Heavily lipidized, calcified giant cell glioblastoma in an 8-year-old patient, associated with neurofibromatosis type 1 (NF1): report of a case with long-term survival.

Giant cell glioblastoma (GCG-BM) with predominance of bizarre, multinucleated giant cells is a rare subtype of glioblastoma, however, its clinical behavior and histological features are still not fully understood. We report an unusual case of a heavily lipidized form of giant cell glioma corresponding mostly to GCGBM in a young patient with neurofibromatosis 1 (NF1). Histologically, the tumor revealed numerous characteristic histopathological features of giant cell glioblastoma including cellular pleomorphism with numerous giant tumor cells, pseudopalisades around necrotic foci and mitotic activity, accompanied by additional unique morphological elements such as massive lipidization of the neoplastic cells, abundant microcalcifications and angiomatous pattern of vascularization. Such aberrant morphology might be associated with the unusually long survival period of 12 years without clinical evidence of tumor recurrence. The coexistence of intracerebral heavily lipidized, calcified giant cell glioblastoma with NF1 has not been previously reported in literature.

Pedunculated subependymomas of lateral ventricle.

The authors describe two cases of extremely rare, pedunculated subependymomas of lateral ventricle. One patient presented with symptoms of intracranial hypertension (case 2); CT scan revealed evidence of intraventricular mass, but in second patient (case 1) coexisting symptomatic anaplastic astrocytoma masked on CT-scan the presence of asymptomatic intraventricular tumour, which was found no earlier than at autopsy. The cellular origin of this rare intracranial neoplasm is discussed.

[Primary brain lymphomas]. / Pierwotne chloniaki mózgowia.

In the Department of Neurosurgery of the Medical University of Warsaw 11 patients with primary central nervous system lymphomas were diagnosed and treated between 1990 and 1999. The patients comprised of 6 females and 5 males, aged 23 to 86 years, with most patients presenting in the sixth decade. The time from first symptoms to the diagnosis ranged from a few days to 5 months. In the majority of patients, the leading symptoms were: headaches, motor weakness, aphasia and memory disturbance. Initial diagnosis was based on MR in 3 patients, CT in 7 and on both CT and MR in 1 patient. The frontal lobe was the most common site of involvement. All the patients underwent surgery. The histological diagnosis was confirmed by immunohistochemical analysis using monoclonal antibodies: CD 20, CD3, CKMNF 116. In all cases, a B-cell type lymphoma was diagnosed. Radiotherapy was administered to 4 patients, 2 received chemotherapy and 1 received combined treatment. The median survival time so far is 17.6 months.

[Light microscopic and ultrastructural features of nerve fibres in glomus tumor in two patients with long-term pain]. / Mikroskopowy i ultrastrukturalny obraz wlókien nerwowych w klebczaku u 2 pacjentów z wieloletnimi bólami.

Morphologic features of nerve fibres in two cases of glomus tumour, localized in thigh skin of 57- and 72-year-old men are reported. In the patients, the paroxysms of severe pain occurred 10 and over 11 years respectively. Histological, immunohistochemical and ultrastructural examination revealed tumour cells with features resembling that of epithelioid smooth muscle cells of normal glomus body. In addition, NF-immunopositive nerve fibres were identified in connective stroma of the tumour. Ultrastructural examination revealed bundles of thin, nonmyelinated axons, surrounded by Schwann cell cytoplasm. The majority of axons were under 0.5 micron in diameter and contained densely packed neurofilaments, whereas only few axons of larger diameter exhibited electron-lucent axoplasm with granular and clear vesicles. The bundles of nonmyelinated nerves were observed always within spaces filled by collagen fibrils and did not show any special relationship to glomus cells.

Epithelioid schwannomas of the acoustic nerve.

Epithelioid schwannomas occur predominantly in relation to peripheral nerves and are associated with histological and clinical malignancy. However, a variant of the epithelioid schwannoma involving cranial nerves is extremely rare. In this study we report three cases of epithelioid schwannomas originating from the acoustic nerves and located in the cerebello-pontine angles. In the first case, the tumor was histopathologically entirely solid and demonstrated biphasic pattern with both spindle-shaped cells and a population of round or polygonal epithelioid cells. The second one consisted of the smaller part exhibiting typical Antoni B and A tissue and large areas containing clusters and bundles of epithelioid cells. Purely epithelioid schwannoma composed predominantly of cords or nests of round and polygonal epithelioid cells were observed in the third case. All schwannomas revealed marked polymorphism and nuclear hyperchromasia. Immunohistochemical studies showed a diffuse, strong positivity for S-100 protein in the cytoplasm of the spindle and epithelioid tumor cells. These two populations of cells were positively stained for vimentin, but were negative for EMA, cytokeratin and HMB45. Patchy GFAP-immunoreactivity was also noticed at the peripheral parts of the tumors. The authors discuss differential diagnosis of this unusual variant of schwannoma in relation to malignant transformation of the epithelioid component.

