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Biomedical subjects

J M Kros

Publications and source records attributed to J M Kros.

At least 55 records · Page 3Linked to original sources
PubMed1997

Hereditary frontotemporal dementia is linked to chromosome 17q21-q22: a genetic and clinicopathological study of three Dutch families.

Hereditary frontotemporal dementia (HFTD) is a rare autosomal dominant form of presenile dementia characterized by behavioral changes and reduced speech. Three multigeneration kindreds with this condition, in the Netherlands, were investigated for clinicopathological comparison and linkage analysis. Frontotemporal atrophy on computed tomographic scanning and/or magnetic resonance imaging was usually present. Single-photon emission computed tomography (SPECT) showed frontal hypoperfusion in the early phase of the disease. Brain tissue showed moderate to severe atrophy of frontal and temporal cortex with neuronal loss, gliosis, and spongiosis. Pick bodies were lacking in all cases of the 3 families. The mean age of onset varied significantly between families. We report here evidence for linkage to chromosome 17q21-q22 with a maximum lod score of 4.70 at theta = 0.05 with the marker D17S932. Recombination analysis positions the gene for HFTD in a region of approximately 5 cM between markers D17S946 and D17S791. Three other neurodegenerative disorders with a strong clinical and pathological resemblance have recently been mapped to the same chromosomal region, suggesting that a group of clinically related neurodegenerative disorders may originate from mutations in the same gene.

Chromosomes, Human, Pair 17↗
PubMed1997

Signet-ring cell oligodendroglioma--report of two cases and discussion of the differential diagnosis.

Two cases of oligodendroglioma consisting largely of signet-ring cells were analyzed histopathologically, immunohistochemically and at the ultrastructural level. The signet-ring cells were negative for a panel of tumor lineage markers including glial fibrillary acidic protein, and were negative for Ki-67 (MIB-1 immunohistochemistry). In contrast with the abundance of lysosomal structures reportedly present in the so-called eosinophilic granular cells in oligodendrogliomas, degenerating mitochondria were mainly seen in the cytoplasm of the signet-ring cells. The differential diagnosis of the oligodendroglial signet-ring cell tumors occurring in children and in adults is discussed.

Adolescent↗
PubMed1997

Detection of chromosomal changes by interphase cytogenetics in biopsies of recurrent astrocytomas and oligodendrogliomas.

Recently, lineage-specific genetic pathways of tumor progression have been suggested in both oligodendrogliomas and astrocytomas. Aberrations consistently reported in gliomas include chromosomes 1, 7, 10, 17 and 19. Identification of specific genetic damage may have important clinical consequences, because oligodendrogliomas, unlike astrocytomas, are responsive to chemotherapy. Genetic alterations specific for tumor type and tumor progression were investigated in 5 pairs of recurrent astrocytomas and 8 pairs of recurrent oligodendrogliomas by means of interphase in situ hybridization (ISH) to paraffin-embedded, formalin-fixed tissue sections. A set of DNA probes specific for the centromeric regions of chromosomes 1, 7, 10, 17, X and Y was applied. Since LOH studies on oligodendrogliomas have revealed losses in the region of 1p32-1p36, a DNA probe specific for the 1p36.3 locus was included. Hybridization with the 1p36.3 probe revealed loss of 1p in 5 of the 8 oligodendroglioma recurrences, the aberration being present in the primary tumors in 2 cases. In none of the astrocytomas was loss of 1p observed. Numerical aberrations were found in one astrocytoma pair (+7) and in the second biopsy of an oligodendroglioma (+7, -10). Aneuploidy was found by in situ hybridization in 8 of the 13 tumor pairs. Detection of aberrations in the 1p36.3 locus by interphase in situ hybridization to paraffin-embedded, formalin-fixed tumors may become a very useful tool in delineation of oligodendroglial from astrocytic genotypes, directing tumor specific therapy. The technique may be of crucial importance in tumor cases in which histologic criteria of lineage are not obvious.

Adult↗
PubMed1996

Ring chromosome 18 in a fetus with only facial anomalies.

