1/29. optic nerve sheath fenestration for a reversible optic neuropathy in radiation oncology.To the authors' knowledge, there is a paucity of published accounts of management of radiation-induced optic neuropathy (RION) by optic nerve sheath fenestration (ONSF) in the conventional medical literature. With higher doses of radiation being given by using conformal techniques, more radiation-induced optic neuritis and neuropathy will be identified. We report here the successful use of ONSF to restore vision to three consecutive patients with pending anterior RION, and the importance of early identification and intervention in these potentially reversible cases.- - - - - - - - - - ranking = 1keywords = radiation-induced (Clic here for more details about this article) |
2/29. glioblastoma multiforme occurring in a patient treated with gamma knife surgery. Case report and review of the literature.Stereotactic radiosurgery is being increasingly advocated as the primary modality for treatment of vestibular schwannomas (VS). This modality has been shown to arrest tumor growth, with few associated short-term morbidities, and with possibly better hearing and facial nerve preservation rates than microsurgery. Radiation-induced oncogenesis has long been recognized, although stereotactic radiosurgery de novo induction of a secondary tumor has never been clearly described. The authors report on a patient with a VS who did not have neurofibromatosis Type 2 and who underwent gamma knife surgery (GKS). This patient required microsurgical removal of the VS within 8 months because of development of a tumor cyst with associated brainstem compression and progressive hydrocephalus. The operation resulted in clinical stabilization and freedom from tumor recurrence. Seven and a half years after undergoing GKS, the patient presented with symptoms of raised intracranial pressure. magnetic resonance imaging demonstrated a new ring-enhancing lesion in the inferior temporal lobe adjacent to the area of radiosurgery, which on craniotomy was confirmed to be a glioblastoma multiforme (GBM). Despite additional conventional external-beam radiation to the temporal lobe, the GBM has progressed. Whereas this first reported case of a GBM within the scatter field of GKS does not conclusively prove a direct causal link, it does fulfill all of Cahan's criteria for radiation-induced neoplasia, and demands increased vigilance for the potential long-term complications of stereotactic radiosurgery, and reporting of any similar cases.- - - - - - - - - - ranking = 0.5keywords = radiation-induced (Clic here for more details about this article) |
3/29. Radiation leukoencephalopathy associated with moderate hydrocephalus: intracranial pressure monitoring and results of ventriculoperitoneal shunting.Delayed neurological sequelae of radiotherapy have several manifestations; leukoencephalopathy is one of the most common. Pathogenetic relationships between radiation leukoencephalopathy and other findings of diffuse radiation injury (brain atrophy and progressive ventriculomegaly) are not well defined. Moreover, no guidelines have been established for the treatment of hydrocephalus when associated with radiation leukoencephalopathy. Our study reports intracranial pressure (ICP) monitoring in two patients with radiation leukoencephalopathy with moderate hydrocephalus. High intraventricular mean pressure and high peaks were found, and marked improvement of clinical status after shunting was achieved. This study, although restricted to only two patients, shows that ventriculoperitoneal shunting insertion is useful in radiation-induced hydrocephalus, when ICP monitoring detects high mean pressure. A hypothesis is advanced concerning radiation-induced hydrocephalus with high ICP, emphasizing periventricular astrocytosis and its connections with cerebral compliance.- - - - - - - - - - ranking = 1keywords = radiation-induced (Clic here for more details about this article) |
4/29. Leukemic dissemination within a glioblastoma in a patient with chronic lymphoid leukemia.Metastasis from an extracranial tumor to a primary central nervous system tumor is a rare event, and most reported cases concern metastases to meningiomas. The authors describe the first case of leukemic cell dissemination within a glioblastoma. The patient likely presented a genetic predisposition to multiple neoplasms, and the unusual localization of leukemic cells might be partly related to the characteristic microvascular proliferation in glioblastoma.- - - - - - - - - - ranking = 0.008328249161694keywords = leukemia (Clic here for more details about this article) |
5/29. prevalence of glioblastoma multiforme in subjects with prior therapeutic radiation.This retrospective study profiled subjects with glioblastoma multiforme (GBM) who had previously received therapeutic radiation. A chart review was conducted of 100 adult patients diagnosed with GBM and referred to a major medical center in the southwestern united states. Seventeen patients received previous radiation therapy with an average dose of 48.5 Grey (Gy) and an average latency period of 15 years between initial therapy and GBM diagnosis. Of these 17, four white females fit all four attribution criteria for radiation-induced GBM. Two had been treated with radiation for prolactinomas, one for pinealoma and one for squamous cell cancer of the ethmoid sinus. The addition of these four case studies to the previously published descriptions of 80 cases of gliomas, 36 of which were GBM, subsequent to radiation therapy provides additional support for considering therapeutic radiation as a risk factor for GBM development.- - - - - - - - - - ranking = 0.5keywords = radiation-induced (Clic here for more details about this article) |
