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Sample records for optic nerve sheath

  1. Primary optic nerve sheath meningioma

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    Jeremic, Branislav [International Atomic Energy Agency, Vienna (Austria); Pitz, Susanne (eds.) [University Eye Hospital, Mainz (Germany)

    2008-07-01

    Optic nerve sheath meningioma (ONSM) is a rare tumour. Cases are usually separated into primary ONSM, which arises either intraorbitally or, less commonly, intracanalicularly, and secondary ONSM, which arises intracranially and subsequently invades the optic canal and orbit. This is the first book to cover all important aspects of the diagnosis and treatment of primary ONSM. After a general introduction, individual chapters discuss the clinical presentation, clinical examination and diagnosis, imaging, and histology. Treatment options are then addressed in detail, with special emphasis on external beam radiation therapy, and in particular stereotactic fractionated radiation therapy. The latter has recently produced consistently good results and is now considered the emerging treatment of choice for the vast majority of patients with primary ONSM. This well-illustrated book will prove invaluable to all practitioners who encounter primary ONSM in their clinical work. (orig.)

  2. Optic Nerve Sheath Mechanics in VIIP Syndrome

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    Raykin, Julia; Forte, Taylor E.; Wang, Roy; Feola, Andrew; Samuels, Brian; Myers, Jerry; Nelson, Emily; Gleason, Rudy; Ethier, C. Ross

    2016-01-01

    Visual Impairment Intracranial Pressure (VIIP) syndrome is a major concern in current space medicine research. While the exact pathology of VIIP is not yet known, it is hypothesized that the microgravity-induced cephalad fluid shift increases intracranial pressure (ICP) and drives remodeling of the optic nerve sheath. To investigate this possibility, we are culturing optic nerve sheath dura mater samples under different pressures and investigating changes in tissue composition. To interpret results from this work, it is essential to first understand the biomechanical response of the optic nerve sheath dura mater to loading. Here, we investigated the effects of mechanical loading on the porcine optic nerve sheath.Porcine optic nerves (number: 6) were obtained immediately after death from a local abattoir. The optic nerve sheath (dura mater) was isolated from the optic nerve proper, leaving a hollow cylinder of connective tissue that was used for biomechanical characterization. We developed a custom mechanical testing system that allowed for unconfined lengthening, twisting, and circumferential distension of the dura mater during inflation and under fixed axial loading. To determine the effects of variations in ICP, the sample was inflated (0-60 millimeters Hg) and circumferential distension was simultaneously recorded. These tests were performed under variable axial loads (0.6 grams - 5.6 grams at increments of 1 gram) by attaching different weights to one end of the dura mater. Results and Conclusions: The samples demonstrated nonlinear behavior, similar to other soft connective tissue (Figure 1). Large increases in diameter were observed at lower transmural pressures (approximately 0 to 5 millimeters Hg), whereas only small diameter changes were observed at higher pressures. Particularly interesting was the existence of a cross-over point at a pressure of approximately 11 millimeters Hg. At this pressure, the same diameter is obtained for all axial loads applied

  3. The optic nerve sheath on MRI in acute optic neuritis

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    Hickman, S.J. [University College London, NMR Research Unit, Department of Neuroinflammation, Institute of Neurology, London (United Kingdom); Moorfields Eye Hospital, Department of Neuro-Ophthalmology, London (United Kingdom); Miszkiel, K.A. [National Hospital for Neurology and Neurosurgery, Lysholm Department of Neuroradiology, London (United Kingdom); Plant, G.T. [Moorfields Eye Hospital, Department of Neuro-Ophthalmology, London (United Kingdom); Miller, D.H. [University College London, NMR Research Unit, Department of Neuroinflammation, Institute of Neurology, London (United Kingdom)

    2005-01-01

    Optic nerve sheath dilatation or gadolinium-enhancement on magnetic resonance imaging in acute optic neuritis have been previously reported but have been thought to be rare occurrences. This study recruited 33 patients with acute unilateral optic neuritis. All had their optic nerves imaged with fat-saturated fast spin-echo (FSE) imaging, and 28 had imaging before and after triple-dose gadolinium-enhanced fat-saturated T{sub 1}-weighted imaging. Follow-up imaging was performed on 20 patients (15 following gadolinium). A dilated subarachnoid space at the anterior end of the symptomatic optic nerve on FSE imaging was seen in 15/33 cases. In three of these cases, dilatation was visible on short-term follow-up. Optic nerve sheath enhancement was seen in 21/28 cases acutely: seven at the anterior end of the lesion only, five at the posterior end only and nine at both ends. Optic sheath enhancement was seen in 13 patients on follow-up. This study suggests that optic nerve sheath dilatation on FSE images and optic nerve sheath enhancement on triple-dose gadolinium-enhanced images are common findings in acute optic neuritis. Optic nerve sheath dilatation may be due to inflammation of the optic nerve, with its associated swelling, interrupting the communication between the subarachnoid space of the diseased optic nerve and the chiasmal cistern. Optic nerve sheath enhancement suggests that meningeal inflammation occurs in optic neuritis, in agreement with pathological studies of both optic neuritis and multiple sclerosis. (orig.)

  4. An unusual radiological presentation of optic nerve sheath meningiom

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    Chameen Samarawickrama

    2016-04-01

    Full Text Available Our report describes an unusual radiological presentation of optic nerve sheath meningioma. The classic radiological appearance of optic nerve thickening with enhancement and calcification within the tumor was not seen; instead, an elongating gadolinium enhancing band-like area adjacent to the superomedial aspect of the left optic nerve sheath was identified. The diagnosis was confirmed on histopathology. Our report adds to the spectrum of presentations of this relatively common clinical entity.

  5. A rare case of bilateral optic nerve sheath meningioma

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    Somen Misra

    2014-01-01

    Full Text Available A 60-year-old female presented with gradual, painless, progressive diminution of vision, and progressive proptosis of left eye since 7 years. Ophthalmological examination revealed mild proptosis and total optic atrophy in the left eye. Magnetic resonance imaging (MRI and computed tomography (CT brain with orbit showed bilateral optic nerve sheath meningioma (ONSM involving the intracranial, intracanalicular, intraorbital part of the optic nerve extending up to optic chiasma and left cavernous sinus.

  6. Microgravity-Driven Optic Nerve/Sheath Biomechanics Simulations

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    Ethier, C. R.; Feola, A.; Myers, J. G.; Nelson, E.; Raykin, J.; Samuels, B.

    2016-01-01

    Visual Impairment and Intracranial Pressure (VIIP) syndrome is a concern for long-duration space flight. Current thinking suggests that the ocular changes observed in VIIP syndrome are related to cephalad fluid shifts resulting in altered fluid pressures [1]. In particular, we hypothesize that increased intracranial pressure (ICP) drives connective tissue remodeling of the posterior eye and optic nerve sheath (ONS). We describe here finite element (FE) modeling designed to understand how altered pressures, particularly altered ICP, affect the tissues of the posterior eye and optic nerve sheath (ONS) in VIIP. METHODS: Additional description of the modeling methodology is provided in the companion IWS abstract by Feola et al. In brief, a geometric model of the posterior eye and optic nerve, including the ONS, was created and the effects of fluid pressures on tissue deformations were simulated. We considered three ICP scenarios: an elevated ICP assumed to occur in chronic microgravity, and ICP in the upright and supine positions on earth. Within each scenario we used Latin hypercube sampling (LHS) to consider a range of ICPs, ONH tissue mechanical properties, intraocular pressures (IOPs) and mean arterial pressures (MAPs). The outcome measures were biomechanical strains in the lamina cribrosa, optic nerve and retina; here we focus on peak values of these strains, since elevated strain alters cell phenotype and induce tissue remodeling. In 3D, the strain field can be decomposed into three orthogonal components, denoted as first, second and third principal strains. RESULTS AND CONCLUSIONS: For baseline material properties, increasing ICP from 0 to 20 mmHg significantly changed strains within the posterior eye and ONS (Fig. 1), indicating that elevated ICP affects ocular tissue biomechanics. Notably, strains in the lamina cribrosa and retina became less extreme as ICP increased; however, within the optic nerve, the occurrence of such extreme strains greatly increased as

  7. Cystic change in primary paediatric optic nerve sheath meningioma.

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    Narayan, Daniel; Rajak, Saul; Patel, Sandy; Selva, Dinesh

    2016-08-01

    Primary optic nerve sheath meningiomas (PONSM) are rare in children. Cystic meningiomas are an uncommon subgroup of meningiomas. We report a case of paediatric PONSM managed using observation alone that underwent cystic change and radiological regression. A 5-year-old girl presented with visual impairment and proptosis. Magnetic resonance (MR) imaging demonstrated a PONSM. The patient was left untreated and followed up with regular MR imaging. Repeat imaging at 16 years of age showed the tumour had started to develop cystic change. Repeat imaging at 21 years of age showed the tumour had decreased in size.

  8. Optic Nerve Sheath Mechanics and Permeability in VIIP Syndrome

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    Raykin, Julia; Best, Lauren; Gleason, Rudy; Mulugeta, Lealem; Myers, Jerry; Nelson, Emily; Samuels, Brian C.; Ethier, C. R.

    2014-01-01

    Long-duration space flight carries the risk of developing Visual Impairment and Intracranial Pressure (VIIP) syndrome, a spectrum of ophthalmic changes including posterior globe flattening, choroidal folds, distension of the optic nerve sheath (ONS), optic nerve kinking and potentially permanent degradation of visual function. The slow onset of VIIP, its chronic nature, and certain clinical features strongly suggest that biomechanical factors acting on the ONS play a role in VIIP. Here we measure several relevant ONS properties needed to model VIIP biomechanics. The ONS (meninges) of fresh porcine eyes (n7) was reflected, the nerve proper was truncated near the sclera, and the meninges were repositioned to create a hollow cylinder of meningeal connective tissue attached to the posterior sclera. The distal end was cannulated, sealed, and pressure clamped (mimicking cerebrospinal fluid [CSF] pressure), while the eye was also cannulated for independent control of intraocular pressure (IOP). The meninges were inflated (CSF pressure cycling 7-50 mmHg) while ONS outer diameter was imaged. In another set of experiments (n4), fluid permeation rate across the meninges was recorded by observing the drainage of an elevated fluid reservoir (30 mmHg) connected to the meninges. The ONS showed behavior typical of soft tissues: viscoelasticity, with hysteresis in early preconditioning cycles and repeatable behavior after 4 cycles, and nonlinear stiffening, particularly at CSF pressures 15 mmHg (Figure). Tangent moduli measured from the loading curve were 372 101, 1199 358, and 2050 379 kPa (mean SEM) at CSF pressures of 7, 15 and 30 mmHg, respectively. Flow rate measurements through the intact meninges at 30mmHg gave a permeability of 1.34 0.46 lmincm2mmHg (mean SEM). The ONS is a tough, strain-stiffening connective tissue that is surprisingly permeable. The latter observation suggests that there could be significant CSF drainage through the ONS into the orbit, likely important

  9. Biomechanics of the Optic Nerve Sheath in VIIP Syndrome

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    Ethier, C. Ross; Raykin, Julia; Gleason, Rudy; Mulugeta, Lealem; Myers, Jerry; Nelson, Emily; Samuels, Brian C.

    2014-01-01

    Long-duration space flight carries the risk of developing Visual Impairment and Intracranial Pressure (VIIP) syndrome, a spectrum of ophthalmic changes including posterior globe flattening, choroidal folds, distension of the optic nerve sheath (ONS), optic nerve kinking and potentially permanent degradation of visual function. The slow onset of VIIP, its chronic nature, and certain clinical features strongly suggest that biomechanical factors acting on the ONS play a role in VIIP. Here we measure several relevant ONS properties needed to model VIIP biomechanics. The ONS (meninges) of fresh porcine eyes (n7) was reflected, the nerve proper was truncated near the sclera, and the meninges were repositioned to create a hollow cylinder of meningeal connective tissue attached to the posterior sclera. The distal end was cannulated, sealed, and pressure clamped (mimicking cerebrospinal fluid [CSF] pressure), while the eye was also cannulated for independent control of intraocular pressure (IOP). The meninges were inflated (CSF pressure cycling 7-50 mmHg) while ONS outer diameter was imaged. In another set of experiments (n4), fluid permeation rate across the meninges was recorded by observing the drainage of an elevated fluid reservoir (30 mmHg) connected to the meninges. The ONS showed behavior typical of soft tissues: viscoelasticity, with hysteresis in early preconditioning cycles and repeatable behavior after 4 cycles, and nonlinear stiffening, particularly at CSF pressures 15 mmHg (Figure). Tangent moduli measured from the loading curve were 372 101, 1199 358, and 2050 379 kPa (mean SEM) at CSF pressures of 7, 15 and 30 mmHg, respectively. Flow rate measurements through the intact meninges at 30mmHg gave a permeability of 1.34 0.46 lmincm2mmHg (mean SEM). The ONS is a tough, strain-stiffening connective tissue that is surprisingly permeable. The latter observation suggests that there could be significant CSF drainage through the ONS into the orbit, likely important

  10. Optic nerve sheath diameter: A novel way to monitor the brain

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    Seelora Sahu

    2017-01-01

    Full Text Available Measurement and monitoring of intracranial pressure is pivotal in management of brain injured patients. As a rapid and easily done bed side measurement, ultrasonography of the optic nerve sheath diameter presents itself as a possible replacement of the conventional invasive methods of intracranial pressure management. In this review we go through the evolution of optic nerve sheath diameter measurement as a novel marker of predicting raised intracranial pressure, the modalities by which it can be measured as well as its correlation with the invasive methods of intracranial pressure monitoring.

  11. The MRI appearance of the optic nerve sheath following fenestration for benign intracranial hypertension

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    Sallomi, D.; Taylor, H.; Hibbert, J.; Sanders, M.D.; Spalton, D.J.; Tonge, K. [Guys and St. Thomas` Hospitals, London (United Kingdom)

    1998-09-01

    Optic nerve fenestration is carried out in cases of severe benign intracranial hypertension. This study aimed to monitor the optic nerve sheath appearances and orbital changes that occur following this procedure. The eight patients were all female with an average age of 37.3 years and a range of 20-58 years. The duration of symptoms was 2-6 years. Symptoms included headaches, diplopia and visual obscurations. Examination revealed severe papilledema. All investigations, including MRI, biochemical and immunological tests, were negative. Patients had fenestration of a 2 mm x 3 mm segment of the medial aspect of the optic nerve sheath. Imaging was obtained with a 1 T MRI machine using a head coil. Coronal, axial and sagittal 3 mm contiguous sections using STIR sequences with TR 4900 ms, IT 150 ms and TE 60 ms were obtained. Five patients showed clinical improvement. The post-operative MRI findings in four of these included a decreased volume of cerebrospinal fluid (CSF) around the optic nerve sheaths and a localized collection of fluid within the orbit. There were no MRI changes in the three patients with no clinical improvement. Decreased CSF volume around the optic nerve and a fluid collection within the orbit may indicate a favorable outcome in optic nerve fenestration. (orig.) With 3 figs., 12 refs.

  12. Primary radiotherapy in progressive optic nerve sheath meningiomas: a long-term follow-up study

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    Saeed, P.; Blank, L.; Selva, D.; Wolbers, J.G.; Nowak, P.J.C.M.; Geskus, R.B.; Weis, E.; Mourits, M.P.; Rootman, J.

    2010-01-01

    Background/aims To report the outcome of primary radiotherapy in patients with progressive optic nerve sheath meningioma (ONSM). Methods The clinical records of all patients were reviewed in a retrospective, observational, multicentre study. Results Thirty-four consecutive patients were included. Tw

  13. Optic Nerve Sheath Diameter Remains Constant during Robot Assisted Laparoscopic Radical Prostatectomy

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    Verdonck, Philip; Kalmar, Alain F.; Suy, Koen; Geeraerts, Thomas; Vercauteren, Marcel; Mottrie, Alex; De Wolf, Andre M.; Hendrickx, Jan F. A.

    2014-01-01

    Background: During robot assisted laparoscopic radical prostatectomy (RALRP), a CO2 pneumoperitoneum (CO2PP) is applied and the patient is placed in a head-down position. Intracranial pressure (ICP) is expected to acutely increase under these conditions. A non-invasive method, the optic nerve sheath

  14. Dural ectasia of the optic nerve sheath: is it always benign?

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    Berker Bakbak

    2009-11-01

    Full Text Available Berker Bakbak1, Hava Dönmez2, Tülay Kansu3, Hayyam Kiratli41Hacettepe University Institute of Neurological Sciences and Psychiatry, Neuro-ophthalmology Unit, Ankara, Turkey; 2Diskapi Yildirim Beyazid Education and Research Hospital Neurology Clinic, Ankara, Turkey; 3Hacettepe University Medical Faculty, Department of Neurology, Neuro-Ophthalmology Unit, Ankara, Turkey; 4Hacettepe University Medical Faculty, Department of Ophthalmology, Ocular Oncology Unit, Ankara, TurkeyAbstract: A 36-year-old woman with a 3-month history of progressive visual loss had papilledema, dilatation of the optic nerve sheaths and normal cerebrospinal fluid pressures. She was diagnosed as dural ectasia of the optic nerve sheaths and surgical decompression was performed. In this case report, severe visual loss is described as a serious complication of this rare disease and the importance of early surgical intervention is emphasized.Keywords: optic nerve, dural ectasia, meningocele

  15. Optic Nerve Sheath as a Novel Mechanical Load on the Globe in Ocular Duction

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    Demer, Joseph L.

    2016-01-01

    Purpose The optic nerve (ON) sheath's role in limiting duction has been previously unappreciated. This study employed magnetic resonance imaging (MRI) to demonstrate this constraint on adduction. Methods High-resolution, surface coil axial MRI was obtained in 11 normal adults, 14 subjects with esotropia (ET) having normal axial length (AL) globe retraction in adduction, suggesting ON tethering. Conclusions Large adduction may exhaust length redundancy in the normally sinuous ON and sheath, so that additional adduction must stretch the sheath and retract or deform the globe. These mechanical effects are most significant in ET with axial myopia, but may also exert traction on the posterior sclera absent strabismus or myopia. Tethering by the ON sheath in adduction is an important, novel mechanical load on the globe. PMID:27082297

  16. Optic pathway glioma associated with orbital rhabdomyosarcoma and bilateral optic nerve sheath dural ectasia in a child with neurofibromatosis-1

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    Nikas, Ioannis; Theofanopoulou, Maria; Lampropoulou, Penelope; Hadjigeorgi, Christiana [Aghia Sophia Children' s Hospital, Imaging Department, Athens (Greece); Pourtsidis, Apostolos; Kosmidis, Helen [Children' s Hospital, A. Kyriakou, Department of Oncology, Athens (Greece)

    2006-11-15

    Neurofibromatosis-1 (NF-1) is a multisystem disorder presenting with a variety of clinical and imaging manifestations. Neural and non-neural tumours, and unusual benign miscellaneous conditions, separately or combined, are encountered in variable locations. We present a 21/2-year-old boy with NF-1 who demonstrated coexisting optic pathway glioma with involvement of the chiasm and optic nerve, orbital alveolar rhabdomyosarcoma and bilateral optic nerve sheath dural ectasia. (orig.)

  17. [Optic nerve sheath meningioma: diagnosis and new treatment options, a case study of monocular blindness during pregnancy].

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    Berete, R; Vignal-Clermont, C; Boissonnet, H; Héran, F; Morax, S

    2006-04-01

    Optic nerve sheath meningiomas are challenging lesions to manage. We report here a case of primary optic nerve sheath meningioma in a pregnant woman with sudden unilateral vision loss. Then we review the current literature on the subject, prognosis factors, and report the results of fractionated radiotherapy and current therapeutic guidelines. Pregnancy may accelerate growth of meningiomas and this diagnosis must be considered in all cases of optic neuropathy in pregnant woman.

  18. Incidence of enhancement of the optic nerve/sheath complex in fat-suppression orbit MRI

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    Lee, Ho Kyu; Yoon, Kwon Ha; Choi, Choong Gon; Suh, Dae Chul [Asan Medical Center, University of Ulsan College of Medicine, Seoul (Korea, Republic of)

    1995-04-15

    To elucidate the incidence of Gd-DTPA enhancement of the optic nerve/sheath complex (ONC) in patients with various ophthalmopathies using fat-suppression MRI. Orbit MRI with fat-suppression technique (ChemSat) was performed in 58 patients with normal and various orbital lesions. The fat-suppression MR was done with and without Gd-DTPA injection in all cases. MR findings were reviewed retrospectively in a blind fashion with respect to presence or absence of contrast enhancement of the ONC. Contrast enhancement of the ONC was seen in 86% (6/7) of cavernous sinus lesions, 80% (8/10) of intraconal lesions excluding the ONC, 57% (16/28) of ONC lesions, 38% (3/8) of ocular lesions, and 2% (1/55) of normal orbits. The ONC enhancement was the most common in optic nerve/sheath tumors (10/10), and pseudotumors (6/6), cavernous sinus dural arteriovenous malformations (3/3) and cavernous sinus thrombosis (2/2), and less frequently seen in optic neuritis (3/14). Enhancement of the ONC may be seen in lesions of the cavernous sinus and orbit other than optic nerve/sheath lesion.

  19. T2-weighted magnetic resonance imaging measurements of optic nerve sheath diameter in dogs with and without presumed intracranial hypertension.

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    Scrivani, Peter V; Fletcher, Daniel J; Cooley, Stacy D; Rosenblatt, Alana J; Erb, Hollis N

    2013-01-01

    Intracranial hypertension is a cause of cerebral ischemia and neurologic deficits in dogs. Goals of this retrospective study were to test interobserver agreement for MRI measurements of optic nerve sheath diameter and associations between optic nerve sheath diameter, signalment data, and presumed intracranial hypertension status in a cohort of dogs. A veterinary radiologist interpreted scans of 100 dogs and dogs were assigned to groups based on presence or absence of at least two MRI characteristics of presumed intracranial hypertension. Two observers who were unaware of group status independently measured optic nerve diameter from transverse T2-weighted sequences. Mean optic nerve sheath diameter for all dogs was 3 mm (1-4 mm). The mean difference between observers was 0.3 mm (limits of agreement, -0.4 and 1.0 mm). There was no correlation between optic nerve sheath diameter and age for either observer (r = -0.06 to 0.00) but a moderate positive correlation was observed between optic nerve sheath diameter and body weight for both observers (r = 0.70-0.76). The 22 dogs with presumed intracranial hypertension weighed less than the 78 dogs without (P = 0.02) and were more often female (P = 0.04). Dogs with presumed intracranial hypertension had a larger ratio of optic nerve sheath diameter to body weight for each observer-side pair (P = 0.01-0.04) than dogs without. Findings indicated that the ratio of MRI optic nerve sheath diameter relative to body weight may be a repeatable predictor of intracranial hypertension in dogs.

  20. Optic Nerve Sheath Diameter Ultrasound and the Diagnosis of Increased Intracranial Pressure.

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    Hylkema, Christopher

    2016-03-01

    Ultrasound has been used for almost 30 years in a wide variety of clinical applications and environments. From the austerity of battlefields to the labor and delivery ward, ultrasound has the ability to give clinicians real-time, noninvasive diagnostic imaging. Ultrasound by emergency physicians (and all nonradiologists) has become more prevalent and has been used for examinations such as the transcranial Doppler to evaluate for stroke, cardiac function, FAST and EFAST examinations for trauma, and now increased intracranial pressure (ICP) via Optic Nerve Sheath Diameter Ultrasound (ONSD). The ONSD is a valid and reliable indicator of ICP.

  1. Primary optic nerve sheath meningiomas: clinical aspects and imaging diagnostic; Meningeoma da bainha do nervo optico: aspectos clinicos e diagnostico por imagem

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    Settanni, F.A.P.; Dumont, P.; Botelho, R.V.; Melo, A.L.; Rabello, J.P.R. [Hospital do Servidor Publico Estadual de Sao Paulo, SP (Brazil)

    1995-03-01

    Primary optic nerve sheath meningiomas are tumors derived from the arachnoid of the sheath nerve. The intra orbital incidence of these tumors is small, and in this case they derive from the arachnoid, not invading the brain tissue. Meningiomas of the optic nerve sheath continue to present many dilemmas to the surgeon who must manage these patients. These tumors behave differently in different age groups: tumors in children and in patients with neurofibromatosis and in the middle age. We present a case of primary optic nerve sheath meningioma. Neuroradiological and characteristic clinic aspects are considered and compared with those of the literature. (author). 13 refs, 3 figs.

  2. Optic Nerve Sheath Diameter: Translating a Terrestrial Focused Technique into a Clinical Monitoring Tool for Spaceflight

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    Mason, Sara; Foy, Millennia; Sargsyan, Ashot; Garcia, Kathleen; Wear, Mary L.; Bedi, Deepak; Ernst, Randy; Van Baalen, Mary

    2015-01-01

    Ultrasonography is increasingly used to quickly measure optic nerve sheath diameter (ONSD) when increased intracranial pressure (ICP) is suspected. NASA Space and Clinical Operations Division has been using ground and on-orbit ultrasound since 2009 as a proxy for ICP in non-acute monitoring for space medicine purposes. In the terrestrial emergency room population, an ONSD greater than 0.59 cm is considered highly predictive of elevated intracranial pressure. However, this cut-off limit is not applicable to the spaceflight setting since over 50% of US Operating Segment (USOS) astronauts have an ONSD greater than 0.60 cm even before launch. Crew Surgeon clinical decision-making is complicated by the fact that many astronauts have history of previous spaceflights. Our data characterize the distribution of baseline ONSD in the astronaut corps, its longitudinal trends in long-duration spaceflight, and the predictive power of this measure related to increased ICP outcomes.

  3. Sonographic Optic Nerve Sheath Diameter as a Screening Tool for Detection of Elevated Intracranial Pressure

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    Afshin Amini

    2013-12-01

    Full Text Available Introduction: Timely diagnosis and treatment of post traumatic, elevated intracranial pressure (EICP, could reduce morbidity and mortality, as well as improve patients’ outcome. This study is trying to evaluate the diagnostic accuracy of sonographic optic nerve sheath diameter (ONSD in detection of EICP. Methods: Sonographic ONSD of patients with head trauma or cerebrovascular accident suspicious for EICP were evaluated by a trained chief resident of emergency medicine, who was blind to the clinical and brain computed tomography scan (BCT findings of patients. Immediately after ultrasonography, BCT was performed and reported by an expert radiologist without awareness from other results of the patients. Finally, ultrasonographic and BCT findings regarding EICP were compared. To evaluate the ability of sonographic ONSD in predicting the BCT findings and obtain best cut-off level, receiver operating characteristic (ROC curve were used. Sensitivity, specificity, positive predictive value (PPV, negative predictive value (NPV, positive likelihood ratio (PLR, and negative likelihood ratio (NLR of sonographic ONSD in determining of EICP was calculated. P < 0.05 was considered to be statistically significant. Results: There were 222 patients (65.3% male, with mean age of 42.2±19.5 years (range: 16-90 years. BCT showed signs of EICP, in 28 cases (12.6%. The means of the ONSD in the patients with EICP and normal ICP were 5.5 ± 0.56 and 3.93 ± 0.53 mm, respectively (P<0.0001. ROC curve demonstrated that the best cut off was 4.85 mm. Sensitivity, specificity, PPV, NPV, PLR, and NLR of ONSD for prediction of EICP were 96.4%, 95.3%, 72.2%, 98.9%, 20.6, and 0.04, respectively. Conclusion: Sonographic diameter of optic nerve sheath could be considered as an available, accurate, and noninvasive screening tool in determining the elevated intracranial pressure in cases with head trauma or cerebrovascular accident. 

  4. Noninvasive and quantitative intracranial pressure estimation using ultrasonographic measurement of optic nerve sheath diameter

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    Wang, Li-juan; Yao, Yan; Feng, Liang-shu; Wang, Yu-zhi; Zheng, Nan-nan; Feng, Jia-chun; Xing, Ying-qi

    2017-01-01

    We aimed to quantitatively assess intracranial pressure (ICP) using optic nerve sheath diameter (ONSD) measurements. We recruited 316 neurology patients in whom ultrasonographic ONSD was measured before lumbar puncture. They were randomly divided into a modeling and a test group at a ratio of 7:3. In the modeling group, we conducted univariate and multivariate analyses to assess associations between ICP and ONSD, age, sex, BMI, mean arterial blood pressure, diastolic blood pressure. We derived the mathematical function “Xing & Wang” from the modelling group to predict ICP and evaluated the function in the test group. In the modeling group, ICP was strongly correlated with ONSD (r = 0.758, p Watson value = 1.94). In the test group, a significant correlation was found between the observed and predicted ICP (r = 0.76, p < 0.001). Bland-Altman analysis yielded a mean difference between measurements of −0.07 ± 41.55 mmH2O. The intraclass correlation coefficient and its 95%CIs for noninvasive ICP assessments using our prediction model was 0.86 (0.79–0.90). Ultrasonographic ONSD measurements provide a potential noninvasive method to quantify ICP that can be conducted at the bedside. PMID:28169341

  5. Controversies: Optic nerve sheath fenestration versus shunt placement for the treatment of idiopathic intracranial hypertension

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    Arielle Spitze

    2014-01-01

    Full Text Available Background: Idiopathic intracranial hypertension (IIH has been increasing in prevalence in the past decade, following the obesity epidemic. When medical treatment fails, surgical treatment options must be considered. However, controversy remains as to which surgical procedure is the preferred surgical option - optic nerve sheath fenestration (ONSF or cerebrospinal fluid (CSF shunting - for the long-term treatment of this syndrome. Purpose: To provide a clinical update of the pros and cons of ONSF versus shunt placement for the treatment of IIH. Design: This was a retrospective review of the current literature in the English language indexed in PubMed. Methods: The authors conducted a PubMed search using the following terms: Idiopathic IIH, pseudotumor cerebri, ONSF, CSF shunts, vetriculo-peritoneal shunting, and lumbo-peritoneal shunting. The authors included pertinent and significant original articles, review articles, and case reports, which revealed the new aspects and updates in these topics. Results: The treatment of IIH remains controversial and lacks randomized controlled clinical trial data. Treatment of IIH rests with the determination of the severity of IIH-related visual loss and headache. Conclusion: The decision for ONSF versus shunting is somewhat institution and surgeon dependent. ONSF is preferred for patients with visual symptoms whereas shunting is reserved for patients with headache. There are positive and negative aspects of both procedures, and a prospective, randomized, controlled trial is needed (currently underway. This article will hopefully be helpful in allowing the reader to make a more informed decision until that time.

  6. Noninvasive intracranial pressure monitoring via optic nerve sheath diameter for robotic surgery in steep Trendelenburg position

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    Shagun Bhatia Shah

    2015-01-01

    Full Text Available Background: Recent reports of increased intracranial pressure (ICP due to steep Trendelenburg (ST position causing neurological deterioration, decreased regional cerebral oxygen saturation and postoperative visual loss after robotic urological and gynecological surgeries led us to consider a simple technique of ICP monitoring. Ours is one of the first instances reported of quantitative noninvasive measurement of increase in ICP with ST position by serial measurement of binocular optic nerve sheath diameter (ONSD in patients undergoing robot assisted urological and gynecological oncosurgery. We tested whether ONSD values rose to above the upper limits of normal and for what length of time they remained elevated. Materials and Methods: Prospective, randomized, interventional, parallel group, active control study conducted on 252 American Society of Anesthesiologists I and II patients. ONSD was measured using 7.5 MHz linear ultrasound probe in supine and Trendelenburg positions. Statistics: Student′s t-test to compare the inter-group mean ONSD and the repetitive t-test for intra-group analysis. Result: Comparison of the mean ONSD values of both groups yielded a 2-tailed significance P <0.01 at all compared time points intra- and post-operatively. In Group-O (open surgery; supine position, the baseline mean bilateral ONSD was 4.36 mm, which did not show any statistically significant change throughout open surgery and postoperative period. On de-docking the robot, 6.2 mm was the mean ONSD value in Group-R (robotic group while 4.3 mm was the corresponding value in control Group-O. Conclusion: ONSD evaluation is a simple, quick, safe, readily available, reliable, cost effective, noninvasive, potential standard of care for screening and monitoring of patients undergoing robotic surgery in ST position.

  7. Intraoral myxoid nerve sheath tumour

    NARCIS (Netherlands)

    Schortinghuis, J; Hille, JJ; Singh, S

    2001-01-01

    A case of an intraoral myxoid nerve sheath tumour of the dorsum of the tongue in a 73-year-old Caucasian male is reported. This case describes the oldest patient with this pathology to date. Immunoperoxidase staining for neuronspecific enolase (NSE) and epithelial membrane antigen (EMA) expression d

  8. Intraoral myxoid nerve sheath tumour

    NARCIS (Netherlands)

    Schortinghuis, J; Hille, JJ; Singh, S

    2001-01-01

    A case of an intraoral myxoid nerve sheath tumour of the dorsum of the tongue in a 73-year-old Caucasian male is reported. This case describes the oldest patient with this pathology to date. Immunoperoxidase staining for neuronspecific enolase (NSE) and epithelial membrane antigen (EMA) expression d

  9. Optic Nerve Sheath Diameter: Translating a Terrestrial Focused Technique Into a Clinical Monitoring Tool for Space Flight

    Science.gov (United States)

    Mason, Sara S.; Foy, Millennia; Sargsyan, Ashot; Garcia, Kathleen; Wear, Mary L.; Bedi, Deepak; Ernst, Randy; Van Baalen, Mary

    2014-01-01

    Emergency medicine physicians recently adopted the use of ultrasonography to quickly measure optic nerve sheath diameter (ONSD) as concomitant with increased intracranial pressure. NASA Space and Clinical Operations Division has been using ground and on-orbit ultrasound capabilities since 2009 to consider this anatomical measure as a proxy for intracranial pressure in the microgravity environment. In the terrestrial emergency room population, an ONSD greater than 0.59 cm is considered highly predictive of elevated intracranial pressure. However, this cut-off limit is not applicable to the spaceflight setting since over 50% of US Operating Segment (USOS) astronauts have an ONSD greater than 0.60 cm even before missions. Crew Surgeon clinical decision-making is complicated by the fact that many astronauts have history of previous spaceflights. Data will be presented characterizing the distribution of baseline ONSD in the astronaut corps, longitudinal trends in-flight, and the predictive power of this measure related to increased intracranial pressure outcomes.

  10. Asymmetric optic nerve sheath diameter as an outcome factor following cranioplasty in patients harboring the 'syndrome of the trephined'

    Directory of Open Access Journals (Sweden)

    Antonio Santos de Araujo Junior

    2013-12-01

    Full Text Available Decompressive craniectomy (DC is gaining an increasing role in the neurosurgical treatment of intractable intracranial hypertension, but not without complications. A rare complication is the “syndrome of the trephined” (ST. It occurs when the forces of gravity overwhelm intracranial pressures, leading the brain to become sunken. Objective To determine the usefulness of asymmetric optic nerve sheath diameter (ONSD as an outcome factor after cranioplasty. Method We followed-up 5 patients submitted to DC and diagnosed with ST. All were submitted to brain MRI to calculate the ONSD. Results Only two patients presented an asymmetric ONSD, being ONSD larger at the site of craniectomy. Surprisingly these patients had a marked neurological improvement after cranioplasty. They became independent a week after and statistically earlier than others. Conclusion It is presumed that the presence of an asymmetric ONSD in trephined patients is an independent factor of good outcome after cranioplasty.

  11. Intrasellar malignant peripheral nerve sheath tumor (MPNST).

    Science.gov (United States)

    Krayenbühl, N; Heppner, F; Yonekawa, Y; Bernays, R L

    2007-02-01

    Intracranial malignant peripheral nerve sheath tumors (MPNST) and intrasellar schwannomas are rare tumors. We describe a case of an intrasellar schwannoma with progression to a MPNST, a finding that, although very rare, extends the differential diagnosis of intrasellar lesions.

  12. Comparison of ultrasonographic measurement of optic nerve sheath diameter (ONSD) versus direct measurement of intracranial pressure (ICP) in traumatic brain injury patients

    OpenAIRE

    2011-01-01

    Aim. To compare ultrasonographic measurement of optic nerve sheath diameter (ONSD) with direct measurement of intracranial pressure (ICP) and evaluate the correlation between them in traumatic brain injury patients. Patients and methods. Twenty traumatic brain injury patients were enrolled in the study. Patients had a median Glasgow Coma Scale (GCS) score of 5. All patients underwent noninvasive measurement of ICP by ultrasonographic measurement of ONSD and invasive ICP measurement using ...

  13. Standard-Fractionated Radiotherapy for Optic Nerve Sheath Meningioma: Visual Outcome Is Predicted by Mean Eye Dose

    Energy Technology Data Exchange (ETDEWEB)

    Abouaf, Lucie [Neuro-Ophthalmology Unit, Pierre-Wertheimer Hospital, Hospices Civils de Lyon, Lyon (France); Girard, Nicolas [Radiotherapy-Oncology Department, Lyon Sud Hospital, Hospices Civils de Lyon, Lyon (France); Claude Bernard University, Lyon (France); Lefort, Thibaud [Neuro-Radiology Department, Pierre-Wertheimer Hospital, Hospices Civils de Lyon, Lyon (France); D' hombres, Anne [Claude Bernard University, Lyon (France); Tilikete, Caroline; Vighetto, Alain [Neuro-Ophthalmology Unit, Pierre-Wertheimer Hospital, Hospices Civils de Lyon, Lyon (France); Claude Bernard University, Lyon (France); Mornex, Francoise, E-mail: francoise.mornex@chu-lyon.fr [Claude Bernard University, Lyon (France)

    2012-03-01

    Purpose: Radiotherapy has shown its efficacy in controlling optic nerve sheath meningiomas (ONSM) tumor growth while allowing visual acuity to improve or stabilize. However, radiation-induced toxicity may ultimately jeopardize the functional benefit. The purpose of this study was to identify predictive factors of poor visual outcome in patients receiving radiotherapy for ONSM. Methods and Materials: We conducted an extensive analysis of 10 patients with ONSM with regard to clinical, radiologic, and dosimetric aspects. All patients were treated with conformal radiotherapy and subsequently underwent biannual neuroophthalmologic and imaging assessments. Pretreatment and posttreatment values of visual acuity and visual field were compared with Wilcoxon's signed rank test. Results: Visual acuity values significantly improved after radiotherapy. After a median follow-up time of 51 months, 6 patients had improved visual acuity, 4 patients had improved visual field, 1 patient was in stable condition, and 1 patient had deteriorated visual acuity and visual field. Tumor control rate was 100% at magnetic resonance imaging assessment. Visual acuity deterioration after radiotherapy was related to radiation-induced retinopathy in 2 patients and radiation-induced mature cataract in 1 patient. Study of radiotherapy parameters showed that the mean eye dose was significantly higher in those 3 patients who had deteriorated vision. Conclusions: Our study confirms that radiotherapy is efficient in treating ONSM. Long-term visual outcome may be compromised by radiation-induced side effects. Mean eye dose has to be considered as a limiting constraint in treatment planning.

  14. Can sonographic measurement of optic nerve sheath diameter be used to detect raised intracranial pressure in patients with tuberculous meningitis? A prospective observational study

    Directory of Open Access Journals (Sweden)

    Shruti V Sangani

    2015-01-01

    Full Text Available CNS Tuberculosis can manifest as meningitis, arachnoiditis and a tuberculoma. The rupture of a tubercle into the subarachnoid space leads to Tuberculosis Meningitis (TBME; the resulting hypersensitivity reaction can lead to an elevation of the intracranial pressure and hydrocephalus. While bedside optic nerve sheath diameter (ONSD ultrasonography (USG can be a sensitive screening test for elevated intracranial pressure in adult head injury, little is known regarding ONSD measurements in Tuberculosis Meningitis. Objectives: The aim of this study was to determine whether patients with TBME had dilation of the optic nerve sheath, as detected by ocular USG performed in the emergency department (ED. Materials and Methods: We conducted a prospective, observational study on adult ED patients with suspected TBME. Patients underwent USG measurements of the optic nerve followed by MRI. The ONSD was measured 3 mm behind the globe in each eye. MRI evidence of basilar meningeal enhancement and any degree of hydrocephalus was suggestive of TBME. Those patients without evidence of hydrocephalus subsequently underwent a lumbar puncture to confirm the diagnosis. Exclusion criteria were age less than 18 and obvious ocular pathology. In total, the optic nerve sheath diameters of 25 adults with confirmed TBME were measured. These measurements were compared with 120 control patients. Results: The upper limit of normal ONSD was 4.37 mm in control group. Those patients with TBME had a mean ONSD of 5.81 mm (SD 0.42. These results confirm that patients with tuberculosis meningitis have an ONSD in excess of the control data (P < 0.001. Conclusion: The evaluation of the ONSD is a simple non-invasive and potentially useful tool in the assessment of adults suspected of having TBME.

  15. Effect of Positive End-Expiratory Pressure on the Sonographic Optic Nerve Sheath Diameter as a Surrogate for Intracranial Pressure during Robot-Assisted Laparoscopic Prostatectomy: A Randomized Controlled Trial

    Science.gov (United States)

    Chin, Ji-Hyun; Kim, Wook-Jong; Lee, Joonho; Han, Yun A.; Lim, Jinwook; Hwang, Jai-Hyun; Cho, Seong-Sik

    2017-01-01

    Background Positive end-expiratory pressure (PEEP) can increase intracranial pressure. Pneumoperitoneum and the Trendelenburg position are associated with an increased intracranial pressure. We investigated whether PEEP ventilation could additionally influence the sonographic optic nerve sheath diameter as a surrogate for intracranial pressure during pneumoperitoneum combined with the Trendelenburg position in patients undergoing robot-assisted laparoscopic prostatectomy. Methods After anesthetic induction, 38 patients were randomly allocated to a low tidal volume ventilation (8 ml/kg) without PEEP group (zero end-expiratory pressure [ZEEP] group, n = 19) or low tidal volume ventilation with 8 cmH2O PEEP group (PEEP group, n = 19). The sonographic optic nerve sheath diameter was measured prior to skin incision, 5 min and 30 min after pneumoperitoneum and the Trendelenburg position, and at the end of surgery. The study endpoint was the difference in the sonographic optic nerve sheath diameter 5 min after pneumoperitoneum and the Trendelenburg position between the ZEEP and PEEP groups. Results Optic nerve sheath diameters 5 min after pneumoperitoneum and the Trendelenburg position did not significantly differ between the groups [least square mean (95% confidence interval); 4.8 (4.6–4.9) mm vs 4.8 (4.7–5.0) mm, P = 0.618]. Optic nerve sheath diameters 30 min after pneumoperitoneum and the Trendelenburg position also did not differ between the groups [least square mean (95% confidence interval); 4.5 (4.3–4.6) mm vs 4.5 (4.4–4.6) mm, P = 0.733]. Conclusions An 8 cmH2O PEEP application under low tidal volume ventilation does not induce an increase in the optic nerve sheath diameter during pneumoperitoneum combined with the steep Trendelenburg position, suggesting that there might be no detrimental effects of PEEP on the intracranial pressure during robot-assisted laparoscopic prostatectomy. Trial Registration ClinicalTrial.gov NCT02516566 PMID:28107408

  16. A rare case of malignant peripheral nerve sheath tumour

    Directory of Open Access Journals (Sweden)

    Anita Harry, Nirankumar Samuel, Vigil TD

    2014-04-01

    Full Text Available Malignant Peripheral Nerve Sheath Tumours are tumours of ectomesenchymal origin often originating from major nerves or their nerve sheaths, they are commonly found in patients with neurofibromatosis-1 though sporadic cases have been reported. We report a rare sporadic case of MPNST in a 20 year old patient arising from the spinal accessory nerve.

  17. Injection inside the paraneural sheath of the sciatic nerve

    DEFF Research Database (Denmark)

    Andersen, Henning Lykke; Andersen, Sofie L; Tranum-Jensen, Jørgen

    2012-01-01

    There exists little anatomic knowledge regarding the structure and sonographic features of the sheath enveloping the sciatic nerve in the popliteal fossa. We investigated the spread of an injection inside the sheath to (1) determine whether the sheath is a structure distinct from the nerve or part...

  18. Retrospective analysis of oral peripheral nerve sheath tumors in Brazilians

    Directory of Open Access Journals (Sweden)

    Juliana Tito Salla

    2009-03-01

    Full Text Available Traumatic neuroma, neurofibroma, neurilemmoma, palisaded encapsulated neuroma and malignant peripheral nerve sheath tumor (MPNST are peripheral nerve sheath tumors and present neural origin. The goal of this study was to describe the epidemiological data of oral peripheral nerve sheath tumors in a sample of the Brazilian population. Biopsies requested from the Oral Pathology Service, School of Dentistry, Federal University of Minas Gerais (MG, Brazil, between 1966 and 2006 were evaluated. Lesions diagnosed as peripheral nerve sheath tumors were submitted to morphologic and to immunohistochemical analyses. All cases were immunopositive to the S-100 protein. Thirty-five oral peripheral nerve sheath tumors were found, representing 0.16% of all lesions archived in the Oral Pathology Service. Traumatic neuroma (15 cases most frequently affected the mental foramen. Solitary neurofibroma (10 cases was more frequently observed in the palate. Neurofibroma associated with neurofibromatosis type I (2 cases was observed in the gingival and alveolar mucosa. Neurilemmoma (4 cases was more commonly observed in the buccal mucosa. Malignant peripheral nerve sheath tumors (3 cases occurred in the mandible, palate, and tongue. Palisaded encapsulated neuroma (1 case occurred in the buccal mucosa. The data confirmed that oral peripheral nerve sheath tumors are uncommon in the oral region, with some lesions presenting a predilection for a specific gender or site. This study may be useful in clinical dentistry and oral pathology practice and may be used as baseline data regarding oral peripheral nerve sheath tumors in other populations.

  19. High-resolution CT of lesions of the optic nerve

    Energy Technology Data Exchange (ETDEWEB)

    Peyster, R.G.; Hoover, E.D.; Hershey, B.L.; Haskin, M.E.

    1983-05-01

    The optic nerves are well demonstrated by high-resolution computed tomography. Involvement of the optic nerve by optic gliomas and optic nerve sheath meningiomas is well known. However, nonneoplastic processes such as increased intracranial pressure, optic neuritis, Grave ophthalmopathy, and orbital pseudotumor may also alter the appearance of the optic nerve/sheath on computed tomography. Certain clinical and computed tomographic features permit distinction of these nonneoplastic tumefactions from tumors.

  20. Malignant peripheral nerve sheath tumour of penis.

    Science.gov (United States)

    Kaur, J; Madan, R; Singh, L; Sharma, D N; Julka, P K; Rath, G K; Roy, S

    2015-04-01

    Malignant peripheral nerve sheath tumour (MPNST) is a rare variety of soft tissue sarcoma that originates from Schwann cells or pluripotent cells of neural crest origin. They have historically been difficult tumours to diagnose and treat. Surgery is the mainstay of treatment with a goal to achieve negative margins. Despite aggressive surgery and adjuvant therapy, the prognosis of patients with MPNST remains poor. MPNST arising from penis is a very rare entity; thus, it presents a diagnostic and therapeutic challenge. We present a case of penile MPNST in a 38-year-old man in the absence of neurofibromatosis treated with surgery followed by post-operative radiotherapy to a dose of 60 Gray in 30 fractions and adjuvant chemotherapy with ifosfamide and adriamycin.

