A report of supratentorial leptomeningeal hemangioblastoma and a literature review.

Hemangioblastomas of the CNS are solid or cystic vascular-rich tumors, most common in the cerebellum, less frequent in the brainstem or spinal cord, and rare in supratentorial locations with meningeal involvement. We document a case in a 58-year-old man who presented with about 2 months history of motor weakness and speech dysfunction. The tumor was a heterogeneously enhanced dural-based tumor with high vascularity and perifocal edema in the left frontal lobe. The tumor was completely removed followed by embolization and preoperative radiotherapy. Histologic examination revealed a hemangioblastoma with features resembling angiomatous meningioma. Immunohistochemistry for epithelial membrane antigen (EMA) and S100 may be helpful to make differential diagnosis. Electron microscopic investigation is essential to differentiate between meningiomas and other leptomeningeal tumors.

Supratentorial cortical ependymoma: report of three cases.

OBJECTIVE AND IMPORTANCE: Extraventricular ependymomas account for 50% of supratentorial ependymomas. Some tumors may extend to the gray matter reaching the pial surface, but pure cortical ependymomas are uncommon. Here, we report three patients with supratentorial intracortical ependymoma. CLINICAL PRESENTATION: We reviewed the clinicopathological findings of all patients operated on for ependymomas at the Bellaria Hospital during an 11-year period and found three lesions described as cortical ependymomas. The three lesions represented 2.5% of all ependymal tumors and 21.4% of supratentorial tumors operated on during the study period. The patient were aged 52, 24, and 11 years (mean, 32.3 yr). One was female. All presented with seizures. On imaging, the lesions were confined to the gray matter, were solid, and demonstrated diffuse enhancement. INTERVENTION: Gross total resection was achieved in all instances. Two patients were treated with surgery, and one was treated with surgery and postoperative radiotherapy. All tumors were low grade. After a mean follow-up of 92.6 months, no patient had recurrence or leptomeningeal dissemination. Review of preoperative magnetic resonance imaging scans confirmed an intracortical location. Routine sections were reviewed, and additional immunoreactions for epithelial membrane antigen, glial fibrillary acidic protein, synaptophysin, neurofilament proteins, S-100 protein, and Ki-67 and electron microscopy were performed. CONCLUSION: Cortical ependymomas seem to behave as benign tumors amenable to surgical removal. Local recurrence and leptomeningeal dissemination seem to be unlikely. Postoperative radiotherapy is unnecessary.

Atypical neuronal-glial tumors of cerebral hemispheres in adults with PNET-like component: clinicopathological features of 5 cases.

We investigated 5 cases with brain tumors composed ofneuronal and astrocytic differentiated tumor cells occurring in the cerebral hemispheres of adults. Patients ranged from 33 - 69 years of age, 3 females and 2 males. Radiologically, contrast enhancement was demonstrated in these tumors. All tumors were surgically resected following radiotherapy and chemotherapy. Four patients have been free of recurrence for 2-5 years. One recurred 15 years after the operation. Histologically, tumor cells were mainly composed of round or oval nucleate cells with scant cytoplasm and compactly arranged with neurocytic features. Immunohistochemically, some tumor cells were immunoreactive for synaptophysin, neurofilament, beta-tubulin, chromogranin A, GFAP and vimentin. There were little immunoreactive cells for myelin basic protein and epithelial membrane antigen. Ultrastructurally, tumor cells were variably differentiated as follows: undifferentiated cells having prominent nuclei and scanty cytoplasm with inconspicuous organelles; neuronal cells consisting of neurosecretory granules or vesicles and abortive synapses, and astrocytic cells with cytoplasmic intermediate filaments. The Ki-67 labeling index ranged from 4.5 - 9.8%. Allelic loss of chromosome Ip occurred in 2 cases (50%) and allelic loss of chromosome 19q occurred in 2 cases (50%) of 4 informative cases. These tumors were characterized as neuronal and astrocytic differentiated tumors with primitive PNET-like component. However, there was little oligodendrocytic or ependymal differentiation in these tumors.

