1/9. ependymoma with extensive lipidization mimicking adipose tissue: a report of five cases.Lipomatous ependymoma is a recently described entity and only 3 cases of this variant have been reported in the literature. We report 5 cases of this rare variant of ependymoma. patients age ranged from 4 years to 45 years and, interestingly, all of them were males. Two tumors were supratentorial in location, 2 in the fourth ventricle and 1 was intramedullary. Microscopically all of them showed the classical histology of ependymoma along with lipomatous differentiation. The lipomatous component was composed of cells with a large clear vacuole pushing the nucleus to the periphery and giving a signet ring cell appearance. This component demonstrated positivity for GFAP and S-100 protein thereby confirming its glial lineage. Three of the 5 tumors were high grade (WHO-grade III), had a high MIB-1 labelling index (MIB-1 LI) and showed recurrence on follow-up. However, 2 were low grade (WHO grade II) and patients are free of disease till the last follow up.- - - - - - - - - - ranking = 1keywords = supratentorial (Clic here for more details about this article) |
2/9. Medulloblastomas in neurofibromatosis type 1. Case report and literature review.A 6-year-old girl, previously diagnosed with neurofibromatosis type 1 (NF-1) presented with ataxia and symptoms of raised intracranial pressure. Diagnostic work up disclosed a posterior fossa tumor. Histopathological study of the excised neoplasm showed a cerebellar medulloblastoma. We review the current literature and suggest that the association of medulloblastoma with NF-1 is not a chance occurrence, and that it might be pathogenically related. We propose that medulloblastoma should be added to the list of malignancies that are apt to occur in NF-1.- - - - - - - - - - ranking = 0.0028810180355259keywords = neoplasm (Clic here for more details about this article) |
3/9. Posterior cervical haemangiopericytoma with intracranial and skull base extension. Diagnostic and therapeutic challenge of a rare hypervascular neoplasm.Haemangiopericytomas are rare hypervascular tumors arising from pericytes. They may occur anywhere in the body, but posterior cervical location is rather uncommon. A case of posterior cervical haemangiopericytoma with posterior fossa and temporal bone extension is reported. Although the patient had undergone preoperative endovascular embolization and surgical resection on three separate occasions, control of the skull base extension was not successful. Following endovascular embolization combined with radiotherapy, the patient has been asymptomatic for 48 months. Angiographic features may help in differentiating haemangiopericytomas from other hypervascular lesions. Preoperative endovascular embolization is recommended due to the pronounced tendency for haemorrhage throughout biopsy and surgical procedures.- - - - - - - - - - ranking = 0.011524072142103keywords = neoplasm (Clic here for more details about this article) |
4/9. Multiple supratentorial epidural haematomas after posterior fossa surgery.Postoperative epidural haematoma distant to a site of craniotomy is a rare but possibly hazardous complication. We report a 31-year-old female who presented with a history of chronic hydrocephalus due to fourth-ventricular plexus papilloma. Following resection of the posterior fossa tumour with intraoperative placement of a ventricular drainage, she consecutively developed four supratentorial epidural haematomas at different locations, all necessitating evacuation. The clinical manifestations ranged from subtle neurological deficits to signs of tentorial herniation; the ultimate outcome was complete recovery. Rapid tapering of CSF pressure after long-standing hydrocephalus and clotting disorders could be implicated as causative factors. We stress the importance of early postoperative CT scan and optimal management of ventricular pressure and coagulation status to detect and prevent this possibly life-threatening complication.- - - - - - - - - - ranking = 5keywords = supratentorial (Clic here for more details about this article) |
5/9. Stereotactic methods in interdisciplinary diagnosis and treatment of posterior fossa tumours.For over 2 years, we have had access to latest generation apparatus and software for planning and stereotactic treatment as well as for x-rays treatment. Until now, we have carried out over 100 procedures. These included 52 stereotactic biopsies of neoplasms, some of them located within the structures of the posterior fossa. In this report, we have discussed the possibilities and effectiveness of diagnosis and treatment of tumours located in different structures, including posterior cave. In the study, we used stereotactic methods. We have described biopsies of the following tumours: cerebellar hemisphere tumour (diagnosed as "metastatic atypic planoepitheliale carcinoma" in a patient with coexisting orbit lymphoma), a bifocal lesion (located in cerebellar hemisphere and cerebellar peduncle, diagnosed as pilocytic astrocytoma of WHO malignancy grade II/III), and lesion (located in the pons, diagnosed as pilocytic astrocytoma WHO grade II).- - - - - - - - - - ranking = 0.0028810180355259keywords = neoplasm (Clic here for more details about this article) |
