Desmoplastic cerebral astrocytoma of infancy: a case report.

We report a case of desmoplastic cerebral astrocytoma of infancy (DCAI), in a 7-month-old boy. DCAI belongs to a group of recently described central nervous system (CNS) tumors, which also includes desmoplastic infantile ganglioglioma (DIG), pleomorphic xanthoastrocytoma (PXA) and dysembryoplastic neuroepithelial tumor (DNT), all characterized by relatively favorable prognosis and occurring mostly in children and young adults. DCAI is a rare neoplasm arising in the cerebral hemispheres within the first two years of life, and histologically is characterized by dense fibrous desmoplasia. In our case, CT scan presents a massive partially cystic tumor of the left cerebral hemisphere with an enlargement of the ventricular system. Histologically, the tumor was composed of cells arranged in fascicles and whorls forming storiform pattern. Immunohistochemical stainings for glial fibrillary acidic protein proved glial histogenesis of this tumor, while no cells were unequivocally immunopositive for neuron specific enolase, neurofilament proteins and synaptophysin what excludes a diagnosis of DIG--a similar entity but containing also a neuronal elements. Our studies, comprising a complete clinical, radiological, histopathological and immunohistochemical data, correspond to a cases of DCAI published before and it is the first one described in Poland.

[Two primary malignant neoplasms and recurrent neoplasms in patients treated at the Regional Cancer Center in Lodz in 1976-1989]. / Dwa nowotwory zlosliwe pierwotne i nowotwory wtórne u chorych leczonych w Regionalnym Osrodku Onkologicznym w Lodzi w latach 1976-1989.

In 1976-1989, 90, 128 patients with malignancies were recorded at the Regional Cancer Center in Lódz. Two different tumors have been diagnosed in 294 of these patients (0.31%), and the second pathologic growth corresponding to the secondary cancer criteria (occurrence within at least 6 months following radio- and/or chemotherapy) only in 148 of patients. Most frequently second tumors have been diagnosed in patients with: lip, skin, uterus and laryngeal cancers (2.8-0.8%). Relatively rare have been second tumors in patients with Hodgkin's disease and non-Hodgkin lymphomas (0.73%). The incidence of each type of the second tumors have been different than the types of neoplasma in all registered patients. Second tumors most frequently diagnosed in the same patient have been: skin cancer of different histological structure (two cases), breast cancer and tumors of the reproductive tract as well as laryngeal or lip cancer, and carcinoma of the lungs. A risk of the secondary malignancy in patients treated with radio- and chemotherapy in comparison with the risk of the second cancer in patients treated with chemotherapy only have been 2.71% and 1.36%, respectively.

Ultrastructural study of subependymal giant cell astrocytoma: unusual paracrystalloid inclusions in tumour cells.

Subependymal giant cell astrocytoma (SEGA) is a tumour with a broad range of morphological, immunohistochemical and ultrastructural neoplastic cellular features. We report here the presence of this tumor in two 7-year-old-boys presented with a lateral intraventricular mass. Further clinical investigation confirmed the diagnosis of tuberous sclerosis. Ultrastructural and immunohistochemical investigation revealed that glial differentiation was more pronounced in these cases. Interestingly, tumour cells containing unusual paracrystalline inclusions, infrequently described in SEGA, were identified ultrastructurally. These inclusions are probably not related to other cellular organelles.

Metaplastic meningioma with lipomatous changes.

We report here relatively rare lipomatous metaplastic changes, probably related to a prolonged clinical course, in not otherwise specified transitional meningioma. This lesion appears to have evolved over 30 years, with the initial diagnosis of multiple sclerosis. Histologically, it was composed of clusters and whorls of polygonal and spindle cells characteristic for transitional meningioma, with large areas of cells with lipid cytoplasmic accumulation, closely resembling mature adipocytes. The lipidization of other brain tumors and the histogenesis of these lesions in meningiomas on the background of meningeal embryogenesis are also discussed.

Analysis of 200 consecutive neuropathologically verified brain tumors of childhood.

Brain tumors are the most frequent solid neoplasms of childhood. We present here a series of 200 consecutive cases of neuropathologically verified brain tumors in children under 18, operated on between 1990-1996 at the Polish Mother Memorial Hospital in Lódz. The respective diagnoses were established on the basis of light microscopy, ultrastructure and immunohistochemistry. The criteria of the World Health Organization (WHO) classification of central nervous system (CNS) tumors were used in all but one (superficial desmoplastic cerebral astrocytoma of infancy) case. The location of tumors, age and sex of children and tumors' histology in our material were compared with those of previously published series of pediatric brain tumors.