Primary intraspinal cervical melanoma.

A case of primary intraspinal melanoma is presented. This is a very rarely observed lesion, as only 37 cases have been described up to now. Patient presenting with signs and symptoms of spinal cord compression after MRI and spinal angiography was operated upon. Total removal of an intraspinal cervical tumour was performed. Histopathological investigation confirmed melanoma. Subsequent radiotherapy was given. Follow up of 4 years duration showed no signs of recurrence in MRI and full motor and sensory recovery.

Intrasellar neuronal hamartoma associated with pituitary adenoma. Case report.

46-year-old acromegalic women presenting high level of growth hormone (32 ng/ml) in the serum underwent surgery. The intrasellar tumor, 16 mm in diameter, has been removed. The biopsy material consisted of two types of closely adjacent and intermingled tissues, one of which was growth hormone positive acidophilic adenoma, the second component were haphazardly oriented ganglion cells of various size and shape, also multinuclear, with bundles of unmyelinated fibers. The cytoplasm and processes of ganglion cells were immunopositive for neurofilaments and for synaptophysin on cellular membranes and processes. There were none glial fibrillary acidic protein positive cellular elements. The authors discuss commonly used name of choristoma for this type of tumor and the problem of possible neurosecretory stimulation of pituitary adenoma by neuronal hamartoma.

[Anaplastic oligoastrocytoma of the spinal cord: diagnostic difficulties. Case report]. / Oligoastrocytoma anaplasticum rdzenia kregowego: trudnosci diagnostyczne. Opis przypadku.

The authors report a rare case of 49-years old woman with rapidly progressing anaplastic oligoastrocytoma primarily localized in the spinal cord. Increasing spastic paresis of the right lower limb was concomitant with decrease in superficial sensibility in this limb and the right side of the trunk below Th10 level, suggested a lesion within the spinal cord. However, it was the difficult confirming the diagnosis by spinal MR imaging, and the negative result of the first MR examination (performed 5 weeks after manifestation of first clinical symptoms of the disease) delayed surgical treatment. During the next 3 weeks the neurological syndrome increased to spastic paraparesis with sphincters dysfunction and decrease in superficial and vibratory sensibility within the lower limbs and the trunk below the Th10 level. The second MR examination of the spine revealed an intraspinal tumour at Th8-Th10 levels. Surgical (partial excision of the tumour) and radiation treatment was given. Histopathological examination of tumour tissue showed the presence of anaplastic oligoastrocytoma. During the follow-up of our patient we found cerebral foci, probably of metastatic origin ascending with cerebrospinal fluid. More than 5 months after the diagnosis was established the patient died of primary disease. The imaging parameters of both spinal MR examinations were similar, however, on second examination narrower field of vision was used. In both cases Magnevist was administered. Discussing factors which might be responsible for the false-negative result of spinal MR examination--the examination of choice for detection of proliferative transformation--the authors take artefacts connected with respiratory and circulatory function, peristaltic movements, and with field of vision into consideration.

Disorganization of cortical structure and the brain tumors.

In the course of histopathological investigation of the temporal lobe sections, selected from 63 patients treated surgically for intractable epilepsy and finally presented with primary temporal tumors, we found 12 cases expressed both neoplastic process' and developmental disorders. The temporal mass lesions consisting of neuro-glial or pure glial tumors were associated with some developmental abnormalities such as cortical dysplasia, neuronal heterotopias and additional cortical neoplastic nodules. The possible "dual pathology" concerning these lesions are discussed in this paper.

[The value of structural neuroimaging in the selection of patients for epileptic surgery]. / Znaczenie strukturalnych badan neuroobrazujacych, w kwalifikacji chorych do leczenia operacyjnego padaczki.