We report on a prenatally detected case of ring chromosome 18 [46,XX,r(18)] in amniotic fluid cells of a fetus with an abnormal facial profile on ultrasound as the only malformation. The chromosome 18 origin of the ring chromosome, of a supernumerary marker chromosome in some cells, and of micronuclei was demonstrated by fluorescent in situ hybridization with a whole chromosome 18 paint (Cambio) and 18 centromere probe L1.84. DNA investigations showed deletions of 18p as well as 18q material of r(18), which turned out to be of paternal origin. Autopsy of the fetus after termination of pregnancy at 20 weeks of gestation showed no additional malformations, in agreement with the previous ultrasound findings.

Abortion, Induced↗
PubMed1996

Prognostic value of the proliferation-related antigen Ki-67 in oligodendrogliomas.

BACKGROUND: In various neoplasms expression of the proliferation-related Ki-67 antigen has successfully been linked with parameters of progression of disease. While a definite grading scheme for oligodendrogliomas has still not been agreed upon, any parameter of tumor proliferation that might be helpful in predicting clinical outcome should be evaluated. METHODS: The expression of the proliferation related nuclear antigen Ki-67 (MIB-1 monoclonal antibody) was investigated in a series of 108 verified oligodendrogliomas, and the percentages of immunoreactive cells were compared with the survival times of the patients. Using multivariate analysis the prognostic value of MIB-1 labeling index (LI) was assessed taking into account age, tumor site, grade, and mitotic index (MI). RESULTS: Patient's age, tumor site, grading according to the grading scheme by Smith, MI and MIB-1 LI were all significantly related to survival. Whereas in a multivariate analysis, age, site, grade and MIB-1 LI had independent prognostic significance, MI failed to add significant prognostic value. CONCLUSIONS: The MIB-1 LI is an important addition to histopathologic grading for predicting clinical outcome of patients with oligodendrogliomas. The MIB-1 LI adds prognostic information independent of patient's age, tumor site and grade. The MI might be incorporated into a grading scheme for oligodendrogliomas, but is not by itself an additional prognosticator.

Adult↗
PubMed1996

Proliferation of gemistocytic cells and glial fibrillary acidic protein (GFAP)-positive oligodendroglial cells in gliomas: a MIB-1/GFAP double labeling study.

Large gemistocytic cells are well-known elements of glial tumors. Recently, miniature gemistocytic cells and neoplastic glial fibrillary acidic protein (GFAP)-positive oligodendroglial cells, which are regularly seen in oligodendrogliomas, have been termed "transitional cells". The proliferative activity of the gemistocytic cell types and the GFAP-positive (gliofibrillary) oligodendrocytes was determined in eight astrocytomas, seven gemistocytic astrocytomas, eight glioblastomas, two monstrocellular glioblastomas, seven oligodendrogliomas and three mixed oligo-astrocytomas by immunohistochemical staining of the proliferation marker MIB-1 in combination with immunostaining for GFAP. Both large gemistocytic cells and the transitional cells showed cytoplasmic GFAP-positive staining. Neither in the classic gemistocytes nor in the minigemistocytes nuclear immunostaining for the MIB-1 antibody was observed. In contrast, MIB-1 staining was seen in the gliofibrillary oligodendrocytes. It is concluded that both large and miniature gemistocytic cell types contrast with gliofibrillary oligodendrocytes by their inability to proliferate.

Antigens, Nuclear↗
PubMed1994

Familial occurrence of polymorphous oligodendroglioma.

A case of familial polymorphous oligodendroglioma, occurring in a brother and sister, is presented. Polymorphous oligodendrogliomas have a characteristic histopathology consisting of scattered multinucleated giant cells against a typical oligodendroglial background. The oligodendroglial character of the tumors was underlined by positive immunostaining for antigalactocerebroside, anticarbonic anhydrase, and anti-leu-7, without expression of glial fibrillary acidic protein. Both tumors were immunopositive for p53, suggesting a mutation in the p53 gene. No incidence of cancer was recorded in the family. This is the first report of familial occurrence of this particular subtype of oligodendroglioma.

Adult↗
PubMed1994

Oligodendroglioma: the Rotterdam-Dijkzigt experience.