Comparative evaluation of p53-protein expression and the PCNA and Ki-67 proliferating cell indices in human astrocytomas.

Mutations of the p53 gene are one of the most frequent genomic alterations of human tumours of astrocytic lineage. Because the physiological role of this gene is a suppression of cellular proliferation and growth, the overexpression of p53-protein may correlate with the expression of PCNA or Ki-67, established markers of cell proliferation. Paraffin-embedded surgical specimens from 60 human astrocytomas (9 pilocytic tumours, 12 WHO grade II, 9 anaplastic astrocytomas [WHO grade III] and 30 glioblastomas [WHO grade IV]) were stained with anti-PCNA (PC10), anti-p53(DO-7) and anti-Ki-67 antibodies (DAKO). Approximately 40% of all the cases were p53-protein immunopositive (53.3% glioblastomas, 33.3% anaplastic, 41.7% low grade astrocytomas but no pilocytic tumor). Statistical analysis did not reveal statistically significant correlation between p53-immunopositivity and PCNA or Ki-67 labeling indices. The Ki-67- and PCNA LI-s were statistically correlated, and the former better discriminated groups of different grades of malignancy.

Proliferating cell nuclear antigen (PCNA) and Ki-67 immunopositivity in human astrocytic tumours.

To compare the Ki-67 and Proliferating Cell Nuclear Antigen (PCNA), markers of cell proliferation, paraffin embedded surgical specimens from 56 human astrocytic tumours (8 pilocytic tumours, grade I; 9 low (II) grade and 9 anaplastic (grade III) astrocytomas and 30 glioblastomas, grade IV) were immunolabelled with the anti-PCNA (PC 10, DAKO) and anti-Ki-67 (DAKO) antibodies. For the latter immunostaining the microwave oven processing was performed. The Ki-67 and PCNA labelling indices (LIs) were statistically compared. For the majority of cases, PCNA LI was higher than that obtained with anti-Ki-67 antibody and the intensity of staining for PCNA was more variable. The statistically significant difference of the percentage of PCNA LIs was found only between low grade (I and II) and high grade (III and IV) tumours, while Ki-67 LIs discriminates each group of glial tumours; thus this latter marker is more sensitive and specific.

The immunohistochemistry and ultrastructure of ganglioglioma with chromosomal alterations: a case report.

Ganglioglioma, together with its counterparts-ganglioneuroma and gangliocytoma are relatively uncommon neoplasms of the brain composed of neoplastic neurons (ganglion and ganglioid cells) and glial cells. We report here a case of ganglioglioma studied by electron microscopy. The case was further characterized by peculiar chromosomal alterations, 46,XX[6]/43,XX[1], der(1)t(1;5)(q21;q12), der(8;13)(q10;q10),-9,i(10)(q10). Routine light microscopy revealed mixed neuro-glial tumor composed of pilocytic astrocytes with abundant Rosenthal fibers and relatively numerous ganglion cells. The latter were immunoreactive with antibodies (Abs) against synaptophysin and neurofilament protein (NFP). Anti-NFP Abs also immunostained numerous distorted axons embedded in the tumor mass. Some of these showed bullous swellings and thus were analogous to dystrophic neurites or spheroids. Ganglion cells were characterized by abundant intracytoplasmic dense-core vesicles, absence of intermediate filaments and numerous microtubules. Occasionally a close apposition of ganglion cells and Rosenthal fibers were seen. Dense-cored vesicles were pleomorphic and ranged in diameter from small synaptic vesicles to large lysosome-like neurosecretory granules. The former occasionally formed characteristic dumbbell shapes. Neoplastic astrocytes were identical to those of other glial tumors of astrocytic lineage; numerous Rosenthal fibers were frequently seen.

Clinical, radiological and histological presentations of dysembryoplastic neuroepithelial tumors (DNT). Report of two cases.