  1. Malignant Peripheral Nerve Sheath Tumour of the Maxilla

    Directory of Open Access Journals (Sweden)

    Puja Sahai

    2014-01-01

    Full Text Available A 38-year-old man was diagnosed with malignant peripheral nerve sheath tumour of the maxilla. He was treated with total maxillectomy. Histopathological examination of the resected specimen revealed a close resection margin. The tumour was of high grade with an MIB-1 labelling index of almost 60%. At six weeks following the surgery, he developed local tumour relapse. The patient succumbed to the disease at five months from the time of diagnosis. The present report underlines the locally aggressive nature of malignant peripheral nerve sheath tumour of the maxilla which necessitates an early therapeutic intervention. A complete resection with clear margins is the most important prognostic factor for malignant peripheral nerve sheath tumour in the head and neck region. Adjuvant radiotherapy may be considered to improve the local control. Future research may demarcate the role of targeted therapy for patients with malignant peripheral nerve sheath tumour.

  2. 超声评估中国健康成年人视神经鞘直径%Ultrasonographic Evaluation of Optic Nerve Sheath Diameter among Healthy Chinese Adults

    Institute of Scientific and Technical Information of China (English)

    王丽娟; 冯良枢; 姚燕; 邓方; 王玉芝; 冯加纯; 邢英琦

    2016-01-01

    The aim of the work described here was to establish the range for optic nerve sheath diameter (ONSD) and potential factors influencing ONSD in healthy Chinese adults. Both ONSDs were measured twice in the sagittal and transversal planes by two observers. The final ONSD value for each participant was the average of 16 measurements of both eyes. The ONSD range (N=3680) among 230 participants was 2.65~4.30 mm. The upper ONSD limit was lower than those in previous studies in Caucasian and African samples. Simple linear regression analyses revealed that the ONSD was correlated with sex, body mass index and waistline and head circumference. After adjustment for potential confounds between these factors, sex (coefficient=0.225,P<0.001) and body mass index (coefficient=0.042,P<0.001) were independently associated with ONSD. Underweight women had the smallest ONSD. These results suggest that racial, sex, and body mass index differences should be noted when assessing ultrasonographic criteria.%目的:确定中国正常成人视神经鞘直径(optic nerve sheath diameter,ONSD)的参考值范围,并且探索与其相关的影响因素。方法以2013年1月-2014年1月健康体检的成年人作为研究对象,每位受试者的双眼视神经鞘的矢状位和横断面均由两位医生分别测量两次。每位受试者ONSD的最终报告值是双眼视神经鞘的16个测量值的平均值。结果共有230例受试者,获得3680个ONSD测量值,范围为2.65~4.30 mm。ONSD的上限值低于以往白种人及黑人的研究结果。简单线性回归分析发现ONSD与性别、体质指数(body mass index,BMI)、腰围、头围相关。在调整了其他可能的影响因素后发现性别(偏回归系数0.189,P<0.001)、BMI(偏回归系数0.032,P<0.001)是ONSD的独立影响因素。低体重组的女性ONSD最小。结论在确立ONSD的正常值标准时应考虑种族、性别、BMI的差别。

  3. [Transformation of trigeminal nerve tumor into malignant peripheral nerve sheath tumor (MPNST)].

    Science.gov (United States)

    Nenashev, E A; Cherekaev, V A; Kadasheva, A B; Kozlov, A V; Rotin, D L; Stepanian, M A

    2012-01-01

    Malignant peripheral nerve sheath tumor (MPNST) is a rare entity with only 18 cases of trigeminal nerve MPNST described by now and only one report of malignant transformation of trigeminal nerve tumor into MPNST published up to date. One more case of malignant transformation of trigeminal nerve (1st division) tumor into MPNST is demonstrated.

  4. Malignant Peripheral Nerve Sheath Tumor: MRI and CT Findings

    Directory of Open Access Journals (Sweden)

    K. O. Kragha

    2015-01-01

    important in its diagnosis. A rare case of MPNST that produced urinary retention and bowel incontinence is presented that may aid clinicians in the diagnosis of this rare clinical entity. Motor weakness, central enhancement, and immunohistochemistry may assist in the diagnosis of MPNST and differentiation between benign peripheral nerve sheath tumor (BPNST and MPNST.

  5. Malignant peripheral nerve sheath tumour of the bladder associated with neurofibromatosis I.

    LENUS (Irish Health Repository)

    O'Brien, Julie

    2008-12-01

    Neurofibromatosis is a hamartomatous disorder of autonomic peripheral nerve sheaths associated with peripheral nerve sheath tumours. Most tumours are neurofibromas; however, the genitourinary system is rarely involved. We present a rare case of a nerve sheath tumour of the bladder in a young patient, which was discovered to be malignant.

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  6. File list: His.Neu.50.AllAg.Nerve_Sheath_Neoplasms [Chip-atlas[Archive

    Lifescience Database Archive (English)

    Full Text Available His.Neu.50.AllAg.Nerve_Sheath_Neoplasms mm9 Histone Neural Nerve Sheath Neoplasms h...ttp://dbarchive.biosciencedbc.jp/kyushu-u/mm9/assembled/His.Neu.50.AllAg.Nerve_Sheath_Neoplasms.bed ...

  7. File list: Oth.Neu.05.AllAg.Nerve_Sheath_Neoplasms [Chip-atlas[Archive

    Lifescience Database Archive (English)

    Full Text Available Oth.Neu.05.AllAg.Nerve_Sheath_Neoplasms mm9 TFs and others Neural Nerve Sheath Neop...lasms SRX337965 http://dbarchive.biosciencedbc.jp/kyushu-u/mm9/assembled/Oth.Neu.05.AllAg.Nerve_Sheath_Neoplasms.bed ...

  8. File list: DNS.Neu.10.AllAg.Nerve_Sheath_Neoplasms [Chip-atlas[Archive

    Lifescience Database Archive (English)

    Full Text Available DNS.Neu.10.AllAg.Nerve_Sheath_Neoplasms mm9 DNase-seq Neural Nerve Sheath Neoplasms... http://dbarchive.biosciencedbc.jp/kyushu-u/mm9/assembled/DNS.Neu.10.AllAg.Nerve_Sheath_Neoplasms.bed ...

  9. File list: Unc.Neu.50.AllAg.Nerve_Sheath_Neoplasms [Chip-atlas[Archive

    Lifescience Database Archive (English)

    Full Text Available Unc.Neu.50.AllAg.Nerve_Sheath_Neoplasms mm9 Unclassified Neural Nerve Sheath Neopla...sms http://dbarchive.biosciencedbc.jp/kyushu-u/mm9/assembled/Unc.Neu.50.AllAg.Nerve_Sheath_Neoplasms.bed ...

  10. Leptomeningeal metastasis of an intradural malignant peripheral nerve sheath tumor.

    Science.gov (United States)

    Stark, Andreas M; Mehdorn, H Maximilian

    2013-08-01

    Malignant peripheral nerve sheath tumors (MPNST) are defined as any malignant tumor arising from or differentiating towards the peripheral nerve sheath. Intradural MPNST metastases are very rare. We report, to our knowledge, the first case of leptomeningeal metastasis of a MPNST to the spine and intracranial space. A 56-year-old woman with primary intradural MPNST of the S1 nerve root developed leptomeningeal metastases as well as brain metastases 19 months after diagnosis. The patient had a history of non-Hodgkins lymphoma for which she had received irradiation to the spine 15 years prior to this presentation. She had no stigmata of neurofibromatosis type 1. Patients with MPNST may also develop leptomeningeal metastases as demonstrated in this patient with intradural post-radiation MPNST.

  11. Ewing sarcoma mimicking a peripheral nerve sheath tumor.

    Science.gov (United States)

    Mitchell, B D; Fox, B D; Viswanathan, A; Mitchell, A H; Powell, S Z; Cech, D A

    2010-10-01

    We describe the first patient with an extradural, extramedullary Ewing's sarcoma tumor mimicking a nerve sheath tumor with no overt evidence of metastasis. A 28-year-old woman with no past medical history presented with a progressive 3-year history of low back pain and right-sided lower extremity radiculopathy after having failed conservative therapies. MRI of the lumbar spine revealed a right-sided enhancing, dumbbell-shaped lesion at the right neural foramen appearing to originate from the L4 nerve root, suspicious for a peripheral nerve sheath tumor or schwannoma. The patient and findings are discussed in the context of the literature, including an update on the relatively recent diagnostic redesignation of the Ewing's sarcoma family tumors.

  12. Support of Nerve Conduction by Respiring Myelin Sheath: Role of Connexons.

    Science.gov (United States)

    Ravera, Silvia; Bartolucci, Martina; Adriano, Enrico; Garbati, Patrizia; Ferrando, Sara; Ramoino, Paola; Calzia, Daniela; Morelli, Alessandro; Balestrino, Maurizio; Panfoli, Isabella

    2016-05-01

    Recently, we have demonstrated that myelin conducts an extramitochondrial oxidative phosphorylation, hypothesizing a novel supportive role for myelin in favor of the axon. We have also hypothesized that the ATP produced in myelin could be transferred thought gap junctions. In this work, by biochemical, immunohistochemical, and electrophysiological techniques, the existence of a connection among myelin to the axon was evaluated, to understand how ATP could be transferred from sheath to the axoplasm. Data confirm a functional expression of oxidative phosphorylation in isolated myelin. Moreover, WB and immunohistochemistry on optic nerve slices show that connexins 32 and 43 are present in myelin and colocalize with myelin basic protein. Interestingly, addition of carbenoxolone or oleamide, two gap junction blockers, causes a decrease in oxidative metabolism in purified myelin, but not in mitochondria. Similar effects were observed on conduction speed in hippocampal Schaffer collateral, in the presence of oleamide. Confocal analysis of optic nerve slices showed that lucifer yellow (that only passes through aqueous pores) signal was found in both the sheath layers and the axoplasma. In the presence of oleamide, but not with oleic acid, signal significantly decreased in the sheath and was lost inside the axon. This suggests the existence of a link among myelin and axons. These results, while supporting the idea that ATP aerobically synthesized in myelin sheath could be transferred to the axoplasm through gap junctions, shed new light on the function of the sheath.

  13. Optic nerve oxygenation

    DEFF Research Database (Denmark)

    Stefánsson, Einar; Pedersen, Daniella Bach; Jensen, Peter Koch

    2005-01-01

    at similar levels of perfusion pressure. The levels of perfusion pressure that lead to optic nerve hypoxia in the laboratory correspond remarkably well to the levels that increase the risk of glaucomatous optic nerve atrophy in human glaucoma patients. The risk for progressive optic nerve atrophy in human...... glaucoma patients is six times higher at a perfusion pressure of 30 mmHg, which corresponds to a level where the optic nerve is hypoxic in experimental animals, as compared to perfusion pressure levels above 50 mmHg where the optic nerve is normoxic. Medical intervention can affect optic nerve oxygen......-oxygenase inhibitor, indomethacin, which indicates that prostaglandin metabolism plays a role. Laboratory studies suggest that carbonic anhydrase inhibitors might be useful for medical treatment of optic nerve and retinal ischemia, potentially in diseases such as glaucoma and diabetic retinopathy. However, clinical...

  14. Optic nerve oxygenation

    DEFF Research Database (Denmark)

    Stefánsson, Einar; Pedersen, Daniella Bach; Jensen, Peter Koch;

    2005-01-01

    at similar levels of perfusion pressure. The levels of perfusion pressure that lead to optic nerve hypoxia in the laboratory correspond remarkably well to the levels that increase the risk of glaucomatous optic nerve atrophy in human glaucoma patients. The risk for progressive optic nerve atrophy in human...... glaucoma patients is six times higher at a perfusion pressure of 30 mmHg, which corresponds to a level where the optic nerve is hypoxic in experimental animals, as compared to perfusion pressure levels above 50 mmHg where the optic nerve is normoxic. Medical intervention can affect optic nerve oxygen......-oxygenase inhibitor, indomethacin, which indicates that prostaglandin metabolism plays a role. Laboratory studies suggest that carbonic anhydrase inhibitors might be useful for medical treatment of optic nerve and retinal ischemia, potentially in diseases such as glaucoma and diabetic retinopathy. However, clinical...

  15. Genetic instability in nerve sheath cell tumors

    DEFF Research Database (Denmark)

    Rogatto, Silvia Regina; Casartelli, Cacilda; Rainho, Claudia Aparecida

    1995-01-01

    After in vitro culture, we analyzed cytogenetically four acoustic nerve neurinomas, one intraspinal neurinoma and one neurofibroma obtainedfrom unrelated patients. Monosomy of chromosomes 22 and 16 was an abnormality common to all cases, followed in frequency by loss of chromosomes 18 (three cases......) and chromosomes 8, 17 and 19 (two cases). Trisomy of chromosome 20 was also detected in two cases. Structural rearrangements were detected at low frequencies, with del(10)(p12) being present in two cases. In addition, we observed cell subpopulations showing a certain degree of genetic instability, reflected...

  16. Malignant Peripheral Nerve Sheath Tumor - A Case Report

    Directory of Open Access Journals (Sweden)

    Anju N Duttargi

    2007-01-01

    Full Text Available Malignant Peripheral Nerve Sheath Tumor [MPNST] is an extremely rare tumor affecting the oral cavity. It refers to sarcomas that arise from nerve or display features of neural differentiation. Here we present a case of 30-year old male patient with MPNST of right side of the mandible. There was a family history of neurotibromatosis in this case. Histologically, pleomorphic spindle cells with wavy nuclei, light stained cytoplasm, and mitotic activity were observed. The clinical presentation, radiological findings, and light microscopic findings are described in detail. The criteria for diagnosing these tumors and recent advances for diagnosis have also been highlighted.

  17. Differential expression of angiogenic factors in peripheral nerve sheath tumors.

    Science.gov (United States)

    Wasa, Junji; Nishida, Yoshihiro; Suzuki, Yoshitaka; Tsukushi, Satoshi; Shido, Yoji; Hosono, Kozo; Shimoyama, Yoshie; Nakamura, Shigeo; Ishiguro, Naoki

    2008-01-01

    It is difficult to differentiate some malignant peripheral nerve sheath tumors (MPNST) from benign peripheral nerve sheath tumors (BPNST) histologically, and to predict the clinical outcome of patients with MPNST. In this study, the expression of VEGF and MVD were evaluated immunohistochemically in 22 cases of MPNST, 14 of neurofibroma and 19 of schwannoma and correlation of the staining grade of VEGF or MVD and the various clinical factors were analyzed, and statistically evaluated. Levels of VEGF mRNA expression were also determined with real-time RT-PCR. Statistically higher positive staining for VEGF was observed in MPNST compared to neurofibroma (P=0.004) and schwannoma (PMPNST showed higher VEGF positive staining than neurofibroma. Moreover, high VEGF expression statistically correlated with the poor prognosis of the patients with MPNST (P=0.015). Although MVD in MPNST was significantly higher than that in neurofibroma (P=0.038) and schwannoma (PMPNST. Although VEGF mRNA expression tended to be higher in MPNST compared to neurofibroma, the difference was not significant. Levels of VEGF protein expression serve as a novel diagnostic and prognostic tools for peripheral nerve sheath tumors.

  18. Novel Therapeutic Development of NF1-Associated Malignant Peripheral Nerve Sheath Tumor (MPNST)

    Science.gov (United States)

    2016-08-01

    AWARD NUMBER: W81XWH-15-1-0124 TITLE: Novel Therapeutic Development of NF1-Associated Malignant Peripheral Nerve Sheath Tumor (MPNST...Nerve Sheath Tumor (MPNST) 5b. GRANT NUMBER W81XWH-15-1-0124 5c. PROGRAM ELEMENT NUMBER 6. AUTHOR(S) 5d. PROJECT NUMBER Ping Chi, MD, PhD 5e. TASK...that affects approximately 1 in 3000 people. Although multiple defects can arise, malignant peripheral nerve sheath tumor (MPNST) represents the most

  19. Extratemporal Malignant Nerve Sheath Tumor of Facial Nerve with Coexistent Intratemporal Neurofibroma Mimicking Malignant Intratemporal Extension

    Directory of Open Access Journals (Sweden)

    Mitsuhiko Nakahira

    2015-01-01

    Full Text Available We present an extremely unusual case of an extratemporal facial nerve malignant peripheral nerve sheath tumor (MPNST arising from preexistent intratemporal neurofibroma, illustrating a difficulty in discriminating between perineural spread of the MPNST and the preexistent intratemporal neurofibroma on preoperative radiographic images. The most interesting point was that preoperative CT scan and MR images led to misinterpretation that MPNST extended proximally along the facial nerve canal. It is important to recognize that the intratemporal perineural spread of neurofibromas and MPNST share common imaging characteristics. This is the first report (to our knowledge of these 2 lesions coexisting in the facial nerve, leading to misinterpretation on preoperative images.

  20. Comparative Oncogenomics for Peripheral Nerve Sheath Cancer Gene Discovery

    Science.gov (United States)

    2015-06-01

    pathway is often dysregulated (7-15). This can occur via multiple mechanisms including CDK4 overexpression, a loss of Rb expression or inappropriate...Malignant transformation of neurofibromas in neurofibromatosis 1 is associated with CDKN2A/ p16 inactivation. Am J Pathol. 1999;155:1879-84. 10. Birindelli S...nerve sheath tumors. Lab Invest. 2001;81:833-44. 11. Perry A, Kunz SN, Fuller CE, Banerjee R, Marley EF, Liapis H, et al. Differential NF1, p16 , and

  1. Malignant peripheral nerve sheath tumor with divergent differentiation

    Directory of Open Access Journals (Sweden)

    Suresh T

    2009-01-01

    Full Text Available A malignant peripheral nerve sheath tumor (MPNST is an uncommon spindle cell sarcoma accounting for approximately 5% of all soft tissue sarcomas. A 55-year-old female with a right suprarenal tumor showed MPNST with additional foci of epithelioid, rhabdomyoblastic, osteogenic and lipogenic differentiation. Although the capacity of MPNST to undergo epithelioid, rhabdomyoblastic, osteogenic and very rarely lipogenic differentiation is reported in literature, the occurrence of all these differentiation in one case has not been described in literature before. To the best of our knowledge, this is only the second MPNST case with lipomatous differentiation

  2. Malignant Peripheral Nerve Sheath Tumour: CT and MRI Findings

    Directory of Open Access Journals (Sweden)

    Massimiliano Sperandio

    2013-01-01

    Full Text Available Malignant peripheral nerve sheath tumour (MPNST is extremely rare malignancy in the general population, occurring more frequently in patients with Neurofibromatosis type 1 (NF1. In the literature five cases of MPNST arising from the parapharyngeal space (PPS in patients without neurofibromatosis have been reported. We report imaging techniques in a patient with MPNST in the PPS, who had neither a family history nor sign of NF1. Computed tomography (CT scan and magnetic resonance imaging (MRI were performed for a correct therapeutic planning. CT and MRI findings were correlated with hystopathological diagnosis.

  3. Malignant peripheral nerve sheath tumour: An elusive diagnosis

    Directory of Open Access Journals (Sweden)

    Patil Karthikeya

    2007-01-01

    Full Text Available Malignant peripheral nerve sheath tumour (MPNST also termed as spindle cell malignancy of the peripheral nerve Schwann cells or neurogenic sarcoma, represents approximately 10% of all soft tissue sarcomas. This tumour is usually found in the lower extremities and only 10-12% of all lesions occur in the head and neck region, which makes it a rare entity. The diagnosis of MPNST has been described as one of the most difficult and elusive diagnosis in the soft tissue diseases because of its non-specific presentation both clinically and histopathologically. This was overcome by the use of immunohistochemistry. A case of MPNST of the left maxillary antrum in a 45 -year -old male patient is reported.

  4. Study of malignant peripheral nerve sheath tumor in cerebellopontine angle.

    Science.gov (United States)

    Hong, WenMing; Cheng, HongWei; Wang, XiaoJie; Hu, XiaoPeng; Feng, ChunGuo

    2014-01-01

    Malignant peripheral nerve sheath tumors (MPNSTs) are very rare soft tissue sarcomas, usually arising from somatic soft tissues or peripheral nerves. Primary MPNST of the cerebellopontine angle is extremely rare, with only a single case reported so far. Here, we report an unusual case of MPNST in cerebellopontine angle in a 25-year-old man presented with dizziness, left facial numbness, and tinnitus. After hospitalization, the tumor was treated with complete surgical excision followed by adjuvant chemotherapy and radiotherapy. Histologically, the tumor showed malignant spindle cells, which were with focal S-100 positivity on immunohistochemistry, and a diagnosis of the MPNST was made. This case is being reported for its rarity and presence in cerebellopontine and illustrated the difficulties in the diagnosis and treatment of MPNST, which to the best of our knowledge, has not been described before in the soft tissue sarcomas.

  5. Primary malignant peripheral nerve sheath tumor at unusual location

    Directory of Open Access Journals (Sweden)

    Souvagya Panigrahi

    2013-01-01

    Full Text Available Malignant peripheral nerve sheath tumor (MPNST is a rare soft tissue sarcoma. Most arise in association with major nerve trunks. Their most common anatomical sites are the proximal portions of the upper and lower extremities and the trunk. MPNSTs have rarely been reported in literature to occur in other unusual body parts. We review all such cases reported till now in terms of site of origin, surgical treatment, adjuvant therapy and outcome and shortly describe our experience with two of these cases. Both of our case presented with lump at unusual sites resembling neurofibroma, one at orbitotemporal area and other in the paraspinal region with characteristic feature of neurofibroma with the exception that both had very short history of progression. They underwent gross total removal of the tumor with adjuvant radiotherapy postoperatively. At 6-month follow-up both are doing well with no evidence of recurrence.

  6. Comparative analysis of CT and pathological findings of peripheral nerve sheath tumors

    Institute of Scientific and Technical Information of China (English)

    张雪林; 王晓琪; 邱士军

    2003-01-01

    Objective: To improve the qualitative diagnosis of peripheral nerve sheath tumors by computed tomography (CT). Methods: CT findings of 64 cases of pathologically confirmed nerve sheath tumors were compared with the pathological findings of the tumors. Results: Low density of the tumors shown in plain CT images was related to dominating reticular structure in the tumor as found pathologically. Tumors with intact capsule found by pathological findings were shown with smooth margin in CT images. Inhomogeneous density and enhancement of the tumors in CT images was related to tumor necrosis, liquefaction and cystic degeneration, and inhomogeneous enhancement also involved the reticular structure. Conclusion: Nerve sheath tumors are characterized by distribution along the nerves, lower density than that of muscles in plain CT images, and inhomogeneous enhancement in enhanced CT, which can help differentiate nerve sheath tumors from other soft tissue tumors. When nerve sheath tumors lack distinctive CT features, the diagnoses have to depend on their pathological findings.

  7. Malignant peripheral nerve cell sheath tumour of the upper lip: a rare case

    Directory of Open Access Journals (Sweden)

    Joseph Ward

    2010-07-01

    Full Text Available We present the case of a malignant peripheral nerve sheath tumour (MPNST that developed on the upper lip of an 86 year old woman. MPNSTs are highly aggressive sarcomas that very rarely occur in the face. We know of no other reported cases of a malignant peripheral nerve sheath tumour arising from the upper lip.

  8. Optic nerve oxygen tension

    DEFF Research Database (Denmark)

    la Cour, M; Kiilgaard, Jens Folke; Eysteinsson, T

    2000-01-01

    To investigate the influence of acute changes in intraocular pressure on the oxygen tension in the vicinity of the optic nerve head under control conditions and after intravenous administration of 500 mg of the carbonic anhydrase inhibitor dorzolamide....

  9. Composite pheochromocytoma with a malignant peripheral nerve sheath tumor: Case report and review of the literature.

    Science.gov (United States)

    Namekawa, Takeshi; Utsumi, Takanobu; Imamoto, Takashi; Kawamura, Koji; Oide, Takashi; Tanaka, Tomoaki; Nihei, Naoki; Suzuki, Hiroyoshi; Nakatani, Yukio; Ichikawa, Tomohiko

    2016-07-01

    Adrenal tumors with more than one cellular component are uncommon. Furthermore, an adrenal tumor composed of a pheochromocytoma and a malignant peripheral nerve sheath tumor is extremely rare. A composite pheochromocytoma with malignant peripheral nerve sheath tumor in a 42-year-old man is reported here. After adequate preoperative control, left adrenalectomy was performed simultaneously with resection of the ipsilateral kidney for spontaneous rupture of the left adrenal tumor. Pathological findings demonstrated pheochromocytoma and malignant peripheral nerve sheath tumor in a ruptured adrenal tumor. To date, there have been only four reported cases of composite pheochromocytoma with malignant peripheral nerve sheath tumor, so the present case is only the fifth case in the world. Despite the very poor prognosis of patients with pheochromocytoma and malignant peripheral nerve sheath tumors reported in the literature, the patient remains well without evidence of recurrence or new metastatic lesions at 36 months postoperatively. Copyright © 2012. Published by Elsevier Taiwan.

  10. Feline cutaneous nerve sheath tumours: histological features and immunohistochemical evaluations.

    Science.gov (United States)

    Mandara, M T; Fabriani, E; Pavone, S; Pumarola, M

    2013-10-01

    Feline cutaneous nerve sheath tumours (CNSTs) are uncommonly reported in the skin, since they are underestimated relative to the more common spindle cell tumours of soft tissue. In this study, 26 nerve sheath tumours selected from 337 skin neoplasms of cats were examined. Histologically, they were classified into malignant (MPNSTs) and benign tumours (BPNSTs) based on degree of cellular atypia and polymorphism as well as mitotic rate and diffuse necrosis. CPNSTs were tipically characterised by Antoni A pattern, in some cases associated with Antoni B pattern. In the malignant peripheral nerve sheath tumours (MPNSTs) the polymorphism was marked, while it was mild to moderate in the benign forms (BPNSTs). In the MPNSTs the mitotic activity was generally higher than in the BPNSTs. In five cases, including three MPNSTs and two BPNSTs, there were multinucleated giant cells. Necrotic foci occurred in a BPNST and in two MPNSTs, while osseous/chondroid metaplasia was found in two cases. Immunohistochemically, all the tumours showed a marked diffuse vimentin expression. S-100 protein was expressed in 17 cases, including 81.8% of BPNSTs and 57.14% of MPNSTs. Twenty-five tumours expressed NSE and twenty-four cases showed immunoreaction for laminin. Thirteen tumours were positive for GFAP, while five tumours were positive for SMA. PGP 9.5 expression was detected in all cases, except for two MPNSTs. NGFR was expressed in eleven cases, including four MPNSTs and seven BPNSTs. Ki67 was expressed in twenty tumours without any relationship with morphologic malignancy of the neoplasm. In this case series we confirmed neoplastic spindloid cells with wavy cytoplasm arranged in compact areas, with occasional nuclear palisading or whirls, and interchanged with loosely arranged areas, as the morphological features supporting a diagnosis of CPNST. A constant concurrent expression of vimentin, NSE, and laminin might confirm the diagnosis of PNST in the absence of clear S-100 protein

  11. Benign and malignant neurogenic tumors of nerve sheath origin on FDG PET

    Energy Technology Data Exchange (ETDEWEB)

    Yun, M. J.; Go, D. H.; Yoo, Y. H.; Shin, K. H.; Lee, J. D [College of Medicine, Yonsei University, Seoul (Korea, Republic of)

    2004-07-01

    The differentiation between benign and malignant nerve sheath tumors is difficult based on conventional radiological imaging. This study was undertaken to investigate the value of FDG PET in distinguishing benign from malignant neurogenic tumors of nerve sheath origin. We performed a retrospective review of the medical record to select patients with nerve sheath tumors who had underdone FDG PET imaging. Fifteen patients (7F: 8M) with benign or malignant nerve sheath tumors were included in this study. Of the 15 patients, 9 were diagnosed with the known neurofibromatosis type I. A total of 19 nerve sheath tumors were included from the 15 patients. All patients had undergone FDG PET to evaluate for malignant potential of the known lesions. Images of FDG PET were semi-quantitatively analyzed and a region of interest (ROI) was placed over the area of the maximum FDG uptake and an average standardized uptake value was taken for final analysis. There were 5 malignant peripheral nerve sheath tumors, 5 schwannomas, and 9 neurofibromas. The mean SUV was 2 (ranged from 1.6 to 3.3) for schwannomas, 1.3 (0.7 to 2.5) for neurofibromas, and 8.4 (4.6 to 12.2) for malignant peripheral nerve sheath tumors. Of 14 benign tumors, all except one schwannoma showed a SUV less than 3. When a cutoff SUV of 4 was used to differentiate the nerve sheath tumors, all tumors were correctly classified as benign or malignant, respectively. Among the 9 patients diagnosed with neurofibromatosis type I. 4 had malignant peripheral nerve sheath tumors and FDG PET accurately detected all the 4 lesions with malignant transformation. According to our results, FDG PET seems to have a great potential for accurately characterizing benign versus malignant nerve sheath tumors. It appears to be extremely useful for patients with neurofibromatosis to localize the lesion with malignant transformation.

  12. Can preoperative MR imaging predict optic nerve invasion of retinoblastoma?

    Energy Technology Data Exchange (ETDEWEB)

    Song, Kyoung Doo, E-mail: kdsong0308@gmail.com [Department of Radiology and Center for Imaging Science, Samsung Medical Center, Sungkyunkwan University School of Medicine, 50, Ilwon-Dong, Kangnam-Ku, Seoul 135-710 (Korea, Republic of); Eo, Hong, E-mail: rtombow@gmail.com [Department of Radiology and Center for Imaging Science, Samsung Medical Center, Sungkyunkwan University School of Medicine, 50, Ilwon-Dong, Kangnam-Ku, Seoul 135-710 (Korea, Republic of); Kim, Ji Hye, E-mail: jhkate.kim@samsung.com [Department of Radiology and Center for Imaging Science, Samsung Medical Center, Sungkyunkwan University School of Medicine, 50, Ilwon-Dong, Kangnam-Ku, Seoul 135-710 (Korea, Republic of); Yoo, So-Young, E-mail: sy1131.yoo@samsung.com [Department of Radiology and Center for Imaging Science, Samsung Medical Center, Sungkyunkwan University School of Medicine, 50, Ilwon-Dong, Kangnam-Ku, Seoul 135-710 (Korea, Republic of); Jeon, Tae Yeon, E-mail: hathor97.jeon@samsung.com [Department of Radiology and Center for Imaging Science, Samsung Medical Center, Sungkyunkwan University School of Medicine, 50, Ilwon-Dong, Kangnam-Ku, Seoul 135-710 (Korea, Republic of)

    2012-12-15

    Purpose: To evaluate the accuracy of pre-operative MRI for the detection of optic nerve invasion in retinoblastoma. Materials and methods: Institutional review board approval and informed consent were waived for this retrospective study. A total of 41 patients were included. Inclusion criteria were histologically proven retinoblastoma, availability of diagnostic-quality preoperative MR images acquired during the 4 weeks before surgery, unilateral retinoblastoma, and normal-sized optic nerve. Two radiologists retrospectively reviewed the MR images independently. Five imaging findings (diffuse mild optic nerve enhancement, focal strong optic nerve enhancement, optic sheath enhancement, tumor location, and tumor size) were evaluated against optic nerve invasion of retinoblastoma. The predictive performance of all MR imaging findings for optic nerve invasion was also evaluated by the receiver operating characteristic curve analysis. Results: Optic nerve invasion was histopathologically confirmed in 24% of study population (10/41). The differences in diffuse mild enhancement, focal strong enhancement, optic sheath enhancement, and tumor location between patients with optic nerve invasion and patients without optic nerve invasion were not significant. Tumor sizes were 16.1 mm (SD: 2.2 mm) and 14.9 mm (SD: 3.6 mm) in patients with and without optic nerve involvement, respectively (P = 0.444). P-Values from binary logistic regression indicated that all five imaging findings were not significant predictors of tumor invasion of optic nerve. The AUC values of all MR imaging findings for the prediction of optic nerve invasion were 0.689 (95% confidence interval: 0.499–0.879) and 0.653 (95% confidence interval: 0.445–0.861) for observer 1 and observer 2, respectively. Conclusion: Findings of MRI in patients with normal-sized optic nerves have limited usefulness in preoperatively predicting the presence of optic nerve invasion in retinoblastoma.

  13. Excellent response of malignant peripheral nerve sheath tumour of retroperitoneum to radiation therapy

    Science.gov (United States)

    Akhavan, Ali; Binesh, Fariba; Ghannadi, Fazlollah; Navabii, Hossein

    2012-01-01

    Malignant peripheral nerve sheath tumours are high-grade sarcomas originating from Schwann cells or nerve sheath cells. Most of these tumours are associated with major nerves of the body wall and extremities. The lower extremity and the retroperitoneum are the most common sites. Surgery is the cornerstone of treatment, however, radiation therapy is usually used as an adjuvant treatment. In this paper we present a 57-year-old Iranian woman with malignant peripheral nerve sheath tumour of retroperitoneum who was operated subtotally and then underwent radiation therapy which led to disappearance of all gross residual disease. PMID:23257269

  14. File list: InP.Neu.20.AllAg.Nerve_Sheath_Neoplasms [Chip-atlas[Archive

    Lifescience Database Archive (English)

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    Lifescience Database Archive (English)

    Full Text Available NoD.Neu.10.AllAg.Nerve_Sheath_Neoplasms mm9 No description Neural Nerve Sheath Neop...lasms http://dbarchive.biosciencedbc.jp/kyushu-u/mm9/assembled/NoD.Neu.10.AllAg.Nerve_Sheath_Neoplasms.bed ...

  2. Regeneration of Optic Nerve

    Directory of Open Access Journals (Sweden)

    Kwok-Fai So

    2011-05-01

    Full Text Available The optic nerve is part of the central nervous system (CNS and has a structure similar to other CNS tracts. The axons that form the optic nerve originate in the ganglion cell layer of the retina and extend through the optic tract. As a tissue, the optic nerve has the same organization as the white matter of the brain in regard to its glia. There are three types of glial cells: Oligodendrocytes, astrocytes, and microglia. Little structural and functional regeneration of the CNS takes place spontaneously following injury in adult mammals. In contrast, the ability of the mammalian peripheral nervous system (PNS to regenerate axons after injury is well documented. A number of factors are involved in the lack of CNS regeneration, including: (i the response of neuronal cell bodies against the damage; (ii myelin-mediated inhibition by oligodendrocytes; (iii glial scarring, by astrocytes; (iv macrophage infiltration; and (v insufficient trophic factor support. The fundamental difference in the regenerative capacity between CNS and PNS neuronal cell bodies has been the subject of intensive research. In the CNS the target normally conveys a retrograde trophic signal to the cell body. CNS neurons die because of trophic deprivation. Damage to the optic nerve disconnects the neuronal cell body from its target-derived trophic peptides, leading to the death of retinal ganglion cells. Furthermore, the axontomized neurons become less responsive to the peptide trophic signals they do receive. On the other hand, adult PNS neurons are intrinsically responsive to neurotrophic factors and do not lose trophic responsiveness after axotomy. In this talk different strategies to promote optic-nerve regeneration in adult mammals are reviewed. Much work is still needed to resolve many issues. This is a very important area of neuroregeneration and neuroprotection, as currently there is no cure after traumatic optic nerve injury or retinal disease such as glaucoma, which

  3. Malignant peripheral nerve sheath tumor arising from solitary neurofibroma

    Directory of Open Access Journals (Sweden)

    Pei-I Chung

    2014-09-01

    Full Text Available Malignant peripheral nerve sheath tumors (MPNSTs are rare sarcomas that are strongly associated with neurofibromatosis type I (NF-1. We describe a 71-year-old woman with no stigmata of neurofibromatosis, who presented with recurrent subcutaneous tumor on her left upper back. She received two excisional biopsies on the back of her trunk at our hospital and both pathology reports revealed neurofibromas. Three years after the last skin biopsy, a rapidly growing subcutaneous tumor emerged at the same site. This tumor was totally resected and the histopathology showed an ill-defined tumor in the dermis and subcutaneous tissue. The tumor was composed of spindle cells in a myxoid stroma with a transition from the area of typical neurofibroma to the hypercellular area. The hypercellular area consisted of atypical, hyperchromatic spindled cells with frequent mitotic figures. She was therefore diagnosed with MPNST.

  4. Impaired Pten expression in human malignant peripheral nerve sheath tumours.

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    Maren Bradtmöller

    Full Text Available Malignant peripheral nerve sheath tumours (MPNST are aggressive sarcomas that develop in about 10% of patients with the genetic disease neurofibromatosis type 1 (NF1. Molecular alterations contributing to MPNST formation have only partially been resolved. Here we examined the role of Pten, a key regulator of the Pi3k/Akt/mTOR pathway, in human MPNST and benign neurofibromas. Immunohistochemistry showed that Pten expression was significantly lower in MPNST (n=16 than in neurofibromas (n=16 and normal nervous tissue. To elucidate potential mechanisms for Pten down-regulation or Akt/mTOR activation in MPNST we performed further experiments. Mutation analysis revealed absence of somatic mutations in PTEN (n=31 and PIK3CA (n=38. However, we found frequent PTEN promotor methylation in primary MPNST (11/26 and MPNST cell lines (7/8 but not in benign nerve sheath tumours. PTEN methylation was significantly associated with early metastasis. Moreover, we detected an inverse correlation of Pten-regulating miR-21 and Pten protein levels in MPNST cell lines. The examination of NF1-/- and NF1+/+Schwann cells and fibroblasts showed that Pten expression is not regulated by NF1. To determine the significance of Pten status for treatment with the mTOR inhibitor rapamycin we treated 5 MPNST cell lines with rapamycin. All cell lines were sensitive to rapamycin without a significant correlation to Pten levels. When rapamycin was combined with simvastatin a synergistic anti-proliferative effect was achieved. Taken together we show frequent loss/reduction of Pten expression in MPNST and provide evidence for the involvement of multiple Pten regulating mechanisms.

  5. Malignant peripheral nerve sheath tumor of facial nerve: Presenting as parotid mass

    Directory of Open Access Journals (Sweden)

    Bageshri P Gogate

    2013-01-01

    Full Text Available Malignant peripheral nerve sheath tumor (MPNST is very uncommon tumor of parotid gland and it is an uncommon spindle cell sarcoma accounting for approximately 5% of all soft-tissue sarcoma. There is strong association between MPNSTs and neurofibromatosis (NF-1 and previous irradiation. Structural abnormality of chromosome 17 is associated with NF-1 and so MPNST. We present a case of a 78-year-old male presenting with slowly growing parotid mass who underwent tumor resection.

  6. Sciatica due to malignant nerve sheath tumour of sciatic nerve in the thigh.

    Directory of Open Access Journals (Sweden)

    Sharma R

    2001-04-01

    Full Text Available Malignant peripheral nerve sheath tumour (MPNST is a rare malignant neoplasm arising from the supportive non-neural component of the peripheral nerves. An unusual case of pain and weakness of the foot and calf muscles due to a giant MPNST of the sciatic nerve in the posterior compartment of the thigh is presented. The patient was already investigated as a case of sciatica due to a lumbar disc disease with a negative magnetic resonance imaging and then unsuccessfully operated elsewhere twice, with a misdiagnosis of tarsal tunnel syndrome. Neurosurgical referral prompted a diagnostic magnetic resonance study of the thigh, revealing the lesion, which was completely excised microsurgically with total relief in the pain and partial improvement in the weakness and sensations in the sole of the foot.

  7. Updates on the diagnosis and treatment of intracranial nerve malignant peripheral nerve sheath tumors

    Directory of Open Access Journals (Sweden)

    L'Heureux-Lebeau B

    2013-04-01

    Full Text Available Bénédicte L'Heureux-Lebeau,1 Issam Saliba2 1University of Montreal, 2Department of Otolaryngology Head and Neck Surgery, Montreal University Hospital Center (CHUM, University of Montreal, Montreal, Quebec, Canada Background: Malignant peripheral nerve sheath tumors (MPNSTs are rare entities and MPNSTs of intracranial nerves are even more sporadic. MPNSTs present diagnosis and treatment challenges since there are no defined diagnosis criteria and no established therapeutic strategies. Methods: We reviewed literature for MPNST-related articles. We found 45 relevant studies in which 60 cases were described. Results: We identified 60 cases of intracranial nerve MPNSTs. The age ranged from 3 to 75 years old. Male to female ratio was 1.5:1. The most involved cranial nerves (CNs were CN VIII (60%, CN V (27%, and CN VII (10%. Most of the MPNSTs reported (47% arose sporadically, 40% arose from a schwannoma, 8% arose from a neurofibroma, and 6% arose from an unspecified nerve tumor. Twenty patients had a history of radiation exposure, four patients had neurofibromatosis type 1 (NF1, four patients had neurofibromatosis type 2 (NF2, and NF2 was suspected in two other patients. Twenty-two patients were treated with radiotherapy and presented a higher survival rate. Seventy-two percent of patients died of their disease while 28% of patients survived. One-year survival rate was 33%. Forty-five percent of tumors recurred and 19% of patients had metastases. Conclusion: MPNSTs involving CNs are very rare. Diagnosis is made in regards to the histological and pathological findings. Imaging may help orient the diagnosis. A preexisting knowledge of the clinical situation is more likely to lead to a correct diagnosis. The mainstay of treatment is radical surgical resection with adjuvant radiotherapy. Since these tumors are associated with a poor prognosis, a close follow-up is mandatory. Keywords: malignant peripheral nerve sheath tumor, MPNST, neurofibroma

  8. Ultrastructural changes in the optic nerve and capillary vessels during early stages of optic nerve injury

    Institute of Scientific and Technical Information of China (English)

    Xuehong Ju; Xiuyun Li; Xiaoshuang Li; Hongtao Tang; Hongguo Liu

    2008-01-01

    BACKGROUND: Capillaries are the only blood supply for optic nerves, which makes the system more vulnerable to impaired blood circulation. OBJECTIVE: To observe the ultrastructural changes in the optic nerves and capillaries in rabbits following intracanalicular segment injury to the optic nerve. DESIGN, TIME AND SETTING: Comparative, observational, pathological morphology was performed at the Department of Anatomy, Weifang Medical College from September to November 2007. MATERIALS: Models of intracanalicular segment injury to the optic nerve were induced in the right eye of thirty healthy, adult rabbits by a flee-falling metal cylinder. The H-7500 transmission electron microscope was provided by Hitachi, Japan. METHODS: All rabbits were randomly assigned into experimental (n = 25) and control (n = 5) groups. Optic nerve specimens were obtained from the experimental group at 0.5, 6, 12, 48, and 96 hours, respectively, following injury. Uitrastructural changes to the optic nerves and their capillaries were observed by electron microscopy. Optic nerve injury was not established in the control group, but optic nerve specimens were collected similarly to the experimental group. MAIN OUTCOME MEASURES: Ultrastructural changes in the injured optic nerves and their capillaries. RESULTS: Thirty rabbits were included in the final analysis. In the control group, cross-sections of the optic nerves exhibited varied thicknesses with regularly arranged fibers. The axons appeared to be smooth with condensed myelin sheaths and oval mitochondria. The microtubules and mierofilaments were clearly seen. The lumens of the capillaries were regular with densely arranged endothelial cells and visible mitochondria. In the experimental group, 30 minutes after injury to the optic nerves, swollen axons, sparse myelin sheath, disordered microtubules and microfilaments, swollen mitochondria, and a decreased number of pinocytosis vesicles and microfilaments in endothelial cells of the capillaries

  9. Optic nerve oxygen tension

    DEFF Research Database (Denmark)

    Kiilgaard, Jens Folke; Pedersen, D B; Eysteinsson, T

    2004-01-01

    The authors have previously reported that carbonic anhydrase inhibitors such as acetazolamide and dorzolamide raise optic nerve oxygen tension (ONPO(2)) in pigs. The purpose of the present study was to investigate whether timolol, which belongs to another group of glaucoma drugs called beta...

  10. Optic nerve oxygen tension

    DEFF Research Database (Denmark)

    Kiilgaard, Jens Folke; Pedersen, D B; Eysteinsson, T

    2004-01-01

    The authors have previously reported that carbonic anhydrase inhibitors such as acetazolamide and dorzolamide raise optic nerve oxygen tension (ONPO(2)) in pigs. The purpose of the present study was to investigate whether timolol, which belongs to another group of glaucoma drugs called beta block...

  11. ECHOGRAPHIC PICTURE OF OPTIC NERVE GLIOMA IN NEUROFIBROMATOSIS TYPE-1

    Directory of Open Access Journals (Sweden)

    Biljana Kuzmanović

    2002-12-01

    Full Text Available Background. Authors want to present echographic picture of orbital part of low-grade pilocytic astrocytoma involving the optic nerve and/or chiasm and optic tract (optic pathway glioma or visual pathway glioma.Methods. 4 children with neurofibromatosis type-1 complicated with optic pathway glioma diagnosed earlier with magnetic resonance were examined by ultrasound. Standardised A-scan technique was used for optic nerve width measurement. The 30° test and B-scan (axial, transverse and longitudinal sections of both eyes and orbits were performed as well.Results. The optic nerve diameter in our cases ranged from 4.48 to 8.5 mm. Two children had the left side optic pathway glioma, one boy had the right side optic pathway glioma and in one tumour was bilateral. The transversal section of the nerve revealed dark oval and in more perpendicular sections round void of the nerve. As the beam is swept towards the orbital apex void becomes more fusiform. The nerve and its sheaths are markedly widened. An abnormal increase in reflectivity and irregularity of the spike’s pattern is exhibited as well. No calcification along the sheaths is noticed. The transverse section of the tumour demonstrated an »inverse doughnut« sign. The outer whiter outline of the widened sheaths surrounds an inner darker circle. The longitudinal section revealed the optic nerve head continuing into the widened optic nerve. The 30° test was negative. The differential diagnosis of meningeoma, optic neuritis and orbital cysticercosis should be considered.Conclusions. Ultrasound as a cheap, safe, easily repeatable imaging method should become a method of choice for screening optic nerve tumours in neurofibromatosis type-1, especially in children, as well as for follow-up after treatment.

  12. Benign Nerve Sheath Myxoma in an Infant Misdiagnosed as Infantile Digital Fibromatosis.

    Science.gov (United States)

    Güngör, Şule; Şişman, Servet; Kocaturk, Emek; Oguz Topal, Ilteris; Yıldırım, Selda

    2016-07-01

    Herein we present the case of a 16-month boy, clinically diagnosed with infantile digital fibromatosis, but 9 months after continued growth, the mass was excised and the histopathologic diagnosis was that of a benign nerve sheath myxoma. We present this case to emphasize that nerve sheath myxomas (also known as myxoid neurothekeoma) should be included in the differential diagnosis of dermal nodules in infants.

  13. Tamoxifen inhibits malignant peripheral nerve sheath tumor growth in an estrogen receptor–independent manner

    OpenAIRE

    Byer, Stephanie J.; Eckert, Jenell M.; Brossier, Nicole M.; CLODFELDER-MILLER, BUFFIE J.; Turk, Amy N.; Carroll, Andrew J.; John C Kappes; Zinn, Kurt R; Prasain, Jeevan K.; CARROLL, STEVEN L.

    2010-01-01

    Few therapeutic options are available for malignant peripheral nerve sheath tumors (MPNSTs), the most common malignancy associated with neurofibromatosis type 1 (NF1). Guided by clinical observations suggesting that some NF1-associated nerve sheath tumors are hormonally responsive, we hypothesized that the selective estrogen receptor (ER) modulator tamoxifen would inhibit MPNST tumorigenesis in vitro and in vivo. To test this hypothesis, we examined tamoxifen effects on MPNST cell proliferati...