In a retrospective study of 82 cases of oligodendroglioma, the influences of tumor size, site, and grade, the age of the patient, the extent of surgical excision, and the effect of additional radiation therapy on the clinical course, as well as their dependencies, were investigated. Tumor grade and tumor site significantly affected the survival rates of the patients. When tumor site was kept constant by distinguishing between frontal and nonfrontal localized oligodendrogliomas, the grading system still showed discriminating power. Conversely, when controlled for grade, the frontal site appeared to be favorable for prognosis. Age was only faintly correlated with survival, with younger patients tending to survive longer. This was compatible with the finding that, in young patients, more frontal localized tumors were found and that older patients had oligodendrogliomas with higher grades. Tumor volumes did not correlate with survival. No correlation between tumor volume and tumor grade was found. Patients who had undergone a decompression tended toward longer survival, although this trend did not reach significance. No beneficial effect of radiation therapy on the survival rate was demonstrable.

Adolescent↗
PubMed1993

Expression of p53 in oligodendrogliomas.

The expression of the nuclear protein p53 in oligodendrogliomas was investigated by immunohistochemistry, using a monoclonal anti-p53 antibody (DO-7) on formalin-fixed, paraffin-embedded material in 84 histologically verified cases, and compared with the histopathological grade and survival. p53-immunoreactive cells were found in 75 per cent of the samples acquired at the first biopsy. The p53 labelling index was not related to the degree of nuclear anaplasia. Tumour cases with more than 75 per cent p53 immunostained cells had a rapidly fatal clinical course. However, no significant correlation was found between p53 labelling index and tumour grade, mitotic index, or ploidy status. In most tumour recurrences (n = 25), the p53 labelling index increased or remained at the level of the first biopsy. In five cases (6 per cent), p53 was absent in the first sample as well as in the recurrence. Irrespective of the underlying aberration of either the gene or the metabolic pathway of p53, it is concluded that a high percentage (i.e., more than 75 per cent) of p53-immunolabelled cells is predictive of an unfavourable clinical course, while a percentage lower than 75 per cent immunoreactive cells does not exclude a rapid fatal outcome.

Adult↗
PubMed1992

Prognostic relevance of DNA flow cytometry in oligodendroglioma.

In a retrospective study of 85 cases, the prognostic value of DNA flow cytometry in oligodendrogliomas was evaluated. Paraffin-embedded material was processed for flow cytometry, and the survival rates of the patients with DNA diploid, aneuploid, and tetraploid tumors were compared using analysis of variance. In addition, the mitotic index was correlated with the results of flow cytometry. Finally, the results of flow cytometry, histopathologic grading, and counting mitoses were tested for dependency. Thirty-one percent of the tumors were diploid, 39% were tetraploid, and 31% were aneuploid. The results of the DNA flow cytometry did not correlate with the survival times (P = 0.798) or with tumor degree. In contrast, the number of mitoses (P less than 0.05), and the grades of the grading system of Smith (P less than 0.003) had relevance for the prognosis. No correlation between flow cytometry, histopathologic grading, and mitotic index was found. It is concluded that flow cytometry has no value in predicting the biologic behavior of oligodendrogliomas, whereas the number of mitoses is a valuable prognostic parameter and thus is considered to be incorporated into the grading system for oligodendrogliomas.

Adolescent↗
PubMed1992

Ultrastructural characterization of transitional cells in oligodendrogliomas.

In oligodendroglial tumors the expression of glial fibrillary acidic protein (GFAP) is found in cells with an astrocytic morphology representing preexistent or neoplastic astrocytes. In addition, a proportion of the GFAP-positive cells has the morphology of miniature gemistocytes (minigemistocytes) or oligodendrocytes (gliofibrillary oligodendrocytes or GFOC). Both minigemistocytes and GFOC are considered as cells transitional between astrocytic and oligodendroglial lineage. Though minigemistocytes can readily be distinguished in routinely stained histological sections, GFAP immunostaining is obligatory for the identification of the GFOC. In the present study, the GFOC is characterized at the ultrastructural level using an immunogold-silver stain on semithin (1 micron) slides for identification of GFAP immunoreactivity and subsequent processing of the adjacent slide for immunoelectron microscopy. In analogy with the minigemistocytes, the glial filaments in the GFOC are arranged in parallel bundles. The finding of cells with ultrastructural features intermediate between those of GFOC and minigemistocytes suggests a close relationship and a possible interconvertibility between the two transitional cell types in oligodendrogliomas.