Dysembryoplastic neuroepithelial tumor (DNT) is a recently described rare brain neoplasm with characteristic clinical and morphological features and favorable prognosis. We report here two cases of DNT. The first concerned a 12 years old girl who presented complex seizures preceded by acoustic aura (melodies). Computed tomography revealed a hypodense tumor measuring 2 x 2.5 cm in diameter, located paracortically in the left temporal lobe. The second tumor was removed from a 21-year-old man with partial complex seizures. Nine years earlier patient underwent neurosurgery with partial removal of the tumor The tumor's histopathologic diagnosis is unfortunately lacking. Computed and magnetic resonance imaging showed a mass occupying the cortex and paracortical areas of the anterior pole of the temporal lobe. Histologically, both tumors consisted of small, S-100 protein immunopositive oligodendrocyte-like cells (OLCs) arranged between synaptophysin- and, to a lesser degree, NFP-immunopositive axons (glioneuronal element). In the second case, an area of pilocytic astrocytoma-like appearance was also found, these cells were immunopositive for GFAP. The present study provides clinical, radiological and histological data, which may be helpful in differential diagnosis of this newly recognised brain tumor.

Accumulation of chromosomal changes in human glioma progression. A cytogenetic study of 50 cases.

Cytogenetic studies of 50 human gliomas, including three oligodendrogliomas, 16 grade I-III astrocytomas, and 31 glioblastomas multiforme, were performed using the short-term tissue culture method. The most common numerical chromosome aberrations were +7, -9, -10, -14, and loss of a sex chromosome. Structural changes involved predominantly the following chromosome arms: 1q, 2q, 6q, 7q, 9p, 14q, 17p, and 18p. Losses of chromosomes 9, 10, and 14, often occurring simultaneously and in polyploid clones, were observed almost exclusively in high-grade gliomas, and appear to constitute important events during glioma progression.

p53 protein and epidermal growth factor receptor expression in human astrocytomas.

p53 mutations are the most frequently detected genetic alterations of gliomas, appearing in a similar proportion of low and high grade astrocytomas, while the amplification of epidermal growth factor receptor (EGFR) gene appears mainly in glioblastomas. Thus, these changes seem to delineate two subgroups of high grade astrocytomas: those originating from preexistent low grade astrocytomas and those originating de novo. Paraffin-embedded surgical specimens from 56 human astrocytomas (8 pilocytic (I.) astrocytomas, 9 low grade (II.) fibrillary astrocytomas, 9 high grade (III.) anaplastic astrocytomas and 30 glioblastomas) were analyzed immunohistochemically for the presence of p53 protein and EGFR. Approximately 41% of all cases were p53-protein-positive while 23% were EGFR-positive. Five cases (8.9%) were double-positive for p53 protein and EGFR. The p53-immunopositive nuclei were revealed in 16 cases (53.3%) of glioblastomas, 3 cases (33.3%) of high grade and 4 cases (44.4%) of low grade astrocytomas. None of pilocytic tumors was p53-positive. EGFR immunopositivity increased with the grade of malignancy (11.1%, 22.2% and 33.3%). Double EGFR-p53-positive cases occuried in similar proportions in all grades (approximately 10%) and did not show different survival rate. There were no differences between average age of patients with only-p53-positive, p53-negative (pilocytic tumors excluded) and only-EGFR-positive tumors.

Primitive neuroectodermal tumors: ultrastructural and immunohistochemical studies.

We report here ultrastructural and immunohistochemical studies of neuroblastic differentiation in the retrospective (n = 17) and prospective (n = 26) series of primitive neuroectodermal tumors (PNETs). By electron microscopy, neuritelike structures containing parallel-oriented microtubules, adhesive plaque junctions, and pleomorphic dense-core vesicles were found in the majority of tumor specimens while synaptic specializations were very rare. By immunohistochemistry, synaptophysin appeared to be the most reliable marker for neuroblastic differentiation present in the most reliable marker for neuroblastic differentiation present in the majority of tumors, while 200 kDa neurofilament protein was immunovisualized in a lower proportion of tumors. Glial fibrillary acidic protein (GFAP) was expressed in both reactive astrocytes and in a small proportion of otherwise typical neoplastic cells. We conclude that the majority of PNETs revealed diverse differentiation and that electron microscopy is still the most reliable tool for its detection followed by immunohistochemistry for synaptophysin.

Pleomorphic xanthoastrocytoma with a gangliomatous component: an immunohistochemical and ultrastructural study.