  14. MRI of intraspinal nerve sheath tumours presenting with sciatica

    Energy Technology Data Exchange (ETDEWEB)

    Loke, T.K.L.; Chan, C.S. [United Christian Hospital (Hong Kong). Dept. of Diagnostic Radiology; Ma, H.T.G. [St Teresa`s Hospital, Kowloon (Hong Kong). MRI and CT scanning Dept.; Ward, S.C.; Metreweli, C. [Prince of wales Hospital, New Territories (Hong Kong). Dept. of Diagnostic Radiology

    1995-08-01

    The magnetic resonance imaging (MRI) characteristics of 14 intraspinal nerve sheath tumours (NST) presenting with sciatica were reviewed. The group comprised seven schwannomas, six neurofibromas and one perineuroma. The tumours were either iso- or hypointense with respect to spinal cord on T1-weighted (T1W) images; almost all tumours were hyperintense compared with spinal cord on T2-weighted (T2W) images. The tumours were all detectable on unenhanced T1 W images. Nine NST were scanned following Gadolinium-Diethylenetriamine penta acetic acid (DTPA) injection and all showed intense enhancement. This aids differentiation from sequestrated disc fragments. Tumours were more likely to show homogeneous enhancement unless they were recurrent tumours. Rim enhancement occurs more commonly in schwannomas and this can be used to differentiate these from neurofibromas. It is estimated that on unenhanced images, schwannomas cannot be distinguished from neurofibromas. Four tumours occurred at T1 1-T12. There was poor correlation of the site of the lesion with the clinical findings. It is recommended that the MRI studies in patients with sciatica should include the lower thoracic region especially if no protruded disc was found in the lumbar region. 15 refs., 4 figs.

  15. Malignant peripheral nerve sheath tumours in inherited disease

    Directory of Open Access Journals (Sweden)

    Evans D

    2012-10-01

    Full Text Available Abstract Background Malignant peripheral nerve sheath tumours (MPNST are rare tumours known to occur at high frequency in neurofibromatosis 1 (NF1, but may also occur in other cancer prone syndromes. Methods The North West Regional Genetic Register covers a population of 4.1 million and was interrogated for incidence of MPNST in 12 cancer prone syndromes. Age, incidence and survival curves were generated for NF1. Results Fifty two of 1254 NF1 patients developed MPNST, with MPNST also occurring in 2/181 cases of schwannomatosis and 2/895 NF2 patients. Three cases were also noted in TP53 mutation carriers. However, there were no cases amongst 5727BRCA1/2 carriers and first degree relatives, 2029 members from Lynch syndrome families, nor amongst 447 Familial Adenomatous Polyposis, 202 Gorlin syndrome, nor 87 vHL cases. Conclusion MPNST is associated with schwannomatosis and TP53 mutations and is confirmed at high frequency in NF1. It appears to be only increased in NF2 amongst those that have been irradiated. The lifetime risk of MPNST in NF1 is between 9–13%.

  16. Palliative Epineurotomy for Focal Radial Malignant Peripheral Nerve Sheath Tumor in a Dog.

    Science.gov (United States)

    Gibson, Andrew David; Davies, Emma; Lara-Garcia, Ana; Lafuente, Pilar

    2016-01-01

    This case report describes the diagnosis of a peripheral nerve sheath tumor of the deep branch of the radial nerve distal to the elbow in a dog. The lesion was identified using computed tomography and ultrasonography and confirmed as sarcoma on histopathological analysis of incisional biopsies. Clinical signs dramatically improved following surgical biopsy before recurring three months later. Repeat epineurotomy of the deep branch of the radial nerve resulted in clinical improvement for a further month before signs once again returned. Epineurotomy as a palliative treatment for peripheral nerve sheath tumors has not been previously described, but may have a place in palliation of clinical signs in specific cases of peripheral nerve sheath tumors in which limb amputation is not an option.

  17. Optic nerve hypoplasia

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    Savleen Kaur

    2013-01-01

    Full Text Available Optic nerve hypoplasia (ONH is a congenital anomaly of the optic disc that might result in moderate to severe vision loss in children. With a vast number of cases now being reported, the rarity of ONH is obviously now refuted. The major aspects of ophthalmic evaluation of an infant with possible ONH are visual assessment, fundus examination, and visual electrophysiology. Characteristically, the disc is small, there is a peripapillary double-ring sign, vascular tortuosity, and thinning of the nerve fiber layer. A patient with ONH should be assessed for presence of neurologic, radiologic, and endocrine associations. There may be maternal associations like premature births, fetal alcohol syndrome, maternal diabetes. Systemic associations in the child include endocrine abnormalities, developmental delay, cerebral palsy, and seizures. Besides the hypoplastic optic nerve and chiasm, neuroimaging shows abnormalities in ventricles or white- or gray-matter development, septo-optic dysplasia, hydrocephalus, and corpus callosum abnormalities. There is a greater incidence of clinical neurologic abnormalities in patients with bilateral ONH (65% than patients with unilateral ONH. We present a review on the available literature on the same to urge caution in our clinical practice when dealing with patients with ONH. Fundus photography, ocular coherence tomography, visual field testing, color vision evaluation, neuroimaging, endocrinology consultation with or without genetic testing are helpful in the diagnosis and management of ONH. (Method of search: MEDLINE, PUBMED.

  18. CT-diagnosis of optic nerve lesions. Differential diagnostic criteria

    Energy Technology Data Exchange (ETDEWEB)

    Unsoeld, R.

    1982-01-01

    Computed tomograms of 166 optic nerve lesions were analyzed: 97 were mainly orbital and 69 mainly intracranial. The criteria were clinical course, size, density and delineation of the optic nerve shadow, orbital and cerebral soft tissue abnormalities, and bony changes in the optic canal. Characteristic CT features are described of individual disease entities such as optic gliomas, optic nerve sheath meningiomas, neoplastic and inflammatory infiltrations. The differential diagnostic importance of individual CT criteria is evaluated and discussed. Simultaneous visualization of orbital and intracranial soft tissue changes as well as bony changes in the optic canal allow the location and identification of the majority of optic nerve lesions based on the criteria mentioned above, and optic nerve tumors can be differentiated. In 9 patients with optic neuritis due to clinically proven encephalitis and in 17 patients with total optic atrophy, no changes in the size of the optic nerve could be found. CT evaluation of the intraorbital portion of the optic nerve requires special examination techniques. Oblique computer reformations through the optic canal provide excellent visualization of bony changes in the optic canal. The exclusion of intracranial causes of optic nerve lesions requires intravenous injection of contrast material.

  19. Reconstruction and measurement of optic nerve sheath imaging in normal population with multi-slice spiral CT%正常人眶内段视神经的CT重建和参数测量

    Institute of Scientific and Technical Information of China (English)

    游勇; 成洪波; 樊宁; 王宁利; 杨洁; 刘璐; 刘旭阳

    2015-01-01

    Background Optical nerve damage of glaucomatous eyes is associated with intracranial pressure.Conventional method of evaluating intracranial pressure is to measure cerebrospinal pressure by lumber puncture.However,the measurement of intraorbital optical nerve parameters,a novel method of evaluating intracranial pressure,is introduced in this field.Objective This study was to measure and analyze the intraorbital optic nerve sheath diameter (ONSD) and cross sectional area (ONSA) in normal population using multi-slice spiral CT.Methods This study protocol was approved by Clinical Ethic Committee of Shenzhen Chinese Traditional Medical Hospital and followed Hersinki Declaration.Informed consent was obtained from each individual prior to any medical examination.One hundred and five eyes of 105 normal persons with normal cerebral CT image were enrolled in Shenzhen Chinese Traditional Medical Hospital from January 2012 to September 2013.Cerebral volume was scanned in all the individuals by 64 slice spiral CT.The brain images were obtained for the curve planar rebuilding of intraorbital optical nerve on image post-processing workstation.The maximum and minimum of ONSD and the ONSA in axial sections at 3,6,9,12 and 15 mm far away from globe wall were measured using a standardized technique to analyze the change of optical nerve parameters at different point locations.These parameters were compared in different gender or eyes.The correlation among age and the optical nerve parameters at 3 mm far away from globe wall was evaluated by multivariate regression analysis.Results The average maximal ONSDs were (6.24±0.47), (5.56±0.44),(5.18±0.43),(4.82±0.41) and (4.69±0.41) mm;the average minimal ONSDs were (5.56±0.50),(4.97± 0.41) ,(4.55±0.35),(4.26±0.39) and (4.10±0.40) mm;the average ONSAs were (27.68±4.40),(22.02±3.35) , (18.74± 2.75) , (16.34±2.72) , (15.40±2.68) mm2 at 3,6,9,12 and 15 mm far away from posterior eyeball wall,respectively, showing significant

  20. Alterations at chromosome 17 loci in peripheral nerve sheath tumors

    Energy Technology Data Exchange (ETDEWEB)

    Lothe, R.A.; Slettan, A.; Saeter, G. [Norwegian Radium Hospital, Oslo (Norway)] [and others

    1995-01-01

    Little is known about the molecular genetic changes in malignant peripheral nerve sheath tumors (MPNST). Inactivation of the TP53 gene in l7p has been reported in a few tumors. The MPNST is one of the manifestations of neurofibromatosis 1 (NF1), suggesting that the NF1 gene in 17q might be important. We present a study of 15 neurofibromas and MPNST from nine individuals. Seven patients had NF1 and six of these developed MPNST. Genetic alterations at nine polymorphic loci on chromosome 17 were examined. Allelic imbalance was detected only in the malignant tumors from NF1 patients (4/6). Complete loss of heterozygosity of 17q loci was found in three of these tumors, all including loci within the NF1 gene. Two of the malignant tumors also showed deletions on 17p. No mutations were detected within exon 5-8 of the TP53 in any of the MPNST, and none of them were TP53 protein-positive using immunostaining with mono- and polyclonal antibodies against TP53. The numbers of chromosome 17 present in each tumor were evaluated by use of fluorescence in situ hybridization (FISH) on interphase nuclei with a centromere-specific probe. A deviation from the disomic status of chromosome 17 was observed in two of the MPNST from NF1 patients. These results support the hypothesis of inactivation of both NF1 gene alleles during development of MPNST in patients with NF1. In contrast to other reports, we did not find evidence for a homozygous mutated condition of the TP53 gene in the same tumors. Finally, FISH analysis was in accordance with the DNA analysis in the deduction of the numbers of chromosome 17 in these tumors. 29 refs., 3 figs., 2 tabs.

  1. Optic Nerve Hemangioblastoma: A Case Report

    Directory of Open Access Journals (Sweden)

    Holly Zywicke

    2012-01-01

    Full Text Available Hemangioblastomas are World Health Organization (WHO grade I tumors of uncertain histologic origin. These central nervous system tumors are most often found in the posterior fossa, brainstem, and spinal cord. There are fewer than 20 reported cases of optic nerve hemangioblastomas in the literature. We present a patient with visual decline found to have a mass arising from within the posterior orbital canal that grossly involved the optic nerve sheath. Neuropathologic evaluation showed hemangioblastoma. Although not a common tumor in this location, consideration of hemangioblastoma in the differential diagnosis is important as they can have a more aggressive course than other tumors of this region and have a detrimental effect on visual prognosis.

  2. A Rare Malignant Peripheral Nerve Sheath Tumor of the Maxilla Mimicking a Periapical Lesion

    Science.gov (United States)

    Álvares, Pamella; Silva, Luciano; Pereira dos Santos Neto, Alexandrino; Rodrigues, Cleomar Donizeth; Caubi, Antônio; Silveira, Marcia; Sayão, Sandra; Sobral, Ana Paula

    2016-01-01

    Malignant peripheral nerve sheath tumor is a malignant neoplasm that is rarely found in the oral cavity. About 50% of this tumor occurs in patients with neurofibromatosis type I and comprises approximately 10% of all soft tissue sarcomas of head and neck region. Intraosseous malignant peripheral nerve sheath tumor of the maxilla is rare. This article is the first to address malignant peripheral nerve sheath tumor of the maxilla presenting as a periapical radiolucency on nonvital endodontically treated teeth in the English medical literature. Surgical approaches to malignant soft tissue tumor vary based on the extent of the disease, age of the patient, and pathological findings. A rare case of intraosseous malignant peripheral nerve sheath tumor is reported in a 16-year-old woman. The patient presented clinically with a pain involving the upper left incisors region and with defined unilocular periapical radiolucency lesion involved between the upper left incisors. An incisional biopsy was made. Histological and immunohistochemical examination were positive for S-100 protein and glial fibrillary acidic protein showed that the lesion was an intraosseous malignant peripheral nerve sheath tumor of the maxilla. Nine years after the surgery, no regional recurrence was observed. PMID:27994888

  3. A Rare Malignant Peripheral Nerve Sheath Tumor of the Maxilla Mimicking a Periapical Lesion

    Directory of Open Access Journals (Sweden)

    José Alcides Arruda

    2016-01-01

    Full Text Available Malignant peripheral nerve sheath tumor is a malignant neoplasm that is rarely found in the oral cavity. About 50% of this tumor occurs in patients with neurofibromatosis type I and comprises approximately 10% of all soft tissue sarcomas of head and neck region. Intraosseous malignant peripheral nerve sheath tumor of the maxilla is rare. This article is the first to address malignant peripheral nerve sheath tumor of the maxilla presenting as a periapical radiolucency on nonvital endodontically treated teeth in the English medical literature. Surgical approaches to malignant soft tissue tumor vary based on the extent of the disease, age of the patient, and pathological findings. A rare case of intraosseous malignant peripheral nerve sheath tumor is reported in a 16-year-old woman. The patient presented clinically with a pain involving the upper left incisors region and with defined unilocular periapical radiolucency lesion involved between the upper left incisors. An incisional biopsy was made. Histological and immunohistochemical examination were positive for S-100 protein and glial fibrillary acidic protein showed that the lesion was an intraosseous malignant peripheral nerve sheath tumor of the maxilla. Nine years after the surgery, no regional recurrence was observed.

  4. Accuracy and complications of CT-guided core needle biopsy of peripheral nerve sheath tumours

    Energy Technology Data Exchange (ETDEWEB)

    Pianta, Marcus; Chock, Eric; Schlicht, Stephen [St Vincent' s Hospital, Fitzroy, VIC (Australia); McCombe, David [St Vincent' s Hospital and Victorian Hand Surgery Associates, Victoria (Australia)

    2015-09-15

    This single-centre study retrospectively reviews the complications in patients that have occurred following peripheral nerve sheath tumour biopsy, and assesses whether there is an association with biopsy technique or underlying lesion characteristics. 41 consecutive core needle biopsies of proven peripheral nerve sheath tumours over a 2-year period in a tertiary teaching hospital were reviewed. Patient demographics and symptoms, tumour characteristics and radiological appearances were recorded. Biopsy and surgical histology were correlated, and post-biopsy and surgical complications analyzed. 41 biopsies were performed in 38 patients. 68 % schwannomas, 24 % neurofibromas and 7 % malignant peripheral nerve sheath tumours. Biopsy histology correlated with surgery in all cases. 71 % of lesions were surgically excised. 60 % of patients reported pain related to their lesion. Following the biopsy, 12 % reported increased pain, which resolved in all cases. Pain exacerbation was noted in tumours smaller in size, more superficial and in closer proximity of the biopsy needle tip to the traversing nerve. Number of biopsy needle passes was not associated with an increased incidence of procedure-related pain. Core biopsy of a suspected peripheral nerve sheath tumour may be performed safely before excisional surgery to confirm lesion histology and assist prognosis. There is excellent correlation between core biopsy and excised surgical specimen histology. The most common complication of pain exacerbation is seen in a minority and is temporary, and more likely with smaller, more superficial lesions and a closer needle-tip to traversing nerve distance during biopsy. (orig.)

  5. Imaging of peripheral nerve sheath tumors with pathologic correlation Pictorial review

    Energy Technology Data Exchange (ETDEWEB)

    Pilavaki, M.; Chourmouzi, D. E-mail: d.chourmouzi@ips.gr; Kiziridou, A.; Skordalaki, A.; Zarampoukas, T.; Drevelengas, A

    2004-12-01

    Peripheral neurogenic tumors include neurilemoma, neurinoma, and malignant peripheral nerve sheath tumors. All neurogenic tumors share common imaging features. Although differentiation between them is difficult, neurogenic origin can be suggested from their imaging appearances, including fusiform shape, relation to the nerve, 'split-fat' sign, associated muscle atrophy and intrinsic imaging characteristics including 'target sign' as well as from lesion location along a typical nerve distribution. Our purpose is to make an overview of imaging findings of each type of peripheral nerve sheath tumor with emphasis on characteristic signs and correlate with histologic features. Morton's neuroma and intraneural ganglion are also included as tumors of nerve origin.

  6. Atypical inguinal malignant peripheral nerve sheath tumour with arteriovenous fistula of the left femoral nerve in a child

    Energy Technology Data Exchange (ETDEWEB)

    Melloni, Pietro; Veintemillas, Maite [Corporacio Sanitaria Parc Tauli, Unitat de Diagnostic per Imatge d' Alta Tecnologia, Barcelona (Spain); Olsina, Gustavo; Oliva, Eulalia; Garcia-Continente, Gemma [Capio Hospital General de Catalunya, Servei de Diagnostic per la Imatge, Barcelona (Spain); Garcia-Hernandez, Felip [Capio Hospital General de Catalunya, Servei de Anatomia Patalogica, Barcelona (Spain)

    2008-07-15

    We report a 9-year-old girl who developed a malignant peripheral nerve sheath tumour (MPNST) with an arteriovenous fistula arising from the left femoral nerve and adjacent to the iliofemoral vessels in the ipsilateral groin, but without infiltrating them. We describe the MRI and MRA findings. Although MPNST is relatively well known and widely studied, the location of this mass is unique in a child. The mass was surgically removed. (orig.)

  7. Evaluation of Bcl-2, Bcl-x and Cleaved Caspase-3 in Malignant Peripheral Nerve Sheath Tumors and Neurofibromas

    Directory of Open Access Journals (Sweden)

    KARIN S. CUNHA

    2013-11-01

    Full Text Available AIMS: To study the expression of Bcl-2, Bcl-x, as well the presence of cleaved caspase-3 in neurofibromas and malignant peripheral nerve sheath tumors. The expression of Bcl-2 and Bcl-x and the presence of cleaved caspase 3 were compared to clinicopathological features of malignant peripheral nerve sheath tumors and their impact on survival rates were also investigated. MATERIALS AND METHODS: The evaluation of Bcl-2, Bcl-x and cleaved caspase-3 was performed by immunohistochemistry using tissue microarrays in 28 malignant peripheral nerve sheath tumors and 38 neurofibromas. Immunoquantification was performed by computerized digital image analysis. CONCLUSIONS: Apoptosis is altered in neurofibromas and mainly in malignant peripheral nerve sheath tumors. High levels of cleaved caspase-3 are more common in tumors with more aggressive histological features and it is associated with lower disease free survival of patients with malignant peripheral nerve sheath tumors.

  8. A new association – multiple endocrine neoplasia type 1 and malignant peripheral nerve sheath tumor

    OpenAIRE

    Preda, Veronica; Sywak, Mark; Learoyd, Diana

    2014-01-01

    Key Clinical Message We report a patient with multiple endocrine neoplasia type 1 (MEN-1) and an aggressive malignant peripheral nerve sheath tumor (MPNST) arising from a ganglioneuroma of the adrenal gland. Patients with MEN-1 require careful consideration of other tumor associations, including MPNST, as it can portend a poor prognosis. MEN-1 and MPNST have not been reported. We report a patient with multiple endocrine neoplasia type 1 (MEN-1) and an aggressive malignant peripheral nerve she...

  9. Malignant peripheral nerve sheath tumor of the cervical vagus nerve in a neurofibromatosis type 1 patient - An unusual presentation

    Directory of Open Access Journals (Sweden)

    Ashok Gupta

    2010-07-01

    Full Text Available Malignant peripheral nerve sheath tumors (MPNST’S of the head and neck comprise 2% to 6% of head and neck sarcomas. These tumors may arise as sporadic variants or in patients with neurofibromatosis (NF. Development of these MPNST’s is one of the serious complications of neurofibromatosis type 1(NF1. To our knowledge there are only two reported cases of MPNST’s arising in the cervical vagal nerve, occurring in NF1 patients. We present here an NF1 patient who developed an MPNST of the cervical vagus nerve and presented only with a cervical swelling and hoarseness.

  10. A hereditary disposition for bovine peripheral nerve sheath tumors in Danish Holstein cattle

    DEFF Research Database (Denmark)

    Grossi, Anette Blak; Agerholm, Jørgen Steen; Christensen, Knud Arnbjerg;

    2014-01-01

    Background: Peripheral nerve sheath tumors (PNSTs) are frequently found in Danish cattle at slaughter. Bovine PNSTs share several gross and histopathological characteristics with the PNSTs in humans with heritable neurofibromatosis syndromes. The aim of the present study was to investigate a poss...

  11. Plexiform malignant peripheral nerve sheath tumour of infancy and childhood of the index finger : Surgical treatment

    NARCIS (Netherlands)

    Meek, Marcel F.; Wolf, Rinze; Coert, J. Henk; Hoekstra, Harald J.; Nicolai, Jean-Philippe A.

    2009-01-01

    We describe a rare case of plexiform malignant peripheral nerve sheath tumour (MPNST) of infancy and childhood in a 3.5-year-old girl. The tumour was located in the proximal phalanx of the left index finger. After initial excisions and a ray amputation, exarticulation of the third and fourth rays wa

  12. Malignant peripheral nerve sheath tumors in patients with and without neurofibromatosis.

    NARCIS (Netherlands)

    Doorn, P F; Molenaar, W M; Buter, J; Hoekstra, H J

    1995-01-01

    Malignant peripheral nerve sheath tumors (MPNST) are rare. They account for 10% of all soft tissue sarcomas. The incidence of MPNST in patients with neurofibromatosis type 1 (NF-1) is 4%. A retrospective study was undertaken to evaluate the prognosis of patients with MPNST and NF-1 vs patients with

  13. Malignant peripheral nerve sheath tumor of the anterior mediastinum: a rare presentation

    Directory of Open Access Journals (Sweden)

    Babusha Kalra

    2014-12-01

    Full Text Available Malignant peripheral nerve sheath tumor (MPNST is a rare tumor that accounts for 5% of all thoracic neoplasm usually located in the posterior mediastinum and is generally associated with a poor outcome. We present a case of MPNST of the anterior mediastinum presenting in a rare location leading to diagnostic dilemmas and treated primarily by surgical resection.

  14. Morphometric Analysis of Connective Tissue Sheaths of Sural Nerve in Diabetic and Nondiabetic Patients

    Directory of Open Access Journals (Sweden)

    Braca Kundalić

    2014-01-01

    Full Text Available One of the most common complications of diabetes mellitus is diabetic neuropathy. It may be provoked by metabolic and/or vascular factors, and depending on duration of disease, various layers of nerve may be affected. Our aim was to investigate influence of diabetes on the epineurial, perineurial, and endoneurial connective tissue sheaths. The study included 15 samples of sural nerve divided into three groups: diabetic group, peripheral vascular disease group, and control group. After morphological analysis, morphometric parameters were determined for each case using ImageJ software. Compared to the control group, the diabetic cases had significantly higher perineurial index (P<0.05 and endoneurial connective tissue percentage (P<0.01. The diabetic group showed significantly higher epineurial area (P<0.01, as well as percentage of endoneurial connective tissue (P<0.01, in relation to the peripheral vascular disease group. It is obvious that hyperglycemia and ischemia present in diabetes lead to substantial changes in connective tissue sheaths of nerve, particularly in peri- and endoneurium. Perineurial thickening and significant endoneurial fibrosis may impair the balance of endoneurial homeostasis and regenerative ability of the nerve fibers. Future investigations should focus on studying the components of extracellular matrix of connective tissue sheaths in diabetic nerves.

  15. Intraosseous malignant peripheral nerve sheath tumor of maxilla: A case report with review of the literature

    Directory of Open Access Journals (Sweden)

    Sandhya Tamgadge

    2014-01-01

    Full Text Available Malignant peripheral nerve sheath tumor (MPNST, the principle malignancy of peripheral nerve origin, though rare in the general population, occurs with excessive frequency among patients with neurofibromatosis. This tumor always arises in soft-tissues, usually found in the lower extremities and only 10-12% of all lesions occur in the head and neck region, which makes it a rare entity. The primary intraosseous MPNST is rare and has been reported most frequently in the mandible. This article discusses a case report of MPNST of the left maxilla without a history of benign nerve tissue tumor and the diagnostic difficulties associated with MPNST.

  16. Superficial malignant peripheral nerve sheath tumor arising from diffuse neurofibroma in a neurofibromatosis type 1 patient.

    Science.gov (United States)

    Inoue, Takuya; Kuwashiro, Maki; Misago, Noriyuki; Narisawa, Yutaka

    2014-07-01

    Malignant peripheral nerve sheath tumors (MPNST) are regarded as sarcomas that arise from peripheral nerves or that display differentiation along the lines of the various elements of the nerve sheath. These tumors occur in deep soft tissues, but superficial primary MPNST with a cutaneous or subcutaneous origin have rarely been reported. A 70-year-old woman presented with a 3-4-year history of a slowly enlarging soft nodule on the left side of her neck. The histopathological diagnosis of the nodule was low-grade MPNST arising from diffuse neurofibroma. There was increased cellularity, but no necrosis or mitotic activity. These histopathological findings pose difficulties in differential diagnosis from a neurofibroma with atypical histological features. We report a rare case of superficial MPNST arising from diffuse neurofibroma associated with underlying occipital bone dysplasia in a neurofibromatosis type 1 patient.

  17. Optic nerve atrophy

    Science.gov (United States)

    Optic atrophy; Optic neuropathy ... There are many causes of optic atrophy. The most common is poor blood flow. This is called ischemic optic neuropathy. The problem most often affects older adults. The optic ...

  18. Epicardial ablation: prevention of phrenic nerve damage by pericardial injection of saline and the use of a steerable sheath.

    Science.gov (United States)

    Neven, Kars; Fernandez-Armenta, Juan; Andreu, David; Berruezo, Antonio

    2014-03-01

    Because of the close proximity of the phrenic nerve to the pericardium, phrenic nerve damage caused by epicardial ablation can easily occur. We report two cases of epicardial VT ablation where pericardial injection of saline, combined with the use of a steerable sheath, successfully prevents the phrenic nerve from being damaged.

  19. Epicardial Ablation: Prevention of Phrenic Nerve Damage by Pericardial Injection of Saline and the Use of a Steerable Sheath

    Directory of Open Access Journals (Sweden)

    Kars Neven, MD

    2014-03-01

    Full Text Available Because of the close proximity of the phrenic nerve to the pericardium, phrenic nerve damage caused by epicardial ablation can easily occur. We report two cases of epicardial VT ablation where pericardial injection of saline, combined with the use of a steerable sheath, successfully prevents the phrenic nerve from being damaged.

  20. 伽玛刀治疗22例原发视神经鞘脑膜瘤的疗效分析%Hyper-SGSI gamma knife in 22 patients with primary optic nerve sheath meningioma

    Institute of Scientific and Technical Information of China (English)

    宁健; 穆晓峰; 赵水喜; 杨书明; 杨远游; 赵卫东; 程海民; 肖利华

    2012-01-01

    Objective To assess the efficacy of Hyper-SGSI gamma knife with different dose fractionations in patients with primary optic nerve sheath meningioma(ONSM). Methods From Aug. 2004 to Mar. 2010, 22 patients with ONSM were treated with fractionated stereotactic radiotherapy ( FSRT) by Hyper-SGSI gamma knife treatment system. 1 case was treated with conventional frac-tionation, the radiation dose was 50 Gy in 25 fractions; 21 patients were treated with hypofraction, the radiation dose were 36-40 Gy in 8-12 fractions. Clinical and radiographic follow-up were performed every 6 months after FSRT therapy, Efficacy of FSRT and improvement of symptoms were evaluated by clinic manifestation and change of tumor maximum diameter in different follow-up time. Results After a median follow-up of 25 months,the visual control rate in all patients was 77. 3% ( 17/22) . Visual acuity was improved in 4 patients, remained stable in 13 patients,and deteriorated in 3patients,blindness in 2 patients. The visual control rate would decline with time of follow-up, with one year follow-up was 95. 0% (19/20 ) , with two years follow-up was 75. 0% ( 9/12). The tumor control rate was 100. 0% (22 cases). The maximum diameter of the tumor was reduced more than 50% in 8 patients(36. 4% ) ;25% - 50% in 11 patients(50. 0% ); 10% ~ 25% in 3 patients( 13. 6% ). The rate of improvement with proptosis was 100. 0% (17/17). The degree of proptosis at pro and post treatment were ( 17. 3 ± 2. 7 ) mm and ( 14. 9 ± 1. 5 ) mm,respectively. There were statistical significance( P <0. 05). Ten cases with reversible complications could be recovered after symptomatic treatment. Conclusion Fractionated stereotactic radiotherapy can improves tumor control and proptosis for ONSM, while control rate for the visual observation is still needed for a long time. The best dose fractionation with FSRT for ONSM will be required for further study.%目的 探讨采用伽玛刀进行低分割、高剂量的分次立体定向放

  1. Tumors of the optic nerve

    DEFF Research Database (Denmark)

    Lindegaard, Jens; Heegaard, Steffen

    2009-01-01

    A variety of lesions may involve the optic nerve. Mainly, these lesions are inflammatory or vascular lesions that rarely necessitate surgery but may induce significant visual morbidity. Orbital tumors may induce proptosis, visual loss, relative afferent pupillary defect, disc edema and optic...... tumor-specific histologic features are given. Finally, treatment modalities and prognosis are discussed....

  2. Retroperitoneal Malignant Peripheral Nerve Sheath Tumor Replacing an Absent Kidney in a Child

    Directory of Open Access Journals (Sweden)

    Samin Alavi

    2013-01-01

    Full Text Available Malignant peripheral nerve sheath tumors (MPNSTs are nonrhabdomyosarcoma soft tissue sarcomas with rare occurrence in children specially in the retroperitoneum. We describe a young child who presented with an abdominal mass. Both ultrasound and computed tomography revealed a large right-sided abdominal mass in the anatomic place of right kidney, while no kidney or ureter was observed at that side. He underwent surgical resection of the tumor with a primary impression of Wilms tumor. To the authors’ knowledge, this is the first case of retroperitoneal malignant peripheral nerve sheath tumor and absent kidney. This case suggests the very rare probability of association of MPNSTs in children with genitourinary tract anomalies such as renal agenesis.

  3. Malignant Peripheral Nerve Sheath Tumor in the Maxilla: Report of a Rare Case

    Directory of Open Access Journals (Sweden)

    Jahanshah Salehinejad

    2013-12-01

    Full Text Available Malignant peripheral nerve sheath tumor (MPNST is a rare malignant tumor that develops either from a preexisting neurofibroma or de novo. The cell of origin is believed to be the Schwann cell and possibly other nerve sheath cells. In this report, we describe a rare case of MPNST that arise from the socket of second left maxillary molar that has been already extracted in a young man. He was referred to a dentist’s office with a tumor-like mass of soft tissue on his left maxillary gingiva. Biopsy and histopathologic examination was performed and based on histologic and immuno-histochemical findings, the diagnosis of MPNST was made. MPNST is a rare malignant tumor in the oral cavity. Dentists must be careful and conscious because this rare malignancy can occur in gingiva and can mimic the clinical feature of any benign gingival enlargements.

  4. Malignant Peripheral Nerve Sheath Tumor in the Maxilla: Report of a Rare Case

    Directory of Open Access Journals (Sweden)

    Jahanshah Salehinejad

    2013-01-01

    Full Text Available Malignant peripheral nerve sheath tumor (MPNST is a rare malignant tumor that develops either from a preexisting neurofibroma or de novo. The cell of origin is believed to be the Schwann cell and possibly other nerve sheath cells. In this report, we describe a rare case of MPNST that arise from the socket of second left maxillary molar that has been already extracted in a young man. He was referred to a dentist’s office with a tumor-like mass of soft tissue on his left maxillary gingiva. Biopsy and histopathologic examination was performed and based on histologic and immuno-histochemical findings, the diagnosis of MPNST was made. MPNST is a rare malignant tumor in the oral cavity. Dentists must be careful and conscious because this rare malignancy can occur in gingiva and can mimic the clinical feature of any benign gingival enlargements.

  5. HDAC8, A Potential Therapeutic Target for the Treatment of Malignant Peripheral Nerve Sheath Tumors (MPNST)

    OpenAIRE

    Gonzalo Lopez; Bill, Kate Lynn J.; Hemant Kumar Bid; Danielle Braggio; Dylan Constantino; Bethany Prudner; Abeba Zewdu; Kara Batte; Dina Lev; Pollock, Raphael E.

    2015-01-01

    Introduction HDAC isoform-specific inhibitors may improve the therapeutic window while limiting toxicities. Developing inhibitors against class I isoforms poses difficulties as they share high homology among their catalytic sites; however, HDAC8 is structurally unique compared to other class I isoforms. HDAC8 inhibitors are novel compounds and have affinity for class I HDAC isoforms demonstrating anti-cancer effects; little is known about their activity in malignant peripheral nerve sheath tu...

  6. EGFR-STAT3 signaling promotes formation of malignant peripheral nerve sheath tumors

    OpenAIRE

    Wu, Jianqiang; Deanna M. Patmore; Jousma, Edwin; Eaves, David W.; Breving, Kimberly; Patel, Ami V.; Schwartz, Eric B.; Fuchs, James R.; Cripe, Timothy P.; Stemmer-Rachamimov, Anat O.; Ratner, Nancy

    2013-01-01

    Malignant peripheral nerve sheath tumors (MPNSTs) develop sporadically or in the context of neurofibromatosis type 1 (NF1). EGFR overexpression has been implicated in MPNST formation, but its precise role and relevant signaling pathways remain unknown. We found that EGFR overexpression promotes mouse neurofibroma transformation to aggressive MPNST (GEM-PNST). Immunohistochemistry demonstrated phosphorylated STAT3 (Tyr705) in both human MPNST and mouse GEM-PNST. A specific JAK2/STAT3 inhibitor...

  7. Intraosseous malignant peripheral nerve sheath tumor with local recurrence, lung metastases and death

    Energy Technology Data Exchange (ETDEWEB)

    Kendi, Tuba Karaguelle [Integra MR Imaging Center, Tahran Cad., No. 38, Kavaklidere, Ankara (Turkey); Center for MR Research, University of Minnesota, 2021 6th Street SE, MN 55455, Minneapolis (United States); Erakar, Aziz; Yildiz, H.Yusuf [Department of Orthopaedic Surgery, Ankara University School of Medicine, 06100, Ankara (Turkey); Saglik, Yener; Erekul, Selim [Department of Pathology, Ankara University School of Medicine, 06100, Ankara (Turkey)

    2004-04-01

    Malignant peripheral nerve sheath tumor (MPNST) is almost always seen in soft tissue. Skeletal involvement by MPNST is uncommon and usually results from secondary invasion. Primary MPNSTs are exceptionally rare. We report a surgically proven case of intraosseous MPNST, with local recurrence and lung metastasis during follow-up. The imaging and histological features of the case are described and the literature on the subject briefly reviewed. (orig.)

  8. Lost in Translation: Ambiguity in Nerve Sheath Tumor Nomenclature and Its Resultant Treatment Effect

    Energy Technology Data Exchange (ETDEWEB)

    Bernthal, Nicholas M., E-mail: Nicholas.bernthal@hsc.utah.edu; Jones, Kevin B.; Monument, Michael J. [Sarcoma Services, Department of Orthopaedics, Huntsman Cancer Institute and Primary Childrens Medical Center, University of Utah, Salt Lake City, UT 84112 (United States); Liu, Ting [Department of Pathology, Huntsman Cancer Institute, University of Utah, Salt Lake City, UT 84112 (United States); Viskochil, David [Division of Medical Genetics, Department of Pediatrics, University of Utah, Salt Lake City, UT 84112 (United States); Randall, R. Lor, E-mail: Nicholas.bernthal@hsc.utah.edu [Sarcoma Services, Department of Orthopaedics, Huntsman Cancer Institute and Primary Childrens Medical Center, University of Utah, Salt Lake City, UT 84112 (United States)

    2013-05-08

    There is much ambiguity surrounding the diagnosis of nerve sheath tumors, including atypical neurofibroma and low-grade MPNST, and yet, the distinction between these entities designates either benign or malignant behavior and thus carries presumed profound prognostic importance that often guides treatment. This study reviews the diagnostic criteria used to designate atypical neurofibroma from low-grade MPNSTs and reviews existing literature the natural history of each of these tumors to see if the distinction is, in fact, of importance.

  9. Sporadic Multifocal Malignant Peripheral Nerve Sheath Tumor-A Rare Presentation: Multifocal MPNST.

    Science.gov (United States)

    Leena, J B; Fernandes, Hilda; Swethadri, G K

    2013-06-01

    Malignant peripheral nerve sheath tumors(MPNST) are uncommon neoplasms with an incidence of 0.001% in general population. Multifocality is a rare manifestation of MPNST . A case of a 65 year old patient who presented with multiple swellings involving the neck, extremity and back without associated neurofibromatosis is reported for its rarity of presentation.. Diagnosis was made by FNAC and confirmed by peroperative findings and histopathology.

  10. Engineered Herpes Simplex Viruses for the Treatment of Malignant Peripheral Nerve Sheath Tumors

    Science.gov (United States)

    2014-09-01

    treating nervous system tumors. We have genetically modified these viruses to make them safe and unable to grow in normal cells, but they will grow in...methods (plaque-titering at 24hr-intervals boost infection; single-step & multi-step replication assays) and FACS monitoring the extent and time course...INTRODUCTION Malignant peripheral nerve sheath tumors (MPNSTs) are a highly aggressive cancer of the peripheral nervous tissue believed to originate within

  11. Intraosseous malignant peripheral nerve sheath tumor in a patient with neurofibromatosis

    Energy Technology Data Exchange (ETDEWEB)

    Terry, D.G. [VA Medical Center, Imaging Service (114-P), 3710 S.W. US Veterans Hospital Road, Portland, OR 97201 (United States); Sauser, D.D. [Department of Radiology, Oregon Health Sciences University Portland, Oregon (United States); Gordon, M.D. [Department of Pathology, Oregon Health Sciences University, Portland, Oregon (United States)

    1998-06-01

    Malignant peripheral nerve sheath tumors (MPNSTs) are uncommon sarcomas that almost always arise in soft tissue. They can develop in pre-existing neurofibromas or schwannomas, de novo from peripheral nerves, or following radiation therapy. Primary intraosseous MPNST is rare and has been reported most frequently in the mandible. Of the reported cases involving the long bones, none has been associated with neurofibromatosis type 1 (NF-1). We report a case of MPNST arising in the femur in a patient with NF-1. (orig.) With 2 figs., 2 tabs., 6 refs.

  12. Thoracic malignant peripheral nerve sheath tumour mimicking a pleural tumour: a rare pedunculated appearance

    Energy Technology Data Exchange (ETDEWEB)

    Komori, Masahiro [Department of Radiology, National Kyushu Cancer Centre, 3-1-1 Notame, Minami-ku, Fukuoka 811-1395 (Japan); Department of Radiology, Munakata Medical Association Hospital, 1201-1 Taguma, Munakata 811-3431 (Japan); Yabuuchi, Hidetake; Kuroiwa, Toshiro [Department of Radiology, National Kyushu Cancer Centre, 3-1-1 Notame, Minami-ku, Fukuoka 811-1395 (Japan); Nagatoshi, Yoshihisa [Department of Paediatrics, National Kyushu Cancer Centre, Fukuoka (Japan); Ichinose, Yukito [Department of Thoracic Surgery, National Kyushu Cancer Centre, Fukuoka (Japan); Hachitanda, Yoichi [Department of Pathology, National Kyushu Cancer Centre, Fukuoka (Japan)

    2003-08-01

    A malignant peripheral nerve sheath tumour (MPNST) generally occurs in adults and often in patients with neurofibromatosis-1 (NF-1). We present a rare case of a huge thoracic MPNST arising from the intercostal nerve in a 12-year-old girl without NF-1. In addition to the unusual occurrence in a child without NF-1, MRI demonstrated a unique pedunculated appearance mimicking a pleural tumour. In this report, we present the CT and MRI findings of our case, together with the histopathological findings, and review previous reports. (orig.)

  13. Multimodal imaging in neurofibromatosis type 1-associated nerve sheath tumors; Multimodale Bildgebung bei Neurofibromatose-Typ-1-assoziierten Nervenscheidentumoren

    Energy Technology Data Exchange (ETDEWEB)

    Salamon, J.; Adam, G. [University Medical Center Hamburg-Eppendorf, Hamburg (Germany). Dept. of Diagnostic and Interventional Radiology; Mautner, V.F. [University Medical Center Hamburg-Eppendorf, Hamburg (Germany). Dept. of Neurology; Derlin, T. [Hannover Medical School, Hannover (Germany). Dept. of Nuclear Medicine

    2015-12-15

    Neurofibromatosis type 1 (NF1) is a neurogenetic disorder. Individuals with NF1 may develop a variety of benign and malignant tumors of which peripheral nerve sheath tumors represent the most frequent entity. Plexiform neurofibromas may demonstrate a locally destructive growth pattern, may cause severe symptoms and may undergo malignant transformation into malignant peripheral nerve sheath tumors (MPNSTs). Whole-body magnetic resonance imaging (MRI) represents the reference standard for detection of soft tissue tumors in NF1. It allows for identification of individuals with plexiform neurofibromas, for assessment of local tumor extent, and for evaluation of whole-body tumor burden on T2-weighted imaging. Multiparametric MRI may provide a comprehensive characterization of different tissue properties of peripheral nerve sheath tumors, and may identify parameters associated with malignant transformation. Due to the absence of any radiation exposure, whole-body MRI may be used for serial follow-up of individuals with plexiform neurofibromas. {sup 18}F-fluorodeoxyglucose positron-emission-tomography (FDG PET/CT) allows a highly sensitive and specific detection of MPNST, and should be used in case of potential malignant transformation of a peripheral nerve sheath tumor. PET/CT provides a sensitive whole-body tumor staging. The use of contrast-enhanced CT for diagnosis of peripheral nerve sheath tumors is limited to special indications. To obtain the most precise readings, optimized examination protocols and dedicated radiologists and nuclear medicine physicians familiar with the complex and variable morphologies of peripheral nerve sheath tumors are required.

  14. Imaging of the optic nerve

    Energy Technology Data Exchange (ETDEWEB)

    Becker, Minerva [Head and Neck and Maxillofacial Radiology, Department of Radiology, Geneva University Hospital, Rue Gabrielle-Perret-Gentil 4, CH - 1211 Geneva 14 (Switzerland)], E-mail: minerva.becker@hcuge.ch; Masterson, Karen [Head and Neck and Maxillofacial Radiology, Department of Radiology, Geneva University Hospital, Rue Gabrielle-Perret-Gentil 4, CH - 1211 Geneva 14 (Switzerland); Delavelle, Jacqueline [Neuroradiology, Department of Radiology, Geneva University Hospital, Rue Gabrielle-Perret-Gentil 4, CH - 1211 Geneva 14 (Switzerland); Viallon, Magalie [Department of Radiology, Geneva University Hospital, Rue Gabrielle-Perret-Gentil 4, CH - 1211 Geneva 14 (Switzerland); Vargas, Maria-Isabel [Neuroradiology, Department of Radiology, Geneva University Hospital, Rue Gabrielle-Perret-Gentil 4, CH - 1211 Geneva 14 (Switzerland); Becker, Christoph D. [Department of Radiology, Geneva University Hospital, Rue Gabrielle-Perret-Gentil 4, CH - 1211 Geneva 14 (Switzerland)

    2010-05-15

    This article provides an overview of the imaging findings of diseases affecting the optic nerve with special emphasis on clinical-radiological correlation and on the latest technical developments in MR imaging and CT. The review deals with congenital malformations, tumors, toxic/nutritional and degenerative entities, inflammatory and infectious diseases, compressive neuropathy, vascular conditions and trauma involving the optic nerve from its ocular segment to the chiasm. The implications of imaging findings on patient management and outcome and the importance of performing high-resolution tailored examinations adapted to the clinical situation are discussed.

  15. Optic nerve invasion of uveal melanoma

    DEFF Research Database (Denmark)

    Lindegaard, Jens; Isager, Peter; Prause, Jan Ulrik

    2007-01-01

    The aim of the study was to identify the histopathological characteristics associated with the invasion of the optic nerve of uveal melanoma and to evaluate the association between invasion of the optic nerve and survival. In order to achieve this, all uveal melanomas with optic nerve invasion...... in Denmark between 1942 and 2001 were reviewed (n=157). Histopathological characteristics and depth of optic nerve invasion were recorded. The material was compared with a control material from the same period consisting of 85 cases randomly drawn from all choroidal/ciliary body melanomas without optic nerve......; and 4) in one case a tumor spread along the inner limiting membrane to the optic nerve through the lamina cribrosa. Invasion of the optic nerve had no impact on all-cause mortality or melanoma-related mortality in multivariate analyses. The majority of melanomas invading the optic nerve are large...

  16. Initial Pattern of Optic Nerve Enhancement in Korean Patients with Unilateral Optic Neuritis

    Science.gov (United States)

    Son, Dae Yong; Park, Kyung-Ah; Seok, Su Sie; Lee, Ju-Yeun

    2017-01-01

    Purpose The purpose of this study was to demonstrate whether the pattern of optic nerve enhancement in magnetic resonance imaging (MRI) can help to differentiate between idiopathic optic neuritis (ON), neuromyelitis optica (NMO), and multiple sclerosis (MS) in unilateral ON. Methods An MRI of the brain and orbits was obtained in patients with acute unilateral ON. Patients with ON were divided into three groups: NMO, MS, and idiopathic ON. The length and location of the abnormal optic nerve enhancement were compared for ON eyes with and without NMO or MS. The correlation between the pattern of optic nerve enhancement and the outcome of visual function was analyzed. Results Of the 36 patients with ON who underwent an MRI within 2 weeks of the onset, 19 were diagnosed with idiopathic ON, 9 with NMO, and 8 with MS. Enhancement of the optic nerve occurred in 21 patients (58.3%) and was limited to the orbital segment in 12 patients. Neither the length nor the location of the optic nerve enhancement was significantly correlated with visual functions other than contrast sensitivity or the diagnosis of idiopathic ON, MS, or NMO. Patients with greater extent of optic nerve sheath enhancement and more posterior segment involvement showed higher contrast sensitivity. Conclusions Our data revealed that the pattern of optic nerve enhancement was not associated with diagnosis of idiopathic ON, NMO, or MS in Korean patients with unilateral ON. We believe further studies that include different ethnic groups will lead to a more definitive answer on this subject. PMID:28243026

  17. Phase I Trial of Intratumoral Administration of NIS Expressing Strain of Measles Virus in Unresectable or Recurrent Malignant Peripheral Nerve Sheath Tumor

    Science.gov (United States)

    2016-10-01

    or Recurrent Malignant Peripheral Nerve Sheath Tumor PRINCIPAL INVESTIGATOR: Dusica Babovic-Vuksanovic, MD CONTRACTING ORGANIZATION: Mayo Clinic...20164. TITLE AND SUBTITLE Recurrent Malignant Peripheral Nerve Sheath Tumor 5a. CONTRACT NUMBER Phase I Trial of Intratumoral Administration of NIS...Expressing Strain of Measles Virus in Unresectable or Recurrent Malignant Peripheral Nerve Sheath Tumor 5b. GRANT NUMBER W81XWH-15-1-0115 5c. PROGRAM

  18. Malignant peripheral nerve sheath tumor arising from the greater omentum: Case report

    Directory of Open Access Journals (Sweden)

    Tokunaga Masakazu

    2011-03-01

    Full Text Available Abstract Malignant peripheral nerve sheath tumors (MPNSTs are rare soft tissue tumors that arise from a peripheral nerve or exhibit nerve sheath differentiation. Most of these tumors arise on the trunk, extremities, or head and neck regions; they are very rarely located in the abdominal cavity. The patient was a 71-year-old man who was referred to our hospital for a mass and pain in the right lower abdomen. Abdominal computed tomography revealed a large (9 × 9 cm, well-circumscribed, lobulated, heterogeneously enhanced mass in the pelvis. Exploratory laparotomy revealed a large mass in the greater omentum, and the tumor was completely excised. Histopathological analysis revealed that the tumor was composed of spindle cells with high mitotic activity. On staining the tumor, positive results were obtained for S-100 but negative results were obtained for c-kit, cluster of differentiation (CD34, α-smooth muscle actin, and desmin. These findings strongly supported a diagnosis of MPNST primarily arising from the greater omentum. To the best of our knowledge, this is the first reported case of an MPNST arising from the greater omentum. In this report, we have described the case of a patient with an MPNST arising from the greater omentum and have discussed the clinical characteristics and management of MPNSTs.

