen utilization secondary to myoglobin alterations.
The immunohistochemical distribution of glial fibrillary acidic protein (GFAP) in neoplastic lesions induced in the rat by ENU is reported. GFAP was present in hypertrophic reactive astrocytes, which were numerous in early neoplastic proliferations, in microtumors of the white matter, and in those collected at the periphery of large tumors. They were absent in cortical oligodendroglial foci and microtumors. No GFAP-positive cells were observed in hyperplasias of the white matter: astrocyte-like cells of large tumors were GFAP-negative. The significance of reactive astrocytes and the problem of the astrocytic component in transplacental ENU tumors are discussed.
We performed a population based survival analysis of all incident cases (220) of motor neuron disease (MND) in the province of Turin, Italy, during the period 1966-1980. 175 cases were diagnosed as amyotrophic lateral sclerosis (ALS), 43 as progressive muscular atrophy (PMA) and 2 as progressive bulbar palsy (PBP). The life-tables of MND, adjusted as to the "expected" mortality, showed a survival rate of 27.8% and 22.0% at 5 and 10 years, respectively. The course of PMA and ALS cases was different, with a 5-year survival rate of 66.8% and 17.7%, respectively. Nevertheless both life-tables showed a similar pattern with a rapidly fatal outcome in the first 3 years after diagnosis and a slower death rate in the following years. In each curve, the slopes suggested the presence of 2 subgroups with different prognosis. It is to be stressed that a percentage of PMA patients (25.7%) showed a rapidly fatal outcome and that a subgroup of ALS patients (26.6%) showed a relatively benign course. This might suggest a different individual susceptibility to the disease.
Open biopsy was performed in right quadriceps muscle (vastus lateralis) in 30 healthy female volunteers between 20 and 50 years of age. Histometric analysis was carried out on ATPase stained sections preincubated at pH 4.5. Type 1, 2a, 2b, and 2 fibres were quantified taking into account the following parameters: percentage of fibre types, mean diameter, atrophy and hypertrophy factor, variability coefficient of mean diameter and mean diameter ratio of type 1: type 2 fibres. Results showed that there is a large variation in fibre type percentage, mean diameter and hypertrophy factor, and consequently ranges of normal values are wider than those previously reported. Atrophy factor and variability coefficient are the least variable parameters. The importance of normal controls is stressed to avoid false positives in histometric evaluation of muscle biopsy with minimal changes.
In this case report we describe the development in the cerebellopontine angle of a very rare tumor, ceruminous adenoma. In the few cases described in the literature this tumor occurred in the external acoustic meatus. In four cases it developed in the cerebellopontine angle by infiltration of the petrous bone or by subcutaneous spread. In the present case no connection was found between the cerebellopontine angle and the external acoustic meatus. The most likely pathogenetic hypothesis in this case is that of a tumor of dysembryogenetic origin.
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AIMS AND BACKGROUND: Medulloblastoma in adults is a rare tumor. The small number of cases in the reported series has not permitted a definite assessment of the prognostic role of clinical, pathologic and cell kinetics factors. The largest series of medulloblastoma in adults treated in a single institution is herein reported. METHODS: The clinical, therapeutic, pathologic and proliferation features of medulloblastoma in 44 adult patients (> 18 years) were analyzed retrospectively with regard to postoperative survival. The proliferation potential of each tumor was evaluated by the immunohistochemical demonstration of proliferating cell nuclear antigen (PCNA) and Ki-67, clone MIB-1, in paraffin sections. RESULTS: The overall 5- and 10-year survival rates were 40% and 35.6%, respectively. Significant factors in predicting a longer postoperative survival were: age < 37 years, decade of management (1977-1990), radiotherapy (50-55 Gy on the posterior fossa and 30-35 Gy on the spinal cord) and nuclear isomorphism. When corrected for adequacy of radiotreatment, desmoplastic type and differentiation were significantly correlated with a shorter survival. The PCNA-labelling index (LI) ranged from 34.5 to 82.2%, the MIB-1-LI ranged from 9.6 to 64.7%. No association was found between PCNA- or MIB-1-LI values and microscopic features, or between LI values and prognosis. CONCLUSIONS: Contrary to a general assumption, desmoplastic medulloblastoma and differentiated medulloblastoma are negative prognostic factors in adequately radiotreated adult patients. This may possibly be referred to lower radiosensitivity of these tumor variants. The LI with PCNA or Ki-67 is of no help in identifying aggressive tumors.
Twenty-one malignant cerebral tumors treated with chemotherapy after operation or clinical diagnosis were studied. The brains were cut in coronal slices that were embedded in paraffin. The histological picture of the whole tumor and of the adjacent normal tissue was carefully investigated, and it was compared with the histological features noted on biopsy. The general characteristics of glioblastoma, including wide central necrosis, were present in all of the cases. No specific alterations referable to chemotherapy were found. The increase of monstrous cells and the decrease of mitoses, although observable in some areas, were not of general importance. There was a statistical relationship between the occurrence of reactive astrocytes with bizarre nuclei and repeated chemotherapy or length of time after radiotherapy.
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