UNLABELLED: The aim of this study was to investigate the value of structural neuroimaging with MRI in the selection of patients for epilepsy surgery. We sought to determine whether MRI influenced decision concerning resective surgery and whether MRI provided much more useful information than enhanced CT. MATERIALS AND METHODS: Neuroimaging studies, MRI and CT, of 300 patients; 265 with partial and 35 with primary generalized seizures, evaluated for surgical treatment of epilepsy were analysed. The MRIs and CTs were interpreted using visual diagnostic criteria and findings were correlated with the EEG changes and clinical semiology. RESULTS: MRIs identified structural lesions in 142, CTs in 96 of all patients. The clinical semiology (partial seizures), MRI, CT and EEG focal findings were concordant in 72 cases. The group of 34 patients had resective surgery. The 7 patients were also operated with MRI and CT focal abnormalities discordant with EEG changes. Also one patient with primary generalized epilepsy and temporal lobe lesion (glioma) had resective surgery. MRI studies revealed structural lesions in 48 patients with normal CT studies. The 43 patients with partial epilepsy had normal CTs and lesions in MRIs; the 34 cases revealed correlation with the EEG findings in 29 temporal and 5 extratemporal regions. Surgery were performed in 23 cases. Also one with partial seizures and MRI detected hippocampal atrophy was operated, despite of generalized EEG patterns. In contrast CT revealed two patients with normal MRI and focal changes. The patients with partial seizures and only CT abnormalities (focal calcifications) were not operated due to discordant EEG findings. In group of 132 patients with normal neuroimaging studies and EEG identified seizure focus only 27 had anterior temporal lobectomy. CONCLUSION: MRI studies gave additional information in case of 16% patients with intractable epilepsy in comparison with CT findings. Resective epilepsy surgery was almost twice as often performed when MRIs revealed structural abnormality. In operated patients, diagnostic sensitivity of structural MRI, CT and EEG to neurophatology were 70.6%, 46.7 and 92.4% respectively.

Lectin histochemistry of ethylnitrosourea-induced oligodendrogliomas in the rat.

Oligodendrogliomas (n = 26) induced by ethylnitrosourea (ENU) in wistar rats were examined to assess the lectin specificity to oligodendroglial membranes. Two different types of oligodendrogliomas were found in our material: an isomorphous type (n = 12), and a polymorphous type (n = 14). The first one, with two variants according to its size, macro- (n = 9) and microtumors (n = 3), had predominantly a honey-comb pattern with 'clear halos' around the nuclei without anaplasia. The second type, composed mostly by macrotumours, was anaplastic, with high cellular density, necrosis and intratumoral hemorrhages. Peanut agglutinin (PNA) labelled plasma membranes of well-differentiated cellular components of the first group. The tumoral oligodendrocytes lost the property to bind PNA in the second group of tumours, while Concanavalin A (Con A) showed affinity to intracytoplasmic structures of these tumours. PNA is a reliable marker of oligodendroglial plasma membrane of well-differentiated ENU-induced oligodendrogliomas. This experimental model, using PNA and Con A, may have important clinical applications regarding the biological behaviour of this type of neoplasm.

Intracranial hemangiopericytomas. Histological and immunohistochemical study.

The authors report on 8 cases of intracranial supratentorial tumors which clinically corresponded to meningeal neoplasms. Only one patient revealed the recurrence. The age of patients was 39-74, prevailed women (5). Histological features of tumors, except some variations in two cases agreed with common morphologic pattern of hemangiopericytomas. Immunohistochemical study with following antibodies was performed: vimentin, epithelial membrane antigen, cytokeratin, S-100 protein, fibronectin, desmin, collagen IV, factor RAg, and glial fibrillary acidic protein. Two atypical cases which presented scarce pseudocalcifications and tendency to concentric cellular structures did not differ from other hemangiopericytomas by their intrinsic immunohistochemical properties such as negative reaction to epithelial membrane antigen and cytokeratin. Atypical positive immunostaining with S-100 protein and with glial fibrillary acidic protein was found solely in the group or perivascular cells in one of those atypical cases reflecting probably local astrocytes embedded in tumor tissue. Immunohistochemical investigations did not present univocal data to explain controversial origin of hemangiopericytomas either from meningeal cup cells or from vascular pericytes.

Subependymal giant cell astrocytoma: clinical, histologic and immunohistochemical characteristic of 3 cases.