  19. Optic Nerve Decompression for Orbitofrontal Fibrous Dysplasia

    OpenAIRE

    Abe, Takumi; Sato, Kaneshige; Otsuka, Takaharu; Kawamura, Noriyoshi; Shimazu, Motohiko; Izumiyama, Hitoshi; Matsumoto, Kiyoshi

    2002-01-01

    Orbitofrontal fibrous dysplasia often involves the bony orbit and the optic canal. Although fibrous dysplasia reportedly produces compression of the optic nerve leading to visual distrubances, optic nerve decompression in patients without clinical signs of optic neuropathy is still controversial. We describe two patients with orbitofrontal fibrous dysplasia without signs of visual disturbance and one patient with McCune-Albright syndrome and progressive visual impairment. Optic nerve decompre...

  20. Lost in Translation: Ambiguity in Nerve Sheath Tumor Nomenclature and Its Resultant Treatment Effect

    Directory of Open Access Journals (Sweden)

    R. Lor Randall

    2013-05-01

    Full Text Available There is much ambiguity surrounding the diagnosis of nerve sheath tumors, including atypical neurofibroma and low-grade MPNST, and yet, the distinction between these entities designates either benign or malignant behavior and thus carries presumed profound prognostic importance that often guides treatment. This study reviews the diagnostic criteria used to designate atypical neurofibroma from low-grade MPNSTs and reviews existing literature the natural history of each of these tumors to see if the distinction is, in fact, of importance.

  1. Potential of boron neutron capture therapy (BNCT) for malignant peripheral nerve sheath tumors (MPNST).

    Science.gov (United States)

    Fujimoto, Takuya; Andoh, Tooru; Sudo, Tamotsu; Fujita, Ikuo; Fukase, Naomasa; Takeuchi, Tamotsu; Sonobe, Hiroshi; Inoue, Masayoshi; Hirose, Tkanori; Sakuma, Toshiko; Moritake, Hiroshi; Sugimoto, Tohru; Kawamoto, Teruya; Fukumori, Yoshinobu; Yamamoto, Satomi; Atagi, Shinji; Sakurai, Yoshinori; Kurosaka, Masahiro; Ono, Koji; Ichikawa, Hideki; Suzuki, Minoru

    2015-12-01

    Malignant peripheral nerve sheath tumors (MPNST) are relatively rare neoplasms with poor prognosis. At present there is no effective treatment for MPNST other than surgical resection. Nonetheless, the anti-tumor effect of boron neutron capture therapy (BNCT) was recently demonstrated in two patients with MPNST. Subsequently, tumor-bearing nude mice subcutaneously transplanted with a human MPNST cell line were injected with p-borono-L-phenylalanine (L-BPA) and subjected to BNCT. Pathological studies then revealed that the MPNST cells were selectively destroyed by BNCT.

  2. Malignant peripheral nerve sheath tumour (MPNST) of mandible: solving the perplexity.

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    Patel, Shilpa; Pathak, Jigna; Dekate, Kamlesh; Mohanty, Neeta

    2015-03-11

    We present an extremely rare case of malignant peripheral nerve sheath tumour (MPNST) in a 30-year-old woman without associated neurofibromatosis 1. The patient presented with an 8 cm×4 cm lesion extending from 46 to the retro molar region involving the ramus of the right mandible associated with regional paraesthesia. Incisional biopsy revealed spindle cells with vesicular nuclei arranged in fascicles leading to a diagnosis of spindle cell lesion. Posterior segmental mandibulectomy was performed under general anaesthesia. On excisional biopsy, a definitive diagnosis of low-grade MPNST was established on the basis of immunohistochemistry. The patient was then lost to follow-up.

  3. Huge Intrathoracic Malignant Peripheral Nerve Sheath Tumor in an Adolescent with Neurofibromatosis Type 1

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    Jong Hyung Yoon

    2014-01-01

    Full Text Available Malignant peripheral nerve sheath tumor (MPNST is a rare soft tissue malignancy usually found in patients with neurofibromatosis type 1 (NF1 with a poor outcome. Although MPNST can be found in any part of the body including head and neck or extremities, intrathoracic MPNST with or without NF1 is uncommon, especially in children or adolescents. Reported herein is a case of huge intrathoracic MPNST in a 16-year-old girl with NF1, and a brief review of the literature.

  4. A CASE REPORT OF LOW GRADE MALIGNANT PERIPHERAL NERVE SHEATH TUMOUR

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    Sampath Kumar

    2015-01-01

    Full Text Available Malignant nerve sheath tumours [syn : Neurogenic sarcoma , Neuro fibro sarcoma] comprise approximately 5% of all soft tissue sarcomas. Only 8to15% of these tumours arises in Head and neck region. Commonest sites in head and neck region a re lateral skull base or along the course of cranial nerves. These tumours in sino nasal region are extreme rare. In this article we report a rare case of low grade MPNST. The tumour was resected by medial maxillectomy via modified Denker extended maxillar y anterior antrostomy , resected tumour Histopathological examination and immunohistochemistry study reported as low grade MPNST and negative for s - 100 protein. Patient was relieved from symptoms and followed for 18 months.

  5. Intraosseous malignant peripheral nerve sheath tumor with focal epithelioid differentiation of the thoracic spine

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    Miyakoshi Naohisa

    2007-01-01

    Full Text Available The authors describe an extremely rare case with malignant peripheral nerve sheath tumor (MPNST with focal epithelioid differentiation presenting as an intraosseous lesion of the spine. A 75-year-old woman presented with progressive paraplegia caused by epidural mass arising from the posterior element of the T7 vertebra. At surgery, the lesion was noted to originate from the T7 vertebra and separate from the dura and spinal nerve roots. The patient died of tumor metastases to the lungs six months after the initial presentation. Histological diagnosis was MPNST. However, the tumor also contained cystic structures lined by epithelioid cells, requiring differentiation from synovial sarcoma. From the histological and immunohistochemical features, as well as the absence of SYT-SSX fusion gene expression, the diagnosis of MPNST with focal epithelioid differentiation was made. This is the first case report of intraosseous MPNST of the spine with a peculiar biphasic appearance.

  6. Giant Sporadic Low Grade Malignant Peripheral Nerve Sheath (MPNST) of Left Thigh.

    Science.gov (United States)

    Nikumbh, Dhiraj B; Suryawanshi, K H; Dravid, N V; Patil, T B; Rokade, C M

    2013-06-01

    Malignant Peripheral Nerve Sheath Tumours (MPNST) are rare spindle- cell sarcomas which are derived from the schwann cells or the pleuripotent cells of the neural crest. MPNSTs are usually located in the peripheral nerve trunk roots, extremities and in the head and neck region. These tumours may arise as sporadic variants or in patients with neurofibromatosis1 (NF1). The estimated incidence of MPNSTs in the patients with NF1is 2-5% as compared to a 0.001% incidence in the general population. Herein, we are reporting a case of a giant sporadic (NF1 independent) low grade MPNST of the left thigh in 65 year female patient, which had a good prognosis.

  7. Epineurial sheath tube (EST) technique: an experimental peripheral nerve repair model.

    Science.gov (United States)

    Bozkurt, Ahmet; Dunda, Sebastian E; Mon O'Dey, Dan; Brook, Gary A; Suschek, Christoph V; Pallua, Norbert

    2011-12-01

    Here we present the epineurial sheath tube (EST) technique as a modified microsurgical rat sciatic nerve model. The EST technique provides a cavity or pouch consisting of an outer epineurial sleeve that has been freed from nerve fascicles. This cavity may be appropriate to test the effectiveness and biocompatibility of implanted growth factors, cell suspensions (embedded in solutions or gels), or bioartificial nerve guide constructs. A total number of 10 rats underwent the surgical procedure for the EST technique. Cylinders made of fibrin gel served as implants and place-holders. Three animals were euthanized directly after operation, while the others survived for 6 weeks. After immersion fixation (3·9% glutaraldehyde), both conventional histology [semi-thin sections (1 μm), toluidine blue] and scanning electron microscopy were performed. Conventional histology and scanning electron microscopy of samples that had been fixed directly after the surgical procedure displayed the integrity of the closed epineurial tube with the fibrin cylinder in its center. Even after 6 weeks, the outer epineurium was not lacerated, the stitches did not loosen, and the lumen did not collapse, but remained open. The practicability of the EST technique could be verified regarding feasibility, reproducibility, mechanical stability, and openness of the lumen. The EST technique can be adapted to other nerve models (e.g. median or facial nerve). It provides a cavity or pouch, which can be used for different neuroscientific approaches including concepts to improve the therapeutic benefit of autologous nerve grafting or therapies to be used as an alternative to autologous nerve grafting.

  8. Feline peripheral nerve sheath tumors: histologic, immunohistochemical, and clinicopathologic correlation (59 tumors in 53 cats).

    Science.gov (United States)

    Schulman, F Y; Johnson, T O; Facemire, P R; Fanburg-Smith, J C

    2009-11-01

    Feline peripheral nerve sheath tumors are uncommonly reported, and their clinical behavior has not been well documented. Fifty-nine peripheral nerve sheath tumors were collected from 53 cats. All of the tumors involved skin, subcutis, skeletal muscle, and/or mucous membranes. Histologically, the tumors were composed of compact to loosely arranged streams and fascicles of spindled cells with eosinophilic, often wavy cytoplasmic processes; small to occasionally moderate amounts of collagenous to myxoid matrix; and nuclear palisading. Immunohistochemically, all tumors were positive for vimentin and S-100 protein, 44 of 59 were positive for glial fibrillary acidic protein (GFAP), and all were negative for muscle specific actin. The tumors fell into 3 histologic categories: 34 benign tumors with Antoni A areas that were S-100 protein and GFAP positive, 9 benign tumors that lacked Antoni A areas and were S-100 protein positive and GFAP negative, and 16 tumors with features of malignancy. Seventy-five percent of these cases involved the head, neck, or limbs. Recurrent tumors were submitted or tumors were reported to have recurred in 9 cases. Tumor recurrence was reported for all 3 of the histologic subtypes. None was documented as having metastasized.

  9. MMP-13, p53 in the Progression of Malignant Peripheral Nerve Sheath Tumors

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    Nikola Holtkamp

    2007-08-01

    Full Text Available Malignant peripheral nerve sheath tumors (MPNST are sarcomas with poor prognosis, limited treatment options. Factors contributing to tumor progression are largely unknown. We therefore examined MPNST from 22 neurofibromatosis type 1 (NF1 patients, 14 nonNF1 patients, 14 neurofibroma patients for matrix metalloproteinase 13 (MMP-13 expression. Because wild-type, mutant p53 were shown to differentially regulate MMP-13 expression, TP53 status, protein levels were also determined. MMP-13 expression was detected in 58% of MPNST, was significantly associated with recurrent MPNST (P = .019. p53 was observed in 78% of MPNST, was found to be strongly associated with MMP-13 expression (P = .005. In contrast, 14 neurofibromas lacked MMP-13, p53 expressions. TP53 mutations were found in only 11% of MPNST, were associated with high tumor grades (P = .029. No significant association between mutant TP53, MMP-13 was observed, indicating that other factors drive MMP-13 expression in MPNST. The presence of metastasis was linked to p53Pro72 polymorphism (P= .041, shorter survival. In summary, our data suggest that MMP-13 expression in nerve sheath tumors is coupled with malignant progression. Therefore, MMP-13 may serve as a marker for progression, as a therapeutic target.

  10. Effects of pigment epithelium derived factor (PEDF) on malignant peripheral nerve sheath tumours (MPNSTs).

    Science.gov (United States)

    Demestre, Maria; Terzi, Menderes Yusuf; Mautner, Victor; Vajkoczy, Peter; Kurtz, Andreas; Piña, Ana Luisa

    2013-12-01

    Neurofibromatosis type 1 (NF1) is an inherited genetic disease affecting 1 in 3,500 individuals. A prominent feature of NF1 is the formation of benign tumours of the peripheral nerve sheath (neurofibromas). However, these can become malignant and form highly metastatic malignant peripheral nerve sheath tumours (MPNST), which are usually fatal despite aggressive surgery, chemotherapy, and radiotherapy. Recent studies have shown that pigment epithelium-derived factor (PEDF) can induce differentiation and inhibit angiogenesis in several kinds of tumours. The present study was designed to determine the in vitro and in vivo effects of PEDF on MPNST angiogenesis and tumour growth. PEDF inhibited proliferation and augmented apoptosis in S462 MPNST cells after 48 h of treatment in culture. In xenografts of S462 MPNST cells in athymic nude mice, PEDF suppressed MPNST tumour burden, due mainly to inhibition of angiogenesis. These results demonstrate for the first time inhibitory effects of PEDF on the growth of human MPNST via induction of anti-angiogenesis and apoptosis. Our results suggest that PEDF could be a novel approach for future therapeutic purposes against MPNST.

  11. Cancer stem cell-like cells derived from malignant peripheral nerve sheath tumors.

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    Melanie Spyra

    Full Text Available This study aims to examine whether or not cancer stem cells exist in malignant peripheral nerve sheath tumors (MPNST. Cells of established lines, primary cultures and freshly dissected tumors were cultured in serum free conditions supplemented with epidermal and fibroblast growth factors. From one established human MPNST cell line, S462, cells meeting the criteria for cancer stem cells were isolated. Clonal spheres were obtained, which could be passaged multiple times. Enrichment of stem cell-like cells in these spheres was also supported by increased expression of stem cell markers such as CD133, Oct4, Nestin and NGFR, and decreased expression of mature cell markers such as CD90 and NCAM. Furthermore, cells of these clonal S462 spheres differentiated into Schwann cells, smooth muscle/fibroblast and neurons-like cells under specific differentiation-inducing cultural conditions. Finally, subcutaneous injection of the spheres into immunodeficient nude mice led to tumor formation at a higher rate compared to the parental adherent cells (66% versus 10% at 2.5 × 10(5. These results provide evidence for the existence of cancer stem cell-like cells in malignant peripheral nerve sheath tumors.

  12. Breast metastases from a malignant peripheral nerve sheath tumor of the kidney: An unusual presentation

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    Shalini Koppisetty

    2016-01-01

    Full Text Available Malignant peripheral nerve sheath tumors (MPNSTs are extremely rare soft tissue sarcomas of ectomesenchymal origin. They are commonly seen in association with neurofibromatosis type 1 (NF-1, but can also occur without a history of NF (isolated MPNST. MPNSTs are most commonly located on the extremities (brachial and sacral plexus, head and neck, and trunk regions and are rarely reported in genitourinary organs. These tumors are aggressive, with a high recurrence rate and distant metastases. MPNST involving the kidney is extremely rare, and review of the literature using PubMed from 2001 to 2014 revealed eight cases of MPNST involving the kidney (seven, primarily involving the kidney and one metastatic MPNST of the kidney. Herein, we describe a case of breast metastases from an MPNST of the kidney without a history of NF-1. The patient was initially diagnosed with a spindle cell neoplasm of the kidney with peripheral nerve sheath differentiation. Eventually, the patient developed a right breast mass that was diagnosed as metastatic MPNST. The patient refused any kind of treatment and died 6 months later in hospice care.

  13. Breast metastases from a malignant peripheral nerve sheath tumor of the kidney: An unusual presentation

    Science.gov (United States)

    Koppisetty, Shalini; Alessio, Ricardo C.; Rajpurkar, Atul

    2016-01-01

    Malignant peripheral nerve sheath tumors (MPNSTs) are extremely rare soft tissue sarcomas of ectomesenchymal origin. They are commonly seen in association with neurofibromatosis type 1 (NF-1), but can also occur without a history of NF (isolated MPNST). MPNSTs are most commonly located on the extremities (brachial and sacral plexus), head and neck, and trunk regions and are rarely reported in genitourinary organs. These tumors are aggressive, with a high recurrence rate and distant metastases. MPNST involving the kidney is extremely rare, and review of the literature using PubMed from 2001 to 2014 revealed eight cases of MPNST involving the kidney (seven, primarily involving the kidney and one metastatic MPNST of the kidney). Herein, we describe a case of breast metastases from an MPNST of the kidney without a history of NF-1. The patient was initially diagnosed with a spindle cell neoplasm of the kidney with peripheral nerve sheath differentiation. Eventually, the patient developed a right breast mass that was diagnosed as metastatic MPNST. The patient refused any kind of treatment and died 6 months later in hospice care. PMID:27453670

  14. Nerve sheath myxoma (neurothekeoma) of the skin: light microscopic and immunohistochemical reappraisal of the cellular variant.

    Science.gov (United States)

    Argenyi, Z B; LeBoit, P E; Santa Cruz, D; Swanson, P E; Kutzner, H

    1993-08-01

    Nerve sheath myxoma (NSM) is a rare cutaneous neoplasm, the histogenesis of which is controversial. Fifteen cases of NSM were studied by routine light microscopy and with a broad panel of immunohistochemical stains. NSM were classified into three groups based on cellularity, mucin content and growth pattern. 1) The hypocellular (myxoid) type (5/15 cases) showed frequent encapsulation or sharp circumscription. Immunohistochemically this type was strongly positive for S-100 protein and collagen type IV and variably positive for epithelial membrane antigen. 2) The cellular type (4/15 cases) had scant mucin and ill-defined nodular or infiltrating growth. Immunostaining showed positive reaction for neuron specific enolase (2/4), Leu-7 (1/4) and smooth muscle specific actin (2/4), and was negative with the other antibodies. 3) The "mixed type" (6/15 cases) had variable cellularity and mucin content with poor demarcation and variable immunolabeling. We conclude that: 1) there are major light microscopic and immunohistochemical differences between the classical hypocellular (myxoid) and the cellular forms of NSM (neurothekeoma); 2) while the immunohistochemical results support the presence of nerve sheath differentiation in the classical forms of NSM, and to some extent in the mixed forms, there is an absence of convincing evidence of neural differentiation in the cellular variant by either light microscopy or immunohistochemistry; 3) the variable immunophenotypes suggest that differentiation other than neural may take place in CNT.

  15. Diffusion tensor tractography for the surgical management of peripheral nerve sheath tumors.

    Science.gov (United States)

    Schmidt, Manfred; Kasprian, Gregor; Amann, Gabriele; Duscher, Dominik; Aszmann, Oskar C

    2015-09-01

    OBJECT Peripheral nerve sheath tumors (PNSTs) are uncommon but bear a significant risk of malignancy. High-resolution MRI is the standard technique for characterizing PNSTs. However, planning the appropriate extent of resection and subsequent reconstructive strategies is highly dependent on the intraoperative findings because preoperative MRI evaluation can be insufficient. Diffusion tensor tractography (DTT) represents a recently developed advanced MRI technique that reveals the microstructure of tissues based on monitoring the random movement of water molecules. DTT has the potential to provide diagnostic insights beyond conventional MRI techniques due to its mapping of specific fibrillar nerve structures. Here, DTT was applied to evaluate PNSTs and to examine the usefulness of this method for the correct delineation of tumor and healthy nerve tissue and the value of this information in the preoperative planning of surgical interventions. METHODS In this prospective study, patients with the clinical symptoms of a PNST were investigated using DTT 3-Tesla MRI scans. Image data processing and tractography were performed using the FACT (fiber assessment by continuous tracking) algorithm and multiple-regions-of-interest approach. The surgical findings were then compared with the results of the DTT MRI scans. Preoperative fascicle visualization and the correlation with the intraoperative findings were graded. RESULTS In a 21-month period, 12 patients with PNSTs were investigated (7 female and 5 male patients with a mean age of 46.2 ± 19.2 years). All patients underwent surgical removal of the tumor. Schwannoma was the most common benign histopathological finding (n = 7), whereas 2 malignant lesions were detected. In 10 of 12 patients, good preoperative nerve fascicle visualization was achieved using DTT scans. In 9 of 10 patients with good preoperative fascicle visualization, good intraoperative correlation between the DTT scans and surgical anatomy was found

  16. A multi-walled silk fibroin/silk sericin nerve conduit coated with poly(lactic-co-glycolic acid) sheath for peripheral nerve regeneration.

    Science.gov (United States)

    Rao, Jianwei; Cheng, Yan; Liu, Yanxiao; Ye, Zhou; Zhan, Beilei; Quan, Daping; Xu, Yangbin

    2017-04-01

    The linearly oriented multi-walled silk fibroin/silk sericin (SF/SS) nerve conduits (NCs) can provide physical cues similar to native peripheral nerve fasciculi, but the mechanical properties of which are not excellent enough. In this study, NCs with a novel and bionic design with dual structures were developed. The important features of our NCs is that the internal skeleton (the multi-walled SF/SS conduits) has a bionic structure similar to the architecture of native peripheral nerve fasciculi, which is beneficial for nerve regeneration, and the outer sheath (the hollow poly(lactic-co-glycolic acid) [PLGA] conduits) could provide strong mechanical protection for the internal skeleton. The linearly oriented multi-walled SF/SS conduit was fabricated and inserted in the hollow PLGA sheath lumen and then used for the bridge across the sciatic nerve defect in rats. The outcome of the peripheral nerve repair post implantation was evaluated. The functional and morphological parameters were examined and showed that the novel PLGA-coated SF/SS NCs could promote peripheral nerve regeneration, approaching those elicited by nerve autografts that are the first candidate for repair of peripheral nerve defects. Thus, these updated NCs have potential usefulness to enhance functional recovery after repair of peripheral nerve defect. Copyright © 2016 Elsevier B.V. All rights reserved.

  17. Unexpected Effect of Calcium Channel Blockers on the Optic Nerve Compartment Syndrome.

    Science.gov (United States)

    Konieczka, K; Todorova, M G; Bojinova, R I; Binggeli, T; Chackathayil, T N; Flammer, J

    2016-04-01

    The optic nerve compartment syndrome is a pathological condition in which cerebrospinal fluid of the subarachnoid space surrounding the optic nerve is partly or totally segregated from the cerebrospinal fluid of the intracranial subarachnoid space, leading - inter alia - to an increase in the diameter of the optic nerve sheath. The pathogenesis of this condition remains unclear. We have observed clinically that optic nerve compartment syndrome often occurs in normal tension glaucoma patients with Flammer syndrome. To treat Flammer syndrome, some glaucoma patients received a low dose of a calcium channel blocker and we analysed whether this treatment also had an effect on the optic nerve compartment syndrome. We retrospectively analysed the data of 10 eyes of seven patients suffering from a combination of primary open angle glaucoma, optic nerve compartment syndrome, and Flammer syndrome. We included subjects who had eye socket echography before and after a few months of therapy with a calcium channel blocker. All patients received a low dose of a calcium channel blocker (nifedipine or amlodipine) to treat Flammer syndrome. As expected, the symptoms of Flammer syndrome were mitigated. To our surprise, the optic nerve compartment syndrome also improved in eight of the 10 eyes (80 %), but remained unchanged in the remainder. To some extent, the optic nerve compartment syndrome is related to the combination of primary open angle glaucoma and Flammer syndrome. On the basis of our results, we hypothesise that treatment of Flammer syndrome may also improve the optic nerve compartment syndrome. Georg Thieme Verlag KG Stuttgart · New York.

  18. Electrophoresis of polar fluorescent tracers through the nerve sheath labels neuronal populations for anatomical and functional imaging

    Science.gov (United States)

    Isaacson, Matthew D.; Hedwig, Berthold

    2017-01-01

    The delivery of tracers into populations of neurons is essential to visualize their anatomy and analyze their function. In some model systems genetically-targeted expression of fluorescent proteins is the method of choice; however, these genetic tools are not available for most organisms and alternative labeling methods are very limited. Here we describe a new method for neuronal labelling by electrophoretic dye delivery from a suction electrode directly through the neuronal sheath of nerves and ganglia in insects. Polar tracer molecules were delivered into the locust auditory nerve without destroying its function, simultaneously staining peripheral sensory structures and central axonal projections. Local neuron populations could be labelled directly through the surface of the brain, and in-vivo optical imaging of sound-evoked activity was achieved through the electrophoretic delivery of calcium indicators. The method provides a new tool for studying how stimuli are processed in peripheral and central sensory pathways and is a significant advance for the study of nervous systems in non-model organisms. PMID:28084413

  19. Magnetic resonance imaging of optic nerve

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    Foram Gala

    2015-01-01

    Full Text Available Optic nerves are the second pair of cranial nerves and are unique as they represent an extension of the central nervous system. Apart from clinical and ophthalmoscopic evaluation, imaging, especially magnetic resonance imaging (MRI, plays an important role in the complete evaluation of optic nerve and the entire visual pathway. In this pictorial essay, the authors describe segmental anatomy of the optic nerve and review the imaging findings of various conditions affecting the optic nerves. MRI allows excellent depiction of the intricate anatomy of optic nerves due to its excellent soft tissue contrast without exposure to ionizing radiation, better delineation of the entire visual pathway, and accurate evaluation of associated intracranial pathologies.

  20. Convection-Enhanced Delivery (CED) in an Animal Model of Malignant Peripheral Nerve Sheath (MPNST) Tumors and Plexiform Neurofibromas (PN)

    Science.gov (United States)

    2012-09-01

    convection enhanced delivery in malignant peripheral nerve sheath tumors or plexiform neurofibromas at this time. References Perrin GQ, Fishbein L...2007. 85(6): p. 1347-1357. Perrin GQ, Li H, Fishbein L, et al., An orthotopic xenograft model of intraneural NF1 MPNST suggests a potential

  1. Germline and somatic NF1 mutations in sporadic and NF1-associated malignant peripheral nerve sheath tumours

    NARCIS (Netherlands)

    Bottillo, Irene; Ahiquist, Terje; Brekke, Helge; Danielsen, Stine A.; van den Berg, Eva; Mertens, Fredrik; Lothe, Ragnhild A.; Dallapiccola, Bruno

    2009-01-01

    Malignant peripheral nerve sheath tumours (MPNSTs) are a malignancy occurring with increased frequency in patients with neurofibromatosis type 1 (NF1). In contrast to the well-known spectrum of germline NF1 mutations, the information on somatic mutations in MPNSTs is limited. In this study, we scree

  2. Expression patterns of cell cycle components in sporadic and neurofibromatosis type 1-related malignant peripheral nerve sheath tumors

    NARCIS (Netherlands)

    Agesen, Trude Holmeide; Florenes, Viva Ann; Molenaar, Willemina M.; Lind, Guro E.; Berner, Jeane-Marie; Plaat, Boudewijn E.C.; Komdeur, Rudy; Myklebost, Ola; van den Berg, Eva; Lothe, Ragnhild A.

    2005-01-01

    The molecular biology underlying the development of highly malignant peripheral nerve sheath tumors (MPNSTs) remains mostly unknown. In the present study, the expression pattern of 10 selected cell cycle components is investigated in a series of 15 MPNSTs from patients with (n = 9) or without (n = 5

  3. Malignant transformation in a hybrid schwannoma/perineurioma: Addition to the spectrum of a malignant peripheral nerve sheath tumor

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    Bharat Rekhi

    2011-01-01

    Full Text Available Benign nerve sheath tumors include schwannomas, neurofibromas and perineuriomas. The malignant counterpart of a nerve sheath tumor is designated as a malignant peripheral nerve sheath tumor (MPNST. Lately, benign nerve sheath tumors comprising more than one component have been described, including hybrid schwannomas/perineuriomas. However, malignant transformation in a hybrid schwannoma/perineurioma has not been documented so far. Herein, we present a rare case of a young adult male who presented with a soft tissue mass in his right thigh that was excised elsewhere and submitted to us for histopathological review. One of the tissue sections displayed histopathological features of a hybrid schwannoma/perineurioma, including alternate arrangement of benign schwann and perineurial cells, reinforced with S100-P and epithelial membrane antigen positivity, respectively, along with low MIB1 and negative p53 immunostaining. The other two tissue sections showed a spindly sarcomatous tumor that was immunohistochemically positive for S100-P, CD34, p53 and exhibited high MIB1 (30-40%. Diagnosis of a MPNST arising in a hybrid schwannoma/perineurioma was made. This unusual case forms yet another addition to the spectrum of a MPNST.

  4. Epigenetic mechanisms drive the progression of neurofibromas to malignant peripheral nerve sheath tumors

    Science.gov (United States)

    Suresh, Krish; Kliot, Tamara; Piunti, Andrea; Kliot, Michel

    2016-01-01

    Thinking Outside the Box: The polycomb repressive complex 2 (PRC2) is a histone methyltransferase complex known to repress gene expression. There is a large body of experimental evidence that supports its role in promoting tumorigenicity by suppressing tumor suppressor genes. Here, we discuss the surprising findings that, in neurofibromas, it may have a completely different role as a tumor suppressor; mutations of PRC2 lead to conversion of benign neurofibromas into malignant peripheral nerve sheath tumors (MPNSTs) by de-repressing and thereby activating genes driving cell growth and development. These findings have potentially powerful clinical applications in both diagnosing and treating MPNSTs. Hypothesis: PRC2 loss drives malignant transformation of neurofibromas. PMID:27920939

  5. Malignant primary nerve sheath tumor of the cauda equina in a patient without Von Recklinghausen disease

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    Amit Shankar Singh

    2013-01-01

    Full Text Available Malignant peripheral nerve sheath tumor (MPNST is a rare variety of soft-tissue sarcoma of ecto-mesenchymal origin. Various sites have been mentioned, but it is rare in cauda equina. Only five cases of MPNST of cauda equina are reported till date. A 42-year-old male presented with complaints of weakness in lower limbs for last 5 months with bladder and bowel dysfunction. On magnetic resonance imaging, an isointense lesion on T1 and a hyperintense on T2 imaging was found at terminal spinal cord level. The lesion was excised following laminectomy and showed varied cellularity with thin oval, polyhedral and spindle-shaped cells arranged in bundles on microscopic examination suggesting MPNST. MPNST of cauda equina is rare and is associated with Neurofibromatosis Type-1 or radiation exposure, but is rarer without these risk factors. Prognosis is favorable in these cases.

  6. BNIP3 regulates AT101 [(--gossypol] induced death in malignant peripheral nerve sheath tumor cells.

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    Niroop Kaza

    Full Text Available Malignant peripheral nerve sheath tumors (MPNSTs are aggressive Schwann cell-derived sarcomas and are the leading cause of mortality in patients with neurofibromatosis type 1 (NF1. Current treatment modalities have been largely ineffective, resulting in a high rate of MPNST recurrence and poor five-year patient survival. This necessitates the exploration of alternative chemotherapeutic options for MPNST patients. This study sought to assess the cytotoxic effect of the BH3-mimetic AT101 [(--gossypol] on MPNST cells in vitro and to identify key regulators of AT101-induced MPNST cell death. We found that AT101 caused caspase-independent, non-apoptotic MPNST cell death, which was accompanied by autophagy and was mediated through HIF-1α induced expression of the atypical BH3-only protein BNIP3. These effects were mediated by intracellular iron chelation, a previously unreported mechanism of AT101 cytotoxicity.

  7. BNIP3 regulates AT101 [(-)-gossypol] induced death in malignant peripheral nerve sheath tumor cells.

    Science.gov (United States)

    Kaza, Niroop; Kohli, Latika; Graham, Christopher D; Klocke, Barbara J; Carroll, Steven L; Roth, Kevin A

    2014-01-01

    Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive Schwann cell-derived sarcomas and are the leading cause of mortality in patients with neurofibromatosis type 1 (NF1). Current treatment modalities have been largely ineffective, resulting in a high rate of MPNST recurrence and poor five-year patient survival. This necessitates the exploration of alternative chemotherapeutic options for MPNST patients. This study sought to assess the cytotoxic effect of the BH3-mimetic AT101 [(-)-gossypol] on MPNST cells in vitro and to identify key regulators of AT101-induced MPNST cell death. We found that AT101 caused caspase-independent, non-apoptotic MPNST cell death, which was accompanied by autophagy and was mediated through HIF-1α induced expression of the atypical BH3-only protein BNIP3. These effects were mediated by intracellular iron chelation, a previously unreported mechanism of AT101 cytotoxicity.

  8. Synchronous Malignant Peripheral Nerve Sheath Tumor and Adenocarcinoma of the Prostate: Case Report and Literature Review

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    Nikolaos Ferakis

    2016-01-01

    Full Text Available Malignant Peripheral Nerve Sheath Tumors (MPNSTs of the prostate are extremely rare. A very unusual case of simultaneous adenocarcinoma and MPNST of the prostate is reported. A 60-year-old Caucasian male presented for annual urologic examination. Digital rectal examination revealed a painless, toughish, and asymmetrically enlarged prostate. Serum prostate-specific antigen was 1 ng/mL. Radiologic examinations demonstrated a large mass, which was arising from the left peripheral lobe of the prostate. The patient underwent transrectal ultrasound-guided biopsy of the prostate which revealed a smooth muscle tumor of uncertain malignant potential. Radical retropubic prostatectomy with en bloc removal of the mass and the seminal vesicles was performed and histology demonstrated low-grade MPNST and adenocarcinoma of the prostate. To the best of our knowledge, this is the first report of simultaneous prostatic adenocarcinoma and MPNST in the English literature.

  9. Malignant Peripheral Nerve Sheath Tumor of Prostate: A Rare Case Report and Literature Review

    Science.gov (United States)

    Lu, Chih-Cheng; Li, Chien-Feng

    2016-01-01

    A mid-aged male presented with progressive lower urinary tract symptoms (LUTS) for years. Huge prostate with low serum prostate-specific antigen (PSA) level was detected. The specimen from transurethral resection revealed surprising pathology finding as malignant peripheral nerve sheath tumor (MPNST). Considering its huge size (more than 300 gm) and location, we prescribed neoadjuvant chemotherapy firstly. The tumor became regressive and then radical surgical resection was achieved. Adjuvant multimodality treatment including concurrent chemoradiotherapy (CCRT) and target therapy was given. However, he expired about one year later. MPNST originating from prostate is very rare and seldom reported before. We here present this extremely rare disease and share our treatment experience. PMID:27872789

  10. Malignant Peripheral Nerve Sheath Tumor of Prostate: A Rare Case Report and Literature Review

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    Kun-Lin Hsieh

    2016-01-01

    Full Text Available A mid-aged male presented with progressive lower urinary tract symptoms (LUTS for years. Huge prostate with low serum prostate-specific antigen (PSA level was detected. The specimen from transurethral resection revealed surprising pathology finding as malignant peripheral nerve sheath tumor (MPNST. Considering its huge size (more than 300 gm and location, we prescribed neoadjuvant chemotherapy firstly. The tumor became regressive and then radical surgical resection was achieved. Adjuvant multimodality treatment including concurrent chemoradiotherapy (CCRT and target therapy was given. However, he expired about one year later. MPNST originating from prostate is very rare and seldom reported before. We here present this extremely rare disease and share our treatment experience.

  11. Intraoral presentation of multiple malignant peripheral nerve sheath tumors associated with neurofibromatosis-1

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    Mahija Janardhanan

    2011-01-01

    Full Text Available Neurofibromatosis-1 (NF-1 is a relatively common autosomal dominant disease characterized by multiple cutaneous fibromatoses and café au lait spots. It is associated with the mutation of NF-1 gene, a tumor suppressor gene located on chromosome 17q11.2. Hence, it can be considered as a familial cancer predisposition syndrome in which the affected individuals are at increased risk of developing malignancies. Intraoral neurofibromas associated with NF-1 are quite common, but the occurrence of malignant peripheral nerve sheath tumor (MPNST in the oral cavity is very rare. Oral MPNST can occur either de novo or by malignant transformation of neurofibromas or very rarely can represent a metastatic lesion. Here, we present a case of MPNST involving the maxillary region, in a patient with NF-1. Since MPNST often creates a diagnostic dilemma, histopathologic criteria for the diagnosis of MPNST are also discussed.

  12. Synchronous Malignant Peripheral Nerve Sheath Tumor and Adenocarcinoma of the Prostate: Case Report and Literature Review

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    Bouropoulos, Konstantinos; Farmakis, Antonios

    2016-01-01

    Malignant Peripheral Nerve Sheath Tumors (MPNSTs) of the prostate are extremely rare. A very unusual case of simultaneous adenocarcinoma and MPNST of the prostate is reported. A 60-year-old Caucasian male presented for annual urologic examination. Digital rectal examination revealed a painless, toughish, and asymmetrically enlarged prostate. Serum prostate-specific antigen was 1 ng/mL. Radiologic examinations demonstrated a large mass, which was arising from the left peripheral lobe of the prostate. The patient underwent transrectal ultrasound-guided biopsy of the prostate which revealed a smooth muscle tumor of uncertain malignant potential. Radical retropubic prostatectomy with en bloc removal of the mass and the seminal vesicles was performed and histology demonstrated low-grade MPNST and adenocarcinoma of the prostate. To the best of our knowledge, this is the first report of simultaneous prostatic adenocarcinoma and MPNST in the English literature. PMID:27872787

  13. Primary peripheral nerve sheath tumors of the thyroid gland: A case report and literature review.

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    Chen, Guang; Liu, Zengguang; Su, Chang; Guan, Qiang; Wan, Fang; Dong, Bingfei; Bao, Liang; Zhang, Wenxin; Wang, Yinping; Wang, Guimin

    2016-02-01

    Primary peripheral nerve sheath tumors (PNSTs) of the thyroid gland are rare, with fewer than 30 cases reported in the medical literature to date. Primary PNSTs of the thyroid gland are classified into malignant and benign PNSTs. The benign PNSTs may be further subclassified into neurofibromas and Schwannomas. This is the case report of a 51-year-old male patient presenting with multiple primary PNSTs involving the left lobe of the thyroid gland. The patient underwent total excision of the thyroid gland and the pathological results indicated a Schwannoma with Antoni type A and B cells. The literature was reviewed briefly and, to the best of our knowledge, this is the first case report of multiple primary PNSTs of the thyroid gland.

  14. An atypical peripheral nerve sheath tumour with pseudoglandular architecture in a dog.

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    Volmer, Christelle; Caplier, Laura; Reyes-Gomez, Edouard; Huet, Hélène; Owen, Roger A; Fontaine, Jean-Jacques

    2010-02-01

    This case describes a subcutaneous soft tissue tumour in a German Shepherd dog. Histologically, the lesion was characterized by proliferating ovoid cells, loosely arranged in a collagenous to myxoid stroma, and by numerous pseudoglandular structures lined by neoplastic cells. Immunohistochemically, neoplastic cells were labelled with vimentin, glial fibrillary acidic protein and S100 antibodies, but not with cytokeratin, desmin and smooth muscle actin antibodies. Ultrastructurally, neoplastic cells were characterized by numerous mitochondria surrounded by endoplasmic reticulum and contained few secondary lysosomes. This tumour was diagnosed as a subcutaneous peripheral nerve sheath tumour (PNST) with pseudoglandular architecture. This case illustrates the morphological diversity of PNST and provides new insight into the differential diagnosis of cutaneous tumours of similar morphology in the dog.

  15. Multicentric benign peripheral nerve sheath tumors in two related bearded dragons, Pogona vitticeps.

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    Lemberger, K Y; Manharth, A; Pessier, A P

    2005-07-01

    Multiple subcutaneous masses from two sibling bearded dragons were removed. Nodules were well demarcated, restricted to the subcutis, and soft, white to yellow, resembling adipose tissue. Histologically, the masses were composed of short interlacing streams and bundles of spindle cells, with regions of vague nuclear palisading. Two of the tumors contained a subpopulation of polygonal cells with abundant periodic acid-Schiff (PAS)-positive cytoplasmic granules. Neoplastic cells were immunohistochemically positive for S100 and neuron-specific enolase (NSE) but negative for desmin and smooth muscle actin. Electron microscopy and reticulin stains demonstrated a continuous basal lamina separating intertwining cells. Histologic, ultrastructural, and immunohistochemical features were consistent with a peripheral nerve sheath origin. At 1 year postexcision, local reoccurrence of a single incompletely excised mass from the left shoulder was noted.

  16. Epineural Sheath Jacket as a New Surgical Technique for Neuroma Prevention in the Rat Sciatic Nerve Model.

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    Siemionow, Maria; Bobkiewicz, Adam; Cwykiel, Joanna; Uygur, Safak; Francuzik, Wojciech

    2017-10-01

    Terminal neuromas resulting from severe nerve injuries and traumatic or surgical limb amputations can become a source of pain, and significantly impair patients' quality of life. Recently, the number of patients with peripheral nerve injuries increased due to modern war conflicts, natural disasters, and traffic accidents. This study investigated the efficacy of the epineural sheath jacket (ESJ) as a novel technique for neuroma prevention in the rat sciatic nerve model. A 20-mm segment of the right sciatic nerve was excised in 18 Lewis rats, and the animals were divided into 3 experimental groups (n = 6/group): group I-control, nerve stump without protection; group II-muscle burying group, nerve stump buried in the muscle; group III-ESJ group, nerve stump protected by ESJ. The ESJ was created from the excised sciatic nerve and applied as a "cap" over the proximal nerve stump. The presence of neuropathic pain was assessed weekly by pinprick test and Tinel sign, up to 24 weeks postsurgery. At 24 weeks, assessments, such as macroscopic evaluation, retrograde neuronal labeling analysis, histomorphometry, and neural/connective tissue ratio were performed. Epineural sheath jacket significantly reduced neuroma formation, which was associated with decreased Tinel sign (16.7%, P < 0.05) response compared with the nerve stump control. Moreover, ESJ reduced axonal sprouting, bulb-shaped nerve ending formation and perineural adhesions, as confirmed by macroscopic evaluation. Histological evaluation confirmed that nerve stumps protected with the ESJ showed less fibrosis and presented well-organized axonal structure. Neural/connective tissue ratio and retrograde neuronal labeling analysis revealed significantly improved results in the ESJ group compared to the control nerve stump group (P = 0.032 and P = 0.042, respectively). The protective effect of the ESJ against neuroma formation was confirmed by behavioral and histological analyses, showing outcomes comparable to the muscle

  17. Unusual brachial plexus lesion: Hematoma masquerading as a peripheral nerve sheath tumor

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    Khaled M Krisht

    2016-01-01

    Full Text Available Background: Malignant peripheral nerve sheath tumors (MPNSTs of the brachial plexus have unique radiographic and clinical findings. Patients often present with progressive upper extremity paresthesias, weakness, and pain. On magnetic resonance (MR imaging, lesions are isointense on T1-weighted and hyperintense on T2-weighted sequences, while also demonstrating marked enhancement on MR studies with gadolinium diethylenetriamine pentaacetic acid. On the basis of their characteristic MR imaging features and rapid clinical progression, two brachial plexus lesions proved to be organizing hematomas rather than MPNST. Methods: A 51-year-old male and a 31-year-old female were both assessed for persistent and worsened left-sided upper extremity pain, paresthesias, and weakness. In both cases, the MR imaging of the brachial plexus demonstrated an extraspinal enhancing lesion located within the left C7-T1 neuroforamina. Results: Although the clinical and radiographic MR features for these 2 patients were consistent with MPNSTs, both lesions proved to be benign organizing hematomas. Conclusions: These two case studies emphasize that brachial plexus hematomas may mimic MPNSTs on MR studies. Accurate diagnosis of these lesions is critical for determining the appropriate management options and treatment plans. Delaying the treatment of a highly aggressive nerve sheath tumor can have devastating consequences, whereas many hematomas resolve without surgery. Therefore, if the patient has stable findings on neurological examination and a history of trauma, surgical intervention may be delayed in favor of repeat MR imaging in 2-3 months to re-evaluate the size of the mass.

  18. DNA Ploidy and Chromosome (FISH Pattern Analysis of Peripheral Nerve Sheath Tumors

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    Anett Hruska

    2004-01-01

    Full Text Available Background and methods: 44 peripheral nerve sheath tumors (PNST (27 schwannomas, 9 neurofibromas and 8 malignant peripheral nerve sheath tumors (MPNST were analyzed to determine DNA ploidy pattern and to clarify the conflicting data in the literature concerning this topic (whether benign PNSTs are aneuploid or not. For further insight we analyzed 6 schwannomas, one atypical neurofibroma and five MPNSTs by fluorescence in situ hybridization (FISH technique using centromeric chromosome probes (7, 17 and 18 and automatic image analysis station, Metafer 4. Results: Benign schwannomas (including the problematic variants as ancient, cellular, neuroblastoma like and multiplex schwannomas could be characterized by euploid‐polyploidisation and by their 4c peak height value which was usually more than 10% of total cell number measured. These characters were not found among neurofibromas and MPNST‐s. FISH analysis revealed and confirmed that the ‘normal’ euploid–polyploid cells are mainly eusomic–polysomic containing two, four, eight or sixteen signals for each chromosomes examined, but in a small proportion aneusomy was found among tumor cells of benign schwannomas (average: 2.58; range 1.33–3.44. In contrast, the atypical neurofibroma displayed marked aneusomy (18.44% but it contained normal eusomic and polysomic cells too. Two diploid MPNSTs proved to be clearly aneusomic with trisomy of chromosome 17 and monosomy of chromosome 18. Conclusions: All these data suggest that ploidy pattern determination combined with FISH analysis may be a very useful supplementary tool for making a right diagnosis (to differentiate benign versus malignant schwannomas in problematic variants and to understand better the malignant transformation in PNSTs.