6/29. cafe-au-lait spots and early onset colorectal neoplasia: a variant of HNPCC?BACKGROUND: cafe-au-lait spots (CALS) are classically found in neurocutaneous syndromes such as neurofibromatosis, but have not been associated with hereditary colorectal cancer. However, review of hereditary colorectal cancer case reports reveals occasional description of CALS on physical exam. methods: We describe the colonic and extracolonic phenotype in a family with CALS and early onset colorectal neoplasia (adenomas and/or cancer) and review 23 additional families reported in the literature. RESULTS: Among the 24 families, 32/59 (54.2%) individuals had colorectal adenomas diagnosed at a mean age of 15.7 /- 1.1 (SE) years (range 5-38 years). The majority (24/32, 75.0%) of persons at first colorectal examination had oligopolyposis (< 100 polyps) versus polyposis (> or = 100 polyps). Forty-two of 59 (71.2%) individuals were affected with colorectal cancer, diagnosed at a mean age of 31.9 /- 2.7 years (range 5-70 years). A brain tumor was found in 28/59 (47.5%) affected individuals (4 families with 2 or more cases) with an overall mean age of diagnosis of 16.5 /- 1.2. lymphoma and/or leukemia was found in 8/24 (33.3%) families (one family with 3 cases). Two families had mutation of the mismatch repair gene, hPMS2 (1 with homozygous germline mutation), while two carried homozygous germline mutations of another mismatch repair gene, hMLH1. CONCLUSIONS: cafe-au-lait spots with early onset colorectal neoplasia may identify families with a variant of HNPCC characterized by oligopolyposis, glioblastoma at young age, and lymphoma. This variant may be caused by homozygous mutation of the mismatch repair genes, such as hPMS2 or hMLH1.- - - - - - - - - - ranking = 0.0020820622904235keywords = leukemia (Clic here for more details about this article) |
7/29. Phenotypic and genotypic characterization of glioblastoma multiforme with epithelial differentiation and adenoid formations.Rare examples of high-grade gliomas show focal epithelial differentiation, which may require distinction from colliision tumors. These epithelial constituents are not well-characterized immunophenotypically and have rarely been subjected to genotyping. We describe a case of a 54-year-old female with a short history of hemiparesis who was found to have an absolute lymphocytosis and a heterogeneously enhancing frontotemporal tumor. Cytological and histological examination of brain biopsies confirmed the presence of a glioblastoma multiforme also containing CAM5.2/CK7/BerEP4/CEA/EMA-immunopositive and GFAP-immunonegative nests of epithelial cells with a high proliferative index and focal glandular differentiation. Hematological investigations confirmed a diagn of chronic lymphocytic leukemia (CLL) with no demonstrable CNS involvement. Genetic analysis using microsatellite markers and specimens obtained by laser capture microdissection of the CNS tumor and normal brain tissue, showed that both the glial and epithelial components of the brain tumor had identical losses of 2/2 informative markers on chromosome 17p13 and 5/5 informative markers on chromosome 10q22-26. The glial and epithelial components also shared an identical 2 base pair deletion in the TP53 gene at codon 209, exon 6, introducing a stop codon at codon 214. No losses at any of the above loci and no TP53 mutation were detected in the leukemic cells. These results suggest both components of the brain tumor, although differing in phenotype, share the same genetic lineage.- - - - - - - - - - ranking = 0.0020820622904235keywords = leukemia (Clic here for more details about this article) |
8/29. glioblastoma multiforme in a child with acute lymphoblastic leukemia: case report and review of literature.An 11-year-old boy with acute lymphoblastic leukemia had received prophylactic cranial irradiation (1800 cGy /10 fractions) and intrathecal methotrexate. Five years later, he developed a glioblastoma multiforme in the right frontal region while the leukemia was in remission. It is possible that the glioma may have been induced by radiation and /or chemotherapy.- - - - - - - - - - ranking = 0.012492373742541keywords = leukemia (Clic here for more details about this article) |
9/29. Radiation-induced cerebellar glioblastoma at the site of a treated medulloblastoma: case report.Radiation-induced glioblastoma multiforme (GBM) is a rare complication of radiotherapy. The authors report such a case occurring 10 years after treatment of cerebellar medulloblastoma. The patient was a 15-year-old boy who had undergone a gross-total removal of a medulloblastoma and received radiation therapy at the age of 5 years. He had experienced no tumor recurrences for 10 years until a new enhancing mass was found at the original site of the medulloblastoma. Following its resection the new lesion was found to be a GBM and there was no evidence of a medulloblastoma. The second tumor developed at the same site as the previous one after a sufficient latent period and fulfilled the criteria for a radiation-induced neoplasm. The original tumor cells expressed synaptophysin without p53 overexpression, a characteristic feature of medulloblastomas. In contrast, cells from the later tumor expressed glial fibrillary acidic protein and p53 but not synaptophysin. A sequence analysis of the p53 gene showed deletion at codon 233 and a C to G transition at codon 278 in the GBM but no mutation in the medulloblastoma. A GBM specimen revealed no amplification of the epidermal growth factor receptor compared with a normal control specimen. In conclusion, the clinical features of a radiation-induced GBM are similar to that of the primary GBM, whereas its genetic alterations render it a secondary GBM. These findings indicate that radiation-induced GBM should be considered a distinct clinical entity.- - - - - - - - - - ranking = 1.5keywords = radiation-induced (Clic here for more details about this article) |
10/29. [Isolated Richter's syndrome in central nervous system: case report.]Diffuse large cell non Hodgkin's lymphoma associated with chronic lymphoid leukemia (CLL), or Richter's syndrome, is a rare and serious complication. Isolated Richter's syndrome in the central nervous system is very rare; only 12 cases have been reported. We describe a 74-year-old patient with diffuse large cell non Hodgkin's lymphoma in the right frontal region with the appearance of multiform glioblastoma.- - - - - - - - - - ranking = 0.0020820622904235keywords = leukemia (Clic here for more details about this article) |
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