Best cases from the AFIP: myxopapillary ependymoma of the sacrum.
Department of Diagnostic Radiology and Nuclear Medicine and the Department of Pathology, Rush University Medical Center, 1653 W Congress Pkwy, Chicago, IL 60612, USA.Radiographics (Impact Factor: 2.73). 11/2006; 26 Suppl 1:S111-6. DOI: 10.1148/rg.26si065020
Article: [Chordoma.][Show abstract] [Hide abstract]
ABSTRACT: To review in the literature, all the epidemiological, clinical, radiological, histological and therapeutic data regarding chordomas as well as various notochordal entities: ecchordosis physaliphora, intradural and intraparenchymatous chordomas, benign notochordal cell tumors, parachordomas and extra-axial chordomas. To identify different types of chordomas, including familial forms, associations with tuberous sclerosis, Ollier's disease and Maffucci's syndrome, forms with metastasis and seeding. To assess the recent data regarding molecular biology and progress in targeted therapy. To compare the different types of radiotherapy, especially protontherapy and their therapeutic effects. To review the largest series of chordomas in their different localizations (skull base, sacrum and mobile spine) from the literature.
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ABSTRACT: Myxopapillary ependymomas rarely present as a primary intrasacral lesion, and extensive sacral osteolysis is unusual. The authors report a case series of 6 patients with these complex tumors causing extensive sacral destruction, who underwent resection, lumbopelvic reconstruction, and fusion. The operative procedure, complications, and outcome are summarized after a mean follow-up of 3.55 years (range 18-80 months).Journal of neurosurgery. Spine 02/2010; 12(2):154-9. DOI:10.3171/2009.9.SPINE08968 · 1.61 Impact Factor
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ABSTRACT: Myxopapillary ependymomas are slow-growing and highly vascular tumours that occur almost exclusively in the cauda equina [Gerber S, Ollivier L, Leclère J, et al. Imaging of sacral tumours. Skeletal Radiol 2008;37:277–89; Shors SM, Jones TA, Jhaveri MD, Huckman MS. Best cases from the AFIP: myxopapillary ependymoma of the sacrum. Radiographics 2006;26:111–6]. Their clinical manifestations are frequently nonspecific, such as low back pain, leg or sacral pain. Lower motor neuron symptoms such as weakness of lower limbs, loss of sensation, and erectile and sphincter dysfunction can be caused by disruption of the cauda equina by a spinal tumour, which is termed cauda equine syndrome [Shors SM, Jones TA, Jhaveri MD, Huckman MS. Best cases from the AFIP: myxopapillary ependymoma of the sacrum. Radiographics 2006;26:111–6].Faecal incontinence as the first symptom of a primary spinal tumour is rare, although it has been reported with lumbar schwannoma of the cauda equina, metastatic spinal deposits, and lumbosacral intraspinal lipomas. Ependymomas are not frequently associated with this symptom, however. Here we report a case of a myxopapillary ependymoma presenting with faecal incontinence and discuss the radiological appearance of myxopapillary ependymomas.European Journal of Radiology Extra 09/2009; DOI:10.1016/j.ejrex.2009.04.001
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