  19. Retrospective canine skin peripheral nerve sheath tumors data with emphasis on histologic, immunohistochemical and prognostic factors

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    Gisele S. Boos

    2015-12-01

    Full Text Available Abstract: In this retrospective study was determined the frequency of canine skin peripheral nerve sheath tumors (PNST in cases diagnosed by the Setor de Patologia Veterinária of the Universidade Federal do Rio Grande do Sul (SPV-UFRGS, Brazil, between the years 2000 and 2012. The canine profiles, as well as histological, immunohistochemical and prognostic aspects of the tumors were based on 70 samples, comprising 40 females, 29 males and one unspecified sample. Between 2000 and 2012, 2,984 skin tumors of dogs were diagnosed in the SPV-UFRGS, totaling 2.34% of skin neoplasms in dogs. Animals that comprised the largest amount of samples (43% were those with no breed (SRD, followed by German Shepherds (10%. Females were more affected than males (40/70 - 57% and 29/70 - 41% respectively. Skin PNST of this research showed predominant localization on the limbs (40% in the forelimbs and 29% in the hindlimbs; affecting adult dogs, mostly aged between 8 and 11 years (54%. The samples were routinely processed for hematoxylin and eosin, and were also evaluated by toluidine blue and Masson's trichrome staining, and immunohistochemistry (IHC anti-vimentin, -S-100, -GFAP, -actin, von Willebrand factor and neurofilament. Anisocytosis and anisokaryosis, mitotic index, intratumoral necrosis, invasion of adjacent tissues, tumor location, local recurrence and metastasis were related to the diagnosis of benign (49/70 or malignant tumor (21/70. The Antoni A histological pattern was observed more frequently in benign tumors. The immunohistochemistry helped to diagnose PNST, and anti-vimentin and anti-protein S-100 showed the highest rates of immunostaining. Throughout statistical analysis of animals with tumor recurrence, it was found that the chance of an animal with a malignant peripheral nerve sheath tumor to develop recurrence is 4.61 times higher than in an animal that had a benign tumor.

  20. The maximum tolerated dose of gamma radiation to the optic nerve during γ knife radiosurgery in an animal study.

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    Deng, Xingli; Yang, Zhiyong; Liu, Ruen; Yi, Meiying; Lei, Deqiang; Wang, Zhi; Zhao, Hongyang

    2013-01-01

    The safety of gamma knife radiosurgery should be considered when treating pituitary adenomas. To determine the maximum tolerated dose of radiation delivered by gamma knife radiosurgery to optic nerves. An animal model designed to establish prolonged balloon compression of the optic chiasm and parasellar region was developed to mimic the optic nerve compression caused by pituitary adenomas. Twenty cats underwent surgery to place a balloon for compression effect and 20 cats in a sham operation group received microsurgery without any treatment. The effects of gamma knife irradiation at 10-13 Gy on normal (sham operation group) and compressed (optic nerve compression group) optic nerves were investigated by pattern visual evoked potential examination and histopathology. Gamma knife radiosurgery at 10 Gy had almost no effect. At 11 Gy, P100 latency was significantly prolonged and P100 amplitude was significantly decreased in compressed optic nerves, but there was little change in the normal optic nerves. Doses of 11 Gy and higher induced significant electrophysiological variations and degeneration of the myelin sheath and axons in both normal and compressed optic nerves. Compressed optic nerves are more sensitive to gamma knife radiosurgery than normal optic nerves. The minimum dose of gamma knife radiosurgery that causes radiation injury in normal optic nerves is 12 Gy; however, the minimum dose is 11 Gy in compressed optic nerves. Copyright © 2013 S. Karger AG, Basel.

  1. A clinicopathological and immunohistochemical study of malignant peripheral nerve sheath tumors

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    Siny Vellukara Sasidharan

    2014-08-01

    Full Text Available Background: Malignant Peripheral Nerve Sheath Tumor (MPNST is a rare aggressive sarcoma that develops within a peripheral nerve and forms a diagnostic challenge in view of its varied histomorphology. This short series highlights the clinicopathological spectrum of 11 cases of MPNST and the incidence of neurofibromatosis 1 (NF1 association. Methods: This retrospective and descriptive study on MPNST was done in the department of pathology, Kasturba medical college Mangalore (Manipal University, India over a period of three years from January 2008 to December 2010. Cases which were histopathologically diagnosed as MPNST were reviewed and immunostains was done where ever indicated to rule out the differentials. Results: A total of 11 cases of MPNST were documented with a wide age range of 17-85 years. Male:female ratio was 2.6:1. Extremities (63.64% were found to be the most common site. Location wise most of the tumors were deep seated (63.64% and maximum cases were high grade (54.55%. NF1 association was seen in 2 cases. Heterologous elements in the form of chondroid differentiation was seen in one case. Immunostain with S-100 was focally positive in all the cases. Conclusion: MPNST is a highly aggressive sarcoma with poor prognosis characterized by a challenge in its diagnosis as it has several mimics. Its diagnosis necessitates the incorporation of clinicopathological features and IHC with S-100 protein. [Int J Res Med Sci 2014; 2(4.000: 1307-1312

  2. Medullary metastasis of a malignant peripheral nerve sheath tumor: A case report

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    Hagi, Tomohito; Nakamura, Tomoki; Yokoji, Ayumu; Matsumine, Akihiko; Sudo, Akihiro

    2016-01-01

    The present study reports a case of medullary metastasis without lung metastasis that occurred as a result of a malignant peripheral nerve sheath tumor (MPNST). An 81-year-old woman presented with a MPNST in the left brachial plexus, arising from the cervical nerve root. The patient underwent carbon ion radiotherapy; however, tumor recurrence was identified in the left shoulder. Subsequently, the patient underwent wide excision. Three weeks subsequent to surgery, imbalance and dysarthria developed suddenly. Dysphagia emerged and left upper limb pain disappeared on the day after symptom development. Magnetic resonance imaging (MRI) revealed that this was due to metastasis to the medulla. Five days subsequent to the onset of dysarthria, the patient succumbed due to respiratory failure. To the best of our knowledge, no previous cases of medullary metastasis arising from a MPNST in the absence of lung metastasis have been reported. MRI is a useful examination tool for the identification of brain metastases; however, the high cost of MRI as a routine examination must be considered due to the rarity of brain metastases. Therefore, methods to detect brain metastasis warrant further investigation. PMID:27588138

  3. Malignant peripheral nerve sheath tumour (MPNST): the clinical implications of cellular signalling pathways.

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    Katz, Daniela; Lazar, Alexander; Lev, Dina

    2009-10-19

    Malignant peripheral nerve sheath tumour (MPNST) is a rare malignancy accounting for 3-10% of all soft tissue sarcomas. Most MPNSTs arise in association with peripheral nerves or deep neurofibromas and may originate from neural crest cells, although the specific cell of origin is uncertain. Approximately half of MPNSTs occur in the setting of neurofibromatosis type 1 (NF1), an autosomal dominant disorder with an incidence of approximately one in 3500 persons; the remainder of MPNSTs develop sporadically. In addition to a variety of clinical manifestations, approximately 8-13% of NF1 patients develop MPNSTs, which are the leading cause of NF1-related mortality. Surgical resection is the mainstay of MPNST clinical management. However, because of invasive growth, propensity to metastasise, and limited sensitivity to chemotherapy and radiation, MPNST has a guarded to poor prognosis. Five-year survival rates of only 20-50% indicate an urgent need for improved therapeutic approaches. Recent work in this field has identified several altered intracellular signal transduction cascades and deregulated tyrosine kinase receptors, posing the possibility of personalised, targeted therapeutics. However, expanded knowledge of MPNST molecular pathobiology will be needed to meaningfully apply such approaches for the benefit of afflicted patients.

  4. Retrobulbar diameter of optic nerve in glaucoma

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    Stefanović Ivan

    2009-01-01

    Full Text Available Introduction. The ultrasound diagnostics of the optic nerve includes the analysis of the optic nerve disc (PNO and measuring of its retrobulbar diameter. With B-scan, by Schraeder's method, it is possible to measure very precisely the optic nerve, the pial diameter, the normal values for the pial diameter being 2.8-4.1 mm. In glaucoma, the disease that is most frequently associated with higher intraocular pressure, there comes the destruction of nerve fibres, which can be visualized as the excavation of the optic nerve disc. Objective. In this paper, we were interested in finding whether in glaucoma, and in what phase of the disease, the optic nerve starts growing thinner. Aware of many forms of this very complex disease, we were interested in knowing if the visualization of excavation on the optic nerve disc is related to diminishing of the pial diameter of the retrobulbar nerve part. Methods. There were treated the patients who had already had the diagnosis of glaucoma and the visualized excavation of the optic disc of various dimensions. Echographically, there was measured the thickness of the retrobulbar part of the optic nerve and the finding compared in relation to the excavation of the optic disc. Results. In all eyes with glaucoma, a normal size of the retrobulbar part of the optic nerve was measured, ranging from 3.01 to 3.91 mm with the median of 3.36 mm. Also, by testing the correlation between the thickness of the optic nerve and the excavation of the PNO, by Pearson test, we found that there was no correlation between these two parameters (r=0.109; p>0.05. Conclusion. In the patients with glaucoma, the retrobulbar part of the optic nerve is not thinner (it has normal values, even not in the cases with a totally excavated optic disc. There is no connection between the size of the PNO excavation and the thickness of the retrobulbar part of the optic nerve.

  5. The impact of optic nerve movement on optic nerve magnetic resonance diffusion parameters

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    Anand Moodley

    2014-04-01

    Full Text Available Background: Optic nerve diffusion imaging is a useful investigational tool of optic nerve microstructure, but is limited by eye-movement-induced optic nerve movement and artifacts from surrounding cerebrospinal fluid, fat, bone and air. Attempts at improving patient cooperation, thus voluntarily limiting eye movement during a standard diffusion imagingsequence, are usually futile. The aim of this study was to establish the impact of optic nerve movement on clinical diffusion parameters of the optic nerve.Method: Twenty-nine healthy volunteers with intact vision and intact conjugate gaze were recruited and subjected to magnetic resonance diffusion-weighted imaging (DWI and diffusion-tensor imaging (DTI of the optic nerves. Twenty right eyes had nerve tracking done using single-shot echo-planar imaging at 20 time points over 3 minutes. Optic nerve movement measurements were correlated with diffusion parameters of apparent diffusion coefficient (ADC, mean diffusivity (MD, fractional anisotropy (FA and anisotropic index(AI using Spearman’s rank correlation.Results: No significant correlations were noted between optic nerve movement parameters and ADC in the axial plane and MD of the optic nerve. Low to moderate negative correlations were noted between optic nerve movement parameters and AI and FA and positive correlation with ADC in the radial plane.Conclusion: Optic nerve movement documented during the timespan of standard diffusion sequences (DWI and DTI has a negative effect on the anisotropic diffusion parameters of the optic nerve. With greater eye movement, optic nerve diffusion appears less anisotropic owing to greater radial diffusion.

  6. Congenital optic nerve anomalies and hereditary optic neuropathies.

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    Heidary, Gena

    2014-12-01

    Congenital and hereditary optic nerve anomalies represent a significant cause of visual dysfunction. While some optic nerve abnormalities affect the visual system alone, others may be associated with neurologic and systemic findings. Correct identification of the optic nerve disease therefore is crucial both for developing a treatment plan with respect to visual rehabilitation, but also for initiating the appropriate multidisciplinary evaluation. The purpose of this review is to highlight common examples of congenital and inherited optic nerve abnormalities in an effort to familiarize the clinician with salient clinical features of these diseases and to review important systemic testing when relevant.

  7. Unilateral optic nerve aplasia associated with microphthalmos

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    Stanković-Babić Gordana

    2012-01-01

    Full Text Available Intraduction. Optic nerve aplasia is a rare developmental anomaly characterised by the congenital absence of the optic nerve, central retinal vessels and retinal ganglion cells that is seen most often in a unilaterally malformed eye. Case report. We reported a girl with a very rare anomaly of the eye, unilateral aplasia of the optic nerve and microphthalmia. We carried out a complete ophthalmological examination, A- and Bscan ultrasonography, magnetic resonance imaging (MRI of the orbit and brain, pediatrician, neurological examinations and karyotype determination. The examined child was a third child from the third regular pregnancy, born at term (39 GS, BM 3100 g. Family ocular history was negative. The right corneal diameter was 7.5 mm and left 10 mm. On dilated fundus examination, the right eye showed the absence of optic nerve and central retinal vessels. B-scan echography showed a small right globe (axial length 13.80 mm, normal size left globe (axial length 18.30 mm and the absence of optic nerve on the right eye. Physical and neurological findings and karyotype was normal. MRI of the orbits and brain marked asymmetry of globe size and unilateral absence of the optic nerve. The patient is under the control of a competent ophthalmologist and prosthetic. Conclusion. Further aesthetic and functional development of a young person is the primary goal in tracking this rare congenital optic nerve anomalies in the malformed eye.

  8. GEMSP exerts a myelin-protecting role in the rat optic nerve.

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    Mangas, Arturo; Vecino, Elena; David Rodríguez, F; Geffard, Michel; Coveñas, Rafael

    2013-11-01

    Chronic experimental autoimmune encephalomyelitis (EAE) was induced in rats to evaluate the potential protective effect of GEMSP, a mixture made up of fatty acids (FA), vitamins, and amino acids or their derivatives, linked to Poly-L-Lysine, on the myelin sheath of the optic nerve. To evaluate the effects of GEMSP on the optic nerve, animals were divided into three experimental groups: (1) EAE rats treated with GEMSP; (2) EAE rats treated with 0.9% NaCl; and (3) control, non-EAE rats. Using electron microscopy, we investigated the possibility that this new drug candidate has a myelin-protective role. A marginally significant reduction in the thickness of the myelin around optic nerve medium-size axons (diameter between 0.8-1.3 μm) was found in EAE rats. Treatment of EAE rats with GEMSP ameliorated myelin damage. Significantly increased myelin thickness was found when animals in groups 2 and 3 were compared. However, the number of myelinated axons studied was not altered in groups 1 or 2 when compared to controls. Our results suggest that in a model of demyelination, GEMSP protects and enhances the formation of the myelin sheath of the optic nerve and therefore could be a potential drug candidate to reduce optic nerve pathogenesis in multiple sclerosis (MS).

  9. Malignant peripheral nerve sheath tumor associated with neurofibromatosis type 1, with metastasis to the heart: a case report

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    Araki Nobuhito

    2010-01-01

    Full Text Available Abstract A rare case is presented of a 61-year-old man with a malignant peripheral nerve sheath tumor associated with neurofibromatosis type 1, with metastasis to the heart. The primary tumor originated in the right thigh in 1982. Since then, the patient has had repeated local recurrences in spite of repeated surgical treatment and adjuvant chemotherapy. He has developed previous metastases of the lung and heart. The patient died of cardiac involvement.

  10. Malignant Peripheral Nerve Sheath Tumors: Differentiation Patterns and Immunohistochemical Features - A Mini-Review and Our New Findings

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    Guo, Aitao; Liu,Aijun; Wei, Lixin; Song, Xin

    2012-01-01

    Malignant peripheral nerve sheath tumors (MPNST) represent a group of highly heterogeneous human malignancies often with multiple histological origins, divergent differentiation patterns, and diverse immunohistochemical presentations. The differential diagnosis of MPNST from other spindle cell neoplasms poses great challenges for pathologists. This report provides a mini-review of these unique features associated with MPNST and also presents the first cases of MPNST with six differentiation p...

  11. Malignant Peripheral Nerve Sheath Tumors: Differentiation Patterns and Immunohistochemical Features - A Mini-Review and Our New Findings

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    Aitao Guo, Aijun Liu, Lixin Wei, Xin Song

    2012-01-01

    Full Text Available Malignant peripheral nerve sheath tumors (MPNST represent a group of highly heterogeneous human malignancies often with multiple histological origins, divergent differentiation patterns, and diverse immunohistochemical presentations. The differential diagnosis of MPNST from other spindle cell neoplasms poses great challenges for pathologists. This report provides a mini-review of these unique features associated with MPNST and also presents the first cases of MPNST with six differentiation patterns.

  12. A Case of Malignant Peripheral Nerve Sheath Tumor with Rhabdomyoblastic Differentiation: Malignant Triton Tumor

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    Kenichiro Mae

    2013-12-01

    Full Text Available Malignant peripheral nerve sheath tumors (MPNST constitute a rare variety of soft tissue sarcomas thought to originate from Schwann cells or pluripotent cells of the neural crest. Malignant triton tumor (MTT, a very rare, highly aggressive soft tissue tumor, is a subgroup of MPNST and is comprised of malignant Schwann cells coexisting with malignant rhabdomyoblasts. We herein report the case of a 24-year-old man who presented a subcutaneous mass in his right thigh. The mass was removed surgically in its entirety and radiation therapy was applied locally to prevent tumor regrowth. Nonetheless, the patient died 10 months after surgery from metastases to the lung and brain. He presented neither cafe-au-lait spots nor cutaneous neurofibromas. The histopathology showed a transition from a neurofibroma to an MTT, making this the second report of an MTT arising from a neurofibroma without neurofibromatosis type 1, an autosomal dominant disorder with which 50-70% of tumors reported in previous studies were associated. A histopathological examination using immunostaining with desmin confirmed this diagnosis. MTT has a poorer prognosis than MPNST and should therefore be regarded as a distinct clinical entity.

  13. Survivin expression and prognostic significance in pediatric malignant peripheral nerve sheath tumors (MPNST).

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    Alaggio, Rita; Turrini, Riccardo; Boldrin, Daniela; Merlo, Anna; Gambini, Claudio; Ferrari, Andrea; Dall'igna, Patrizia; Coffin, Cheryl M; Martines, Annalisa; Bonaldi, Laura; De Salvo, Gian Luca; Zanovello, Paola; Rosato, Antonio

    2013-01-01

    Malignant peripheral nerve sheath tumors (MPNST) are very aggressive malignancies comprising approximately 5-10% of all soft tissue sarcomas. In this study, we focused on pediatric MPNST arising in the first 2 decades of life, as they represent one the most frequent non-rhabdomyosarcomatous soft tissue sarcomas in children. In MPNST, several genetic alterations affect the chromosomal region 17q encompassing the BIRC5/SURVIVIN gene. As cancer-specific expression of survivin has been found to be an effective marker for cancer detection and outcome prediction, we analyzed survivin expression in 35 tumor samples derived from young patients affected by sporadic and neurofibromatosis type 1-associated MPNST. Survivin mRNA and protein expression were assessed by Real-Time PCR and immunohistochemical staining, respectively, while gene amplification was analyzed by FISH. Data were correlated with the clinicopathological characteristics of patients. Survivin mRNA was overexpressed in pediatric MPNST and associated to a copy number gain of BIRC5; furthermore, increased levels of transcripts correlated with a higher FNCLCC tumor grade (grade 1 and 2 vs. 3, p = 0.0067), and with a lower survival probability (Log-rank test, p = 0.0038). Overall, these data support the concept that survivin can be regarded as a useful prognostic marker for pediatric MPNST and a promising target for therapeutic interventions.

  14. Poly (ADP) ribose polymerase inhibition: A potential treatment of malignant peripheral nerve sheath tumor.

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    Kivlin, Christine M; Watson, Kelsey L; Al Sannaa, Ghadah A; Belousov, Roman; Ingram, Davis R; Huang, Kai-Lieh; May, Caitlin D; Bolshakov, Svetlana; Landers, Sharon M; Kalam, Azad Abul; Slopis, John M; McCutcheon, Ian E; Pollock, Raphael E; Lev, Dina; Lazar, Alexander J; Torres, Keila E

    2016-01-01

    Poly (ADP) ribose polymerase (PARP) inhibitors, first evaluated nearly a decade ago, are primarily used in malignancies with known defects in DNA repair genes, such as alterations in breast cancer, early onset 1/2 (BRCA1/2). While no specific mutations in BRCA1/2 have been reported in malignant peripheral nerve sheath tumors (MPNSTs), MPNST cells could be effectively targeted with a PARP inhibitor to drive cells to synthetic lethality due to their complex karyotype and high level of inherent genomic instability. In this study, we assessed the expression levels of PARP1 and PARP2 in MPNST patient tumor samples and correlated these findings with overall survival. We also determined the level of PARP activity in MPNST cell lines. In addition, we evaluated the efficacy of the PARP inhibitor AZD2281 (Olaparib) in MPNST cell lines. We observed decreased MPNST cell proliferation and enhanced apoptosis in vitro at doses similar to, or less than, the doses used in cell lines with established defective DNA repair genes. Furthermore, AZD2281 significantly reduced local growth of MPNST xenografts, decreased the development of macroscopic lung metastases, and increased survival of mice with metastatic disease. Our results suggest that AZD2281 could be an effective therapeutic option in MPNST and should be further investigated for its potential clinical use in this malignancy.

  15. Appropriate modulation of autophagy sensitizes malignant peripheral nerve sheath tumor cells to treatment with imatinib mesylate.

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    Okano, Munehiro; Sakata, Naoki; Ueda, Satoshi; Takemura, Tsukasa

    2014-04-01

    Malignant peripheral nerve sheath tumor (MPNST), very rare in childhood, is a highly aggressive soft-tissue tumor. We experienced a case of a 7-year-old boy with MPNST who was treated with imatinib mesylate (imatinib) after the identification of platelet-derived growth factor receptor expression in his tumor. We were unable to observe clinical benefits of imatinib in this patient. Therefore, cellular reactions of imatinib were investigated in vitro using 3 MPNST cell lines. Imatinib induced cytotoxicity in vitro with variable IC50 values (11.7 to >30 μM). Induction of apoptosis was not a pivotal mechanism in the inhibitory effects. We found that the treatment of MPNST cell lines with imatinib induced autophagy. Suppression of the initiation of autophagy by 3-methyladenine or small interfering RNA (siRNA) against beclin-1 attenuated the imatinib-mediated cytotoxicity. In contrast, blocking the formation of autophagosomes or the development of autolysosomes using siRNA against microtubule-associated protein light chain 3B, bafilomycin A1, chloroquine, or an MEK1/2 inhibitor (U0126) enhanced the imatinib-induced cytotoxicity in MPNST cells. Our data showed that the imatinib-mediated autophagy can function as a cytotoxic mechanism and that appropriate modulation of autophagy may sensitize MPNST cells to imatinib, which in turn may be a novel therapeutic strategy for MPNST.

  16. Malignant Peripheral Nerve Sheath Tumors of the Head and Neck: A Case Series and Literature Review

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    Brandon T. Mullins

    2014-01-01

    Full Text Available Background. Malignant peripheral nerve sheath tumors (MPNSTs of the head and neck are rare aggressive neoplasms with a poor prognosis. This study describes the management and outcomes of 3 of our patients with MPNSTs of the head and neck. Methods. We identified 3 patients presenting with MPNST of the head and neck and treated at the University of North Carolina. We compared our results to the literature from 1963 to 2014. Results. Mean follow-up was 31 months. Average age at diagnosis was 44.7 years of age. All patients received wide-local excision and adjuvant radiotherapy. No patients recurred during the series. Recurrence-free survival time for the patients was 45, 37, and 3 months, respectively. Conclusions. Our data series confirms that a combined-modality approach with complete surgical resection and adjuvant radiotherapy leads to improved outcomes in MPNSTs of the head and neck. Nonetheless, due to historically poor outcomes, continued research into newer therapies needs to be explored.

  17. Malignant Peripheral Nerve Sheath Tumors in Children with Neurofibromatosis Type 1

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    Apostolos Pourtsidis

    2014-01-01

    Full Text Available Purpose. Malignant peripheral nerve sheath tumors (MPNSTs are rare in children and account for approximately 5–10% of all soft tissue sarcomas in adults. MPNSTs may occur independently but individuals with neurofibromatosis type 1 (NF1 have a significantly increased risk. Our aim is to present patients with MPNST treated in our department. Cases and Results. In this report we present 4 cases of MPNSTs (3 females: 13, 12, and 13 years old and 1 male: 10 years old arising in patients with NF1. All of them presented with an enlarging mass and pain at diagnosis. Tumor was located in the buttock, the spinal cord, the trunk, and the left leg proximal to the heel. Wide excision of the tumor and radiotherapy were applied to all and adjuvant chemotherapy was given to three of them after the disease was progressed. All four died 32, 18, 10, and 22 months after diagnosis with progressive disease locally and pulmonary metastases in two of them. Conclusions. In conclusion, MPNSTs arising in patients with NF1 are high grade sarcomas with short survival. Individuals with NF1 should be followed closely in order to identify early the development of MPNSTs. Aggressive surgery and complete excision significantly improves disease-free survival. The usefulness of radiation therapy in MPNSTs is not determined although all patients will receive radiation therapy at some stage of the disease. The role of chemotherapy is unclear.

  18. Morphological spectrum of peripheral nerve sheath tumors: A series of 126 cases

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    Gabhane Sushma

    2009-01-01

    Full Text Available By convention, soft tissue tumors include the peripheral nerve sheath tumor (PNST although they arise from neuroectoderm. PNSTs display a wide spectrum of morphology and biological behavior. Malignant transformation in benign tumors, especially neurofibroma, is an acknowledged phenomenon.This article describes clinico-pathological features of 126 PNSTs with their morphological variants and those associated with neurofibromatosis 1 (NF1 encountered in a single center over a 6-year period. Benign PNSTs were more common [118 (93.65%] when compared with their malignant counterparts [8 (6.34%]. Histological variants including plexiform schwannomas and diffuse, plexiform and pigmented forms of neurofibromas, though rare, were observed. Eleven (8.73% patients with NF1 showed a strong association with plexiform neurofibroma. A malignant transformation in pre-existing neurofibroma was seen in 1 patient with NF1. This series highlights the clinico-pathological spectrum of PNSTs, their morphological variants and the incidence of associated NF1. The importance of detecting plexiform variants lies in their vastly different prognostic implications particularly when occurring in the setting of NF1

  19. Primary uterine cervix melanoma resembling malignant peripheral nerve sheath tumor: a case report.

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    Pusceddu, Sara; Bajetta, Emilio; Buzzoni, Roberto; Carcangiu, Maria Luisa; Platania, Marco; Del Vecchio, Michele; Ditto, Antonino

    2008-10-01

    A rare variant of malignant melanoma (MM) of the uterine cervix that mimics a malignant peripheral nerve sheath tumor (MPNST) is described. A 43-year-old white woman was admitted to the hospital complaining of genital discharge and vaginal bleeding. Neoadjuvant chemotherapy and total abdominal hysterectomy and bilateral salpingo-ovariectomy plus pelvic lymphadenectomy were performed, and the diagnosis was MPNST, FIGO IIB. Pathological examination showed a diffuse proliferation of amelanotic spindle cells and large, highly atypical, frequently multinucleated, bizarre, and S100-, HMB-45-, vimentin-positive cells. The patient remained disease-free for 43 months, when an abdominal computed tomographic scan showed local polypoid vaginal lesions, with histological features of typical MM. A pathological review was obtained in our institution by a gynecological pathologist, who defined the primary neoplasm in the cervix as an MM, with a pattern of growth histologically simulating an MPNST, metastatic to the vagina. To our knowledge, this is the first report in literature of MM of the uterine cervix resembling MPNST. Despite its rarity, this variant of MM should be considered when a diagnosis of cervix MPNST is made. The histological and immunohistochemical features of these different entities should be considered in the differential diagnosis.

  20. Survivin expression and prognostic significance in pediatric malignant peripheral nerve sheath tumors (MPNST.

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    Rita Alaggio

    Full Text Available Malignant peripheral nerve sheath tumors (MPNST are very aggressive malignancies comprising approximately 5-10% of all soft tissue sarcomas. In this study, we focused on pediatric MPNST arising in the first 2 decades of life, as they represent one the most frequent non-rhabdomyosarcomatous soft tissue sarcomas in children. In MPNST, several genetic alterations affect the chromosomal region 17q encompassing the BIRC5/SURVIVIN gene. As cancer-specific expression of survivin has been found to be an effective marker for cancer detection and outcome prediction, we analyzed survivin expression in 35 tumor samples derived from young patients affected by sporadic and neurofibromatosis type 1-associated MPNST. Survivin mRNA and protein expression were assessed by Real-Time PCR and immunohistochemical staining, respectively, while gene amplification was analyzed by FISH. Data were correlated with the clinicopathological characteristics of patients. Survivin mRNA was overexpressed in pediatric MPNST and associated to a copy number gain of BIRC5; furthermore, increased levels of transcripts correlated with a higher FNCLCC tumor grade (grade 1 and 2 vs. 3, p = 0.0067, and with a lower survival probability (Log-rank test, p = 0.0038. Overall, these data support the concept that survivin can be regarded as a useful prognostic marker for pediatric MPNST and a promising target for therapeutic interventions.

  1. Optic Nerve Avulsion after Blunt Trauma

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    Hacı Halil Karabulut

    2014-05-01

    Full Text Available Optic nerve avulsion is an uncommon presentation of ocular trauma with a poor prognosis. It can be seen as complete or partial form due to the form of trauma. We assessed the complete optic nerve avulsion in a 16-year-old female patient complaining of loss of vision in her left eye after a traffic accident. (Turk J Ophthalmol 2014; 44: 249-51

  2. Optic Nerve Sonography in the Diagnostic Evaluation of Pseudopapilledema and Raised Intracranial Pressure: A Cross-Sectional Study

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    Masoud Mehrpour

    2015-01-01

    Full Text Available Introduction. Differentiating pseudopapilledema from papilledema which is optic disk edema and a result of increased ICP (intracranial pressure is important and can be done with noninvasive methods like orbital ultrasound examination. Method. This was a cross-sectional study in which patients with optic nerve head swelling were referred for LP exam after optic nerve head swelling diagnosis confirmation and having normal brain imaging (CT scan. Before LP (lumbar puncture exam the patients were referred for optic nerve ultrasound test of both eyes. Results. Considering 5.7 mm as the upper limit for normal ONSD (optic nerve sheath diameter, sensitivity and negative predictive value of optic sonography in diagnosis of pseudopapilledema are 100% for both eyes. Calculated accuracy validity of ONSD measurement in detecting pseudopapilledema is 90% for the right eye and 87% for the left eye. Conclusion. Our study demonstrated a close correlation between optic nerve sheath dilation on ocular ultrasound and evidence of elevated ICP with optic disk swelling. With the aid of noninvasive diagnostic tests we can avoid unnecessary concerns along with expensive and invasive neurological investigations while targeting the correct diagnosis in bilateral optic disk swelling. Our study showed optic nerve sonography as a reliable diagnostic method for further usage.

  3. Optical coherence tomographic findings in optic nerve hypoplasia

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    Daruchi Moon

    2013-01-01

    Full Text Available We investigated a case of unilateral optic nerve hypoplasia using spectral domain optical coherence tomography (SDOCT. Optical coherence tomography was done on both eyes using 5-line Raster scan for the fovea to analyze the retinal nerve fiber layer thickness, inner retinal layer thickness, outer retinal layer thickness, and optic disc cube scan for the disc. Retinal nerve fiber layer thickness, inner retinal layer thickness, and outer retinal layer thickness were manually measured at 21-points of each five lines, and results were compared between both eyes. Retinal nerve fiber layer thickness and inner retinal layer thickness of optic nerve hypoplasia were significantly thinner than the opposite eye, but there was no significant difference in the thickness of the outer retinal layer between both eyes.

  4. Optic nerve decompression for orbitofrontal fibrous dysplasia.

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    Abe, Takumi; Sato, Kaneshige; Otsuka, Takaharu; Kawamura, Noriyoshi; Shimazu, Motohiko; Izumiyama, Hitoshi; Matsumoto, Kiyoshi

    2002-08-01

    Orbitofrontal fibrous dysplasia often involves the bony orbit and the optic canal. Although fibrous dysplasia reportedly produces compression of the optic nerve leading to visual distrubances, optic nerve decompression in patients without clinical signs of optic neuropathy is still controversial. We describe two patients with orbitofrontal fibrous dysplasia without signs of visual disturbance and one patient with McCune-Albright syndrome and progressive visual impairment. Optic nerve decompression was performed prophylactically for two patients and therapeutically for one patient through the transcranial extradural route. Dystopias and craniofacial deformities induced by fibrous dysplasia also were corrected. The micropressure suction-irrigation system was especially effective for decreasing heat transfer and thereby preventing thermal injury of the optic nerve. The orbitofrontal area was reconstructed from cranial bone, iliac bone, and ribs. Postoperative follow-up revealed no disturbances in visual function and no evidence of cerebrospinal fluid leakage. These findings suggest that optic nerve decompression may be effective in preventing visual disturbances with minimal risk of other neurological sequelae. Subsequent orbital reconstruction yielded satisfactory cosmetic results.

  5. Study on the clinic application value of ultrasonography examination of optic nerve sheath diameter in brain injury%超声检查视神经鞘直径在颅脑损伤诊治中的应用价值

    Institute of Scientific and Technical Information of China (English)

    赵万; 柳茂成; 黄斌; 张浚; 丁志海; 苗雨露; 王福明; 余少雄; 刘春霞; 白洪涛; 刘泽; 尹勇; 黄志斌

    2012-01-01

    Objective To explore the clinic application value of ultrasonography examination of optic nerve sheath diameter(ONSD) in brain injury.Methods From July 2008-June 2011,90 cases of brain injured patients were chosen as experimental group including light (A group),medium (B group),and heavy (C group) brain injured patients according to the admission GCS score ;50 cases of conventional physical examination and 90 cases of volunteers 50 in neurosurgical outpatient were chosen as control group.The ONSD of both groups were measured 3 mm behind the globe through orbital using color sonographic with different time after admission.3 times measurements were carried out for every optic nerve sheath.All client's ONSD mean and standard deviation were calculated.In 0.5 h after color dopplar ultrasound examination,lumbar vertebra puncturing measured intracranial pressure in different groups.Results After admission (1d,3 d,7 d,14 d),the ONSD of A group was (4.54 ±0.32)mm,(4.42 ±0.30)mm,(4.44 ±0.32) m,and (4.43 ± 0.25) mm,respectively; The ONSD of B groups was (4.48 ± 0.28) mm,(4.52 ± 0.24) mm,(4.46 ±0.28)mm,and (4.38 ±0.22)mm,respectively; The ONSD of C group was (5.67 ±0.35)mm,(6.36 ± 0.42) mm,(5.65 ± 0.23) mm,and (4.76 ± 0.35) mm,respectively.After admission (1 d,3 d,7 d,14 d),the intracranial pressure (IP) of A group was (82 ± 11) mmH2O,(79 ± 12) mmH2O,(90 ±15) mmH2O,and (86 ± 14) mmH2O,respectively; The IP of B group was (78 ± 15) mmH2O,(85 ± 10)mmH2O,(78 ± 16) mmH2O,(80 ± 11) mmH2O,The IP of C group was (225 ± 26) mmH2 O,(288 ± 23)mmH2O,(256 ± 23) mmH2O,(122 ± 18) mmH2O,respectively.Group D had the ONSD average of (4.58± 0.41)mm and IP of (88 ± 10)mmH2O after eyeball 3-mm place.No difference was found between A and B,A and D,or B and D (P>0.05) ; A difference was found between A and C,B and C,or D and C (t =12.24~24.67,P<0.01).Conclusions The ONSD and IP in light medium brain injured patients had no change.In patients with severe brain injury

  6. The optic nerve head in hereditary optic neuropathies.

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    O'Neill, Evelyn C; Mackey, David A; Connell, Paul P; Hewitt, Alex W; Danesh-Meyer, Helen V; Crowston, Jonathan G

    2009-05-01

    Hereditary optic neuropathies are a prominent cause of blindness in both children and adults. The disorders in this group share many overlapping clinical characteristics, including morphological changes that occur at the optic nerve head. Accurate and prompt clinical diagnosis, supplemented with imaging when indicated, is essential for optimum management of the relevant optic neuropathy and appropriate counseling of the patient on its natural history. Patient history, visual field assessment, optic disc findings and imaging are the cornerstones of a correct diagnosis. This Review highlights the characteristic optic nerve head features that are common to the various hereditary optic neuropathies, and describes the features that enable the conditions to be differentiated.

  7. Neurofibromin specific antibody differentiates malignant peripheral nerve sheath tumors (MPNST) from other spindle cell neoplasms.

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    Reuss, David E; Habel, Antje; Hagenlocher, Christian; Mucha, Jana; Ackermann, Ulrike; Tessmer, Claudia; Meyer, Jochen; Capper, David; Moldenhauer, Gerhard; Mautner, Victor; Frappart, Pierre-Olivier; Schittenhelm, Jens; Hartmann, Christian; Hagel, Christian; Katenkamp, Kathrin; Petersen, Iver; Mechtersheimer, Gunhild; von Deimling, Andreas

    2014-04-01

    Malignant peripheral nerve sheath tumors (MPNST) derive from the Schwann cell or perineurial cell lineage and occur either sporadically or in association with the tumor syndrome neurofibromatosis type 1 (NF1). MPNST often pose a diagnostic challenge due to their frequent lack of pathognomonic morphological or immunohistochemical features. Mutations in the NF1 tumor suppressor gene are found in all NF1-associated and many sporadic MPNST. The presence of NF1 mutation may have the potential to differentiate MPNST from several morphologically similar neoplasms; however, mutation detection is hampered by the size of the gene and the lack of mutational hot spots. Here we describe a newly developed monoclonal antibody binding to the C-terminus of neurofibromin (clone NFC) which was selected for optimal performance in routinely processed formalin-fixed and paraffin-embedded tissue. NFC immunohistochemistry revealed loss of neurofibromin in 22/25 (88 %) of NF1-associated and 26/61 (43 %) of sporadic MPNST. There was a strong association of neurofibromin loss with deletions affecting the NF1 gene (P < 0.01). In a series of 256 soft tissue tumors of different histotypes NFC staining showed loss of neurofibromin in 2/8 myxofibrosarcomas, 2/12 (16 %) pleomorphic liposarcomas, 1/16 (6 %) leiomyosarcomas, and 4/28 (14 %) unclassified undifferentiated pleomorphic sarcomas. However, loss of neurofibromin was not observed in 22 synovial sarcomas, 27 schwannomas, 23 solitary fibrous tumors, 14 low-grade fibromyxoid sarcomas, 50 dedifferentiated liposarcomas, 27 myxoid liposarcomas, 13 angiosarcomas, 9 extraskeletal myxoid chondrosarcomas, and 7 epitheloid sarcomas. Immunohistochemistry using antibody NFC may substantially facilitate sarcoma research and diagnostics.

  8. HDAC8, A Potential Therapeutic Target for the Treatment of Malignant Peripheral Nerve Sheath Tumors (MPNST.

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    Gonzalo Lopez

    Full Text Available HDAC isoform-specific inhibitors may improve the therapeutic window while limiting toxicities. Developing inhibitors against class I isoforms poses difficulties as they share high homology among their catalytic sites; however, HDAC8 is structurally unique compared to other class I isoforms. HDAC8 inhibitors are novel compounds and have affinity for class I HDAC isoforms demonstrating anti-cancer effects; little is known about their activity in malignant peripheral nerve sheath tumors (MPNST. Recently, we demonstrated anti-MPNST efficacy of HDAC8i in human and murine-derived MPNST pre-clinical models; we now seek to consider the potential therapeutic inhibition of HDAC8 in MPNST.Four Human MPNST cell lines, a murine-derived MPNST cell line, and two HDAC8 inhibitors (PCI-34051, PCI-48012; Pharmacyclics, Inc. Sunnyvale, CA were studied. Proliferation was determined using MTS and clonogenic assays. Effects on cell cycle were determined via PI FACS analysis; effects on apoptosis were determined using Annexin V-PI FACS analysis and cleaved caspase 3 expression. In vivo growth effects of HDAC8i were evaluated using MPNST xenograft models. 2D gel electrophoresis and mass spectrometry were used to identify potential HDAC8 deacetylation substrates.HDAC8i induced cell growth inhibition and marked S-phase cell cycle arrest in human and murine-derived MPNST cells. Relative to control, HDAC8i induced apoptosis in both human and murine-derived MPNST cells. HDAC8i exhibited significant effects on MPNST xenograft growth (p=0.001 and tumor weight (p=0.02. Four potential HDAC8 substrate targets were identified using a proteomic approach: PARK7, HMGB1, PGAM1, PRDX6.MPNST is an aggressive sarcoma that is notoriously therapy-resistant, hence the urgent need for improved anti-MPNST therapies. HDAC8 inhibition may be useful for MPNST by improving efficacy while limiting toxicities as compared to pan-HDACis.

  9. Dissecting the roles of Ephrin-A3 in malignant peripheral nerve sheath tumor by TALENs.

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    Wang, Zhengguang; Liu, Zhendong; Liu, Bo; Liu, Gengyan; Wu, Song

    2015-07-01

    Malignant peripheral nerve sheath tumor (MPNST) is a rare and aggressive soft tissue sarcoma for which effective treatments have not yet been established due to poor understanding of its pathogenesis. Our previous study indicated that miR-210-mediated Ephrin-A3 (EFNA3) promotion of proliferation and invasion of MPNST cells plays an important role in MPNST tumorigenesis and progression. The purpose of the present study was to further investigate the roles of EFNA3 in MPNST. Constructed transcription activator-like effector nucleases (TALENs) and lentiviral vectors were transfected into MPNST ST88-14 (NF1 wild-type) and sNF96.2 (NF1 mutant type) cell lines to obtain gain- and loss-of-function cell lines for the EFNA3 function study. The results showed that the knockout of ENFA3 increased cellular viability and invasiveness of the MPNST cells. However, the adhesion ability of MPNST cells was enhanced or inhibited when EFNA3 was overexpressed or knocked out, respectively. It was also observed that knockout of EFNA3 significantly decreased the expression of phosphorylated FAK (p-FAK) and the tumor necrosis factor α (TNF-α) compared to that in the control cells, yet the expression of phosphatidylinositol 3-kinase (PI3K), GTPase, integrins, vascular endothelial growth factor (VEGF) and hypoxia-inducible factor 1α (HIF-α) increased significantly. Inversely, overexpression of EFNA3 significantly increased the expression of p-FAK and TNF-α compared to that in the control cells, yet the expression of PI3K, GTPase, integrins, VEGF and HIF-α decreased significantly. The results indicated that EFNA3 serves as a tumor suppressor in MPNST cells and it may play a critical role in the focal adhesion kinase (FAK) signaling and VEGF-associated tumor angiogenesis pathway. These findings may not only facilitate the better understanding of MPNST pathogenesis, but also suggest EFNA3 as a promising target for MPNST treatment.

  10. Confirmation of mutation landscape of NF1-associated malignant peripheral nerve sheath tumors.

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    Sohier, Pierre; Luscan, Armelle; Lloyd, Angharad; Ashelford, Kevin; Laurendeau, Ingrid; Briand-Suleau, Audrey; Vidaud, Dominique; Ortonne, Nicolas; Pasmant, Eric; Upadhyaya, Meena

    2017-05-01

    The commonest tumors associated with neurofibromatosis type 1 (NF1) are benign peripheral nerve sheath tumors, called neurofibromas. Malignant transformation of neurofibromas into aggressive MPNSTs may occur with a poor patient prognosis. A cooperative role of SUZ12 or EED inactivation, along with NF1, TP53, and CDKN2A loss-of-function, has been proposed to drive progression to MPNSTs. An exome sequencing analysis of eight MPNSTs, one plexiform neurofibroma, and seven cutaneous neurofibromas was undertaken. Biallelic inactivation of the NF1 gene was observed in the plexiform neurofibroma and the MPNSTs, underlining that somatic biallelic NF1 inactivation is likely to be the initiating event for plexiform neurofibroma genesis, although it is unlikely to be sufficient for the subsequent MPNST development. The majority (5/8) of MPNSTs in our analyses demonstrated homozygous or heterozygous deletions of CDKN2A, which may represent an early event following NF1 LOH in the malignant transformation of Schwann cells from plexiform neurofibroma to MPNST. Biallelic somatic alterations of SUZ12 was also found in 4/8 MPNSTs. EED biallelic alterations were detected in 2 of the other four MPNSTs, with one tumor having a homozygous EED deletion. A missense mutation in the chromatin regulator KDM2B was also identified in one MPNST. No TP53 point mutations were found in this study, confirming previous data that TP53 mutations may be relatively rare in NF1-associated MPNSTs. Our study confirms the frequent biallelic inactivation of PRC2 subunits SUZ12 and EED in MPNSTs, and suggests the implication of KDM2B.

  11. [Neuroanatomy of the optic, trigeminal, facial, glossopharyngeal, vagus, accessory and hypoglossal nerves (author's transl)].

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    Lang, J

    1981-01-01

    1. The intracranial and intraorbital course of the optic nerve is described concisely, the intracanicular one in full details. Apart from the wide and small sections of the optic canal, its axis opposite to the cranial planes, the coating of the canal and the adjacency to the paranasal sinuses and arteries are exactly described. 2. At the trigeminal nerve the trigeminal ganglion, its roots and also the mandibular nerve have great importance in the practical medicine considering thermo-coagulation or surgery of the trigeminal nerve. This segments and also the adjacency of the fifth nerve to the internal carotid artery and subarachinoid brain vessels are exactly, the nuclei areas and central tracts are briefly explained. 3. The nuclei of the facial nerve the intracerebral and intracisternal course and its development, the facial canal and its narrow passes are described. Also the position of the internal acoustic pore in the skull, the dimensions of the internal acoustic meatus and the relations between nerves and vessels are explained. In addition to the geniculate ganglion and the chorda tympani the communications of the facial nerve inside the temporal bone, the tympanic intumescentia (ganglion) and the nervus intermedius, also the petrosal nerves are included in the description. The sheaths of the segments of the seventh cranial nerve and also the fasciculation are exactly, the somatotopic organization is briefly described. 4. The extracranial course of the glossopharyngeal nerve is briefly, its intracranial sections are included exactly in the investigation. 5. The nuclei of the vagus nerve and the intra- und extracranial course are described. 6. The accessory nerve, its nucleus and the intra- and extracranial course are concisely explained. 7. The hypoglossal nerve, its nucleus, the emergence of the fibres and also the relations of nerves and vessels in the posterior cranial fossa are described. The hypoglossal canal and also the extracranial course are

  12. The connective tissue and glial framework in the optic nerve head of the normal human eye: light and scanning electron microscopic studies.

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    Oyama, Tokuhide; Abe, Haruki; Ushiki, Tatsuo

    2006-12-01

    The arrangement of connective tissue components (i.e., collagen, reticular, and elastic fibers) and glial elements in the optic nerve head of the human eye was investigated by the combined use of light microscopy and scanning electron microscopy (SEM). Light-microscopically, the optic nerve head could be subdivided into four parts from the different arrangements of the connective tissue framework: a surface nerve fiber layer, and prelaminar, laminar, and postlaminar regions. The surface nerve fiber layer only possessed connective tissue elements around blood vessels. In the prelaminar region, collagen fibrils, together with delicate elastic fibers, formed thin interrupted sheaths for accommodating small nerve bundles. Immunohistochemistry for the glial fibrillary acidic protein (GFAP) showed that GFAP-positive cells formed columnar structures (i.e., glial columns), with round cell bodies piled up into layers. These glial columns were located in the fibrous sheaths of collagen fibrils and elastic fibers. In the laminar region, collagen fibrils and elastic fibers ran transversely to the optic nerve axis to form a thick membranous layer - the lamina cribrosa - which had numerous round openings for accommodating optic nerve fiber bundles. GFAP-positive cellular processes also ran transversely in association with collagen and elastin components. The postlaminar region had connective tissues which linked the lamina cribrosa with fibrous sheaths for accommodating nerve bundles in the extraocular optic nerve, where GFAP-positive cells acquired characteristics typical of fibrous astrocytes. These findings indicate that collagen fibrils, as a whole, form a continuous network which serves as a skeletal framework of the optic nerve head for protecting optic nerve fibers from mechanical stress as well as for sustaining blood vessels in the optic nerve. The lamina cribrosa containing elastic fibers are considered to be plastic against the mechanical force affected by elevation

  13. Anatomy of the optic nerve head and glaucomatous optic neuropathy.

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    Radius, R L

    1987-01-01

    The mechanism of axon damage in eyes with glaucomatous optic neuropathy remains undefined. Interestingly, it has been observed that, although the entire nerve cross-section may be involved by the nerve damage, in many instances, the superior and inferior axon bundles are preferentially affected by the pressure insult. Thus, recent studies by many investigators have stressed a re-examination of the optic nerve head anatomy, including the nerve head microcirculation, the glial and connective tissue elements within the nerve head, and the morphology of the axons themselves. Any correlation between regional differences in this anatomy and the preferential involvement by specific axon bundles within the nerve head by the pressure insult may suggest some further insight into the mechanisms underlying the pressure-induced axon loss in glaucomatous eyes.