We report a case of a 24-year-old woman with left temporal pleomorphic xanthoastrocytoma (PXA) with atypical neuronal cells. Many neoplastic cells, otherwise typical of PXA, expressed glial fibrillary acidic protein, while neuronal cells with marked atypia were immunopositive for synaptophysin and neurofilament protein. This report supports a notion that PXA, like other astrocytic tumors, may have its gangliogliomatous counterpart.

Expression of synaptophysin and neurofilament protein confirms predominantly neuroblastic differentiation of primitive neuroectodermal tumors (PNET).

We report here that neuronal and glial differentiation is easily detected using synaptophysin (SF) and neurofilament protein (NFP) immunohistochemistry in primitive neuroectodermal tumors (PNET). Thus, for all practical reasons, every PNET should be regarded as PNET with "hidden" multipotential or bipotential differentiation.

Diagnosis of Alzheimer's disease with commercially available anti-beta peptide (beta A4) antibodies following microwave oven pretreatment.

Alzheimer's disease (AD), the most common presenile dementia is underdiagnosed in Poland, thus every attempt to make the frequency of this diagnosis approaching standards of Western countries should be recommended. Deposits of beta A4 amyloid in a form of amyloid (senile) plaques, diffuse amyloid deposits and congophilic angiopathy is central to the pathogenesis of AD. These amyloid deposits are virtually invisible in routine pathological stainings like HE but may be visualized with Bielschowsky silver impregnation, other metallic impregnations, and following Thioflavine S or Congo red stainings. We report here that amyloid deposits are as easily immunolabeled with commercially available antibodies against beta A4 (DAKO) and such a staining was greatly enhanced by microwave oven pretreatment. In all cases of AD, the diagnosis could be easily made using either 4GM or commercial DAKO anti-beta A4 antibodies following pretreatment with formic acid or processing in microwave oven. Pretreatment in microwave oven even for only one second was already sufficient to visualize beta A4-immunopositive plaques while after 5 second the intensity of staining approached that obtained after formic acid pretreatment.

Reactive and degenerative changes of tissues surrounding a brain tumor.

We report here immunohistochemical and ultrastructural studies of the pattern of brain degeneration being a consequence of the presence of brain tumors. Robust microglial reaction with upregulation of MHC II type antigens within and around the brain tumor were seen along with the purely degenerative phenomena like neuroaxonal dystrophy (NAD) and myelin dilatation ("ballooning"). The reaction was monotonous and independent of the histological type of the brain tumor.

Recurrent anaplastic ependymoma with an abnormal karyotype and c-myc proto-oncogene overexpression.

Cytogenetic analysis on a supratentorial, recurrent, anaplastic ependymoma from a 29-year-old female disclosed the presence of an abnormal clone with the karyotype 46,XX,der(8)t(8;11)(q24;p11),-11,add(?)t(?;11)(?;q13). By the Northern hybridization assay and immunohistochemical staining, tumor cells revealed overexpression of c-myc proto-oncogene, although no evidence of amplification or structural rearrangement of this gene was found.

Immunohistochemical and ultrastructural studies of stromal cells in hemangioblastoma.

In order to shed more light on the controversial tissue histogenesis of the stromal cells (SC), light microscopic, immunohistochemical and electron microscopic studies were performed on surgical specimens of hemangioblastomas (36, 26 and 7 cases, respectively). SC were immunoreactive for vimentin, S-100 protein, and neuron specific enolase (NSE) in all cases. Occasional SC were also positive for desmin, smooth muscle actin, Factor VIII, Ulex europaeus lectin receptors, GFAP, and Factor XIIIa. However, majority of these cells were negative with all the endothelial and smooth muscle cell markers used. Electron microscopy demonstrated several different types of SC that were reminiscent of pericytes, smooth muscle cells and abnormal endothelium as well as the intermediate forms between all the above cell types. Few SC were found lining the vascular lumina. Some SC formed small cavities reminiscent of early capillaries. However, typical Weibel-Palade bodies were not found in these SC. It is concluded that SC represents a heterogeneous population of lipidized cells, derived predominantly from the vasogenic mesenchyme. Although immunohistochemistry failed to reveal any consistent antigenic property of SC, ultrastructural findings strongly support the hypothesis that these cells are modified or abnormally differentiated endothelial cells and pericytes.

Neoplastic vascular tumors of the central nervous system.

We report here neuropathology of vascular brain neoplastic tumors. These include haemangioblastoma, haemangiopericytoma, angiosarcoma. The most recent molecular data on the histogenesis of gliosarcoma are also discussed.