Three cases of subependymal giant cell astrocytoma (SEGA) in the women aged 23, 26, and 36 years were reported. Two of them had no clinical evidence of tuberous sclerosis complex (TSC) and the one woman presented apparent mental retardation. All patients manifested sudden clinical onset with symptoms of elevated intracranial pressure due to tumor of lateral ventricles and obstructive hydrocephalus. At surgery, the neoplasm was removed totally in one case and resected partially in 2 cases. Histologically, the tumors were composed of large polygonal cells with vesicular nuclei, prominent nucleoli and glassy eosinophilic cytoplasm, intermingled with spindle and small cells. In addition, multinucleated and bizarre giant cells were present, but they were very numerous in one case only. The tumor cells revealed in all cases variegated immunoreactivity for glial fibrillary acidic protein (GFAP), S-100 protein, vimentin (VIM) and neuron-specific enolase (NSE), with stronger expression of VIM than GFAP in 2 cases. Immunostaining of neurofilament proteins and synaptophysin was negative. The results suggest rather astroglial incomplete or aberrant differentiation and maturation than neuronal differentiation of tumor cells. The immunohistochemical variations of SEGA in asymptomatic TSC cases and those associated with tuberous sclerosis are discussed.

Cerebral gangliogliomas. Morphological and immunohistochemical study.

Report concerns 6 uncommon cases of cerebral ganglioglioma in adult patients, the majority of whom (4) demonstrated typical classic features, others were anaplastic. Both types presented neuronal and glial components characterized either by NF, NSE and Syn or GFAP immunoreactivity. Only the tumor of the youngest patient presented moderate desmoplasia. Observations indicate at an easy classification of glial component contrary to neuronal component in relation to the origin of neuronal changes.

Choroid plexus carcinoma. Case report.

Report concerns the uncommon cerebellar neoplasm of choroid plexus in 57-year-old woman, which was devoid of some anaplastic features causing doubts about degree of tumor differentiation. Immuno- and histochemical study presented positive CK, VIM, GFAP, S-100 immunostaining and positive PAS-dimedone reaction conforming histological diagnosis.

Meningiomas and gliomas in juxtaposition: casual or causal coexistence? Report of two cases.

Two cases of meningioma and glioma established in biopsy material from one or more than one operation are reported. In these cases, an originally benign meningioma was followed by the development of anaplastic astrocytoma in close juxtaposition to the site of first operation. The close juxtaposition of two histologically different tumors suggested that one of them might lead to local proliferation and independent growth of the other.

Concanavalin A binding by the hippocampal neurons following brief cerebral ischemia in gerbils.

Twenty two Mongolian gerbils after 5 min bilateral carotid artery occlusion and 6, 12, 24, 48, 72, 94 hours and 5 days survival were investigated for the neuronal changes in dorsal hippocampus. Paraffin sections were stained with cresyl-violet and marked by their binding of Concanavalin A (Con A) labelled with peroxidase. The degeneration and neuronal loss was observed only in CA1 sectors in almost all experimental groups, whereas the decreased binding of Con A by the neurons of CA1 sector corresponded to the intensity of histologic changes but appeared also only in this sector even without any histological changes. These observation can point at the subthreshold damage of CA1 neurons as result of either diminished supply or increased metabolism of d-glucose, or diminished number of Con A receptors or changes of their specificity after ischemic period.

Multiple cerebral cavernous angiomas. Case report.

This 26-year-old woman was admitted to Neurosurgery Department in 1972 for the first time due to increased intracranial pressure and since observed and treated by ventriculo-peritoneal shunt during next twenty years. First symptoms such as visual disturbances, hemiparesis and hemianesthesia appeared 8 years earlier followed 4 years later by epileptic fits. Ventriculography revealed hydrocephalus and the tumor in the region of the third ventricle. CT scan visualized multiple hyperdense foci. Ventriculo-peritoneal shunt provided good improvement. During the next twenty years condition of the patient gradually worsened. Diagnosis of multiple cavernous angiomas was established only at autopsy.

Supratentorial astrocytoma associated with arteriovenous malformation. Case report.

A case of the 18-year-old boy suffering from epilepsy since 8 years and suspected of microangioma is presented. The craniotomy revealed left parietal parasagittal superficially located neoplasm and arteriovenous malformation beneath. Unexpected fibrillary astrocytoma associated with vascular tumor was the diagnostic problem, solved histologically after the operation. The problem of coexistence of various neoplasms with arteriovenous malformations is discussed.