Peripheral primitive neuroectodermal tumor within the spinal epidural space.

A case of an epidural spinal peripheral primitive neuroectodermal tumor (pPNET) in a 13-year-old girl is presented. The tumor was disseminated at the moment of diagnosis, thus only diagnostic oligobiopsy of the epidural mass was performed. Histologically the tumor was composed of small round blue cells. The neoplastic cells expressed MIC2 and features of neural differentiation on immunohistochemical staining (neuron-specific enolase, synaptophysin and NCAM positivity). Fluorescent in situ hybridization (FISH) analysis was performed for the final diagnosis and the translocation t(11;22)(q24;q12) was detected. The present case emphasizes the usefulness of FISH in differential diagnosis of tumors, especially when only routinely fixed material is available.

Meningioangiomatosis with a predominant fibrocalcifying component.

A case of meningioangiomatosis, resected from the parietal lobe in a 31-year-old female is presented. Macroscopically, the lesion was composed of five calcified nodules embedded within hardened elastic tissue. Histologically, cortical and subcortical calcified masses were found surrounded by a palisade of spindle and/or oval cells. In adjacent nervous tissue many pathological microvessels were observed and some were ensheathed by a perivascular proliferation of spindle cells. Moreover, gliosis with Rosenthal fibers and prominent connective tissue elements were observed. Immunohistochemical analysis based on monoclonal antibodies was performed. The spindle cells both within the palisades and the perivascular proliferations were vimentin and usually epithelial membrane antigenpositive. The possible pathogenesis of meningioangiomatosis is discussed and the role of angiogenesis within this lesion emphasized.

Vasculopathy and amyloid beta reactivity in brains of patients with acquired immune deficiency (AIDS).

The authors examined multiple brain sections from 15 autopsy cases of AIDS for the vascular changes, presence of amyloid plaques and signs of axonal damage. The mean patients age was 33.8 years (range 24-48 years). Neuropathological findings included: HIV-specific changes (5), opportunistic infections (7), lymphoma (1) and two cases with nonspecific changes. All sections were stained with hematoxylin-eosin (H-E), selected sections were stained with Masson trichrome, Gomori reticulin, Congo red and thioflavine S method. Two sections from each case were immunostained for the presence of beta-amyloid (4G8). ubiquitin, alpha-smooth muscle actin and CD31. The different forms of vascular changes were found in all cases. The common changes were: lymphocytic perivascular or transmural infiltrations and hyalinization, thickening or fibrosis of the arterial and arteriolar wall. The perivascular space widening up to status lacunaris was a frequent phenomenon in the basal ganglia and deep white matter. Some of the cortical arterioles formed little multiluminal structures. Immunohistochemical examination revealed features of hypertrophy of the vascular muscular layer and signs of the slight endothelial cells proliferation. In three cases 4G8 immunopositive. Congo red and thioflavine S-negative, diffuse beta-amyloid deposits were present in the gray matter, focally their localization was perivascular. Ubiquitin immunoreactivity presented as numerous dot-like structures or focal bundles of positive widened axons in the white matter, spheroids and scattered positive neurons were also found. The authors suggest that some of morphological changes within the brain and consecutive neuropsychological symptoms in AIDS are of the vascular origin. Presence of amyloid plaques and axonal damage are the elements of the complex degenerative and inflammatory process in the brain caused by chronic inflammatory stimulation in HIV infection.

The occurrence of cerebellar hemangioblastoma in numerous first degree relatives with von Hippel-Lindau disease.

Von Hippel-Lindau disease is an autosomal dominant disorder caused by a mutation of VHL gene. The incidence of the disease is one in 36,000 and its clinical manifestation is a familial occurrence of hemangioblastoma of the central nervous system and retina, renal cell cancer and pheochromocytoma. Cerebellar hemangioblastoma is the most frequent or sometimes the only abnormality observed in this syndrome. We present a family with von Hippel-Lindau disease in which four first degree relatives had a cerebellar hemangioblastoma. This neoplasm caused the death of two brothers aged 27 and 24 years old, respectively and their mother aged 62. The third son of this family was affected ten years ago, at the age of 30. The healthy family members are counselled in Oncological Genetic Outpatient Unit in Gdansk.