  14. Neuronal differentiation of PC12 cells induced by sciatic nerve and optic nerve conditioned medium

    Institute of Scientific and Technical Information of China (English)

    DU Chan; YANG De-mei; ZHANG Pei-xun; DENG Lei; JIANG Bao-guo

    2010-01-01

    Background Previous work has shown that optic nerve and sciatic nerve conditional medium had neurotrophic activity on neurons. In order to find if the optic nerve conditioned media (CM) had a similar activity to make PC12 cells differentiate as sciatic nerve CM did, we explored the neurotrophic activity in optic nerve CM in the same in vitro system and compared the neurotrophin expression levels in optic and sciatic nerves under both conditions.Methods PC12 cells were used to examine the effects of neurotrophins secreted by the sciatic nerve and optic nerve. RT-PCR and real-time QPCR showed that the sciatic nerve and optic nerve produced a range of neurotrophins including nerve growth factor (NGF), brain derived neurotrophic factor (BDNF) and neurotrophin-3 (NT-3).Results The effects of sciatic nerve and optic nerve CM on neurite outgrowth were tested against a range of neurotrophins, and they had different neuritogenic activities. Only NGF and sciatic nerve CM had obvious neuritogenic activities, although the concentration of NGF in the sciatic nerve CM was very low.Conclusions Our experiment showed that sciatic nerve CM had a higher neurotrophic activity on PC12 cells than optic nerve CM. These results suggested that peripheral nervous system (PNS) and central nervous system (CNS) had different expression levels of neurotrophin, which may in part explain the lack of ability to regenerate the CNS.

  15. The optic nerve head in glaucoma

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    Rupert RA Bourne

    2006-09-01

    Full Text Available ll types of glaucoma involve glaucomatous optic neuropathy. The key to detection and management of glaucoma is understanding how to examine the optic nerve head (ONH. This pictorial glossary addresses the following issues: how to examine the ONH; normal characteristics of the ONH; characteristics of a glaucomatous ONH; how to tell if the glaucomatous optic neuropathy is getting worse;‘pitfalls and pearls’.

  16. Malignant Trigeminal Nerve Sheath Tumor and Anaplastic Astrocytoma Collision Tumor with High Proliferative Activity and Tumor Suppressor P53 Expression

    Directory of Open Access Journals (Sweden)

    Maher Kurdi

    2014-01-01

    Full Text Available Background. The synchronous development of two primary brain tumors of distinct cell of origin in close proximity or in contact with each other is extremely rare. We present the first case of collision tumor with two histological distinct tumors. Case Presentation. A 54-year-old woman presented with progressive atypical left facial pain and numbness for 8 months. MRI of the brain showed left middle cranial fossa heterogeneous mass extending into the infratemporal fossa. At surgery, a distinct but intermingled intra- and extradural tumor was demonstrated which was completely removed through left orbitozygomatic-temporal craniotomy. Histopathological examination showed that the tumor had two distinct components: malignant nerve sheath tumor of the trigeminal nerve and temporal lobe anaplastic astrocytoma. Proliferative activity and expressed tumor protein 53 (TP53 gene mutations were demonstrated in both tumors. Conclusions. We describe the first case of malignant trigeminal nerve sheath tumor (MTNST and anaplastic astrocytoma in collision and discuss the possible hypothesis of this rare occurrence. We propose that MTNST, with TP53 mutation, have participated in the formation of anaplastic astrocytoma, or vice versa.

  17. Malnutrition and myelin structure: an X-ray scattering study of rat sciatic and optic nerves

    Energy Technology Data Exchange (ETDEWEB)

    Vargas, V.; Vargas, R.; Marquez, G.; Vonasek, E.; Mateu, L. [Dept. de Biologia Estructural, Caracas (Venezuela); Luzzati, V. [Centre de Genetique Moleculaire, CNRS, Gif-sur-Yvette (France); Borges, J. [Servicio de Neurologia, Universidad Central de Venezuela, Caracas (Venezuela)

    2000-07-01

    Taking advantage of the fast and accurate X-ray scattering techniques recently developed in our laboratory, we tackled the study of the structural alterations induced in myelin by malnutrition. Our work was performed on sciatic and optic nerves dissected from rats fed with either a normal or a low-protein caloric diet, as a function of age (from birth to 60 days). By way of electrophysiological controls we also measured (on the sciatic nerves) the height and velocity of the compound action potential. Malnutrition was found to decrease the amount of myelin and to impair the packing order of the membranes in the sheaths. (orig.)

  18. Nerve sheath tumors of the head and neck - a radiological review; Tumores da bainha nervosa em cabeca e pescoco - estudo revisional

    Energy Technology Data Exchange (ETDEWEB)

    Souza, Ricardo Pires de; Carramao, Cintia Fernandes; Soares, Aldemir Humberto [Hospital Heliopolis, Sao Paulo, SP (Brazil). Servico de Diagnostico por Imagem; Chacra Junior, Jose; Rapoport, Abrao [Hospital Heliopolis, Sao Paulo, SP (Brazil). Servico de Cirurgia de Cabeca e Pescoco

    1997-01-01

    Peripheral nerve sheath tumors of the head and neck - a review. Peripheral nerve sheath tumors are derived from neural crest and rare classified as neuroectodermal in origin. They can be divided into neurofibroma, schwannoma and neurogenic sarcoma. Neurofifromas are benign well circumscribed, nonencapsulated tumors which involve all elements of normal peripheral nerves. Schwannomas are beginning encapsulated tumors composed fundamentally by Schwann cells. Neurogenic sarcomas are malignant tumors which can be de novo or arise from preexisting neurofibroma or schwannoma. Peripheral nerve sheath tumors can arise from any nerve that contain myelin sheath, but are more frequent in extremities and trunk, being rare on cervical region. neurogenic tumors of head and neck can arise from cranial nerves, especially vagus nerve, brachial plexus and other small nervous plexus. Computed tomography and magnetic resonance imaging are the methods of choice in the evaluation of those tumors and can demonstrate lesions with several patterns. Areas of cystic degeneration are frequent in schwannomas, while neurofibromas are usually homogeneous. About 1/3 of those tumors are hyper vascularized and those who arise nervous spinal; roots can have an aspect of dumbbell which contain cervical and intravertebral components. (author) 51 refs., 5 figs.

  19. Gender dimorphism and age of onset in malignant peripheral nerve sheath tumor preclinical models and human patients

    OpenAIRE

    Shurell, Elizabeth; Tran, Linh M.; Nakashima, Jonathan; Smith, Kathleen B.; Tam, Brenna M.; Li, Yunfeng; Dry, Sarah M.; Federman, Noah; Tap, William D; Wu, Hong; Eilber, Fritz C.

    2014-01-01

    Background Gender-based differences in disease onset in murine models of malignant peripheral nerve sheath tumor (MPNST) and in patients with Neurofibromatosis type-1-(NF-1)-associated or spontaneous MPNST has not been well studied. Methods Forty-three mGFAP-Cre+;Pten loxp/+;LSL-K-ras G12D/+ mice were observed for tumor development and evaluated for gender disparity in age of MPNST onset. Patient data from the prospectively collected UCLA sarcoma database (1974–2011, n = 113 MPNST patients) a...

  20. DNA copy number changes in high-grade malignant peripheral nerve sheath tumors by array CGH

    Directory of Open Access Journals (Sweden)

    Bjerkehagen Bodil

    2008-06-01

    Full Text Available Abstract Background Malignant peripheral nerve sheath tumors (MPNSTs are rare and highly aggressive soft tissue tumors showing complex chromosomal aberrations. In order to identify recurrent chromosomal regions of gain and loss, and thereby novel gene targets of potential importance for MPNST development and/or progression, we have analyzed DNA copy number changes in seven high-grade MPNSTs using microarray-based comparative genomic hybridization (array CGH. Results Considerable more gains than losses were observed, and the most frequent minimal recurrent regions of gain included 1q24.1-q24.2, 1q24.3-q25.1, 8p23.1-p12, 9q34.11-q34.13 and 17q23.2-q25.3, all gained in five of seven samples. The 17q23.2-q25.3 region was gained in all five patients with poor outcome and not in the two patients with disease-free survival. cDNA microarray analysis and quantitative real-time reverse transcription PCR were used to investigate expression of genes located within these regions. The gene lysyl oxidase-like 2 (LOXL2 was identified as a candidate target for the 8p23.1-p12 gain. Within 17q, the genes topoisomerase II-α (TOP2A, ets variant gene 4 (E1A enhancer binding protein, E1AF (ETV4 and baculoviral IAP repeat-containing 5 (survivin (BIRC5 showed increased expression in all samples compared to two benign tumors. Increased expression of these genes has previously been associated with poor survival in other malignancies, and for TOP2A, in MPNSTs as well. In addition, we have analyzed the expression of five micro RNAs located within the 17q23.2-q25.3 region, but none of them showed high expression levels compared to the benign tumors. Conclusion Our study shows the potential of using DNA copy number changes obtained by array CGH to predict the prognosis of MPNST patients. Although no clear correlations between the expression level and patient outcome were observed, the genes TOP2A, ETV4 and BIRC5 are interesting candidate targets for the 17q gain associated

  1. Intraosseous primary malignant peripheral nerve sheath tumor of the calcaneus: An unusual case and review of literature

    Directory of Open Access Journals (Sweden)

    Gaurav Pratap Singh Gahlot

    2015-01-01

    Full Text Available Malignant peripheral nerve sheath tumors (MPNSTs are uncommon sarcomas that originate from a peripheral nerve or neurofibroma either spontaneously or in association with neurofibromatosis type 1. MPNSTs account for approximately 5% of all soft tissue malignancies. The tumor is commonly seen in the extremities and trunk. Most of these tumors are high-grade with the potential to recur and metastasize. Common metastatic sites include the lungs, bone, and pleura. Primary intraosseous MPNST is rare, and the diagnosis of intraosseous MPNST, especially in an unusual location is difficult because of its cellular origin, histomorphological similarities with other sarcomas, and bone is the most common site for metastasis. We report an unusual case of MPNST of the calcaneus in a young male.

  2. Rexin-G®, a tumor-targeted retrovector for malignant peripheral nerve sheath tumor: A case report

    Science.gov (United States)

    Kim, Seth; Federman, Noah; Gordon, Erlinda M.; Hall, Frederick L.; Chawla, Sant P.

    2017-01-01

    Soft tissue sarcoma is a rare neoplasm of mesenchymal origin, accounting for only ~1% of all adult cancers and consisting of 75 histological subtypes. In the present report, the unique case of a 14 year-old female with metastatic malignant peripheral nerve sheath tumor (formerly, malignant melanotic schwannoma) of the parotid gland, who experienced a durable response and sustained tumor control with Rexin-G®, a tumor-targeted retroviral expression vector encoding an anti-cyclin G1 construct, is described. Post-parotidectomy, and prior to the administration of Rexin-G®, the patient received various chemotherapy regimens, including doxorubicin, ifosfamide, temozolomide, sorafenib, and an immunological therapy with interleukin-2, which only resulted in the further progression of lung metastases. The patient subsequently participated in a Phase 1/2 gene therapy study, during which she received intravenous Rexin-G® as monotherapy for two years with minimal drug-associated adverse events. Currently, the patient has no evidence of active disease 9 years after commencing the Rexin-G® treatment, and with no additional anti-cancer therapy. In conclusion, Rexin-G® may be a viable therapeutic option for malignant peripheral nerve sheath tumors, and should be further investigated in prospective histology-specific clinical trials for this type, and possibly other types, of chemotherapy-resistant sarcoma. PMID:28588778

  3. Giant malignant peripheral nerve sheath tumor of thigh in an adolescent with neurofibromatosis type 1: a case report

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    Tosun HB

    2015-10-01

    Full Text Available Haci Bayram Tosun,1 Sancar Serbest,2 Bilge Aydin Turk,3 Seyit Ali Gumustas,1 Abuzer Uludag1 1Department of Orthopaedics and Traumatology, Faculty of Medicine, Adiyaman University, Adiyaman, 2Department of Orthopaedics and Traumatology, Faculty of Medicine, Kirikkale University, Kirikkale, 3Department of Pathology, Faculty of Medicine, Adiyaman University, Adiyaman, Turkey Abstract: Malignant peripheral nerve sheath tumors (MPNSTs are rare sarcomas of children and adolescents, and they are aggressive tumors with a high rate of local recurrence. We present a 15-year-old boy with neurofibromatosis type 1 (NF1, who had a giant MPNST on the right thigh taking into account the available literature. Diagnosis of MPNST may be delayed in NF1 patients due to confusion with a neurofibroma and/or a plexiform neurofibroma. Malignancy should be considered, especially in cases with big masses, with heterogeneous involvement, or in the presence of cysts or necrotic nodules. The aim of surgical treatment is complete surgical excision. Keywords: nerve sheath neoplasm, sarcoma, adolescent, neurofibromatosis, lower extremity

  4. Malignant peripheral nerve sheath tumours in neurofibromatosis type 1: MRI supports the diagnosis of malignant plexiform neurofibroma

    Energy Technology Data Exchange (ETDEWEB)

    Mautner, V.F. [Department of Neurology, Klinikum Nord Hamburg, Langenhorner Chaussee 560, 22419, Hamburg (Germany); Friedrich, R.E. [Department of Maxillofacial Surgery, Universitaetsklinikum Eppendorf, Hamburg (Germany); Deimling, A. von [Department of Neuropathology, Charite, Berlin (Germany); Hagel, C. [Department of Neuropathology, Universitaetsklinikum Eppendorf, Hamburg (Germany); Korf, B. [Center for Human Genetics, Harvard Institutes of Medicine, Boston, MA (United States); Knoefel, M.T. [Department of Surgery, Universitaetsklinikum Eppendorf, Hamburg (Germany); Wenzel, R.; Fuensterer, C. [MRI-Institute Hamburg Othmarschen, Hamburg (Germany)

    2003-09-01

    Plexiform neurofibroma (PNF) is a typical feature of neurofibromatosis 1 (NF1). About 10% of patients with NF1 develop malignant peripheral nerve-sheath tumours (MPNST), usually arising from PNF, and this is the major cause of poor survival. A better prognosis can be achieved if the tumours are diagnosed at an early stage. Our objective was to establish MRI criteria for MPNST and to test their usefulness in detecting early malignant change in PNF. MRI was performed on 50 patients with NF1 and nerve-sheath tumours, of whom seven had atypical pain, tumour growth or neurological deficits indicative of malignancy; the other 43 were asymptomatic. On MRI all seven symptomatic patients had inhomogeneous lesions, due to necrosis and haemorrhage and patchy contrast enhancement. In one patient, the multiplicity of confluent tumours with inhomogeneous areas in addition to central lesions did not allow exclusion of malignancy. Only three of the 43 asymptomatic patients had comparable changes; the other 40 patients had tumours being of relatively homogeneous structure on T1- and T2-weighted images before and after contrast enhancement. All three asymptomatic patients with inhomogeneous lesions were shown to have MPNST. (orig.)

  5. Acupuncture Treatment for Optic Nerve Contusion

    Institute of Scientific and Technical Information of China (English)

    2008-01-01

    @@ Optic nerve contusion is a commonly-seen eye injury, which is mostly caused by traffic accident, collision, and falling. Early diagnosis and timely emergency treatment can make such patients restore vision to a certain extent. Otherwise, there may appear optic atrophy or loss of vision. At present, in the treatment of this disease, cortical hormone, dehydrating agent, vasodilator, vitamin, energy mixture and neurotrophic agent, or surgical operation can all give certain therapeutic effect. In the recent 5 years, the Department of Ophthalmology of the Hospital Affiliated to Hubei College of Traditional Chinese Medicine has adopted acupuncture for treatment of optic nerve contusion, and obtained quite good therapeutic results. Some typical cases are reported in the following.

  6. Verticillin A Inhibits Leiomyosarcoma and Malignant Peripheral Nerve Sheath Tumor Growth via Induction of Apoptosis

    Science.gov (United States)

    Zewdu, A; Lopez, G; Braggio, D; Kenny, C; Constantino, D; Bid, HK; Batte, K; Iwenofu, OH; Oberlies, NH; Pearce, CJ; Strohecker, AM; Lev, D; Pollock, RE

    2017-01-01

    Objective The heterogeneity of soft tissue sarcoma (STS) represents a major challenge for the development of effective therapeutics. Comprised of over 50 different histology subtypes of various etiologies, STS subsets are further characterized as either karyotypically simple or complex. Due to the number of genetic anomalies associated with genetically complex STS, development of therapies demonstrating potency against this STS cluster is especially challenging and yet greatly needed. Verticillin A is a small molecule natural product with demonstrated anticancer activity; however, the efficacy of this agent has never been evaluated in STS. Therefore, the goal of this study was to explore verticillin A as a potential STS therapeutic. Methods We performed survival (MTS) and clonogenic analyses to measure the impact of this agent on the viability and colony formation capability of karyotypically complex STS cell lines: malignant peripheral nerve sheath tumor (MPNST) and leiomyosarcoma (LMS). The in vitro effects of verticillin A on apoptosis were investigated through annexin V/PI flow cytometry analysis and by measuring fluorescently-labeled cleaved caspase 3/7 activity. The impact on cell cycle progression was assessed via cytometric measurement of propidium iodide intercalation. In vivo studies were performed using MPNST xenograft models. Tumors were processed and analyzed using immunohistochemistry (IHC) for verticillin A effects on growth (Ki67) and apoptosis (cleaved caspase 3). Results Treatment with verticillin A resulted in decreased STS growth and an increase in apoptotic levels after 24 h. 100 nM verticillin A induced significant cellular growth abrogation after 24 h (96.7, 88.7, 72.7, 57, and 39.7% reduction in LMS1, S462, ST88, SKLMS1, and MPNST724, respectively). We observed no arrest in cell cycle, elevated annexin, and a nearly two-fold increase in cleaved caspase 3/7 activity in all MPNST and LMS cell lines. Control normal human Schwann (HSC) and

  7. Traumatic globe luxation with optic nerve transection.

    Science.gov (United States)

    Bajaj, Mandeep S.; Kedar, Sachin; Sethi, Anita; Gupta, Vishal

    2000-09-01

    Luxation of the globe is a rare event that results from severe trauma to the orbit, often causing orbital rim and wall fractures. Though associated globe rupture often necessitates enucleation, repositioning of the globe can be attempted in these cases if the globe is intact. We report a case of globe luxation and optic nerve transection with its surgical management. A 17-year-old male presented with anterior luxation of the right globe and optic nerve transection following blunt trauma to the orbit. Computerized tomography revealed an anteriorly subluxated globe with complete transection of the optic nerve and multiple fractures of the orbital walls. The orbit was explored and the globe repositioned in the orbit with reattachment of the muscles. Postoperatively, the globe was in normal position with moderate motility and excellent overall cosmesis. Though the visual prognosis in these cases is usually extremely poor and depends on the extent and duration of injury, preservation of the globe not only helps the patient recover psychologically from the trauma but also allows better cosmesis.

  8. An ShRNA Screen Identifies MEIS1 as a Driver of Malignant Peripheral Nerve Sheath Tumors

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    Ami V. Patel

    2016-07-01

    Full Text Available Malignant peripheral nerve sheath tumors (MPNST are rare soft tissue sarcomas that are a major source of mortality in neurofibromatosis type 1 (NF1 patients. To identify MPNST driver genes, we performed a lentiviral short hairpin (sh RNA screen, targeting all 130 genes up-regulated in neurofibroma and MPNSTs versus normal human nerve Schwann cells. NF1 mutant cells show activation of RAS/MAPK signaling, so a counter-screen in RAS mutant carcinoma cells was performed to exclude common RAS-pathway driven genes. We identified 7 genes specific for survival of MPSNT cells, including MEIS1. MEIS1 was frequently amplified or hypomethylated in human MPSNTs, correlating with elevated MEIS1 gene expression. In MPNST cells and in a genetically engineered mouse model, MEIS1 expression in developing nerve glial cells was necessary for MPNST growth. Mechanistically, MEIS1 drives MPNST cell growth via the transcription factor ID1, thereby suppressing expression of the cell cycle inhibitor p27Kip and maintaining cell survival.

  9. The occurrence of IgM and complement factors along myelin sheaths of peripheral nerves An immunohistochemical study of the Guillain-Barre syndrome : Preliminary communication

    NARCIS (Netherlands)

    Luijten, J.A.F.M.; Baart de la Faille-Kuyper, E.H.

    In nerve biopsies from 4 of 6 patients with the Guillain-Barré syndrome (GBS), IgM and complement factors were found, probably localized along myelin sheaths. The possible significance of this phenomenon has been discussed. No such findings were obtained in control subjects.

  10. The occurrence of IgM and complement factors along myelin sheaths of peripheral nerves An immunohistochemical study of the Guillain-Barre syndrome : Preliminary communication

    NARCIS (Netherlands)

    Luijten, J.A.F.M.; Baart de la Faille-Kuyper, E.H.

    1972-01-01

    In nerve biopsies from 4 of 6 patients with the Guillain-Barré syndrome (GBS), IgM and complement factors were found, probably localized along myelin sheaths. The possible significance of this phenomenon has been discussed. No such findings were obtained in control subjects.

  11. Computer-assisted cytogenetic analysis of 51 malignant peripheral-nerve-sheath tumors : Sporadic vs. neurofibromatosis-type-1-associated malignant schwannomas

    NARCIS (Netherlands)

    Plaat, B E; Molenaar, W M; Mastik, M F; Hoekstra, H J; te Meerman, G J; van den Berg, E

    1999-01-01

    Cytogenetic studies in small groups of patients with malignant peripheral-nerve-sheath tumors (MPNST) revealed complex karyotypes with no consistent changes. A computer-assisted cytogenetic analysis using a cytogenetic database was performed to determine recurrent cytogenetic alterations in 51 MPNST

  12. The occurrence of IgM and complement factors along myelin sheaths of peripheral nerves An immunohistochemical study of the Guillain-Barre syndrome : Preliminary communication

    NARCIS (Netherlands)

    Luijten, J.A.F.M.; Baart de la Faille-Kuyper, E.H.

    1972-01-01

    In nerve biopsies from 4 of 6 patients with the Guillain-Barré syndrome (GBS), IgM and complement factors were found, probably localized along myelin sheaths. The possible significance of this phenomenon has been discussed. No such findings were obtained in control subjects.

  13. Genomic Changes in Chromosomes 10, 16, and X in Malignant Peripheral Nerve Sheath Tumors Identify a High-Risk Patient Group

    NARCIS (Netherlands)

    Brekke, Helge R.; Ribeiro, Franclim R.; Kolberg, Matthias; Agesen, Trude H.; Lind, Guro E.; Eknaes, Mette; Hall, Kirsten S.; Bjerkehagen, Bodil; van den Berg, Eva; Teixeira, Manuel R.; Mandahl, Nils; Smeland, Sigbjorn; Mertens, Fredrik; Skotheim, Rolf I.; Lothe, Ragnhild A.

    2010-01-01

    Purpose The purpose of this study was to identify genetic aberrations contributing to clinical aggressiveness of malignant peripheral nerve sheath tumors (MPNSTs). Patients and Methods Samples from 48 MPNSTs and 10 neurofibromas were collected from 51 patients with (n = 31) or without (n = 20) neuro

  14. Sheehan's Syndrome with Edema in Optical Neural Sheaths: A Case Report

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    Mehtap Evran

    2013-04-01

    Full Text Available Sheehan syndrome is partial or complete hypophyseal insufficiency due to hypophyseal necrosis secondary to postpartum hemorrhage and hypovolemia. Clinical findings are cessation of lactation, secondary amenorrhea, loss of libido and hypophyseal reserve loss. Treatment is hormone replacement. Forty-four years old women admitted with somnolence, malaise, edema and blurred vision. Physical examination revealed edema and hypotension. She had massive bleeding during her birth 11 years old ago; therefore she had received blood transfusion and could not breast-feed then. She had amenorrhea when she admitted. She was diagnosed to be Sheehan syndrome while glucocorticoid and levothyroxine replacement was started. Hypophyseal and orbital magnetic resonance imaging revealed empty sella and edema in bilateral optical neural sheaths respectively. Empty sella may cause edema in optical neural sheaths by increasing pressure in subarachnoidal space. We present this case to point out this coincidence. [Cukurova Med J 2013; 38(2.000: 299-304

  15. Divergent differentiation in malignant soft tissue neoplasms: the paradigm of liposarcoma and malignant peripheral nerve sheath tumor.

    Science.gov (United States)

    Pytel, Peter; Taxy, Jerome B; Krausz, Thomas

    2005-01-01

    In tumors clonality does not always translate into morphologic uniformity. While most sarcomas exhibit only one line of histologic differentiation, a minority may display a strikingly diverse phenotype in addition to the main lineage. This phenomenon not only presents a diagnostic problem but also raises questions about the commitment of tumor cells toward a specific phenotype. Among sarcomas, malignant peripheral nerve sheath tumor (MPNST) and dedifferentiated liposarcoma are two entities in which divergent differentiation is a relatively frequent event. Diagnostically it is crucial to recognize the "primary" sarcoma in such tumors correctly and distinguish it from the "secondary" divergent elements. The presence of the latter could be the first morphologic clue to a specific sarcoma type. Even though it may be difficult to explain the pathogenesis of divergent differentiation, divergence still illustrates that the phenotype of a tumor cell is not set in stone but can be modulated or switched by a number of factors.

  16. Primary hepatic malignant peripheral nerve sheath tumor successfully treated with combination therapy: a case report and literature review

    Science.gov (United States)

    Jung, Hae Il; Lee, Hyoung Uk; Ahn, Tae Sung; Lee, Jong Eun; Lee, Hyun Yong; Cho, Hyon Doek; Lee, Sang Cheol

    2016-01-01

    Primary malignant peripheral nerve sheath tumor (MPNST) in a young female patient, not associated with neurofibromatosis type-I is extremely rare in the liver. A 33-year-old female was admitted with a right flank pain for a weak. The CT scan showed 12.5-cm-sized mass located at the right hepatic lobe. At laparotomy, about 20.0-cm-sized mass was on the right hepatic lobe with attachment to right diaphragmatic pleura. Right hepatic lobe and adherent part of diaphragmatic pleura were resected. On histology and immunohistochemistry, it was diagnosed MPNST. Adjuvant radiotherapy for the right diaphragmatic pleura and adjuvant chemotherapy with adriamycin, ifosfamide and cisplatin were sequentially performed. The prognosis of MPNST is generally poor and it is associated with a highly aggressive course of recurrence, metastases, and death. Our case is probably a first report about combination therapy. PMID:27904856

  17. BET Bromodomain Inhibition Triggers Apoptosis of NF1-Associated Malignant Peripheral Nerve Sheath Tumors through Bim Induction

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    Amish J. Patel

    2014-01-01

    Full Text Available Malignant peripheral nerve sheath tumors (MPNSTs are highly aggressive sarcomas that develop sporadically or in neurofibromatosis type 1 (NF1 patients. There is no effective treatment for MPNSTs and they are typically fatal. To gain insights into MPNST pathogenesis, we utilized an MPNST mouse model that allowed us to study the evolution of these tumors at the transcriptome level. Strikingly, in MPNSTs we found upregulation of a chromatin regulator, Brd4, and show that BRD4 inhibition profoundly suppresses both growth and tumorigenesis. Our findings reveal roles for BET bromodomains in MPNST development and report a mechanism by which bromodomain inhibition induces apoptosis through induction of proapoptotic Bim, which may represent a paradigm shift in therapy for MPNST patients. Moreover, these findings indicate epigenetic mechanisms underlying the balance of anti- and proapoptotic molecules and that bromodomain inhibition can shift this balance in favor of cancer cell apoptosis.

  18. PIC Simulation of RF Plasma Sheath Formation and Initial Validation of Optical Diagnostics using HPC Resources

    Science.gov (United States)

    Icenhour, Casey; Exum, Ashe; Martin, Elijah; Green, David; Smithe, David; Shannon, Steven

    2014-10-01

    The coupling of experiment and simulation to elucidate near field physics above ICRF antennae presents challenges on both the experimental and computational side. In order to analyze this region, a new optical diagnostic utilizing active and passive spectroscopy is used to determine the structure of the electric fields within the sheath region. Parallel and perpendicular magnetic fields with respect to the sheath electric field have been presented. This work focuses on the validation of these measurements utilizing the Particle-in-Cell (PIC) simulation method in conjunction with High Performance Computing (HPC) resources on the Titan supercomputer at Oak Ridge National Laboratory (ORNL). Plasma parameters of interest include electron density, electron temperature, plasma potentials, and RF plasma sheath voltages and thicknesses. The plasma is modeled utilizing the VSim plasma simulation tool, developed by the Tech-X Corporation. The implementation used here is a two-dimensional electromagnetic model of the experimental setup. The overall goal of this study is to develop models for complex RF plasma systems and to help outline the physics of RF sheath formation and subsequent power loss on ICRF antennas in systems such as ITER. This work is carried out with the support of Oak Ridge National Laboratory and the Tech-X Corporation.

  19. Sera from patients with seropositive neuromyelitis optica spectral disorders caused the degeneration of rodent optic nerve.

    Science.gov (United States)

    Matsumoto, Yoshiko; Kanamori, Akiyasu; Nakamura, Makoto; Takahashi, Toshiyuki; Nakashima, Ichiro; Negi, Akira

    2014-02-01

    Neuromyelitis optica (NMO) is an autoimmune inflammatory, neurodestructive disease primarily targeting the optic nerve and spinal cord. An autoantibody against water channel protein aquaporin-4 (AQP4), which is expressed at endofeet of astrocytes has been implicated in the pathogenesis of NMO. We evaluated the impact of sera of seropositive patients with NMO spectrum disorders (NMOSDs) on the rodent optic nerve and retina. Serum was obtained either from patients with seropositive NMOSD (AQP4+), seronegative patient with idiopathic optic neuritis (AQP4-), and healthy volunteers (control). Anti-AQP4 antibody in a serum was measured by a previously established cell-based assay. The patients' sera were applied on the optic nerve after de-sheathed. Immunohistochemistry showed that at 7 days after the treatment, the area of the optic nerve exposed to the AQP4+ sera lost expression of both AQP4 and glial fibrillary acidic protein. Also, Human-IgG immunoreactivity and marked invasion of inflammation cells were observed in the optic nerve treated with AQP4+ serum. Immnoreactivity of neurofilament was reduced at 14 days after the treatment, not 7 days. Real-time polymerase chain reaction revealed the reduced gene expression of neurofilament in retina from the eye that was exposed to the AQP4+ sera at 14 days. Retrograde fluorogold-labeling on the retinal flatmount disclosed the significantly reduced number of retinal ganglion cells when the AQP4+ sera were applied. The present model has demonstrated that the sera from patients with seropositive NMOSDs led to the regional astrocytic degeneration and inflammatory cell invasion in the optic nerve, resulting in the ultimate loss of RGCs and their axons at areas beyond the injury site.

  20. Growth Factor and Laminin Effect with Muscular Fiber Sheath on Repairing of the Sciatica Nerve

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    S Torabi

    2014-01-01

    Background & aim: Peripheral nerve injuries which can lead to a physical disability. If the defect is very low, direct suture without tension on both ends of the cut nerve regeneration is considered as a standard procedure. Otherwise, to reconstruct the axons, the gap must be filled by graft material in order to the guidance. Due to the similarity of the matrix tubular skeletal muscle and nerve muscles graft was used to repair in this study. Methods: In the present experimental study, 42 female Wistar rats were divided into three groups and underwent surgery. In the first group a narrow strip of muscle was prepared by freezing – thawing, and later sutured between the distal and proximal sciatic nerve. In the second group, the gap caused by muscle graft was regenerated and the nerve growth factor and laminin was injected into the graft. In the control group, the two ends of the cut nerve were hidden beneath the adjacent muscles. Next, a group of rats with sciatic functional index was investigated for the behavioral. On the other group were examined for histological studies after two months. Results: Sciatic functional index and Mean counts of myelinated fibers in two graft groups compared with the control group was significant p<0.05. Statistical analysis was performed using ANOVA test. Conclusion: co-axially aligned muscle grafts were an appropriate alternative substitute for repairing. It seems that the nerve growth factor and laminin have a positive role in axonal regeneration and functional recovery acceleration. Key words: Sciatic Functional Index, muscle graft, NGF, Laminin

  1. A case of amblyopia with contralateral optic nerve hypoplasia.

    Science.gov (United States)

    Frantz, Kelly A; Pang, Yi

    2013-09-01

    We describe an unusual case of unilateral optic nerve hypoplasia (ONH) in a patient with contralateral anisometropic/strabismic amblyopia. A seven-year-old boy presented with visual acuities of 6/12 R and 6/18 L and eccentric fixation in the left eye. Cycloplegic retinoscopy was R +1.50/-0.50 × 180 and L +5.25 DS. Funduscopy revealed optic nerve hypoplasia of the right eye. The patient fixated with his better-seeing right eye, despite the optic nerve hypoplasia. His reduced vision may be attributed to optic nerve hypoplasia in the right eye and amblyopia in the left. Although optic nerve hypoplasia can occur with ipsilateral amblyopia, we believe this is the first reported case of unilateral optic nerve hypoplasia in the fellow eye of an amblyopic patient.

  2. Optic Nerve Lymphoma. Report of Two Cases and Review of the Literature

    Science.gov (United States)

    Kim, Jennifer L.; Mendoza, Pia; Rashid, Alia; Hayek, Brent; Grossniklaus, Hans E.

    2014-01-01

    Lymphoma may involve the optic nerve as isolated optic nerve lymphoma or in association with CNS or systemic lymphoma. We present two biopsy-proven non-Hodgkin lymphomas of the optic nerve and compare our findings with previously reported cases. We discuss the mechanism of metastasis, classification of optic nerve involvement, clinical features, radiologic findings, optic nerve biopsy indications and techniques, histologic features, and treatments. We propose a classification system of optic nerve lymphoma: isolated optic nerve involvement, optic nerve involvement with CNS disease, optic nerve involvement with systemic disease, and optic nerve involvement with primary intraocular lymphoma. Although it is an uncommon cause of infiltrative optic neuropathy, optic nerve metastasis should be considered in patients with a history of lymphoma. The recommended approach to a patient with presumed optic nerve lymphoma includes neuroimaging, and cerebrospinal fluid evaluation as part of the initial work-up, then judicious use of optic nerve biopsy, depending on the clinical situation. PMID:25595061

  3. Epithelioid Sarcoma of the Forearm Arising from Perineural Sheath of Median Nerve Mimicking Carpal Tunnel Syndrome

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    Hiromasa Fujii

    2009-01-01

    Full Text Available We report here a case of epithelioid sarcoma in the forearm of a 33-year-old male presenting with symptoms and signs of carpal tunnel syndrome originating from the direct involvement of the median nerve. Due to the slow growing of the tumor, the patient noticed the presence of tumor mass in his forearm after several months from the initial onset of the symptoms. Magnetic resonance imaging showed an 8×4 cm mass involving the median nerve in the middle part of the forearm, and histological analysis of the biopsy specimen revealed the diagnosis of epithelioid sarcoma. Radical surgical resection was performed in conjunction with adjuvant chemotherapy. The function of the flexors were restored by the multiple tendon transfers (EIP→FDS; ECRL→FDP; BrR→FPL; EDM→opponens with superficial cutaneous branch of radial nerve transfer to the resected median nerve. The function of the affected hand showed excellent with the DASH disability/symptom score of 22.5, and both the grasp power and sensory of the median nerve area has recovered up to 50% of the normal side. The patient returned to his original vocation and alive with continuous disease free at 3.5-year follow-up since initial treatment.

  4. Hemangioblastoma of the optic nerve--case report.

    Science.gov (United States)

    Higashida, Tetsuhiro; Sakata, Katsumi; Kanno, Hiroshi; Kawasaki, Takashi; Tanabe, Yutaka; Yamamoto, Isao

    2007-05-01

    A 64-year-old man presented with a rare sporadic hemangioblastoma arising in the left optic nerve manifesting as left visual disturbance gradually progressive over 5 years. Magnetic resonance imaging revealed a well-enhanced mass in the left optic nerve. Partial resection of the tumor was performed via the frontoorbital approach. The histological diagnosis was optic nerve hemangioblastoma. Hemangioblastoma must be considered in the differential diagnosis of optic nerve tumors even in the absence of other lesions associated with von Hippel-Lindau disease.

  5. Expressions of matrix metalloproteinase-9 and tissue inhibitor of metalloproteinase-1 in malignant peripheral nerve sheath tumor

    Institute of Scientific and Technical Information of China (English)

    2007-01-01

    BACKGROUND: Matrix metalloproteinase-9 (MMP-9) can degrade collagen Ⅳ (the main structural ingredient of basilar membrane), and it also plays an important role in tumor vascularization, tumor cell progression, formation of metastatic focus, etc. Tissue inhibitor of metalloproteinase-1 (TIMP-1) can bind with MMP-9 to form 1∶1 compound and inhibit its activity, and can negatively regulate the tumor progression and metastasis.OBJECTIVE: To analyze the relationship of MMP-9 and TIMP-1 expressions with the pathological grade,metastasis and prognosis of malignant peripheral nerve sheath tumor (MPNST).DESIGN: An observational comparative experiment.SETTING: Heze Medical College.PARTICIPANTS: Fifty-eight surgical pathological samples, which were clearly diagnosed to be MPNST,were collected from the pathological laboratory archives in the Department of Pathology, Heze Municipal Hospital from January 1988 to December 2003. The MPNST pathological types were common tumor in 53 cases, malignant triton tumor in 2 cases, epithelial MPNST in 2 cases and MPNST with gland differentiation in 1 case. The pathological grade was grade 1 in 11 cases, grade 2 in 24 cases and grade 3 in 23 cases.Besides, the resected tumor samples of 20 patients with benign peripheral nerve tumor (10 cases of nerve sheath tumor and 10 cases of neurofibromatosis) and the normal peripheral nerves (by-products of some surgeries) of 5 patients were also collected. The samples were used with the approval of the patients.Rat-anti-human MMP-9, TIMP-1 monoclonal antibody and S-P kit were purchased from Fuzhou Maixin Biotechnology, Co.,Ltd.METHODS: The documented paraffin blocks were again prepared to sections of 5 μ m. The expressions of MMP-9 and TIMP-1 in the samples were detected with mmunohistochemical S-P method. The relationships of the MPNST severity, recurrence, metastasis and survival rate with the expressions of MMP-9 and TIMP-1 were analyzed.MAIN OUTCOME MEASURES: Relationships of MMP-9 and TIMP-1

  6. 视神经减压术对颅脑损伤合并视神经损伤视力康复的意义%The significance of decompression of optic nerve in the rehabilitation of traumatic optic nerve following head trauma

    Institute of Scientific and Technical Information of China (English)

    邱建武; 宋振全; 林军

    2002-01-01

    Objective To investigate the significance of decompression of optic nerve in the rehabilitation of traumatic optic neuropathy following head trauma.Method Surgical treatment was performed on 16 patients suffered from traumatic neuropathy following head trauma.Fragments ,hematoma,necrotic brain tissue resulting from fracture of orbital bone were removed and frontal and orbital fracture or malposition corrected by craniotomy through valve of frontal bone in 15 patients.Optic canal was probed and sheath of optic nerve was cut open for decompression of optic nerve.Frontotemporal wound was made in 1 case with superior orbital fissure syndrome and decompression of optic canal and abrasion of superior orbital fissure were performed through outer side of orbital point.Result Surgical treatment was effective in 12 cases(75% ),exophthalmus in 2 cases was corrected.Conclusion Decompression of optic nerve through frontal bone or frontotemporal is effective in enhancing visual acuity in patients with traumatic optic neuropathy following head trauma.

  7. Intraorbital neuromuscular choristoma adjacent to the optic nerve

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    Arie Perry, M.D.

    2017-03-01

    Full Text Available Neuromuscular choristoma is a rare tumor that incorporates mature skeletal muscle within fascicles of peripheral nerve. The etiology is poorly understood, yet most present in large nerves of children, with a tight link to post-operative fibromatosis recently appreciated. Herein, we report an exceptional intra-orbital example in a 53-year-old man with optic nerve compression.

  8. Integrated Model of the Eye/Optic Nerve Head Biomechanical Environment

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    Ethier, C. R.; Feola, A.; Myers, J. G.; Nelson, E.; Raykin, J.; Samuels, B.

    2017-01-01

    Visual Impairment and Intracranial Pressure (VIIP) syndrome is a concern for long-duration space flight. Previously, it has been suggested that ocular changes observed in VIIP syndrome are related to the cephalad fluid shift that results in altered fluid pressures [1]. We are investigating the impact of changes in intracranial pressure (ICP) using a combination of numerical models, which simulate the effects of various environment conditions, including finite element (FE) models of the posterior eye. The specific interest is to understand how altered pressures due to gravitational changes affect the biomechanical environment of tissues of the posterior eye and optic nerve sheath. METHODS: Additional description of the numerical modeling is provided in the IWS abstract by Nelson et al. In brief, to simulate the effects of a cephalad fluid shift on the cardiovascular and ocular systems, we utilized a lumped-parameter compartment model of these systems. The outputs of this lumped-parameter model then inform boundary conditions (pressures) for a finite element model of the optic nerve head (Figure 1). As an example, we show here a simulation of postural change from supine to 15 degree head-down tilt (HDT), with primary outcomes being the predicted change in strains at the optic nerve head (ONH) region, specifically in the lamina cribrosa (LC), retrolaminar optic nerve, and prelaminar neural tissue (PLNT). The strain field can be decomposed into three orthogonal components, denoted as the first, second and third principal strains. We compare the peak tensile (first principal) and compressive (third principal) strains, since elevated strain alters cell phenotype and induces tissue remodeling. RESULTS AND CONCLUSIONS: Our lumped-parameter model predicted an IOP increase of c. 7 mmHg after 21 minutes of 15 degree HDT, which agreed with previous reports of IOP in HDT [1]. The corresponding FEM simulations predicted a relative increase in the magnitudes of the peak tensile

  9. Morphometry of Axons in Optic Nerves of Siamese's Twins

    Institute of Scientific and Technical Information of China (English)

    Xinzu Gu; Zhenping Zhang; Qi Lin; Jiongji Liang; Wenyu Lu; Xiulan Ye; A A Sadun

    2002-01-01

    Purpose: To observe the development of optic nerve, we examined four optic nerves from Siameses Twins by absolute counts of axons.Methods: Mean axon diameter, mean axon density, totally axonal population and optic nerve area were noted for each optic nerve. The mean axon diameter and the mean axon density were compared between paraxial (inner sectors)and cortical (outer sectors)areas of the nerves.Results: More myelinated axons were seen in the inner sectors as compared to the outer sectors(average 11 axons/1 000 μm2 in inner sectors and 34 axons/l 000 μm2 in outer sectors( P=0. 036) . The myelinated fibers were also smaller(63 microns) in the outer sectors as compared to the inner sectors(72 microns) ( P = 0. 001 ). The average cross sectors area for the four 40 week stage optical nerves of Siamese Twins was 3.32 × 103 as compared to 1 million axons for 32-week-old normals.Conclusion: Our finding of fewer axonal number and small myelinated fibers in the Siamese Twins suggests hypoplasia. Myelination was more abnormal in the paraxial optic nerve than that in the peripheral sectors, suggesting anomalous development of optic nerve peripherally and delayed developnent centrally. Axonal density is higher in inner sectors than that in outer sectors, suggesting delayed development of the outer nerve sector.

  10. Overexpression of cyclooxygenase-2 in malignant peripheral nerve sheath tumor and selective cyclooxygenase-2 inhibitor-induced apoptosis by activating caspases in human malignant peripheral nerve sheath tumor cells.

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    Michiyuki Hakozaki

    Full Text Available BACKGROUND: Cyclooxygenase-2 (COX-2 is a key enzyme in the conversion of arachidonic acid to prostanoids, and its activation is associated with carcinogenesis as well as inflammation. The antitumor effect of selective COX-2 inhibitors has been noted in various malignancies. Malignant peripheral nerve sheath tumor (MPNST is a rare and aggressive soft tissue sarcoma for which effective treatments have not yet been established. The purpose of this study was to investigate a potential therapeutic role of COX-2 in MPNST. METHODS: We evaluated the expression of COX-2 in 44 cases of high-grade MPNST using immunohistochemical staining and compared the staining results with the characteristics and outcome of the patients. We also investigated the antitumor effect of etodolac, a selective COX-2 inhibitor, on MPNST cells in vitro using the MPNST cell line, FMS-1. RESULTS: Overexpression of COX-2 (≥50% positive cells was observed in 29 cases (65.9%, was significantly associated with a poor overall survival (P = 0.0495, and was considered an independent risk factor for a poor outcome by the results of both univariate and multivariate analysis. Etodolac induced apoptosis of FMS-1 cells through the activation of caspase-8, -9, and -3. Moreover, several caspase inhibitors significantly inhibited etodolac-induced apoptosis. CONCLUSIONS: Selective COX-2 inhibitors including etodolac had an antitumor effect on MPNST cells, and their use holds promise as a novel therapeutic strategy for patients with MPNST to improve their prognoses.

  11. Malignant peripheral nerve sheath tumor of adrenal gland with heterologus osseous differentiation in a case of Von Recklinghausen′s disease

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    Manas R Baisakh

    2014-01-01

    Full Text Available Malignant peripheral nerve sheath tumor (MPNST of the adrenal gland is extremely rare. Most of them occur in association with neurofibromatosis, ganglioneuroma or as part of a composite tumor such as pheochromocytoma. Only seven cases of MPNST of the adrenal gland have been reported in the literature till date. Discriminating this entity from other soft tissue sarcomas and gastrointestinal stromal tumor of the adrenal gland has important diagnostic and therapeutic implications. Moreover, the tumor size and pattern of expression for certain immunohistochemical markers may serve as independent predictors of aggressiveness. Herein we present a 24-years-old male with features of Von Recklinghausen′s disease who presented with large left adrenal gland malignant peripheral nerve sheath tumor.

  12. MicroRNA Gene Regulatory Networks in Peripheral Nerve Sheath Tumors

    Science.gov (United States)

    2012-09-01

    NUMBER (include area code) Standard Form 298 (Rev. 8-98) Prescribed by ANSI Std . Z39.18 MicroRNA Gene Regulatory Networks in Peripheral Nerve...Department of Surgery, University of Minnesota, 11-212 Moos Tower (Mail Code: MMC 195), 515 Delaware St, S.E, Minneapolis, MN 55455, USA e-mail...malignant bone tumor with an incidence of 4–5 cases per million. It arises from the metaphysis of the long bones of adolescents and young adults. Two

  13. Long-term progress of six cases of malignant peripheral nerve sheath tumors of the mediastinum that underwent surgical treatment: Case report series

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    Yuki Nakajima

    2016-01-01

    Discussion and conclusions: Nerve sheath tumors localized within the membrane offer good long-term prognosis even if malignant. Furthermore, long-term survival is possible even if the tumor has invaded neighboring organs, provided it can be completely excised. In cases where complete excision is difficult, a multidisciplinary approach including radiotherapy and anti-cancer drug treatment may contribute to improved prognosis but this is a subject that requires further study.

