1/20. Familial gliomas : a case report.Two non-twin brothers were found to have intracranial malignant neoplasms. The age of presentation was third and fourth decade but the onset was simultaneous, at the same time. diagnosis in each of them was made by computed tomography and confirmed by histopathology. Elder among them had cellular ependymoma and the younger had oligodendroglioma. Both the brothers received radiotherapy post operatively and were surviving asymptomatically without any neurological deficit, leading active life as police constable, 12 months after surgical treatment.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
2/20. Spinal oligodendroglioma with gliomatosis in a child. Case report.The authors present a rare case of oligodendrogliomatosis in a child, which they believe originated from a primary spinal cord tumor. At 2.5 years of age this boy developed poor balance, neck stiffness, and a regression in developmental milestones. A computerized tomography (CT) scan of the head initially revealed ventriculomegaly and multiple cystic cerebellar lesions. In addition, magnetic resonance (MR) imaging revealed a cystic intramedullary lesion involving the cervical spinal cord. A CT scan of the head and an MR image obtained 3 years later demonstrated diffuse small cysts on the surface of the brainstem, cerebellum, medial temporal and inferior frontal cortices, subcortical white matter, and corpus callosum suggestive of leptomeningeal tumor spread. Analysis of pathological specimens obtained at surgery showed neoplastic glial cells with small, uniform nuclei and perinuclear clear zones. The cells appeared to migrate along the subpial space but no tumor cells were present in the subarachnoid space. These findings were compatible with a diagnosis of oligodendrogliomatosis cerebri. Despite having a complicated course, chemotherapy with carboplatin has provided the patient with long-term palliation and a high quality of life. This case may represent the fifth report in the literature of oligodendrogliomatosis occurring in a child but only the third occurring with a spinal primary tumor.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
3/20. Gas in the cranium: an unusual case of delayed pneumocephalus following craniotomy.We present the case history of a 23-year-old man who underwent frontal craniotomy followed by radiotherapy for a Grade III anaplastic glioma. magnetic resonance imaging (MRI) at the 3-month follow-up showed significant tumour response. He became unwell some weeks after the MRI with an upper respiratory tract infection, severe headache and mild right-sided weakness. A computed tomographic (CT) scan showed a very large volume of intracranial gas, thought to have entered via a defect in the frontal air sinus after craniotomy and brought to light by blowing his nose. Intracranial air is frequently present after craniotomy, but it is normally absorbed within 34 weeks. The presence of pneumocephalus on a delayed postoperative CT scan should raise the possibility of a cerebrospinal fluid (CSF) fistula, or infection with a gas-forming organism. Many CSF fistulae require surgical closure in order to prevent potentially life-threatening central nervous system infection and tension pneumocephalitis. Immediate neurosurgical review is advisable.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
4/20. A novel germline mutation of PTEN associated with brain tumours of multiple lineages.We have identified a novel germline mutation in the PTEN tumour suppressor gene. The mutation was identified in a patient with a glioma, and turned out to be a heterozygous germline mutation of PTEN (Arg234Gln), without loss of heterozygosity in tumour dna. The biological consequences of this germline mutation were investigated by means of transfection studies of the mutant PTEN molecule compared to wild-type PTEN. In contrast to the wild-type molecule, the mutant PTEN protein is not capable of inducing apoptosis, induces increased cell proliferation and leads to high constitutive PKB/Akt activation, which cannot be increased anymore by stimulation with insulin. The reported patient, in addition to glioma, had suffered from benign meningioma in the past but did not show any clinical signs of Cowden disease or other hereditary diseases typically associated with PTEN germline mutations. The functional consequences of the mutation in transfection studies are consistent with high proliferative activity. Together, these findings suggest that the Arg234Gln missense mutation in PTEN has oncogenic properties and predisposes to brain tumours of multiple lineages.- - - - - - - - - - ranking = 2keywords = life (Clic here for more details about this article) |
5/20. hypersensitivity reactions to procarbazine with mechlorethamine, vincristine, and procarbazine chemotherapy in the treatment of glioma.The authors report the clinical features of hypersensitivity reactions believed to result from procarbazine in eight patients treated with mechlorethamine, vincristine, and procarbazine (MOP) for high-grade glioma. There was one instance of hypersensitivity in 7 patients treated for recurrent disease and seven instances in 16 patients treated with an adjuvant protocol using MOP directly after surgery. Maculopapular rash was seen in seven of eight, fever was seen in four of eight, and reversible abnormal liver function test results were seen in three of four patients. Pulmonary toxic effects were seen in five of eight patients and consisted of isolated interstitial pneumonitis in one, fever and infiltrate after rechallenge with procarbazine after previous rash in two, and cough accompanying rash in two. The toxic effects were mild to moderate in six patients but severe to life threatening in the two who were rechallenged after development of rash. The observed incidence of rash during adjuvant therapy was higher than that previously found by the authors for recurrent disease, and it appears to be higher than has been reported in Hodgkin's disease, lymphoma, and other solid tumors. The findings by the authors suggest that a high index of suspicion be kept for hypersensitivity reactions to procarbazine when treating primary brain tumors and that, contrary to the experience in other settings, procarbazine be stopped if rash develops.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