Recurrent meningiomas--the immunohistochemical analysis of angiogenesis and cellular proliferation. Preliminary study.

Meningiomas exhibit a tendency to the local regrowth after their surgical resection, and some of those recurrent tumors show histological malignancy progression. The present study was performed on 10 cases of recurrent meningiomas chosen out of total 122 meningiomas diagnosed in our Department of Pathomorphology in the period 1993-1998. The tissue material from both operations was examined. One patient was operated three times. In three cases at the second operation there was progression from benign to atypical meningioma. The other tumors did not change their histological grade (four benign, one atypical and two anaplastic). The tumor tissue section were stained immunohistochemically with monoclonal antibodies raised against epithelial membrane antigen (EMA), Ki-67 and von Willebrand factor (vWf). The percentage of EMA-positive cells and Ki-67 immunoreactive cells (proliferation cell index--PCI) were counted. The vascular density (number of blood vessels/mm2) was assessed in the preparations stained with anti-vWf, as the measure of the intensity of angiogenesis. The values of the examined parameters were greatly differentiated within both the initial and the recurrent tumor groups. The values of vascular density ranged 12-96 vessels/mm2, the percentage of EMA-positive cells was 0.75%, PCI was from 0.3 to 9.3%. The values of the examined parameters did not differ significantly between initial and recurrent meningiomas groups. It is however worth to point out that PCI in the most recurrent tumors were higher than in their primary counterparts.

The metastases of lung cancer to the brain--the examination of angiogenesis and p53 expression.

The aim of the present study was to investigate the intensity of angiogenesis and p53 protein expression in metastases of lung cancer to the brain. There were eight cases of squamous cell type and nine adenocarcinomas among 17 examined cases of metastatic carcinomas. The antibodies against von Willebrand factor (vWF)--to highlight the microvessels and against p53 protein--for detection of immunopositive cells were used. The intensity of angiogenesis was represented by the mean number of the blood vessels in three tumor fields with the highest microvascular density ("hot spots"). The measurements were taken in three microscopic fields under 200x magnification (the examined area was 0.785 mm2). The mean number of p53-positive cells in three tumor areas under 200x magnification with the highest number of p53-positive cells was the measure of protein p53 expression. The values of vascular density and p53 expression differed a lot among the examined tumors. The values of vascular density were between 4.2-106 vessels/mm2 (mean value 49.3 vessel/mm2). The number of p53-immunopositive cells was between 0-284 cells/mm2 (mean value 110.6 cells/mm2). There was no significant correlation between examined parameters (correlation coefficient 0.18).

Cytogenetic and proliferative potentials in meningiomas.

Cytogenetic analysis and Ki-67 staining index (SI) were performed on the series of 51 meningiomas, to estimate the relationship between the extent of chromosomal changes and the growth potential of tumors. The tumors were classified according to histological subtype (22 meningiotheliomatous, 15 transitional, 12 fibroblastic and 2 angiomatous) and grade (40 benign, 5 atypical and 6 malignant ones). There was no significant difference in the complexity of chromosomal changes among the histologically distinct tumor subtypes. In contrast, there was a significant association between the number of chromosomal abnormalities and tumor grade. Normal karyotypes were found in 50% and complex in 20% of benign tumors. All grade II or III tumors revealed complex karotype. The tumors classified as benign revealed significantly lower mean Ki-67 SI than atypical or malignant ones (1.6%, 7.4% and 14.7%, respectively). However, within tumors classified as benign, mean Ki-67 SI of these with normal or simple karyotypic changes did not differ significantly from those with complex karyotype (1.6% and 0.9%, respectively). Thus, the extent of genome abnormalities in meningiomas, measured on the chromosomal level, does not relate directly to their proliferative potential.

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.

Antiphospholipid syndrome in systemic lupus erythematosus--immunomorphological study of the central nervous system; case report.