  14. Comprehensive establishment and characterization of orthoxenograft mouse models of malignant peripheral nerve sheath tumors for personalized medicine.

    Science.gov (United States)

    Castellsagué, Joan; Gel, Bernat; Fernández-Rodríguez, Juana; Llatjós, Roger; Blanco, Ignacio; Benavente, Yolanda; Pérez-Sidelnikova, Diana; García-Del Muro, Javier; Viñals, Joan Maria; Vidal, August; Valdés-Mas, Rafael; Terribas, Ernest; López-Doriga, Adriana; Pujana, Miguel Angel; Capellá, Gabriel; Puente, Xose S; Serra, Eduard; Villanueva, Alberto; Lázaro, Conxi

    2015-05-01

    Malignant peripheral nerve sheath tumors (MPNSTs) are soft-tissue sarcomas that can arise either sporadically or in association with neurofibromatosis type 1 (NF1). These aggressive malignancies confer poor survival, with no effective therapy available. We present the generation and characterization of five distinct MPNST orthoxenograft models for preclinical testing and personalized medicine. Four of the models are patient-derived tumor xenografts (PDTX), two independent MPNSTs from the same NF1 patient and two from different sporadic patients. The fifth model is an orthoxenograft derived from an NF1-related MPNST cell line. All MPNST orthoxenografts were generated by tumor implantation, or cell line injection, next to the sciatic nerve of nude mice, and were perpetuated by 7-10 mouse-to-mouse passages. The models reliably recapitulate the histopathological properties of their parental primary tumors. They also mimic distal dissemination properties in mice. Human stroma was rapidly lost after MPNST engraftment and replaced by murine stroma, which facilitated genomic tumor characterization. Compatible with an origin in a catastrophic event and subsequent genome stabilization, MPNST contained highly altered genomes that remained remarkably stable in orthoxenograft establishment and along passages. Mutational frequency and type of somatic point mutations were highly variable among the different MPNSTs modeled, but very consistent when comparing primary tumors with matched orthoxenografts generated. Unsupervised cluster analysis and principal component analysis (PCA) using an MPNST expression signature of ~1,000 genes grouped together all primary tumor-orthoxenograft pairs. Our work points to differences in the engraftment process of primary tumors compared with the engraftment of established cell lines. Following standardization and extensive characterization and validation, the orthoxenograft models were used for initial preclinical drug testing. Sorafenib (a BRAF

  15. Optical coherence tomography of the prostate nerves

    Science.gov (United States)

    Chitchian, Shahab

    Preservation of the cavernous nerves during prostate cancer surgery is critical in preserving a man's ability to have spontaneous erections following surgery. These microscopic nerves course along the surface of the prostate within a few millimeters of the prostate capsule, and they vary in size and location from one patient to another, making preservation of the nerves difficult during dissection and removal of a cancerous prostate gland. These observations may explain in part the wide variability in reported sexual potency rates (9--86%) following prostate cancer surgery. Any technology capable of providing improved identification, imaging, and visualization of the cavernous nerves during prostate cancer surgery would be of great assistance in improving sexual function after surgery, and result in direct patient benefit. Optical coherence tomography (OCT) is a noninvasive optical imaging technique capable of performing high-resolution cross-sectional in vivo and in situ imaging of microstructures in biological tissues. OCT imaging of the cavernous nerves in the rat and human prostate has recently been demonstrated. However, improvements in the OCT system and the quality of the images for identification of the cavernous nerves is necessary before clinical use. The following chapters describe complementary approaches to improving identification and imaging of the cavernous nerves during OCT of the prostate gland. After the introduction to OCT imaging of the prostate gland, the optimal wavelength for deep imaging of the prostate is studied in Chapter 2. An oblique-incidence single point measurement technique using a normal-detector scanning system was implemented to determine the absorption and reduced scattering coefficients, mua and m's , of fresh canine prostate tissue, ex vivo, from the diffuse reflectance profile of near-IR light as a function of source-detector distance. The effective attenuation coefficient, mueff, and the Optical Penetration Depth (OPD) were

  16. Multicentric fibromyxoid peripheral nerve sheath tumor (multicentric schwannoma) in a dromedary camel (Camelus dromedarius): morphopathological, immunohistochemical, and electron microscopic studies.

    Science.gov (United States)

    Khodakaram-Tafti, A; Khordadmehr, M

    2011-11-01

    During postslaughter inspection of a 4-year-old male dromedary camel (Camelus dromedarius), numerous small nodules to large masses up to 4 cm in diameter were found on the serosal surfaces of forestomachs, large intestines, mesentery, liver, and spleen. Grossly, the masses were discrete, round, smooth, and white to gray that bulged from the serosal layer. Cut surfaces of the masses were discrete, round, white, and relatively homogeneous without any necrotic foci. Histopathologically, the masses were encapsulated and composed of a mixture of round and spindle-shaped cells in loose whorls of neoplastic cells with small elongated hyperchromatic wavy nuclei and a small amount of pale eosinophilic, poorly defined cytoplasm. Masson's trichrome staining showed mild amounts of collagen fibers forming an irregular, loose stroma. In immunohistochemistry, immunoreactivity for the Schwann cell marker (S100) was diffusely positive in the neoplastic cells. The immunoreactivity for CK, c-kit, and CD34 were negative. Ultrastructural examination confirmed the tumor was entirely formed of neoplastic Schwann cells. On the basis of the histopathological, immunohistochemical, and ultrastructural findings, the tumors were diagnosed as multicentric fibromyxoid peripheral nerve sheath tumor (multicentric schwannoma). This tumor has not been previously recorded in camel worldwide.

  17. Combinatorial therapeutic targeting of BMP2 and MEK-ERK pathways in NF1-associated malignant peripheral nerve sheath tumors.

    Science.gov (United States)

    Ahsan, Sidra; Ge, Yubin; Tainsky, Michael A

    2016-08-30

    The clinical management of malignant peripheral nerve sheath tumors (MPNSTs) is challenging not only due to its aggressive and invasive nature, but also limited therapeutic options. Using gene expression profiling, our lab identified BMP2-SMAD1/5/8 pathway as a potential therapeutic target for treating MPNSTs. In this study, we explored the therapeutic impact of targeting BMP2-SMAD1/5/8 pathway in conjunction with RAS-MEK-ERK signaling, which is constitutively activated in MPNSTs. Our results indicated that single agent treatment with LDN-193189, a BMP2 Type I receptor inhibitor, did not affect the growth and survival of MPNST cells at biochemically relevant inhibitory concentrations. However, addition of a MEK1/2 inhibitor, selumetinib, to LDN-193189-treated cells resulted in significant inhibition of cell growth and induction of cell death. LDN-193189 at biochemically effective concentrations significantly inhibited motility and invasiveness of MPNST cells, and these effects were enhanced by the addition of selumetinib. Overall, our results advocate for a combinatorial therapeutic approach for MPNSTs that not only targets the growth and survival via inhibition of MEK1/2, but also its malignant spread by suppressing the activation of BMP2-SMAD1/5/8 pathway. Importantly, these studies were conducted in low-passage patient-derived MPNST cells, allowing for an investigation of the effects of the proposed drug treatments in a biologically-relevant context.

  18. Photothermal therapy improves the efficacy of a MEK inhibitor in neurofibromatosis type 1-associated malignant peripheral nerve sheath tumors

    Science.gov (United States)

    Sweeney, Elizabeth E.; Burga, Rachel A.; Li, Chaoyang; Zhu, Yuan; Fernandes, Rohan

    2016-11-01

    Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive tumors with low survival rates and the leading cause of death in neurofibromatosis type 1 (NF1) patients under 40 years old. Surgical resection is the standard of care for MPNSTs, but is often incomplete and can generate loss of function, necessitating the development of novel treatment methods for this patient population. Here, we describe a novel combination therapy comprising MEK inhibition and nanoparticle-based photothermal therapy (PTT) for MPNSTs. MEK inhibitors block activity driven by Ras, an oncogene constitutively activated in NF1-associated MPNSTs, while PTT serves as a minimally invasive method to ablate cancer cells. Our rationale for combining these seemingly disparate techniques for MPNSTs is based on several reports demonstrating the efficacy of systemic chemotherapy with local PTT. We combine the MEK inhibitor, PD-0325901 (PD901), with Prussian blue nanoparticles (PBNPs) as PTT agents, to block MEK activity and simultaneously ablate MPNSTs. Our data demonstrate the synergistic effect of combining PD901 with PBNP-based PTT, which converge through the Ras pathway to generate apoptosis, necrosis, and decreased proliferation, thereby mitigating tumor growth and increasing survival of MPNST-bearing animals. Our results suggest the potential of this novel local-systemic combination “nanochemotherapy” for treating patients with MPNSTs.

  19. Preclinical evaluation of the combination of mTOR and proteasome inhibitors with radiotherapy in malignant peripheral nerve sheath tumors.

    Science.gov (United States)

    Yamashita, A S; Baia, G S; Ho, J S Y; Velarde, E; Wong, J; Gallia, G L; Belzberg, A J; Kimura, E T; Riggins, G J

    2014-05-01

    About one half of malignant peripheral nerve sheath tumors (MPNST) have Neurofibromin 1 (NF1) mutations. NF1 is a tumor suppressor gene essential for negative regulation of RAS signaling. Survival for MPNST patients is poor and we sought to identify an effective combination therapy. Starting with the mTOR inhibitors rapamycin and everolimus, we screened for synergy in 542 FDA approved compounds using MPNST cells with a native NF1 loss in both alleles. We further analyzed the cell cycle and signal transduction. In vivo growth effects of the drug combination with local radiation therapy (RT) were assessed in MPNST xenografts. The synergistic combination of mTOR inhibitors with bortezomib yielded a reduction in MPNST cell proliferation. The combination of mTOR inhibitors and bortezomib also enhanced the anti-proliferative effect of radiation in vitro. In vivo, the combination of mTOR inhibitor (everolimus) and bortezomib with RT decreased tumor growth and proliferation, and augmented apoptosis. The combination of approved mTOR and proteasome inhibitors with radiation showed a significant reduction of tumor growth in an animal model and should be investigated and optimized further for MPNST therapy.

  20. KANK1 inhibits cell growth by inducing apoptosis though regulating CXXC5 in human malignant peripheral nerve sheath tumors

    Science.gov (United States)

    Cui, Zhibin; Shen, Yingjia; Chen, Kenny H.; Mittal, Suresh K.; Yang, Jer-Yen; Zhang, GuangJun

    2017-01-01

    Malignant peripheral nerve sheath tumors (MPNSTs) are a type of rare sarcomas with a poor prognosis due to its highly invasive nature and limited treatment options. Currently there is no targeted-cancer therapy for this type of malignancy. Thus, it is important to identify more cancer driver genes that may serve as targets of cancer therapy. Through comparative oncogenomics, we have found that KANK1 was a candidate tumor suppressor gene (TSG) for human MPNSTs. Although KANK1 is known as a cytoskeleton regulator, its tumorigenic function in MPNSTs remains largely unknown. In this study, we report that restoration of KANK1 in human MPNST cells inhibits cell growth both in human cell culture and xenograft mice by increasing apoptosis. Consistently, knockdown of KANK1 in neurofibroma cells promoted cell growth. Using RNA-seq analysis, we identified CXXC5 and other apoptosis-related genes, and demonstrated that CXXC5 is regulated by KANK1. Knockdown of CXXC5 was found to diminish KANK1-induced apoptosis in MPNST cells. Thus, KANK1 inhibits MPNST cell growth though CXXC5 mediated apoptosis. Our results suggest that KANK1 may function as a tumor suppressor in human MPNSTs, and thus it may be useful for targeted therapy. PMID:28067315

  1. Radiation Therapy in Management of Sporadic and Neurofibromatosis Type 1 (NF1 Associated Malignant Peripheral Nerve Sheath Tumors (MPNST

    Directory of Open Access Journals (Sweden)

    Jenna eKahn

    2014-11-01

    Full Text Available Introduction: Malignant peripheral nerve sheath tumors (MPNST are highly aggressive soft tissue sarcomas in which complete surgical resection is the mainstay of therapy. However, the recurrence rate is high and few options remain for refractory or metastatic MPNST. This study examines the outcomes of adjuvant radiation therapy in MPNST in patients with and without neurofibromatosis-1 (NF1 and reviews the literature on use of radiation for MPNST. Methods: A retrospective review of 33 MPNST patients between 1990 and 2012 evaluated at the NIH. All diagnoses were pathologically confirmed at the NCI. Clinical presentation, treatment, and survival were analyzed. Results: Thirty-three patients were included, 18 NF1-associated, 15 sporadic tumors. Tumor location included extremity (58%, trunk (36%, and head/neck (6%. Histologic grade showed 25 high grade tumors compared to 7 low grade tumors. Twenty patients were treated with radiation therapy, (median total dose of 58.5 Gy with 1.8 Gy/fraction. A median survival of all patients was 46.5 months and 43.7% overall 5 year survival. Prognostic factors include extent of resection, tumor location, and histology grade. Radiation was not found to be a prognostic factor for overall survival. Conclusions: This study is consistent with previous studies regarding the role of radiation in the management of MPNST. Prospective evaluation of adjuvant radiation will allow to more fully define the role of radiation in MPNST.

  2. Pediatric and adult malignant peripheral nerve sheath tumors: an analysis of data from the surveillance, epidemiology, and end results program.

    Science.gov (United States)

    Amirian, E Susan; Goodman, J Clay; New, Pamela; Scheurer, Michael E

    2014-02-01

    Malignant peripheral nerve sheath tumors (MPNSTs) are rare soft tissue sarcomas that arise predominantly from Schwann cells. Despite the fact that MPNSTs have high local recurrence rates and are generally associated with poor prognosis, little is known about prognostic factors or effective clinical management for this tumor type. The purpose of this study was to describe the distributions of patient and tumor characteristics and to identify predictors of cause-specific survival among MPNST cases reported to SEER between 1973 and 2008. Patient and tumor characteristics were compared between pediatric and adult MPNST cases. Cox regression and tree-based survival analysis were used to examine factors associated with MPNST-related mortality separately among adults and children. A total of 1,315 MPNST cases were isolated from the 1973-2008 SEER dataset. Among pediatric cases, sex, race, and radiation therapy predicted MPNST survival, whereas among adults, tumor site, tumor grade, number of primary tumors, and tumor size were significant predictors. As tumor size at diagnosis/resection may be the only somewhat "modifiable" prognostic factor, future studies should aim to identify biological and social attributes associated with tumor size at diagnosis, separately among individuals with and without NF-1, in order to help identify earlier opportunities for clinical intervention.

  3. Combinatorial therapy with tamoxifen and trifluoperazine effectively inhibits malignant peripheral nerve sheath tumor growth by targeting complementary signaling cascades.

    Science.gov (United States)

    Brosius, Stephanie N; Turk, Amy N; Byer, Stephanie J; Longo, Jody Fromm; Kappes, John C; Roth, Kevin A; Carroll, Steven L

    2014-11-01

    Chemotherapeutic agents effective against malignant peripheral nerve sheath tumors (MPNSTs) are urgently needed. We recently found that tamoxifen potently impedes xenograft growth. In vitro, tamoxifen inhibits MPNST proliferation and survival in an estrogen receptor-independent manner; these effects are phenocopied by the calmodulin inhibitor trifluoperazine. The present study was performed to establish the mechanism of action of tamoxifen in vivo and optimize its therapeutic effectiveness. To determine if tamoxifen has estrogen receptor-dependent effects in vivo, we grafted MPNST cells in castrated and ovariectomized mice; xenograft growth was unaffected by reductions in sex hormones. To establish whether tamoxifen and trifluoperazine additively or synergistically impede MPNST growth, mice xenografted with neurofibromatosis type 1-associated or sporadic MPNST cells were treated with tamoxifen, trifluoperazine, or both drugs for 30 days. Both monotherapies inhibited graft growth by 50%, whereas combinatorial treatment maximally reduced graft mass by 90% and enhanced decreases in proliferation and survival. Kinomic analyses showed that tamoxifen and trifluoperazine have both shared and distinct targets in MPNSTs. In addition, trifluoperazine prevented tamoxifen-induced increases in serum/glucocorticoid regulated kinase 1, a protein linked to tamoxifen resistance. These findings suggest that combinatorial therapy with tamoxifen and trifluoperazine is effective against MPNSTs because these agents target complementary pathways that are essential for MPNST pathogenesis.

  4. Combinatorial therapeutic targeting of BMP2 and MEK-ERK pathways in NF1-associated malignant peripheral nerve sheath tumors

    Science.gov (United States)

    Ahsan, Sidra; Ge, Yubin; Tainsky, Michael A.

    2016-01-01

    The clinical management of malignant peripheral nerve sheath tumors (MPNSTs) is challenging not only due to its aggressive and invasive nature, but also limited therapeutic options. Using gene expression profiling, our lab identified BMP2-SMAD1/5/8 pathway as a potential therapeutic target for treating MPNSTs. In this study, we explored the therapeutic impact of targeting BMP2-SMAD1/5/8 pathway in conjunction with RAS-MEK-ERK signaling, which is constitutively activated in MPNSTs. Our results indicated that single agent treatment with LDN-193189, a BMP2 Type I receptor inhibitor, did not affect the growth and survival of MPNST cells at biochemically relevant inhibitory concentrations. However, addition of a MEK1/2 inhibitor, selumetinib, to LDN-193189-treated cells resulted in significant inhibition of cell growth and induction of cell death. LDN-193189 at biochemically effective concentrations significantly inhibited motility and invasiveness of MPNST cells, and these effects were enhanced by the addition of selumetinib. Overall, our results advocate for a combinatorial therapeutic approach for MPNSTs that not only targets the growth and survival via inhibition of MEK1/2, but also its malignant spread by suppressing the activation of BMP2-SMAD1/5/8 pathway. Importantly, these studies were conducted in low-passage patient-derived MPNST cells, allowing for an investigation of the effects of the proposed drug treatments in a biologically-relevant context. PMID:27494873

  5. A Case Report of a Malignant Peripheral Nerve Sheath Tumor of the Oral Cavity in Neurofibromatosis Type 1

    Directory of Open Access Journals (Sweden)

    Özmen Öztürk

    2012-01-01

    Full Text Available Patients with neurofibromatosis type 1 develop both benign and malignant tumors at an increased frequency. Most of the malignant peripheral nerve sheath tumors (MPNSTs are considered as high-grade sarcomas originating from tissues of mesenchymal origin. It is generally accepted that MPNSTs occur in about 2% to 5% of neurofibromatosis patients. In this paper, we present a 16-year-old male patient with neurofibromatosis who developed MPNST of the retromolar area. The mass enlarged rapidly in a period of 6 weeks. The patient was treated surgically, and a tumor mass with a diameter of  cm was excised, but after 8 months a recurrence was observed at the same site. The sarcomatous change in a neurofibroma has an extremely poor prognosis, so patients with neurofibromatosis should be closely monitored for a possible malignancy. A rapid change in size of a preexisting neurofibroma, infiltration of the adjacent structures, intralesional hemorrhage, and pain indicate a possible malignant transformation to MPNST.

  6. Malignant peripheral nerve sheath tumor (MPNST) in the spine: a retrospective analysis of clinical and molecular prognostic factors.

    Science.gov (United States)

    Wang, Ting; Yin, Huabin; Han, Shuai; Yang, Xinhai; Wang, Jing; Huang, Quan; Yan, Wangjun; Zhou, Wang; Xiao, Jianru

    2015-04-01

    Spinal malignant peripheral nerve sheath tumors (MPNSTs) are relatively rare. There is little information published in the literature regarding this subject. The aim of this retrospective study was to evaluate factors that may affect the outcomes of patients with spinal MPNSTs by reviewing 43 patients with spinal MPNST who were treated in our hospital between 2001 and 2012. Univariate and multivariate analyses were performed to identify prognostic variables relative to patient and tumor characteristics, treatment modality and molecules. All 43 MPNST patients (25 men and 18 women; median age 49 years) underwent surgical resection, of whom 15 patients also underwent postoperative radiotherapy. Local recurrence was found in 21 (48.8 %) patients. Twenty-two (51.2 %) patients died during the follow-up periods with a median survival time of 49 months. The 5-year recurrence and survival rate was 53 and 44 % respectively. The statistical analyses suggested that high-grade malignancy and osteolytic destruction were closely associated with recurrence and death. A total of 38 cases accepted postoperative immunohistochemisty examine. S-100 was identified as an independent factor related to both recurrence and survival, adjusting for clinical factors. In conclusion, we confirmed that malignant grade and osteolytic destruction were the two independent factors for both recurrence and survival, while patients with S-100 protein negative had a higher recurrence rate and a lower survival rate.

  7. [Case of abdominal wall malignant peripheral nerve sheath tumor which is difficult to distinguish from a urachal disease].

    Science.gov (United States)

    Tatenuma, Tomoyuki; Sakata, Ryoko; Sugiura, Shinpei; Tajiri, Takehiro; Gondo, Toshikazu; Kitami, Kazuo

    2013-09-01

    Malignant peripheral nerve sheath tumors (MPNST) are highly malignant soft tissue sarcomas. It is very rare for MPNST to arise in the abdominal wall. We report a case of abdominal wall MPNST that was difficult to distinguish from a urachal disease. A 72-year-old woman found a mass of the umbilicus in October 2011. She visited a digestive surgery department in November because it gradually enlarged. Diagnostic imaging suggested a urachal tumor. She was then referred to our clinic. Contrast enhanced CT showed that the 5-cm cystic tumor extended from the umbilicus to abdominal wall. The tumor showed low uptake value in PET-CT. We diagnosed her with a urachal cyst, but could not deny urachal carcinoma. Therefore, we performed surgical resection in January 2012. The pathological diagnosis was MPNST. She has not experienced recurrence for 9 months. MPNST mostly occur in the retroperitoneum close to the spine, extremities, head, and neck. It is very rare for them to occur in the abdominal wall. This is the sixth case including overseas reports. In addition, this is the first case in which it was difficult to distinguish from a urachal disease.

  8. Involvement of PACAP/ADNP signaling in the resistance to cell death in malignant peripheral nerve sheath tumor (MPNST) cells.

    Science.gov (United States)

    Castorina, Alessandro; Giunta, Salvatore; Scuderi, Soraya; D'Agata, Velia

    2012-11-01

    Malignant peripheral nerve sheath tumors (MPNSTs) are sarcomas able to grow under conditions of metabolic stress caused by insufficient nutrients or oxygen. Both pituitary adenylate cyclase-activating polypeptide (PACAP) and activity-dependent neuroprotective protein (ADNP) have glioprotective potential. However, whether PACAP/ADNP signaling is involved in the resistance to cell death in MPNST cells remains to be clarified. Here, we investigated the involvement of this signaling system in the survival response of MPNST cells against hydrogen peroxide (H(2)O(2))-evoked death both in the presence of normal serum (NS) and in serum-starved (SS) cells. Results showed that ADNP levels increased time-dependently (6-48 h) in SS cells. Treatment with PACAP38 (10(-9) to 10(-5) M) dose-dependently increased ADNP levels in NS but not in SS cells. PAC(1)/VPAC receptor antagonists completely suppressed PACAP-stimulated ADNP increase and partially reduced ADNP expression in SS cells. NS-cultured cells exposed to H(2)O(2) showed significantly reduced cell viability (~50 %), increased p53 and caspase-3, and DNA fragmentation, without affecting ADNP expression. Serum starvation significantly reduced H(2)O(2)-induced detrimental effects in MPNST cells, which were not further ameliorated by PACAP38. Altogether, these finding provide evidence for the involvement of an endogenous PACAP-mediated ADNP signaling system that increases MPNST cell resistance to H(2)O(2)-induced death upon serum starvation.

  9. Primary Intraosseous Malignant Peripheral Nerve Sheath Tumor of the Medial Cuneiform: A Case Report and Review of the Literature.

    Science.gov (United States)

    Muthusamy, Saravanaraja; Conway, Sheila A; Pitcher, J David; Temple, H Thomas

    Peripheral nerve sheath tumors (benign and malignant) usually arise in the soft tissues and are unusual in bone. Intraosseous peripheral nerve sheath tumors are usually benign and constitute approximately 0.2% of all bone tumors. Intraosseous malignant peripheral nerve sheath tumors (MPNSTs) are uncommon and usually result from secondary invasion. Only a few cases of primary intraosseous MPNSTs have been reported in published studies, and these were localized mostly in the mandible (approximately 50%) or maxilla, spine, and, occasionally, in the appendicular skeleton. To the best of our knowledge, we report the first case of primary intraosseous MPNST involving a midtarsal bone (medial cuneiform). The patient was a 62-year-old female who presented with pain and tenderness but without swelling. Imaging revealed nonspecific findings, and the preoperative computed tomography-guided biopsy findings were consistent with MPNST. The patient was treated with neoadjuvant radiotherapy, followed by wide local excision and allograft reconstruction. At the final follow-up examination (24 months), the graft had been incorporated without evidence of local recurrence or distant disease. The patient with primary intraosseous MPNST of the medial cuneiform described in the present report presented with nonspecific clinical and radiologic findings. Thus, a high index of suspicion and histopathologic examination, including immunohistochemistry, are necessary for an accurate diagnosis.

  10. Unilateral Optic Nerve Hypoplasia with Contralateral Optic Pathway Hypoplasia: A Case Report.

    Science.gov (United States)

    Nishi, Tomo; Yukawa, Eiichi; Taoka, Toshiaki; Ogata, Nahoko

    2013-01-01

    Optic nerve hypoplasia is diagnosed by the ophthalmoscopic appearance of the fundus of the eye and by standard magnetic resonance imaging of the brain. The ability to study eyes with optic nerve hypoplasia by magnetic resonance diffusion tensor imaging has improved the evaluation of the optic pathways. The authors report a case of unilateral optic nerve hypoplasia with hypoplasia of the contralateral optic pathway. The entire visual pathway of this patient was examined by magnetic resonance and magnetic resonance diffusion tensor imaging. The images show a decrease of the volume of the optic radiation contralateral to the optic nerve abnormality and also pre- and post-chiasmal abnormalities.

  11. MRI of optic nerve lesion in multiple sclerosis

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    Fujii, Koichi; Uehara, Masako; Ashikaga, Ryuuichirou; Inoue, Masaaki; Yamamoto, Takashi; Hamada, Tatsumi; Ishida, Osamu; Nakao, Yuzou; Miyakoshi, Keizou (Kinki Univ., Osakasayama, Osaka (Japan). School of Medicine)

    1992-12-01

    We evaluated the ability of MRI using short TI inversion recovery (STIR) to detect optic nerve lesions in multiple sclerosis (MS). Eleven patients with MS were studied with MRI at 0.5 T. STIR images revealed high signal lesions in all of 14 nerves in 11 patients with previous and recurrent attack of optic neuritis. In addition, two of seven asymptomatic nerves also showed high intensity on STIR images. The high signal lesions on STIR images seemed to reflect demyelination in the nerves with some attacks and to show occult lesions in the nerves without any attack. STIR method was concluded to be a potentially useful procedure in detection of optic nerve lesions in MS. (author).

  12. [Diagnostic difficulties in the laryngeal malignant peripheral nerve sheath tumor (MPNST)].

    Science.gov (United States)

    Pabiszczak, Maciej; Woźniak, Aldona; Wierzbicka, Małgorzata; Leszczyńska, Małgorzata; Szyfter, Witold

    2004-01-01

    The malignant tumor deriving from the peripheral nerve sheet, previously described as malignant Schwannoma or neurosarcoma is extremely rare as malignancy localized in the larynx. The half of cases has been developing on the basis of neurofibromatosis in von Recklinghausen disease type I or seldom, type II. The high grade of malignancy end tendency to reccurences and distant metastases is typical for this tumors. The case of 64 year old man with larynx neurosarcoma was presented. The diagnostic difficulties were caused by clinical presentation of the smooth tumor covered by unchanged mucosa and typical histological features of the tumor. The final histological assessment was complemented by positive immunohistochemical reaction (antigens against protein S-100, NSE and PG 9.5).

  13. Magnetic resonance imaging in optic nerve lesions with multiple sclerosis

    Energy Technology Data Exchange (ETDEWEB)

    Kojima, Shigeyuki; Hirayama, Keizo; Kakisu, Yonetsugu; Adachi, Emiko (Chiba Univ. (Japan). School of Medicine)

    1990-12-01

    Magnetic resonance imaging (MRI) of the optic nerve was performed in 10 patients with multiple sclerosis (MS) using short inversion time inversion recovery (STIR) pulse sequences, and the results were compared with the visual evoked potentials (VEP). The 10 patients had optic neuritis in the chronic or remitting phase together with additional symptoms or signs allowing a diagnosis of clinically definite or probable MS. Sixteen optic nerves were clinically affected and 4 were unaffected. MRI was performed using a 0.5 tesla supeconducting unit, and multiple continuous 5 mm coronal and axial STIR images were obtained. A lesion was judged to be present if a focal or diffuse area of increased signal intensity was detectd in the optic nerve. In VEP, a delay in peak latency or no P 100 component was judged to be abnormal. With regard to the clinically affected optic nerves, MRI revealed a region of increased signal intensity in 14/16 (88%) and the VEP was abnormal in 16/16 (100%). In the clinically unaffected optic nerves, MRI revealed an increased signal intensity in 2/4 (50%). One of these nerves had an abnormal VEP and the other had a VEP latency at the upper limit of normal. The VEP was abnormal in 1/4 (25%). In the clinically affected optic nerves, the degree of loss of visual acuity was not associated with the longitudinal extent of the lesions shown by MRI. The mean length was 17.5 mm in optic nerves with a slight disturbance of visual acuity and 15.0 mm in nerves with severe visual loss. MRI using STIR pulse sequences was found to be almost as sensitive as VEP in detecting both clinically affected and unaffected optic nerve lesions in patients with MS, and was useful in visualizing the location or size of the lesions. (author).

  14. Endoscopic optic nerve decompression for nontraumatic compressive optic neuropathy

    Directory of Open Access Journals (Sweden)

    Cheng-long REN

    2015-11-01

    Full Text Available Objective To describe the preliminary experience with endoscopic optic nerve decompression (EOND for nontraumatic compressive optic neuropathies (NCONs. Methods The clinical data of 10 patients, male 5 and female 5, with a mean age of 44.3±5.1 years, who underwent EOND for visual loss (n=5 or visual deterioration (n=5 due to tumor compression in General Hospital of Armed Police Forces of China in the period from April 2013 to April 2014 were analyzed retrospectively. Preoperative and 6-month-postoperative clinical and imaging data of these patients were reviewed and analyzed. Results Among 5 patients who lost light perception (including 2 patients with bilateral optic nerve compression before operation, 4 of them showed visual improvement to different degrees on the 7th day after operation (with improvement of bilateral visual acuity. The other 5 patients with visual impairment before operation recovered their visual acuity to different extent after the operation. All of the patients had no obvious post-operative complications. Conclusion EOND is a safe, effective, and minimally invasive surgical technique affording recovery of visual function to NCON patients. DOI: 10.11855/j.issn.0577-7402.2015.11.12

  15. Myocilin is involved in NgR1/Lingo-1-mediated oligodendrocyte differentiation and myelination of the optic nerve.

    Science.gov (United States)

    Kwon, Heung Sun; Nakaya, Naoki; Abu-Asab, Mones; Kim, Hong Sug; Tomarev, Stanislav I

    2014-04-16

    Myocilin is a secreted glycoprotein that belongs to a family of olfactomedin domain-containing proteins. Although myocilin is detected in several ocular and nonocular tissues, the only reported human pathology related to mutations in the MYOCILIN gene is primary open-angle glaucoma. Functions of myocilin are poorly understood. Here we demonstrate that myocilin is a mediator of oligodendrocyte differentiation and is involved in the myelination of the optic nerve in mice. Myocilin is expressed and secreted by optic nerve astrocytes. Differentiation of optic nerve oligodendrocytes is delayed in Myocilin-null mice. Optic nerves of Myocilin-null mice contain reduced levels of several myelin-associated proteins including myelin basic protein, myelin proteolipid protein, and 2'3'-cyclic nucleotide 3'-phosphodiesterase compared with those of wild-type littermates. This leads to reduced myelin sheath thickness of optic nerve axons in Myocilin-null mice compared with wild-type littermates, and this difference is more pronounced at early postnatal stages compared with adult mice. Myocilin also affects differentiation of oligodendrocyte precursors in vitro. Its addition to primary cultures of differentiating oligodendrocyte precursors increases levels of tested markers of oligodendrocyte differentiation and stimulates elongation of oligodendrocyte processes. Myocilin stimulation of oligodendrocyte differentiation occurs through the NgR1/Lingo-1 receptor complex. Myocilin physically interacts with Lingo-1 and may be considered as a Lingo-1 ligand. Myocilin-induced elongation of oligodendrocyte processes may be mediated by activation of FYN and suppression of RhoA GTPase.

  16. Intrinsic determinants of optic nerve regeneration

    Institute of Scientific and Technical Information of China (English)

    ZHU Rui-lin; CHO Kin-Sang; GUO Chen-ying; CHEW Justin; CHEN Dong-feng; YANG Liu

    2013-01-01

    Objective To review the functions of these intracellular signals in their regulation of retinal ganglion cell (RGC) axon regeneration.Data sources Relevant articles published in English or Chinese from 1970 to present were selected from PubMed.Searches were made using the terms "intrinsic determinants,axon regeneration,RGC,optic nerve regeneration,and central nervous system axon regeneration."Study selection Articles studying the mechanisms controlling RGC and central nervous system (CNS) axon regeneration were reviewed.Articles focusing on the intrinsic determinants of axon regeneration were selected.Results Like other CNS neurons of mammals,RGCs undergo a developmental loss in their ability to grow axons as they mature,which is a critical contributing factor to the failure of nerve regeneration and repair after injury.This growth failure can be attributed,at least in part,by the induction of molecular programs preventing cellular overgrowth and termination of axonal growth upon maturation.Key intracellular signals and transcription factors,including B cell lymphoma/leukemia 2,cyclic adenine monophosphate,mammalian target of rapamycin,and Krüppel-like transcription factors,have been identified to play central roles in this process.Conclusions Intense effort and substantial progress have been made to identify the various intrinsic growth pathways that regulate RGC axon regeneration.More work is needed to elucidate the mechanisms of and the interrelationship between the actions of these factors and to successfully achieve regeneration and repair of the severed RGC axons.

  17. Optical Biopsy of Peripheral Nerve Using Confocal Laser Endomicroscopy: A New Tool for Nerve Surgeons?

    Directory of Open Access Journals (Sweden)

    Christopher S Crowe

    2015-09-01

    Full Text Available Peripheral nerve injuries remain a challenge for reconstructive surgeons with many patients obtaining suboptimal results. Understanding the level of injury is imperative for successful repair. Current methods for distinguishing healthy from damaged nerve are time consuming and possess limited efficacy. Confocal laser endomicroscopy (CLE is an emerging optical biopsy technology that enables dynamic, high resolution, sub-surface imaging of live tissue. Porcine sciatic nerve was either left undamaged or briefly clamped to simulate injury. Diluted fluorescein was applied topically to the nerve. CLE imaging was performed by direct contact of the probe with nerve tissue. Images representative of both damaged and undamaged nerve fibers were collected and compared to routine H&E histology. Optical biopsy of undamaged nerve revealed bands of longitudinal nerve fibers, distinct from surrounding adipose and connective tissue. When damaged, these bands appear truncated and terminate in blebs of opacity. H&E staining revealed similar features in damaged nerve fibers. These results prompt development of a protocol for imaging peripheral nerves intraoperatively. To this end, improving surgeons' ability to understand the level of injury through real-time imaging will allow for faster and more informed operative decisions than the current standard permits.

  18. Evaluation of the Optic Nerve Head in Glaucoma.

    Science.gov (United States)

    Gandhi, Monica; Dubey, Suneeta

    2013-01-01

    Glaucoma is an optic neuropathy leading to changes in the intrapaillary and parapaillary regions of the optic disk. Despite technological advances, clinical identification of optic nerve head characteristics remains the first step in diagnosis. Careful examination of the disk parameters including size, shape, neuroretinal rim shape and pallor; size of the optic cup in relation to the area of the disk; configuration and depth of the optic cup; ratios of cup-to-disk diameter and cup-to-disk area; presence and location of splinter-shaped hemorrhages; occurrence, size, configuration, and location of parapapillary chorioretinal atrophy; and visibility of the retinal nerve fiber layer (RNFL) is important to differentiate between the glaucomatous and nonglaucomatous optic neuropathy. How to cite this article: Gandhi M, Dubey S. Evaluation of the Optic Nerve Head in Glaucoma. J Current Glau Prac 2013;7(3):106-114.

  19. Malignant peripheral nerve sheath tumors (MPNST – Clinicopathological study and treatment outcome of twenty-four cases

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    DattaGupta Sidharth

    2006-08-01

    Full Text Available Abstract Background Malignant peripheral nerve sheath tumor (MPNST is biologically an aggressive tumor for which the treatment of choice is the surgery. We reviewed the clinical profile, diagnostic methods, treatment patterns, and outcome of twenty-four MPNST patients in this study. Patients and methods A retrospective analysis of 24 MPNST patients, treated from 1994 to 2002, in the department of Surgical Oncology at All India Institute of Medical Sciences, New Delhi, was done. A combination of gross, histopathological and immunohistochemical findings, and proliferation markers (MIB1 were considered for diagnosis and grade of the MPNST. Survival analysis was done by the Kaplan-Meier method and differences were evaluated with the log-rank test. Multivariate analysis was carried out by using Cox's proportional hazards model by using SPSS (Version 9, Chicago, Illinois software. Results MPNST constituted 12% of all soft tissue sarcomas, where 21% (5/24 of patients had associated Von Recklinghausen's disease (VRHD. A higher incidence of male preponderance and multifocal MPNST were noted in the present series. At a mean follow-up of 38 months, 13 (54 % patients had relapse of disease and 5-year over all and disease free survival were 58% and 35% respectively. In univariate analysis, sex (p = 0.05, tumor depth (p p p = 0.04, cellular differentiation (p p = 0.05 for overall survival. However, in multivariate analysis, cellular differentiation (p p Conclusion MPNSTs constituted a significant proportion (12% of soft tissue sarcoma in our medical center. Heterogeneous differentiation and multifocality of the tumor were few distinct features of MPNST. Sex and cellular differentiation were noticed as the new adverse prognostic factors and adjuvant radiotherapy has been proved to be a significant treatment tool in the current series.

  20. Malignant peripheral nerve sheath tumor (MPNST) arising in diffuse-type neurofibroma: clinicopathologic characterization in a series of 9 cases.

    Science.gov (United States)

    Schaefer, Inga-Marie; Fletcher, Christopher D M

    2015-09-01

    Diffuse-type neurofibroma, an uncommon variant of neurofibroma, is associated with neurofibromatosis type 1 in ∼60% of cases. Typically presenting in young adults as ill-defined plaque-like dermal/subcutaneous thickening, most cases are located on the trunk or the head and neck region. Malignant transformation is extremely rare. Nine cases of malignant peripheral nerve sheath tumor (MPNST) arising in diffuse-type neurofibroma (identified in consult files) are described, including clinicopathologic features and follow-up. Five patients were male and 4 female, aged 31 to 59 years (median 49 y). All diffuse-type neurofibromas contained Meissner corpuscles, with tumor sizes ranging between 3.6 and 45 cm (median, 7.4 cm). Five patients had a clinical history of neurofibromatosis type 1, and 1 had Klippel-Trénaunay-Weber syndrome. Six tumors arose on the trunk and 1 each on the leg, arm, and scalp. Increased cellularity, nuclear atypia, and mitoses (range, 1 to 63/50 high-power fields) indicated transition to MPNST, classified as low grade in 5, intermediate to high grade in 1, and high grade in 3 cases, 1 of which exhibited heterologous angiosarcomatous differentiation. S-100 expression was quite strong and diffuse in the neurofibroma components and less extensive or weaker in MPNST. Follow-up, available for all patients (median, 80.5 mo, except 1 recent case), revealed that 1 patient developed local recurrence after 9 months; 1 with metastases at the time of initial diagnosis died 1 month after tumor resection. All other patients were alive without evidence of disease at 15 to 145 months (median, 83 mo). Diffuse-type neurofibroma may show transformation to MPNST in very rare instances. It is important to be aware of possible malignant change, requiring thorough sampling of resection specimens and long-term clinical follow-up of patients with unexcised lesions.

  1. The 4q12 amplicon in malignant peripheral nerve sheath tumors: consequences on gene expression and implications for sunitinib treatment.

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    Jan Zietsch

    Full Text Available BACKGROUND: Malignant peripheral nerve sheath tumors (MPNST are highly aggressive tumors which originate from Schwann cells and develop in about 10% of neurofibromatosis type 1 (NF1 patients. The five year survival rate is poor and more effective therapies are needed. Sunitinib is a drug targeting receptor tyrosine kinases (RTK like PDGFRalpha, c-Kit and VEGFR-2. These genes are structurally related and cluster on chromosomal segment 4q12. METHODOLOGY/PRINCIPAL FINDINGS: Here we characterize this region by multiplex ligation-dependent probe amplification (MLPA in MPNST. Our probe set encompasses the 3 adjacent RTK genes (PDGFRA, KIT, KDR and 6 flanking genes. We found amplification of several genes within this region in a subset of MPNST and MPNST cell lines. Transcript and protein expression of PDGFRA matched well with its increased copy number suggesting a central role of PDGFRA within the amplicon. Studying the effect of sunitinib on 5 MPNST cell lines revealed that cell line S462 harboring the 4q12 amplicon was extremely sensitive to the drug with an IC50 below 1.0 microM. Moreover, sunitinib induced apoptosis and prevented PDGF-AA induced signaling via PDGFRalpha as determined by western blotting. Co-expression of VEGF and its receptor VEGFR-2 (KDR was present in MPNST cell lines suggesting an autocrine loop. We show that VEGF triggered signal transduction via the MAPK pathway, which could be blocked by sunitinib. CONCLUSIONS/SIGNIFICANCE: Since multiple receptors targeted by sunitinib are expressed or over-expressed by MPNST cells sunitinib appears as an attractive drug for treatment of MPNST patients. Presence of the 4q12 amplicon and subsequent over-expression of PDGFRA might serve as predictive markers for efficacy of sunitinib.

  2. A GFP-Tagged Gross Deletion on Chromosome 1 Causes Malignant Peripheral Nerve Sheath Tumors and Carcinomas in Zebrafish.

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    Matteo Astone

    Full Text Available Malignant peripheral nerve sheath tumors (MPNSTs are highly aggressive soft-tissue sarcomas, characterized by complex karyotypes. The molecular bases of such malignancy are poorly understood and efficient targeted molecular therapies are currently lacking. Here we describe a novel zebrafish model of MPNSTs, represented by the transgenic mutant line Tg(-8.5nkx2.2a:GFPia2. ia2 homozygous animals displayed embryonic lethality by 72 hpf, while the heterozygotes develop visible tumor masses with high frequency in adulthood. Histological and immunohistochemical examination revealed aggressive tumors with either mesenchymal or epithelial features. The former (54% of the cases arose either in the abdominal cavity, or as intrathecal/intraspinal lesions and is composed of cytokeratin-negative spindle cells with fascicular/storiform growth pattern consistent with zebrafish MPNSTs. The second histotype was composed by polygonal or elongated cells, immunohistochemically positive for the pan-cytokeratin AE1/AE3. The overall histologic and immunohistochemical features were consistent with a malignant epithelial neoplasm of possible gastrointestinal/pancreatic origin. With an integrated approach, based on microsatellite (VNTR and STS markers, we showed that ia2 insertion, in Tg(-8.5nkx2.2a:GFPia2 embryos, is associated with a deletion of 15.2 Mb in the telomeric portion of chromosome 1. Interestingly, among ia2 deleted genes we identified the presence of the 40S ribosomal protein S6 gene that may be one of the possible drivers for the MPNSTs in ia2 mutants. Thanks to the peculiar features of zebrafish as animal model of human cancer (cellular and genomic similarity, transparency and prolificacy and the GFP tag, the Tg(-8.5nkx2.2a:GFPia2 line provides a manageable tool to study in vivo with high frequency MPNST biology and genetics, and to identify, in concert with the existing zebrafish MPNST models, conserved relevant mechanisms in zebrafish and human cancer

  3. Prognostic relevance of FDG PET in patients with neurofibromatosis type-1 and malignant peripheral nerve sheath tumours

    Energy Technology Data Exchange (ETDEWEB)

    Brenner, Winfried; Buchert, Ralph; Clausen, Malte [University Medical Center Hamburg-Eppendorf, Department of Nuclear Medicine, Hamburg (Germany); Friedrich, Reinhard E.; Mautner, Victor F. [University Medical Center Hamburg-Eppendorf, Department of Oral and Maxillofacial Surgery, Hamburg (Germany); Gawad, Karim A. [University Medical Center Hamburg-Eppendorf, Department of General, Visceral and Thoracic Surgery, Hamburg (Germany); Hagel, Christian [University Medical Center Hamburg-Eppendorf, Institute of Neuropathology, Hamburg (Germany); Deimling, Andreas von [Charite Universitaetsmedizin, Department of Neuropathology, Berlin (Germany); Wit, Maike de [University Medical Center Hamburg-Eppendorf, Department of Medicine II, Hamburg (Germany)

    2006-04-15

    In patients with neurofibromatosis type-1 (NF1) and malignant peripheral nerve sheath tumours (MPNSTs), survival rates are low and time to death is often less than 2 years. However, there are patients with a more favourable prognosis who develop metastases rather late or not at all. Since histopathology and tumour grading are not well correlated with prognosis, we aimed to evaluate the potential of {sup 18}F-fluorodeoxyglucose positron emission tomography (FDG PET) for prediction of patient outcome in MPNST. FDG PET was performed in 16 patients with NF1 and MPNSTs. Standardised uptake values (SUVs) were calculated for each tumour and correlated to tumour grade and patient outcome in terms of survival or death. Three patients with tumour grade II had an SUV <3. None of these patients developed metastases or died during a follow-up of 41-62 months. Thirteen patients with tumour grades II and III had an SUV >3. Only one of these patients is still alive after 20 months; the remaining 12 died within 4-33 months. SUV predicted long-term survival with an accuracy of 94%, compared with 69% for tumour grade. In Kaplan-Meier survival analysis, patients with an SUV >3 had a significantly shorter mean survival time, 13 months, than patients with an SUV <3, in whom the mean survival time was 52 months. Tumour grading did not reveal differences in survival time (15 vs 12 months). Tumour SUV obtained by FDG PET was a significant parameter for prediction of survival in NF1 patients with MPNSTs while histopathological tumour grading did not predict outcome. (orig.)

  4. Relationship Between Optic Nerve Appearance and Retinal Nerve Fiber Layer Thickness as Explored with Spectral Domain Optical Coherence Tomography

    Science.gov (United States)

    Aleman, Tomas S.; Huang, Jiayan; Garrity, Sean T.; Carter, Stuart B.; Aleman, Wendy D.; Ying, Gui-shuang; Tamhankar, Madhura A.