6/20. Papillary glioneuronal tumor.The descriptive term papillary glioneuronal tumor (PGNT) has been repeatedly applied to a morphologic subset of low-grade mixed glial-neuronal neoplasia of juvenile and young adult patients. We report on a 13-year-old boy with PGNT of the left temporal lobe, who presented with headaches and a single generalized seizure. On magnetic resonance imaging, tumor was seen as a large, moderately enhancing paraventricular mass with cyst-mural nodule configuration and slight midline shift. Perifocal edema was virtually absent. Gross total resection could be performed, followed by an uneventful recovery. Histologically, the tumor exhibited similar, if not identical, features as reported previously. These comprised a patterned biphasic mixture of sheets of synaptophysin-expressing small round cells and pseudorosettes of GFAP-positive rudimentary astrocytes along vascular cores. Focally, the latter imprinted a pseudopapillary aspect on this otherwise solid lesion. Both cellular components expressed non-polysialylated neural cell adhesion molecule (NCAM)-L species, and several overlapping areas of synaptophysin and GFAP immunoreactivity were present. The mean MIB-1 labeling index remained below 1%. Signs of anaplasia, in particular mitotic figures, endothelial proliferation, or necrosis were consistently lacking. We perceive PGNT as a clinically and morphologically well-delineated subgroup of extraventricular neurocytic neoplasia, whose paradigmatic presentation may allow for consideration as an entity.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
7/20. 45-year-old male with symptomatic mass in the frontal lobe.A 45-year-old male patient developed focal seizures in his right arm. neuroimaging demonstrated a tumor of the left frontal lobe. Tumor classification was undecided after stereotactic biopsy. Neuropathological examination of the open biopsy specimen revealed overlapping morphological features of an oligodendroglioma and a central neurocytoma. Groups of tumor cell featured the typical "fried egg" appearance seen in oligodendroglioma; microcalcifications and a network of branching non-proliferating vessels were present. neurocytoma-like features included small nucleus-free areas of neuropil and perivascular pseudorosettes. Neuron specific enolase was strongly expressed cytoplasmically in the tumor cells and the "neuropil islands" were found to express synaptophysin. The final diagnosis of an oligodendroglioma with neurocytic differentiation was based on tumor location, clinicopathological findings and diagnostic genotyping. Combined loss of heterozygosity (LOH) on the short arm of chromosome 1 (1p) and the long arm of chromosome 19 (19q), the "molecular signature" of oligodendrogliomas, was revealed. Besides supporting the diagnosis of an oligodendroglioma, the molecular data allow for additional therapeutic options. These tumors may point to the presence of yet another potential tumor precursor cell similar to the recently discovered "N-O"-cells in the cerebral cortex of rats, capable of differentiation into neurons and oligodendrocytes.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
8/20. Papillary glioneuronal tumor with minigemistocytic components and increased proliferative activity.Papillary glioneuronal tumor (PGNT) is a rare and new type of glioneuronal neoplasm of the central nervous system. It is characterized by pseudopapillary structures composed of hyalinized vessels rimmed by cuboidal glial cells and the proliferation of neuronal cells. We report a peculiar PGNT arising in the parietal lobe of a 67-year-old man, which was characterized by proliferation of minigemistocytic cells as well as typical components. The minigemistocytic cells had eccentric nuclei and plump eosinophilic cytoplasm that was filled with glial filaments. The Ki-67 labeling index was as high as 10% in the minigemistocytic areas. Recently, the presence of oligodendroglial-like component was suggested in PGNT. Considering that oligodendroglioma sometimes accompanies minigemistocytic components, the minigemistocytic cells in PGNT were suggested to be a part of oligodendroglial differentiation. Although PGNT is defined as an indolent glioneuronal tumor, the presence of minigemistocytic components with the high Ki-67 labeling index may indicate more aggressive nature.- - - - - - - - - - ranking = 6keywords = life (Clic here for more details about this article) |
9/20. Treatment-related myelodysplasia/AML in a patient with a history of breast cancer and an oligodendroglioma treated with temozolomide: case study and review of the literature.The emergence of temozolomide as an effective alkylating agent with little acute toxicity or cumulative myelosuppression has led to protracted courses of chemotherapy for many patients with gliomas. Secondary, or treatment-related, myelodysplasia (t-MDS) and acute myelogenous leukemia (t-AML) are life-threatening complications of alkylating chemotherapy and have been reported in patients with primary brain tumors. We describe a case of temozolomide-related t-MDS/AML and discuss the clinical features of this condition. Administration of an alkylating agent in patient populations with long median survivals must be undertaken with an understanding of the potential for this treatment complication.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
10/20. Primary disseminated leptomeningeal oligodendroglioma with 1p deletion. Case report.The authors report the case of a 2-year-old boy with a primary, diffuse leptomeningeal oligodendroglioma in which the deletion of chromosome arm lp was identified by performing a fluorescence in situ hybridization (FISH) analysis. This previously healthy child initially presented with malaise, anorexia, nausea, vomiting, and macrocephaly. Imaging studies confirmed the presence of hydrocephalus, and a ventriculoperitoneal shunt was placed. The postoperative course was complicated by emesis, continued weight loss, and numerous seizurelike episodes. A contrast-enhanced magnetic resonance imaging study performed approximately 10 weeks postoperatively showed diffuse leptomeningeal thickening and enhancement without evidence of an intraparenchymal mass lesion. A right frontal lobe brain biopsy revealed a hypercellular proliferation of small oligodendroglioma-like cells, which occupied the leptomeninges diffusely and spared the underlying cortical gray matter. The tumor cells displayed prominent perinuclear clearing and had evenly spaced, uniformly round nuclei. Occasional mitotic figures were observed. Background vessels were thin and delicate, and there was no evidence of necrosis. The tumor cells showed strong immunoreactivity for S100 protein; the results of immunohistochemical staining were negative for glial fibrillary acidic protein, vimentin, epithelial membrane antigen, NeuN, and synaptophysin. The deletion of lp was demonstrated by FISH analysis; lq, 19p, and 19q were intact. This appears to be the first reported case of a primary diffuse leptomeningeal oligodendroglioma in which a lp deletion was identified.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
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