Search results for "Ependymoma"

Childhood supratentorial ependymomas with YAP1-MAMLD1 fusion: an entity with characteristic clinical, radiological, cytogenetic and histopathological…

2018

Ependymoma with YAP1-MAMLD1 fusion is a rare, recently described supratentorial neoplasm of childhood, with few cases published so far. We report on 15 pediatric patients with ependymomas carrying YAP1-MAMLD1 fusions, with their characteristic histopathology, immunophenotype and molecular/cytogenetic, radiological and clinical features. The YAP1-MAMLD1 fusion was documented by RT-PCR/Sanger sequencing, and tumor genomes were studied by molecular inversion probe (MIP) analysis. Significant copy number alterations were identified by GISTIC (Genomic Identification of Significant Targets in Cancer) analysis. All cases showed similar histopathological features including areas of high cellularity…

Spinal ependymoma in adults: a multicenter investigation of surgical outcome and progression-free survival

2018

OBJECTIVESpinal ependymomas are rare glial neoplasms. Because their incidence is low, only a few larger studies have investigated this condition. There are no clear data concerning prognosis and therapy. The aim of the study was to describe the natural history, perioperative clinical course, and local tumor control of adult patients with spinal ependymomas who were surgically treated under modern treatment standards.METHODSThe authors performed a multicenter retrospective study. They identified 158 adult patients with spinal ependymomas who had received surgical treatment between January 2006 and June 2013. The authors analyzed the clinical and histological aspects of these cases to identif…

Spinal cord monitoring during intraspinal extramedullary tumor operations (Peroneal nerve evoked responses)

1990

Longterm scalp recording of early SEP components triggered by peroneal or tibial nerve stimulation detects functional disturbances of spinal cord transmission due to mechanical trauma. We confirm previous observations that preoperative SEP patterns reflect neurological deficits and clearly show functional disturbances even on the side where they are not manifest. Peroneal nerve SEP have a well-known P40-peak corresponding to activities of neurons at the postcentral cortical layers. The P40-peak was identified in only 55% of our recordings. We therefore, tried to use the P50-peak that could be identified in 100% of the recordings under the difficult recording circumstances in the operating r…

Endoscope-assisted microsurgery for tumors of the septum pellucidum: surgical considerations and benefits of the method in the treatment of four seri…

2003

Neoplasms that primarily originate from the septum pellucidum are extremely rare. Generally the septum pellucidum is involved in direct extension of tumors that arise from the neighboring structures, principally the corpus callosum. Endoscope-assisted techniques form a useful adjunct to common microsurgical procedures to completely remove intraventricular lesions. There are two main advantages of endoscope-assisted surgery over common microsurgical techniques: reduction of superficial brain retraction with less iatrogenic trauma to the neighboring structures and inspection of hidden corners depict simultaneously anatomical details which are not precisely visible in the zoomed and thus light…

Preradiation chemotherapy of children and young adults with malignant brain tumors: Results of the german pilot trial HIT'88/'89

1998

Background Preradiation chemotherapy could be beneficial in malignant brain tumors, because the blood-brain tumor-barrier is disrupted after surgery, bone marrow recovery--essential for intense chemotherapy--is still intact, and CNS toxicity and ototoxicity of active drugs are lower before irradiation of a child's brain. Patients and methods A neoadjuvant phase 2 and a single arm pilot trial were initiated to investigate the efficacy and toxicity of an intense multidrug regimen before radiotherapy in 147 patients aged between 3 and 29; 9 years with medulloblastoma (94), malignant glioma (22), ependymoma (21), and stPNET (10). They were treated with one or two cycles consisting of procarbazi…

Rare sacral space-occupying lesions, their surgical management and reconstructive measures involved

1988

Nine cases of space-occupying lesions of the sacral bone are presented. The problems of the clinical diagnosis, which in many cases comes too late, are discussed together with the indications for surgical treatment in this special group of tumours. The main clues are provided by the changes in the X-rays as well as the more modern imaging techniques (CT and MRI). The surgical technique aims at a most radical tumour removal with preservation of the sacral nerve roots, after which stabilisation of the sometimes weakened pelvic girdle may be necessary. The good prospects of complete removal of these tumours of the sacrum with satisfactory results seem to be very little known and justifies furt…

Ependymoma associated protein Zfta is expressed in immature ependymal cells but is not essential for ependymal development in mice

2022

AbstractThe fusion protein of uncharacterised zinc finger translocation associated (ZFTA) and effector transcription factor of tumorigenic NF-κB signalling, RELA (ZFTA-RELA), is expressed in more than two-thirds of supratentorial ependymoma (ST-EPN-RELA), but ZFTA’s expression profile and functional analysis in multiciliated ependymal (E1) cells have not been examined. Here, we showed the mRNA expression of mouse Zfta peaks on embryonic day (E) 17.5 in the wholemount of the lateral walls of the lateral ventricle. Zfta was expressed in the nuclei of FoxJ1-positive immature E1 (pre-E1) cells in E18.5 mouse embryonic brain. Interestingly, the transcription factors promoting ciliogenesis (cilia…

Aspartate aminotransferase in brain tissue cultures

1969

Intermittent Horner's syndrome on alternate sides: a hint for locating spinal lesions.

1980

A patient with a cervical intramedullary ependymoma is described who exhibited a Horner's syndrome on alternate sides. Pharmacological study demonstrated damage to the pre-ganglionic sympathetic neurons. Disregarding cases with the Shy-Drager syndrome, three comparable case reports have been found in the literature where the fluctuating expression of Horner's syndrome, alternating from one side to the other, was attributable to a lesion of the cervical spinal cord. In contrast to the present patient, the pharmacological responses of these patients indicated a lesion of the central sympathetic pathway.

Novel clinical insights into spinal hemangioblastoma in adults: a systematic review

2021

ABSTRACT Background Hemangioblastomas are well vascularized, benign CNS tumors and the third most common primary spinal cord tumor after astrocytoma/ependymoma, occurring sporadically or as a part of an autosomal dominant von Hippel-Lindau disease in which tumors are often multiple and prone to relapse. Spinal hemangioblastomas are commonly located in the cervical cord and associated with a syrinx formation. Due to location and growth trends, they may cause significant neurological deficit, impairing patient quality of life. We conducted a systematic review to understand better clinical insights of spinal hemangioblastoma in adults and compare spinal hemangioblastoma versus posterior crania…