6/9. Infratentorial giant cell ependymoma: a rare variant of ependymoma.We describe a giant cell ependymoma occurring in a 50-year-old man. The mass was located in the posterior aspect of the foramen magnum, extending from the cerebellar tonsil to the upper cervical spine. The tumor was a highly cellular neoplasm showing biphasic histology. Diffuse sheets of non-cohesive atypical giant cells, having eccentrically located single or multiple nuclei and plump eosinophilic cytoplasm, partly infiltrated the desmoplastic inflammatory stroma. Parts of perivascular pseudorosette-forming or pseudopapillary areas were composed of atypically elongated cells, which looked like conventional anaplastic ependymoma. There was a transitional area between two patterns. Numerous mitoses and focal necrosis were observed. Immunohistochemically, the tumor cells were immunoreactive for glial fibrillary acidic protein, vimentin, S-100 protein, and CD99. None of the tumor cells showed immunoreactivity for epithelial membrane antigen except for the intracytoplasmic lumen of a few vacuolated cells. Ultrastructurally, tumor cells were ependymal in nature; we noted cytoplasmic intermediate filaments and intercellular microrosettes with microvilli, cilia, and long zonula adherens. The features of this tumor, e.g. its superficial location, mixed giant cells, perivascular pseudorosettes or papillaries, complicated its differentiation from rhabdoid/papillary meningioma. However, immunohistochemistry and electron microscopy confirmed the diagnosis of ependymoma. The giant cell variant should be included in the subclassification of the ependymoma.- - - - - - - - - - ranking = 0.0028810180355259keywords = neoplasm (Clic here for more details about this article) |
7/9. Meningeal melanocytoma of the posterior fossa: case report and literature review.BACKGROUND: Meningeal melanocytomas are rare primary melanotic tumors of the leptomeninges. According to our review of the literature, just 22 cases of meningeal melanocytoma (MM) of the posterior fossa have been previously reported. Some aspects related to diagnosis, radiological appearance, histopathologic features, and management are discussed in this paper. CASE DESCRIPTION: We describe the case of a 42-year-old female presenting with severe headache, nausea, and vomiting. Computed tomography and magnetic resonance imaging demonstrated a posterior fossa lesion that was surgically treated. Histopathologic examination showed a highly cellular melanocytic neoplasm with numerous dark pigments in the cytoplasm. Immunoperoxidase staining S-100 protein and HMB 45 demonstrated immunoreactivity for both, confirming the diagnosis of MM. CONCLUSIONS: In conclusion, MMs are rare histologically benign tumors that can be cured by complete surgical resection alone, which should be the goal of the treatment. These lesions, although rare, should be considered in the differential diagnosis of tumors of the posterior fossa.- - - - - - - - - - ranking = 0.0028810180355259keywords = neoplasm (Clic here for more details about this article) |
8/9. Genetic profiling of a distant second glioblastoma multiforme after radiotherapy: recurrence or second primary tumor?OBJECT: In nearly all patients with glioblastoma multiforme (GBM) a local recurrence develops within a short period of time. In this paper the authors describe two patients in whom a second GBM developed after a relatively long time interval at a site remote from the primary tumor. The genetic profiles of the tumors were compared to discriminate between distant recurrence and a second primary tumor. methods: Both patients harboring a supratentorial GBM were treated with surgery and local high-dose radiotherapy. Local control of the disease at the primary tumor site was achieved. Within 2 years, a second GBM developed in both patients, not only outside the previously irradiated target areas but infratentorially in one patient and in the opposite hemisphere in the other. The tumors were examined for the presence of several genetic alterations that are frequently found in GBMs--a loss of heterozygosity at chromosome regions 1p36, 10pl5, 19q13, and 22q13, and at the CDKN2A, PTEN, DMBT1, and TP53 gene regions; a TP53 mutation; and EGFR amplification. In the first patient, genetic profiling revealed that the primary tumor had an allelic imbalance for markers in several chromosome regions for which the second tumor displayed a complete loss. In the second patient, genetic profiling demonstrated the presence of genetic changes in the second tumor that were identical with and additional to those found in the primary tumor. CONCLUSIONS: Based on the similarities between the genetic profiles of the primary and the second tumors in these patients, the authors decided that in each case the second distant GBM was a distant recurrence rather than a second independent primary tumor.- - - - - - - - - - ranking = 1keywords = supratentorial (Clic here for more details about this article) |
9/9. Neurobehavioral alterations in an adolescent following posterior fossa tumor resection.The posterior fossa syndrome (PFS) consists of a variety of symptoms, including cerebellar mutism, behavioral disturbances and personality changes. We report longitudinal clinical, neuroradiological and neurobehavioral findings in a 19-year-old left-handed patient, diagnosed with attentional deficit hyperactivity disorder (ADHD) at the age of 12, who underwent posterior fossa tumor resection. Although the patient did not develop cerebellar mutism after surgery, marked apathy and emotional indifference, urinary retention, eye-lid apraxia and visual hallucinosis became apparent after a brief interval of normal functioning. Based on these findings it is argued that the PFS might be considered a semiological heterogeneous condition with variable clinical expressions. Long-term follow-up investigations revealed subtle, but significant cognitive and affective deficits, resembling the cerebellar cognitive affective syndrome in adults. As demonstrated by functional neuroimaging studies with SPECT, symptoms were associated with perfusional deficits in the anatomoclinically suspected supratentorial regions, reflecting the distant impact of the cerebellum on cognitive and affective functions.- - - - - - - - - - ranking = 1keywords = supratentorial (Clic here for more details about this article) |