Supratentorial giant cell ependymoma.

Ependymomas account for 3 to 9% of all neuroepithelial tumors and, although occurring most often within the ventricular system, they may arise from the extraventricular parenchyma as well. Several histologic patterns of these neoplasm are well known, but little attention has been devoted to a variant composed of giant elements. We describe the case of a 13-year-old girl experiencing a 3-month history of partial seizures in whom cranial magnetic resonance imaging showed an extraventricular, right parietotemporal neoplasm, adherent to the overlying dura mater. Histologic, immunohistochemical and ultrastructural findings were consistent with those of a high-grade ependymoma. The tumor was characterized by the presence of a major component of pleomorphic giant cells, as also seen in pleomorphic xantoastrocytoma, subependymal giant cell astrocytoma and glioblastoma multiforme. Similar elements have been described in two filum terminale and one supratentorial, intraventricular ependymoma, respectively. Histologic and evolutional data of those and of our own case suggest that isolated giant cells are not necessarily linked to a bad prognosis in ependymomas.

Clinical application of three-dimensional anisotropy contrast magnetic resonance axonography. Technical note.

The utility of three-dimensional anisotropy contrast (3DAC) magnetic resonance (MR) axonography, a method sensitive to neuronal fibers and their directionality, was investigated in the clinical setting using a 3-tesla MR imaging system based on a General Electric Signa platform. The study focused on healthy volunteers and patients with common structural central nervous system disorders, namely chronic infarction, brainstem cavernous hemangioma, supratentorial meningioma, and astrocytoma. Three orthogonal anisotropic diffusion-weighted images were first obtained. Three primary colors were each assigned to a diffusion-weighted image, respectively, and the images were subsequently combined into a single-color image in full-color spectrum (3DAC MR axonography image). Fiber-tract definition in the cerebral peduncle of the midbrain of healthy volunteers showed intersubject variation, with two general patterns recognized: dispersed (60% of cases) and compact (40% of cases). Pathological alterations in the fiber tracts were readily identified in cases involving wallerian degeneration of the pyramidal tract, as illustrated in the cases of chronic infarction. Displacement of major tracts, such as the medial lemniscus or corticospinal tract, as well as fiber directionality, was also easily recognized in cases of mass lesions. As an imaging method uniquely capable of providing information regarding axonal connectivity, 3DAC MR axonography appears to have promising potential for routine clinical application.

Supratentorial giant cell ependymoma: a case report.

Ependymomas are neoplasms of the central nervous system that are capable of demonstrating remarkably heterogeneous histologic features. These tumors originate from ependymal cells lining the ventricles, the choroid plexus, the central canal of the spinal cord, and the filum terminale, so they are therefore seen throughout the neuraxis. We describe the case of a 26-year-old man who experienced a 3-week history of right-sided numbness and a 1-week history of worsening bifrontal headache. Computed tomographic scanning and magnetic resonance imaging of his head demonstrated an irregularly enhancing mass involving the left medial frontal lobe, with extension across the corpus callosum and expansion into the body and atrium of the left lateral ventricle. Histologic, immunohistochemical, and electron microscopic findings were consistent with an anaplastic ependymoma. Unique to this neoplasm was the presence of multiple tumor giant cells. The presence of pleomorphic tumor giant cells is a characteristic feature of the subependymal giant cell astrocytoma, and it is also commonly seen in pleomorphic xanthoastrocytoma and glioblastoma multiforme. Bizarre giant cells were recently described in two filum terminale ependymomas. This report presents the first case of a supratentorial giant cell ependymoma with anaplastic features.

Central nervous system neoplasms: indications for electron microscopy.