Adult↗
PubMed1991

Ultrastructural and immunohistochemical segregation of gemistocytic subsets.

Gemistocytes are frequently encountered in cases of reactive gliosis as well as in glial tumors. Recently, miniature forms of gemistocytes (minigemistocytes) were recognized as cellular constituents of oligodendrogliomas. Antibodies specific for the intermediate filaments glial fibrillary acidic protein and vimentin are reactive with gemistocytic cells, but do not react specifically with these cells. In a study of 23 glial tumors we found the monoclonal antibody Pm43 selectively reactive with the classical gemistocytes as well as with the minigemistocytes. Nevertheless, at the ultrastructural level a striking difference in the arrangement of the glial filaments between both gemistocytic cell types was found. Immunoelectron microscopy showed that the reactivity for the newly discovered gemistocytic marker Pm43 was confined to identical intermediate filaments. Despite immunohistochemical homology, a clearly different ultrastructure divides classic gemistocytes and minigemistocytes into two subsets.

Adolescent↗
PubMed1991

The pleomorphic xanthoastrocytoma and its differential diagnosis: a study of five cases.

Five brain tumors with the histopathologic features of pleomorphic xanthoastrocytomas (PXAs) are presented. Computed tomography scans showed a remarkable homology. Two cases had atypical localizations for a PXA, while one 46-year-old patient did not conform to the normal age distribution of this tumor. Nevertheless, in these cases, the histopathology was always characteristic for PXA, a remarkable pleomorphism, in addition to simultaneous expression of glial fibrillary acidic protein and histiocytic markers in the various tumor cells. In one of the presented tumors, however, clusters of neoplastic neuronal cells were also found. In this particular case, differential diagnostic criteria to distinguish between a PXA and a desmoplastic infantile ganglioglioma are lacking.

Adolescent↗
PubMed1991

Familial occurrence of tumours of the choroid plexus.

The case histories of two children of consanguineous parents with papillomas of the choroid plexus are presented. Although exogenic factors in the genesis of these neoplasms can not be excluded, autosomal recessive inheritance is proposed.

Carcinoma↗
PubMed1990

Prognostic implications of glial fibrillary acidic protein containing cell types in oligodendrogliomas.

In oligodendroglial tumors the intermediate filament glial fibrillary acidic protein (GFAP) may be expressed by cells with the morphologic characteristics of typical oligodendrocytes (gliofibrillary oligodendrocytes [GFOC]) and by miniature forms of gemistocytes (minigemistocytes) as well. These latter cell types have been regarded as transitional cells that represent intermediate forms between an oligodendroglial and an astrocytic phenotype. Furthermore, in oligodendrogliomas GFAP may be expressed by intermingled classic large gemistocytes, which are not considered transitional cells. In a retrospective study of 111 oligodendrogliomas, the presence of the various GFAP-positive cell types was correlated with the survival rates of the patients. Therefore, GFAP expression was visualized with the use of an indirect conjugated peroxidase method. The survival times of the patients were recorded and statistical comparisons were made. The percentage of GFAP-positive tumor cells is increased in oligodendrogliomas of 28 patients who underwent a second biopsy (all these patients had been treated with radiation therapy as well). It was found that neither the presence of GFOC nor that of minigemistocytes is predictive of the survival. In contrast, patients with classic gemistocytes had survival lengths approximately twice as short as those of patients who did not have these cells in their tumors. No clear correlation was found between tumor grading or any of the individual histopathologic features with the presence of the various GFAP-positive cell types. The ominous sign of the presence of gemistocytes in oligodendrogliomas confirms some earlier reports about the prognostic significance of this cell type in astrocytomas.

Adolescent↗