We present a case of a 44-year-old female with systemic lupus erythematosus (SLE) with secondary antiphospholipid syndrome (APS). The patient died in the 15th year of the disease. The paraffin sections of the brain and spinal cord were examined using routine histological methods and immunohistochemistry with monoclonal antibodies against CD31, FVIIIAg and polyclonal antibodies IgG, fibrinogen and C3. Numerous thrombi-recent, organized and recanalized and focal vasculitis were seen. The immunopositive reaction in thickened vessel walls for CD31 and FVIIIAg indicated the process of the local incorporation of the thrombi. The immunoreactivity for fibrinogen, IgG and C3 suggests the immunological complexes formation in vessel walls, perivascular astrocytes and neurons.

Metastatic carcinoid tumor in the brain.

The case presented describes a metastatic carcinoid in the cerebral hemisphere of a 52-year-old woman. The onset of the disease was in the form of stroke, CT-examination showed hemorrhagic focus and edema. A craniotomy was carried out and the lesion was removed. Histological diagnosis stated that the tumor was a carcinoid accompanied by a massive hemorrhage. Two and a half months later, another CT-examination revealed a second tumor in proximity to the site of operation, which was then treated palliatively by means of Co 60 radiation. The patient died 1.5 months later. General autopsy showed two other neoplastic tumors: a smaller one in the left lung and a bigger one (possible primary) in the stomach. In the report special attention was paid to an unusual cellular polymorphism especially prominent in the tumor formerly irradiated.

Cytogenetic findings in two synovial sarcomas.

Cytogenetic analysis was performed after short-term tissue culture of two recurrent synovial sarcomas. The tumors were classified on the basis of morphology, location, and immunohistochemistry. In a poorly differentiated tumor, the karyotype 49,XY, +7, +8, +19,t(5:18) (q11.2;q11.2), and in a biphasic tumor two clonal cell lines with common translocations t(X;18)(p11.2;q11.2) and t(12;17)(p11.2;q11.2) were present. In the predominant cell line several other structural aberrations including t(1;12)(q21;q24.3), t(3;18)(p23;q21), and 17p+ were found. A comparison of our results with previously published studies suggests that in addition to t(X;18), translocations of chromosome 18 with other chromosomes may represent a consistent feature of chromosomal changes in synovial sarcoma.

Immunological and immunohistochemical studies in brain tumours.

The authors have studied different brain tumours with immunohistochemical technique using fluorescence and peroxidase methods. On frozen sections reactions for fibronectin (FN), prostaglandin E2 (PGE2), carcinoembryonic antigen (CEA) with polyclonal antibodies and for lymphocyte subpopulations B, NK and Pan-T with monoclonal antibodies have been investigated. Both primary and metastatic tumours contained fibronectin in various intensity and showed increased reactions with PGE2 antibodies comparing with surrounding tissues. A well developed FN positive fibrillary network in tumour tissue confirmed the presence of angiogenesis and visualised abnormalities of the vessel walls with focal disruptions. The intracytoplasmatic location of PGE2, more intense than in surrounding tissues revealed in this study, may indicate that tumour cells are capable of its production. Since prostaglandin inhibits lymphocyte cytotoxicity against tumour cells in vitro, this may be suggestive for its activity in tumour defence reaction. CEA was not found in primary brain tumours by immunohistochemical procedure, either fluorescence or peroxidase method. It has been detected only in metastatic carcinomatous tumours in moderate intensity. Immunostaining for subpopulations of lymphocytes in tumour slices with monoclonal antibodies showed only small number of cells, in some specimens. The reasons for this failure are discussed. The meaning of all these findings mentioned above for clinical purposes is considered. The application of immunohistochemical staining may be useful in diagnosing tumour of vague or doubtful microscopic appearances.

[Basilar artery thrombosis. Clinical and morphological aspects]. / Zakrzep tetnicy podstawnej mózgu. Obraz kliniczny i morfologiczny.

On the basis of clinical analysis and pathological findings in 25 patients dying after occlusion of the basilar artery the authors describe the main signs and course of the disease. Their observations made possible isolation of two types of the disease: 1) acute, with sudden consciousness disturbances leading to gradually increasing coma with flaccid extremities or decerebrate rigidity, stiff pupils not reacting to light and, frequently, with divergent squint, 2) subacute--with headache and hemiparaesthesiae and later quadriparesis and paresis of cranial nerves III to VII or IX, X, XII. A characteristic clinical feature is variability of neurological signs in the disease. The most frequent cause of basilar artery occlusion were atherosclerotic changes.