Diagnostic dilemmas of biopsy specimens in the central nervous system (CNS) tumors are often the result of multiple factors, including fixation artifact, biopsy size, lack of immunohistochemical techniques to distinguish cell types, and unawareness of rare entities. Correct diagnosis and confirmation of diagnosis of primary CNS neoplasms is imperative and may require electron microscopic examination. In some instances, use of electron microscopy may be the only approach for accurate recognition of an entity. Although diagnostic electron microscopy is expensive and cost cutting is encouraged in today's practice of medicine, cost must be weighed against the consequences of even 1 patient developing CNS treatment-related necrosis or a radiation-induced neoplasm secondary to misdiagnosis of a benign entity. This study reviews the ultrastructural differences of three groups of diagnostically difficult CNS lesions: clear cell neoplasms (ependymoma, oligodendroglioma, central neurocytoma), rare entities containing astrocytes invested by a basal lamina (pleomorphic xanthoastrocytoma, the desmoplastic neuroepithelial tumors of infancy), and benign entities characterized by transitional cell forms (subependymoma, subependymal giant cell astrocytoma).

Mutations and immunohistochemistry of p53 and proliferation markers in astrocytic tumors of childhood.

Thirty cases of hemispheric astrocytic tumors of childhood, consisting of 11 pilocytic astrocytomas, 2 fibrillary astrocytomas, 9 anaplastic astrocytomas, and 8 glioblastomas, were studied for the presence of p53 mutations and for immunohistochemical demonstrations of p53 and proliferation markers PCNA and Ki-67 MIB-1. The study was performed using polymerase chain reaction (PCR)-assisted single-strand conformation polymorphism analysis of exons 5-8 and direct sequence analysis of PCR products. For immunohistochemistry, DO1 and PAb 1801 were used. No mutation and no positivity for p53 protein were found in pilocytic astrocytomas. Mutations (at codons 144, 202, and 245) were found in 2 out of 8 glioblastomas and in 1 out of 9 anaplastic astrocytomas, whereas positive staining was found in 11 out of 17 malignant gliomas. Cases with mutations showed the highest p53 labeling index and also PCNA and MIB-1 labeling indices. The negative results in pilocytic astrocytomas are in line with the benign course of these tumors, whereas for malignant gliomas no difference seems to exist in comparison with adult cases.

Analysis of morphological changes in recurrent glial tumors after surgery and telegammatherapy.

In 65 cases with supratentorial localized recurrent gliomas the morphological pattern of the tumors from the first and second operation was investigated. Comparison of the tumor tissue obtained for diagnosis from the first and second operation indicated blastic transformation of the tumors observed mainly in the astrocytoma group. It has been found that the transformation proceeds in a longer time since the morphological pattern of the tumors in patients with short survival either did not change or changed only to a minor degree. It was interesting to note at the second operation that tumors with signs of differentiation and clearly defined malignancy did not correlate with survival time. It seems that the primary histological pattern of the tumor determines the survival time after the first operation.

Gliofibroma: immunohistochemical analysis.

A case of gliofibroma occurring in an adult patient as a large circumscribed supratentorial tumor is reported. The bimorphic pattern was substantiated and further analyzed by immunohistochemistry. Some evidence in favor of collagen production by mesenchymal and/or inflammatory cells leading to a progressive fibrous replacement of the glial cells in this particular tumor type is presented.

Pathological studies of CT findings in supratentorial astrocytoma. Correlation between low-density lesions and changes in fine structure.

CT findings and pathological features of 102 supratentorial astrocytomas were evaluated, and the surgically removed specimens of 10 cases studied by electron microscopy. Five pathological indices-anaplasia, vascularity, interstitial water content, necrosis and tumor fibers were graded from 1 to 4, and studies by the double-blind method. CT scans were divided into 4 groups according to attenuation coefficients and contrast enhancement. The data were statistically analysed. The results showed that the interstitial water content and tumor fibers were statistically different in low-density lesions and isodensity or hyperdensity lesions. The peritumoral low-density specimens correlated well with infiltrated tumor cells, which had swollen mitochondria. No definite correlation was found between the histological subtypes of astrocytomas and CT images. These findings suggest that high water content and lower physical density are responsible for the low-density of astrocytomas. Abnormalities of capillary ultrastructure result in the accumulation of contrast medium. The peritumoral area of low-density seen on CT scans is the real extent of the edematous tumor. But it is impossible to distinguish the histological subtypes of astrocytomas by CT findings.