    2014-01-01

    Purpose To study the relationship between the appearance of the optic nerve and the retinal nerve fiber layer (RNFL) thickness determined by spectral domain optical coherence tomography (OCT). Methods Records from patients with spectral domain-OCT imaging in a neuro-ophthalmology practice were reviewed. Eyes with glaucoma/glaucoma suspicion, macular/optic nerve edema, pseudophakia, and with refractive errors > 6D were excluded. Optic nerve appearance by slit lamp biomicroscopy was related to the RNFL thickness by spectral domain-OCT and to visual field results. Results Ninety-one patients (176 eyes; mean age: 49 ± 15 years) were included. Eighty-three eyes (47%) showed optic nerve pallor; 89 eyes (50.6%) showed RNFL thinning (sectoral or average peripapillary). Average peripapillary RNFL thickness in eyes with pallor (mean ± SD = 76 ± 17 μm) was thinner compared to eyes without pallor (91 ± 14 μm, P < 0.001). Optic nerve pallor predicted RNFL thinning with a sensitivity of 69% and a specificity of 75%. Optic nerve appearance predicted RNFL thinning (with a sensitivity and specificity of 81%) when RNFL had thinned by ∼ 40%. Most patients with pallor had RNFL thinning with (66%) or without (25%) visual field loss; the remainder had normal RNFL and fields (5%) or with visual field abnormalities (4%). Conclusions Optic nerve pallor as a predictor of RNFL thinning showed fair sensitivity and specificity, although it is optimally sensitive/specific only when substantial RNFL loss has occurred. Translational Relevance Finding an acceptable relationship between the optic nerve appearance by ophthalmoscopy and spectral domain-OCT RNFL measures will help the clinician's interpretation of the information provided by this technology, which is gaining momentum in neuro-ophthalmic research. PMID:25374773

  5. Neutrophils express oncomodulin and promote optic nerve regeneration

    National Research Council Canada - National Science Library

    Kurimoto, Takuji; Yin, Yuqin; Habboub, Ghaith; Gilbert, Hui-Ya; Li, Yiqing; Nakao, Shintaro; Hafezi-Moghadam, Ali; Benowitz, Larry I

    2013-01-01

    .... In a widely studied instance of this phenomenon, proinflammatory agents have been shown to cause retinal ganglion cells, the projection neurons of the eye, to regenerate lengthy axons through the injured optic nerve...

  6. Analytic methods in assessment of optic nerve cupping.

    Science.gov (United States)

    Jindra, L F; Kuběna, T; Gaudino, R N

    2014-06-01

    The intent of this paper is to provide a systems-based analysis of the methods used to evaluate optic nerve cupping, identify potential flaws in these systems, and propose alternatives better to assess this anatomic quantity. Estimation of optic nerve cupping requires an analytic understanding of both the psychophysical as well as the mathematical bases inherent in this measure. When the (decimal-based) cup-to-disc ratio is used to quantitate optic nerve cupping, a one-dimensional, linear estimate is produced, which in turn is derived from two- or three-dimensional, non-linear physical quantities of area or volume, respectively. When extrapolating from volume, to area, to linear measures, due to the psychophysical constraints which limit this task, such a data-compressed estimate of optic nerve cupping may neither accurately reflect, nor correctly represent, the true amount of cupping actually present in the optic nerve head. This type of one-dimensional metric (when comparing calculations from two- or three-dimensional measures over a range of optic nerve cupping), appears to introduce errors which, while most pronounced earlier on in the disease progression, often overestimate the amount of relative cupping (percent cupping) present in a pathological process like glaucoma. The same systemic errors can also lead to overestimation of the progression in cupping, especially in optic nerves with low cup-to disc values. To provide clinically meaningful estimates of optic nerve cupping, the practitioner needs to be aware of psychophysical and mathematical limitations inherent in using a linear cup-to-disc ratio to estimate the amount of cupping observed in a physical structure like the optic disc. The resultant flaws introduced by observer extrapolation from three, to two, to one dimensions (volume, area, and linear); transposition from non-linear to linear quantities; and optical illusions, caused by factors like disc topology, morphology, and ametropia, can all

  7. Coincidental Optic Nerve Meningioma and Thyroid Eye Disease.

    Science.gov (United States)

    Garg, Aakriti; Patel, Payal; Lignelli, Angela; Baron, Edward; Kazim, Michael

    2015-01-01

    A 57-year-old woman with diabetes mellitus, hypertension, obesity, and Graves disease presented with clinical evidence of thyroid eye disease (TED) and optic neuropathy. She was referred when a tapered dose of steroids prompted worsening of her TED. CT and MRI were consistent with TED and bilateral optic nerve meningioma. To the authors' knowledge, this is the first reported case of concurrent TED and unsuspected bilateral optic nerve meningioma. When investigating the etiology of TED-associated optic neuropathy, careful attention to orbital imaging is required because coexisting pathology may exist.

  8. Morphometric analysis of the fascicular organisation of the optic nerve

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    Radunović Miroslav

    2015-01-01

    Full Text Available Background/Aim. The optic nerve is anatomically observed in four segments: intrabulbar, orbital, canalicular, and cranial. According to the literature, the surface of the transversal cut of the nerve is different through it. The aim of this study was to evaluate the fascicular organisation of the optic nerve, throughout its three segments from the eye. Methods. Five pairs of optic nerves, obtained from the autopsies were examined. Using Heidenhain's (azan staining, the cuts were prepared for microscopy. Morphometric analysis was performed using the stereological methods for morphometric cytology - the Weible’s testing system M42. The following measures were established: the surface of the transverse cut of the nerve, the entire surface of fasciculi, the entire surface of connective tissue and blood vessels, the number of fasciculi, the surface of a single fasciculus. Results. The surface of the transverse cut of the nerve was found to grow from the orbital to the cranial segment, as well as the entire surface of fasciculi. While their number is significantly lower in the cranial segment, the number of fasciculi varied slightly between the orbital and the canalicular segment. The surface of a single fasciculus grows from the bulb to the chiasma. There is probable a cause to believe that this may be due to fusion of the “small” fasciculi in the orbitocranial direction. Conclusion. There are significant differences among the examined parameters of the different parts of the optic nerve.

  9. Dual mTORC1/2 inhibition induces anti-proliferative effect in NF1-associated plexiform neurofibroma and malignant peripheral nerve sheath tumor cells.

    Science.gov (United States)

    Varin, Jennifer; Poulain, Laury; Hivelin, Mikael; Nusbaum, Patrick; Hubas, Arnaud; Laurendeau, Ingrid; Lantieri, Laurent; Wolkenstein, Pierre; Vidaud, Michel; Pasmant, Eric; Chapuis, Nicolas; Parfait, Béatrice

    2016-06-14

    Approximately 30-50% of individuals with Neurofibromatosis type 1 develop benign peripheral nerve sheath tumors, called plexiform neurofibromas (PNFs). PNFs can undergo malignant transformation to highly metastatic malignant peripheral nerve sheath tumors (MPNSTs) in 5-10% of NF1 patients, with poor prognosis. No effective systemic therapy is currently available for unresectable tumors. In tumors, the NF1 gene deficiency leads to Ras hyperactivation causing the subsequent activation of the AKT/mTOR and Raf/MEK/ERK pathways and inducing multiple cellular responses including cell proliferation. In this study, three NF1-null MPNST-derived cell lines (90-8, 88-14 and 96-2), STS26T sporadic MPNST cell line and PNF-derived primary Schwann cells were used to test responses to AZD8055, an ATP-competitive "active-site" mTOR inhibitor. In contrast to rapamycin treatment which only partially affected mTORC1 signaling, AZD8055 induced a strong inhibition of mTORC1 and mTORC2 signaling in MPNST-derived cell lines and PNF-derived Schwann cells. AZD8055 induced full blockade of mTORC1 leading to an efficient decrease of global protein synthesis. A higher cytotoxic effect was observed with AZD8055 compared to rapamycin in the NF1-null MPNST-derived cell lines with IC50 ranging from 70 to 140 nM and antiproliferative effect was confirmed in PNF-derived Schwann cells. Cell migration was impaired by AZD8055 treatment and cell cycle analysis showed a G0/G1 arrest. Combined effects of AZD8055 and PD0325901 MEK inhibitor as well as BRD4 (BromoDomain-containing protein 4) inhibitors showed a synergistic antiproliferative effect. These data suggest that NF1-associated peripheral nerve sheath tumors are an ideal target for AZD8055 as a single molecule or in combined therapies.

  10. Dual mTORC1/2 inhibition induces anti-proliferative effect in NF1-associated plexiform neurofibroma and malignant peripheral nerve sheath tumor cells

    Science.gov (United States)

    Hivelin, Mikael; Nusbaum, Patrick; Hubas, Arnaud; Laurendeau, Ingrid; Lantieri, Laurent; Wolkenstein, Pierre; Vidaud, Michel; Pasmant, Eric; Chapuis, Nicolas; Parfait, Béatrice

    2016-01-01

    Approximately 30-50% of individuals with Neurofibromatosis type 1 develop benign peripheral nerve sheath tumors, called plexiform neurofibromas (PNFs). PNFs can undergo malignant transformation to highly metastatic malignant peripheral nerve sheath tumors (MPNSTs) in 5-10% of NF1 patients, with poor prognosis. No effective systemic therapy is currently available for unresectable tumors. In tumors, the NF1 gene deficiency leads to Ras hyperactivation causing the subsequent activation of the AKT/mTOR and Raf/MEK/ERK pathways and inducing multiple cellular responses including cell proliferation. In this study, three NF1-null MPNST-derived cell lines (90-8, 88-14 and 96-2), STS26T sporadic MPNST cell line and PNF-derived primary Schwann cells were used to test responses to AZD8055, an ATP-competitive “active-site” mTOR inhibitor. In contrast to rapamycin treatment which only partially affected mTORC1 signaling, AZD8055 induced a strong inhibition of mTORC1 and mTORC2 signaling in MPNST-derived cell lines and PNF-derived Schwann cells. AZD8055 induced full blockade of mTORC1 leading to an efficient decrease of global protein synthesis. A higher cytotoxic effect was observed with AZD8055 compared to rapamycin in the NF1-null MPNST-derived cell lines with IC50 ranging from 70 to 140 nM and antiproliferative effect was confirmed in PNF-derived Schwann cells. Cell migration was impaired by AZD8055 treatment and cell cycle analysis showed a G0/G1 arrest. Combined effects of AZD8055 and PD0325901 MEK inhibitor as well as BRD4 (BromoDomain-containing protein 4) inhibitors showed a synergistic antiproliferative effect. These data suggest that NF1-associated peripheral nerve sheath tumors are an ideal target for AZD8055 as a single molecule or in combined therapies. PMID:26840085

  11. A case of a sporadic malignant peripheral nerve sheath tumor of the urinary bladder with concomitant in situ urothelial carcinoma treated by transuretheral resection

    Directory of Open Access Journals (Sweden)

    Aysegul Sari

    2011-01-01

    Full Text Available Malignant peripheral nerve sheath tumor (MPNST of the urinary bladder is a very rare clinical entity. The association of such a tumor with urothelial carcinoma is even more unusual. Differential diagnosis between coexisting two distinct primary tumors and carcinosarcoma of the urinary bladder is very important as both the treatment and prognosis vary widely. Herein, we report a case of an MPNST with a concomitant in situ urothelial carcinoma in a 53-year-old man. To our knowledge, this is the first documented case of MPNST of the bladder that is treated by transuretheral resection which is in contrast with the previous reports that used cystectomy.

  12. Discovery of a small molecule targeting IRA2 deletion in budding yeast and neurofibromin loss in malignant peripheral nerve sheath tumor cells

    OpenAIRE

    Wood, Matthew; Rawe, Melissa; Johansson, Gunnar; Pang, Shu; Soderquist, Ryan S.; Patel, Ami V.; Nelson, Sandra; Seibel, William; Ratner, Nancy; Sanchez, Yolanda

    2011-01-01

    Malignant peripheral nerve sheath tumor (MPNST) is a life-threatening complication of neurofibromatosis type 1 (NF1). NF1 is caused by mutation in the gene encoding neurofibromin, a negative regulator of Ras signaling. There are no effective pharmacologic therapies for MPNST. To identify new therapeutic approaches targeting this dangerous malignancy, we developed assays in NF1+/+ and NF1−/ − MPNST cell lines and in budding yeast lacking the NF1 homologue IRA2 (ira2Δ). Here we describe UC1, a ...

  13. Diagnostic Accuracy of PET/CT-Guided Percutaneous Biopsies for Malignant Peripheral Nerve Sheath Tumors in Neurofibromatosis Type 1 Patients.

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    Mehdi Brahmi

    Full Text Available Malignant peripheral nerve sheath tumors (MPNST are one of the most frequent causes of death in patients with neurofibromatosis type 1 (NF1. Early detection is crucial because complete surgical resection is the only curative treatment. It has been previously reported that an 18F-fluorodeoxyglucose (FDG positron emission tomography/computed tomography (PET/CT image with a T/L (Tumor/Liver SUV max ratio > 1.5 provides a high negative predictive value; however, it is not specific enough to make a NF1-related MPNST diagnosis. A formal proof of malignant transformation from a histological analysis is necessary before surgical excision because the procedure can cause mutilation. The objective of the present work was to investigate the effectiveness of and complications associated with PET/CT-guided percutaneous biopsies for an NF1-related MPNST diagnosis.PET/CT-guided percutaneous biopsy procedures performed on 26 NF1 patients with a clinical suspicion of MPNST and a suspect lesion from a PET/CT scan (T/L SUV max ratio > 1.5 were retrospectively evaluated. The localization of the suspected malignant site was determined using PET/CT. A stereotactic (ultrasonic and CT control core biopsy technique was used with a local anesthesia.The first PET/CT-guided percutaneous biopsies enabled a pathological diagnosis for all of the patients (no "inconclusive " results were obtained, and no secondary procedures were needed. Among the 26 patients, the histopathological results from the biopsy were malignant in 17 cases and benign (BPNST with atypical cells in nine cases. No complications from the diagnostic procedure were observed. A surgical resection was performed in 18 patients (seven benign and 11 malignant biopsies, removing the fine needle biopsy scar. In addition, six locally advanced/metastatic MPNST were treated with chemo/radiotherapy, and two BPNST had no progression after a follow-up of 14 and 39 months, respectively. The PET/CT-guided percutaneous

  14. Malignant peripheral nerve sheath tumors: Clinicopathological profile of 63 cases diagnosed at a tertiary cancer referral center in Mumbai, India

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    Rekhi Bharat

    2010-10-01

    Full Text Available Background: A malignant peripheral nerve sheath tumor (MPNST is a rare sarcoma, characterized by an aggressive course and forms a diagnostic challenge, in view of its varied histomorphology. The present study is a comprehensive analysis, including histopathological spectrum of 63 MPNSTs that forms a substantial study from an Indian perspective. Materials and Methods: Clinicopathological features of 63 MPNSTs, diagnosed during a period from January 2002 to December 2006, at a tertiary cancer referral center in Mumbai, India, were analyzed. Statistical analysis was carried out using SPSS (version 14 and STRATA. Difference in events was noted in 50 cases with selected variables. Disease free survival (DFS was calculated by Kaplan-Meir analysis at the end of 1 year. Results: More cases were identified in > 30 years age (36 cases, 57.14% group; in men (46 cases, 73%, and were deep-seated (38, 60.3%. Ten cases (15.9% showed stigmata of multiple neurofibromatosis type 1. Average tumor (T size was 9.9 cm, with 72.9% cases having T size > 5 cm. More cases were of high grade (56, 88.8% and high stage (22, 34.9%. Histopathologically, most cases showed hypo- and hypercellular areas (marbleized appearance of doubly indented spindle cells. Two cases showed epithelioid differentiation. Heterologous elements in the form of osteoid, chondroid, pigmented neuroectodermal (1 case, glandular (1 case and rhabdomyoblastic differentiation (1 case were identified in 14 cases (22.2%. S-100 protein positivity was noted in 38/54 cases (70.3%. Maximum cases (45, 71.4% underwent surgery, including wide excisions and amputations (R0 in 20 cases, marginal excisions (R1 in 4, and intracapsular excision (R2 in 1 case. Nineteen cases underwent adjuvant treatment. A total of 29 cases (46% showed recurrences and 22 (34.9% showed multifocality and/or metastasis. Four patients succumbed to the disease in 1 year. The DFS was 53.1%. Cases ≤30 years of age (P- value = 0.007, T size

  15. The value of {sup 18}F-FDG PET/CT in the management of malignant peripheral nerve sheath tumors

    Energy Technology Data Exchange (ETDEWEB)

    Khiewvan, Benjapa [University of Texas MD Anderson Cancer Center, Department of Nuclear Medicine, Houston, TX (United States); Mahidol University, Division of Nuclear Medicine, Department of Radiology, Faculty of Medicine Siriraj Hospital, Bangkok (Thailand); Macapinlac, Homer A.; Chuang, Hubert H. [University of Texas MD Anderson Cancer Center, Department of Nuclear Medicine, Houston, TX (United States); Lev, Dina; Al Sannaa, Ghadah [University of Texas MD Anderson Cancer Center, Department of Cancer Biology, Houston, TX (United States); McCutcheon, Ian E. [University of Texas MD Anderson Cancer Center, Department of Neurosurgery, Houston, TX (United States); Slopis, John M. [University of Texas MD Anderson Cancer Center, Department of Neuro-Oncology, Houston, TX (United States); Wei, Wei [University of Texas MD Anderson Cancer Center, Department of Biostatistics, Houston, TX (United States)

    2014-09-15

    Our objective was to determine how positron emission tomography (PET)/CT had been used in the clinical treatment of malignant peripheral nerve sheath tumor (MPNST) patients at The University of Texas MD Anderson Cancer Center. We reviewed a database of MPNST patients referred to MD Anderson Cancer Center during 1995-2011. We enrolled 47 patients who underwent PET/CT imaging. Disease stage was based on conventional imaging and PET/CT findings using National Comprehensive Cancer Network (NCCN) guidelines. Treatment strategies based on PET/CT and conventional imaging were determined by chart review. The maximum and mean standardized uptake values (SUV{sub max}, SUV{sub mean}), metabolic tumor volume (MTV), total lesion glycolysis (TLG), change in SUV{sub max}, change in MTV, and change in TLG were calculated from the PET/CT studies before and after treatment. Response prediction was based on imaging studies performed before and after therapy and categorized as positive or negative for residual tumor. Clinical outcome was determined from chart review. PET/CT was performed for staging in 16 patients, for restaging in 29 patients, and for surveillance in 2 patients. Of the patients, 88 % were correctly staged with PET/CT, whereas 75 % were correctly staged with conventional imaging. The sensitivity to detect local recurrence and distant metastasis at restaging was 100 and 100 % for PET/CT compared to 86 and 83 % for conventional imaging, respectively. PET/CT findings resulted in treatment changes in 31 % (5/16) and 14 % (4/29) of patients at staging and restaging, respectively. Recurrence, MTV, and TLG were prognostic factors for survival, whereas SUV{sub max} and SUV{sub mean} were not predictive. For 21 patients who had imaging studies performed both before and after treatment, PET/CT was better at predicting outcome (overall survival, progression-free survival) than conventional imaging. A decreasing SUV{sub max} ≥ 30 % and decrease in TLG and MTV were significant

  16. Giant cell arteritis mimicking infiltrative leptomeningeal disease of the optic nerves.

    Science.gov (United States)

    Kornberg, Michael D; Ratchford, John N; Subramaniam, Rathan M; Probasco, John C

    2015-04-09

    A 67-year-old man presented with several days of progressive, painless left eye vision loss. He reported mild jaw claudication but denied headache, scalp tenderness or constitutional symptoms. Examination revealed palpable temporal arteries, blurring of the left optic disc, and 20/100 vision in the left eye with mild relative afferent pupillary defect. Inflammatory markers were sent, and methylprednisolone was initiated for presumptive giant cell arteritis (GCA). Erythrocyte sedimentation rate was normal, however, and C reactive protein was only mildly elevated, prompting further investigation. Orbital MRI revealed nodular enhancement of the optic nerve sheaths bilaterally from optic nerve head to chiasm, raising concern for an infiltrative leptomeningeal process such as sarcoidosis or lymphoma. Methylprednisolone was temporarily stopped while a broad work up for inflammatory and neoplastic causes was pursued. Fluorodeoxyglucose-positron emission tomography ultimately revealed hypermetabolism in the temporal, ophthalmic and occipital arteries suggesting GCA, which was confirmed by temporal artery biopsy. Steroids were restarted, and the patient's vision stabilised.

  17. [Up to date methods of optic nerve evaluation in patients with optic neuropathy of various etiology].

    Science.gov (United States)

    Sheremet, N L; Ronzina, I A; Galoian, N S; Kazarian, E E

    2011-01-01

    132 patients (188 eyes) with proven optic neuropathy of non-glaucomatous origin were examined using static perimetry, visual evoked potential (flash and pattern types), optic coherent tomography of papilla and thickness of peripapillary nerve fiber layer. Sensitivity/ specificity of methods for identification of optic nerve diseases in acute stage were 92/75%, 92/84%, 90/92% respectively. Combination of these three methods let us identify optic neuropathy in 100% of cases, including latent forms.

  18. Glaucoma severity affects diffusion tensor imaging (DTI) parameters of the optic nerve and optic radiation

    Energy Technology Data Exchange (ETDEWEB)

    Sidek, S. [Department of Biomedical Imaging, University Malaya, Research Imaging Centre, Faculty of Medicine, University Malaya (Malaysia); Medical Imaging Unit, Faculty of Medicine, Universiti Teknologi MARA, Selangor (Malaysia); Ramli, N. [Department of Biomedical Imaging, University Malaya, Research Imaging Centre, Faculty of Medicine, University Malaya (Malaysia); Rahmat, K., E-mail: katt_xr2000@yahoo.com [Department of Biomedical Imaging, University Malaya, Research Imaging Centre, Faculty of Medicine, University Malaya (Malaysia); Ramli, N.M.; Abdulrahman, F. [Department of Ophthalmology, Faculty of Medicine, University Malaya, Kuala Lumpur (Malaysia); Tan, L.K. [Department of Biomedical Imaging, University Malaya, Research Imaging Centre, Faculty of Medicine, University Malaya (Malaysia)

    2014-08-15

    Objectives: To evaluate whether MR diffusion tensor imaging (DTI) of the optic nerve and optic radiation in glaucoma patients provides parameters to discriminate between mild and severe glaucoma and to determine whether DTI derived indices correlate with retinal nerve fibre layer (RNFL) thickness. Methods: 3-Tesla DTI was performed on 90 subjects (30 normal, 30 mild glaucoma and 30 severe glaucoma subjects) and the FA and MD of the optic nerve and optic radiation were measured. The categorisation into mild and severe glaucoma was done using the Hodapp–Parrish–Anderson (HPA) classification. RNFL thickness was also assessed on all subjects using OCT. Receiver operating characteristic (ROC) analysis and Spearman's correlation coefficient was carried out. Results: FA and MD values in the optic nerve and optic radiation decreased and increased respectively as the disease progressed. FA at the optic nerve had the highest sensitivity (87%) and specificity (80%). FA values displayed the strongest correlation with RNFL thickness in the optic nerve (r = 0.684, p ≤ 0.001) while MD at the optic radiation showed the weakest correlation with RNFL thickness (r = −0.360, p ≤ 0.001). Conclusions: The high sensitivity and specificity of DTI-derived FA values in the optic nerve and the strong correlation between DTI-FA and RNFL thickness suggest that these parameters could serve as indicators of disease severity.

  19. Optic Nerve Injury in a Patient with Chronic Allergic Conjunctivitis

    Directory of Open Access Journals (Sweden)

    Ribhi Hazin

    2014-01-01

    Full Text Available Manipulation of the optic nerve can lead to irreversible vision changes. We present a patient with a past medical history of skin allergy and allergic conjunctivitis (AC who presented with insidious unexplained unilateral vision loss. Physical exam revealed significant blepharospasm, mild lid edema, bulbar conjunctival hyperemia, afferent pupillary defect, and slight papillary hypertrophy. Slit lamp examination demonstrated superior and inferior conjunctival scarring as well as superior corneal scarring but no signs of external trauma or neurological damage were noted. Conjunctival cultures and cytologic evaluation demonstrated significant eosinophilic infiltration. Subsequent ophthalmoscopic examination revealed optic nerve atrophy. Upon further questioning, the patient admitted to vigorous itching of the affected eye for many months. Given the presenting symptoms, history, and negative ophthalmological workup, it was determined that the optic nerve atrophy was likely secondary to digital pressure from vigorous itching. Although AC can be a significant source of decreased vision via corneal ulceration, no reported cases have ever described AC-induced vision loss of this degree from vigorous itching and chronic pressure leading to optic nerve damage. Despite being self-limiting in nature, allergic conjunctivitis should be properly managed as extreme cases can result in mechanical compression of the optic nerve and compromise vision.

  20. Robust Optic Nerve Segmentation on Clinically Acquired CT.

    Science.gov (United States)

    Panda, Swetasudha; Asman, Andrew J; Delisi, Michael P; Mawn, Louise A; Galloway, Robert L; Landman, Bennett A

    2014-03-21

    The optic nerve is a sensitive central nervous system structure, which plays a critical role in many devastating pathological conditions. Several methods have been proposed in recent years to segment the optic nerve automatically, but progress toward full automation has been limited. Multi-atlas methods have been successful for brain segmentation, but their application to smaller anatomies remains relatively unexplored. Herein we evaluate a framework for robust and fully automated segmentation of the optic nerves, eye globes and muscles. We employ a robust registration procedure for accurate registrations, variable voxel resolution and image field-of-view. We demonstrate the efficacy of an optimal combination of SyN registration and a recently proposed label fusion algorithm (Non-local Spatial STAPLE) that accounts for small-scale errors in registration correspondence. On a dataset containing 30 highly varying computed tomography (CT) images of the human brain, the optimal registration and label fusion pipeline resulted in a median Dice similarity coefficient of 0.77, symmetric mean surface distance error of 0.55 mm, symmetric Hausdorff distance error of 3.33 mm for the optic nerves. Simultaneously, we demonstrate the robustness of the optimal algorithm by segmenting the optic nerve structure in 316 CT scans obtained from 182 subjects from a thyroid eye disease (TED) patient population.

  1. Triterpenoid saponin flaccidoside II from Anemone flaccida triggers apoptosis of NF1-associated malignant peripheral nerve sheath tumors via the MAPK-HO-1 pathway

    Directory of Open Access Journals (Sweden)

    Han LT

    2016-04-01

    Full Text Available Lin-tao Han,1 Yin Fang,1 Yan Cao,2 Feng-hua Wu,1 E Liu,2 Guo-yan Mo,2 Fang Huang1 1China Key Laboratory of TCM Resource and Prescription, Ministry of Education, 2Department of Pharmacy, Hubei University of Chinese Medicine, Wuhan, Hubei, People’s Republic of China Abstract: Malignant peripheral nerve sheath tumors (MPNSTs are highly aggressive soft tissue neoplasms that are extremely rare and are frequently associated with neurofibromatosis type 1 patients. MPNSTs are typically fatal, and there is no effective treatment so far. In our previous study, we showed that flaccidoside II, one of the triterpenoid saponins isolated from Anemone flaccida Fr. Schmidt, has antitumor potential by inducing apoptosis. In the present study, we found that flaccidoside II inhibits proliferation and facilitates apoptosis in MPNST cell lines ST88-14 and S462. Furthermore, this study provides a mechanism by which the downregulation of heme oxygenase-1 via extracellular signal-regulated kinase-1/2 and p38 mitogen-activated protein kinase pathways is involved in the apoptotic role of flaccidoside II. This study suggested the potential of flaccidoside II as a novel pharmacotherapeutic approach for MPNSTs. Keywords: flaccidoside II, malignant peripheral nerve sheath tumors, apoptosis, MAPK, HO-1

  2. A Multidisciplinary Approach to Educating Preschool Children with Optic Nerve Hypoplasia and Septo-Optic Nerve Dysplasia.

    Science.gov (United States)

    Bahar, Cheryl; Brody, Jill; McCann, Mary Ellen; Mendiola, Rosalinda; Slott, Gayle

    2003-01-01

    This article discusses the observations and experiences of a multidisciplinary team at the Blind Childrens Center in Los Angeles, which works specifically with children from birth to 5 years of age who have been diagnosed with optic nerve hypoplasia and may have septo-optic displasia. Strategies for educational interventions are explained.…

  3. Long echo time STIR sequence MRI of optic nerves in optic neuritis

    Energy Technology Data Exchange (ETDEWEB)

    Onofrj, M. [Dept. of Neurology, State Univ. of Chieti, Ospedale ex-Pediatrico (Italy); Tartaro, A. [Dept. of Radiology, State Univ. of Chieti (Italy); Thomas, A. [Dept. of Neurology, State Univ. of Chieti, Ospedale ex-Pediatrico (Italy); Gambi, D. [Dept. of Neurology, State Univ. of Chieti, Ospedale ex-Pediatrico (Italy); Fulgente, T. [Dept. of Neurology, State Univ. of Chieti, Ospedale ex-Pediatrico (Italy); Delli Pizzi, C. [Dept. of Radiology, State Univ. of Chieti (Italy); Bonomo, L. [Dept. of Radiology, State Univ. of Chieti (Italy)

    1996-01-01

    MRI of the optic nerves was obtained in 13 patients with acute optic neuritis and 13 with a previous optic neuritis (ON), assessed by clinical features, visual fields and visual evoked potentials. Results of the conventional short tau inversion recovery (STIR) sequence obtained with a short echo time (STE-STIR; 22 ms) were compared with those of a long echo time (LTE-STIR: 80 ms) sequence. The conventional STE-STIR sequence revealed lesions in the optic nerves in 78.5% of acute and 58.8% of previous ON. The LTE-STIR sequence showed abnormalities in 92.8% of acutely symptomatic nerves and 94.1% of nerves with previous ON. The optic nerve lesions appeared significantly longer with the LTE-STIR sequence than with the conventional STE-STIR sequences, in both acute and previous ON. (orig.)

  4. Blunt Facial Trauma Causing Isolated Optic Nerve Hematoma

    Directory of Open Access Journals (Sweden)

    R. Parab

    2013-01-01

    Full Text Available Traumatic optic neuropathy is an uncommon, yet serious, result of facial trauma. The authors present a novel case of a 59-year-old gentleman who presented with an isolated blunt traumatic left optic nerve hematoma causing vision loss. There were no other injuries or fractures to report. This case highlights the importance of early recognition of this rare injury and reviews the current literature and management of traumatic optic neuropathy.

  5. Optical coherence tomography of the rat cavernous nerves

    Science.gov (United States)

    Fried, Nathaniel M.; Rais-Bahrami, Soroush; Lagoda, Gwen A.; Chuang, Ying; Burnett, Arthur L.; Su, Li-Ming

    2007-02-01

    Improvements in identification, imaging, and visualization of the cavernous nerves during radical prostatectomy, which are responsible for erectile function, may improve nerve preservation and postoperative potency. Optical coherence tomography (OCT) is capable of real-time, high-resolution, cross-sectional, in vivo tissue imaging. The rat prostate serves as an excellent model for studying the use of OCT for imaging the cavernous nerves, as the rat cavernous nerve is a large, visible, and distinct bundle allowing for easy identification with OCT in addition to histologic confirmation. Imaging was performed with the Niris OCT system and a handheld 8 Fr probe, capable of acquiring real-time images with 11-μm axial and 25-μm lateral resolution in tissue. Open surgical exposure of the prostate was performed on a total of 6 male rats, and OCT images of the prostate, cavernous nerve, pelvic plexus ganglion, seminal vesicle, blood vessels, and periprostatic fat were acquired. Cavernous nerve electrical stimulation with simultaneous intracorporeal pressure measurements was performed to confirm proper identification of the cavernous nerves. The prostate and cavernous nerves were also processed for histologic analysis and further confirmation. Cross-sectional and longitudinal OCT images of the cavernous nerves were acquired and compared with histologic sections. The cavernous nerve and ganglion could be differentiated from the surrounding prostate gland, seminal vesicle, blood vessels, bladder, and fatty tissue. We report preliminary results of OCT images of the rat cavernous nerves with histologic correlation and erectile stimulation measurements, thus providing interpretation of prostate structures as they appear in OCT images.

  6. The Impact of Ocular Pressures, Material Properties and Geometry on Optic Nerve Head Deformation

    Science.gov (United States)

    Feola, Andrew J.; Myers, Jerry G.; Raykin, Julia; Nelson, Emily S.; Samuels, Brian C.; Ethier C. Ross

    2017-01-01

    Alteration in intracranial pressure (ICP) has been associated with various diseases that cause visual impairment, including glaucoma, idiopathic intracranial hypertension and Visual Impairment and Intracranial Pressure (VIIP) syndrome. However, how changes in ICP lead to vision loss is unclear, although it is hypothesized to involve deformations of the tissues in the optic nerve head (ONH). Recently, understanding the effect of ICP alterations on ocular tissues has become a major concern for NASA, where 42 of astronauts that partake in long duration space missions suffer from VIIP syndrome. Astronauts with VIIP syndrome suffer from visual impairment and changes in ocular anatomy that persist after returning to earth (1). It is hypothesized that the cephalad fluid shift that occurs upon entering microgravity increases ICP, which leads to an altered biomechanical environment in the posterior globe and optic nerve sheath, and subsequently VIIP syndrome. Our goal was to develop a finite element (FE) model to simulate the acute effects of elevated ICP on the posterior eye. Here, we simulated how inter-individual differences affect the deformation of ONH tissues. Further, we examined how several different geometries influenced deformations when exposed to elevated ICP.

  7. Quantitative contrast-enhanced MR imaging of the optic nerve

    Energy Technology Data Exchange (ETDEWEB)

    Simon, J.H. [Depts. of Radiology, Univ. of Colorado Health Sciences Center, Denver, CO (United States)]|[Iowa Univ., Iowa City, IA (United States); Rubinstein, D. [Depts. of Radiology, Univ. of Colorado Health Sciences Center, Denver, CO (United States)]|[Iowa Univ., Iowa City, IA (United States); Brown, M. [Depts. of Radiology, Univ. of Colorado Health Sciences Center, Denver, CO (United States)]|[Iowa Univ., Iowa City, IA (United States); Yuh, W. [Depts. of Radiology, Univ. of Colorado Health Sciences Center, Denver, CO (United States)]|[Iowa Univ., Iowa City, IA (United States); Birch-Iensen, M. [Depts. of Radiology, Univ. of Colorado Health Sciences Center, Denver, CO (United States)]|[Iowa Univ., Iowa City, IA (United States); Szumowski, J. [Depts. of Radiology, Univ. of Colorado Health Sciences Center, Denver, CO (United States)]|[Iowa Univ., Iowa City, IA (United States); Stears, J. [Depts. of Radiology, Univ. of Colorado Health Sciences Center, Denver, CO (United States)]|[Iowa Univ., Iowa City, IA (United States)

    1994-11-01

    During the acute stages of optic neuritis damage to the blood-optic nerve barrier can be detected using i.v. paramagnetic contrast-enhanced MR imaging. Quantification of the enhancement pattern of the optic nerve, intraorbital fat and muscle was determined in 15 normal subjects using 3 fat-suppression MR imaging methods: T1-weighted spin-echo and spoiled gradient-echo sequences preceded by a flat-frequency selective pulse (FATSAT+SE and FATSAT+SPGR, respectively) and a pulse sequence combining CHOPPER fat suppression with a fat-frequency selective preparation pulse (HYBRID). Pre- and postcontrast-enhanced studies were acquired for FATSAT+SE and FATSAT+SPGR. There was no significant enhancement of the optic nerve by either method (mean increase of 0.96% and 5.3%, respectively), while there was significant enhancement in muscle (mean 118.2% and 108.2%, respectively; p<0.005) and fat (mean increase of 13% and 37%, respectively; p<0.05). Postcontrast optic nerve/muscle signal intensity ratios (mean, SD) were 0.51 (0.07), 0.58 (0.05) and 0.75 (0.05) for FATSAT+SE, FATSAT+SPGR and HYBRID, respectively. These results suggest a practical methodology and range of values for normal signal intensity increases and ratios of tissue signal that can be used as objective measures of optic neuritis for natural history studies and treatment trials. (orig.).

  8. Stimulation of the human auditory nerve with optical radiation

    Science.gov (United States)

    Fishman, Andrew; Winkler, Piotr; Mierzwinski, Jozef; Beuth, Wojciech; Izzo Matic, Agnella; Siedlecki, Zygmunt; Teudt, Ingo; Maier, Hannes; Richter, Claus-Peter

    2009-02-01

    A novel, spatially selective method to stimulate cranial nerves has been proposed: contact free stimulation with optical radiation. The radiation source is an infrared pulsed laser. The Case Report is the first report ever that shows that optical stimulation of the auditory nerve is possible in the human. The ethical approach to conduct any measurements or tests in humans requires efficacy and safety studies in animals, which have been conducted in gerbils. This report represents the first step in a translational research project to initiate a paradigm shift in neural interfaces. A patient was selected who required surgical removal of a large meningioma angiomatum WHO I by a planned transcochlear approach. Prior to cochlear ablation by drilling and subsequent tumor resection, the cochlear nerve was stimulated with a pulsed infrared laser at low radiation energies. Stimulation with optical radiation evoked compound action potentials from the human auditory nerve. Stimulation of the auditory nerve with infrared laser pulses is possible in the human inner ear. The finding is an important step for translating results from animal experiments to human and furthers the development of a novel interface that uses optical radiation to stimulate neurons. Additional measurements are required to optimize the stimulation parameters.

  9. Optical stimulation of the facial nerve: a surgical tool?

    Science.gov (United States)

    Richter, Claus-Peter; Teudt, Ingo Ulrik; Nevel, Adam E.; Izzo, Agnella D.; Walsh, Joseph T., Jr.

    2008-02-01

    One sequela of skull base surgery is the iatrogenic damage to cranial nerves. Devices that stimulate nerves with electric current can assist in the nerve identification. Contemporary devices have two main limitations: (1) the physical contact of the stimulating electrode and (2) the spread of the current through the tissue. In contrast to electrical stimulation, pulsed infrared optical radiation can be used to safely and selectively stimulate neural tissue. Stimulation and screening of the nerve is possible without making physical contact. The gerbil facial nerve was irradiated with 250-μs-long pulses of 2.12 μm radiation delivered via a 600-μm-diameter optical fiber at a repetition rate of 2 Hz. Muscle action potentials were recorded with intradermal electrodes. Nerve samples were examined for possible tissue damage. Eight facial nerves were stimulated with radiant exposures between 0.71-1.77 J/cm2, resulting in compound muscle action potentials (CmAPs) that were simultaneously measured at the m. orbicularis oculi, m. levator nasolabialis, and m. orbicularis oris. Resulting CmAP amplitudes were 0.3-0.4 mV, 0.15-1.4 mV and 0.3-2.3 mV, respectively, depending on the radial location of the optical fiber and the radiant exposure. Individual nerve branches were also stimulated, resulting in CmAP amplitudes between 0.2 and 1.6 mV. Histology revealed tissue damage at radiant exposures of 2.2 J/cm2, but no apparent damage at radiant exposures of 2.0 J/cm2.

  10. Optic nerve pH and PO2

    DEFF Research Database (Denmark)

    Pedersen, Daniella B; Stefánsson, Einar; Kiilgaard, Jens Folke

    2006-01-01

    Earlier studies have demonstrated that carbonic anhydrase inhibitors (CAIs) increase optic nerve oxygen tension (ONPO(2)) in pigs. We hypothesized that the mechanism of this effect was either a CO(2) increase or a pH decrease in tissue and blood. To test this hypothesis we investigated and compared...... how optic nerve pH (ONpH) and ONPO(2) are affected by: (1) carbonic anhydrase inhibition; (2) respiratory acidosis, and (3) metabolic acidosis. We measured ONpH with a glass pH electrode and ONPO(2) with a polarographic oxygen electrode. One of the electrodes was placed in the vitreous cavity 0.5 mm...

  11. Convection-Enhanced Delivery (CED) in an Animal Model of Malignant Peripheral Nerve Sheath Tumors and Plexiform Neurofibromas

    Science.gov (United States)

    2013-02-01

    sheath tumors or plexiform neurofibromas. References: Perrin GQ, Fishbein L, Thomson SA, et al., Plexiform-like neurofibromas develop in the...mouse by intraneural xenograft of an NF1 tumor-derived Schwann cell line. J Neurosci Res, 2007. 85(6): p. 1347-1357. 3 Perrin GQ, Li H, Fishbein L

  12. Chemical shift selective magnetic resonance imaging of the optic nerve in patients with acute optic neuritis

    DEFF Research Database (Denmark)

    Larsson, H B; Thomsen, C; Frederiksen, J

    1988-01-01

    of the 16 patients, abnormalities were seen. In one patient with bilateral symptoms, signal hyperintensity and swelling of the right side of the chiasm were found. In another patient the optic nerve was found diffusely enlarged with only a marginally increased signal in the second echo. In the third patient......Optic neuritis is often the first manifestation of multiple sclerosis (MS). Sixteen patients with acute optic neuritis and one patient with benign intracranial hypertension (BIH) were investigated by magnetic resonance imaging, using a chemical shift selective double spin echo sequence. In 3...... an area of signal hyperintensity and swelling was seen in the left optic nerve. In the patient with BIH the subarachnoid space which surrounds the optic nerves was enlarged. Even using this refined pulse sequence, avoiding the major artefact in imaging the optic nerve, the chemical shift artefact, lesions...

  13. Comprehensive adipocytic and neurogenic tissue microarray analysis of NY-ESO-1 expression - a promising immunotherapy target in malignant peripheral nerve sheath tumor and liposarcoma.

    Science.gov (United States)

    Shurell, Elizabeth; Vergara-Lluri, Maria E; Li, Yunfeng; Crompton, Joseph G; Singh, Arun; Bernthal, Nicholas; Wu, Hong; Eilber, Fritz C; Dry, Sarah M

    2016-11-08

    Immunotherapy targeting cancer-testis antigen NY-ESO-1 shows promise for tumors with poor response to chemoradiation. Malignant peripheral nerve sheath tumors (MPNSTs) and liposarcomas (LPS) are chemoresistant and have few effective treatment options. Materials Methods: Using a comprehensive tissue microarray (TMA) of both benign and malignant tumors in primary, recurrent, and metastatic samples, we examined NY-ESO-1 expression in peripheral nerve sheath tumor (PNST) and adipocytic tumors. The PNST TMA included 42 MPNSTs (spontaneous n = 26, NF1-associated n = 16), 35 neurofibromas (spontaneous n = 22, NF-1 associated n = 13), 11 schwannomas, and 18 normal nerves. The LPS TMA included 48 well-differentiated/dedifferentiated (WD/DD) LPS, 13 myxoid/round cell LPS, 3 pleomorphic LPS, 8 lipomas, 1 myelolipoma, and 3 normal adipocytic tissue samples. Stained in triplicate, NY-ESO-1 intensity and density were scored. NY-ESO-1 expression was exclusive to malignant tumors. 100% of myxoid/round cell LPS demonstrated NY-ESO-1 expression, while only 6% of WD/DD LPS showed protein expression, one of which was WD LPS. Of MPNST, 4/26 (15%) spontaneous and 2/16 (12%) NF1-associated MPNSTs demonstrated NY-ESO-1 expression. Strong NY-ESO-1 expression was observed in myxoid/round cell and dedifferentiated LPS, and MPNST in primary, neoadjuvant, and metastatic settings. We found higher prevalence of NY-ESO-1 expression in MPNSTs than previously reported, highlighting a subset of MPNST patients who may benefit from immunotherapy. This study expands our understanding of NY-ESO-1 in WD/DD LPS and is the first demonstration of staining in a WD LPS and metastatic/recurrent myxoid/round cell LPS. These results suggest immunotherapy targeting NY-ESO-1 may benefit patients with aggressive tumors resistant to conventional therapy.

  14. Determining degree of optic nerve edema from color fundus photography

    Science.gov (United States)

    Agne, Jason; Wang, Jui-Kai; Kardon, Randy H.; Garvin, Mona K.

    2015-03-01

    Swelling of the optic nerve head (ONH) is subjectively assessed by clinicians using the Frisén scale. It is believed that a direct measurement of the ONH volume would serve as a better representation of the swelling. However, a direct measurement requires optic nerve imaging with spectral domain optical coherence tomography (SD-OCT) and 3D segmentation of the resulting images, which is not always available during clinical evaluation. Furthermore, telemedical imaging of the eye at remote locations is more feasible with non-mydriatic fundus cameras which are less costly than OCT imagers. Therefore, there is a critical need to develop a more quantitative analysis of optic nerve swelling on a continuous scale, similar to SD-OCT. Here, we select features from more commonly available 2D fundus images and use them to predict ONH volume. Twenty-six features were extracted from each of 48 color fundus images. The features include attributes of the blood vessels, optic nerve head, and peripapillary retina areas. These features were used in a regression analysis to predict ONH volume, as computed by a segmentation of the SD-OCT image. The results of the regression analysis yielded a mean square error of 2.43 mm3 and a correlation coefficient between computed and predicted volumes of R = 0:771, which suggests that ONH volume may be predicted from fundus features alone.

  15. Optic nerve histopathology in a case of Wolfram Syndrome

    DEFF Research Database (Denmark)

    Ross-Cisneros, Fred N; Pan, Billy X; Silva, Ruwan A

    2013-01-01

    Mitochondrial dysfunction in Wolfram Syndrome (WS) is controversial and optic neuropathy, a cardinal clinical manifestation, is poorly characterized. We here describe the histopathological features in postmortem retinas and optic nerves (ONs) from one patient with WS, testing the hypothesis...... that mitochondrial dysfunction underlies the pathology. Eyes and retrobulbar ONs were obtained at autopsy from a WS patient, and compared with those of a Leber hereditary optic neuropathy (LHON) patient and one healthy control. Retinas were stained with hematoxylin & eosin for general morphology and ONs were...... of axonal degeneration, involving primarily the papillomacular bundle, closely resembled LHON and other mitochondrial optic neuropathies, supporting that mitochondrial dysfunction underlies its pathogenesis....

  16. Spontaneous resolution of optic nerve coloboma-associated retinal detachment.

    Science.gov (United States)

    Ying, Michelle S; Fuller, Jeff; Young, Joshua; Marcus, Dennis M

    2004-01-01

    We report a congenital neurosensory retinal detachment associated with an optic nerve coloboma with subsequent spontaneous reattachment. This represents the earliest reported case of such a clinical situation. An observation period is recommended for infants with this clinical course to allow for the opportunity of spontaneous reattachment.

  17. Bilateral iris, choroid, optic nerve colobomas and retinal detachment ...

    African Journals Online (AJOL)

    Rabah M. Shawky

    2013-12-05

    Dec 5, 2013 ... low posterior hair line, partial left simian crease, and short fingers. MRI brain shows ... The patient has mild mental retardation, now she can walk, dress, and feed ... and acoustic evidence of optic nerve head coloboma. B-scan.

  18. Optic nerve pH and PO2

    DEFF Research Database (Denmark)

    Pedersen, Daniella B; Stefánsson, Einar; Kiilgaard, Jens Folke;

    2006-01-01

    how optic nerve pH (ONpH) and ONPO(2) are affected by: (1) carbonic anhydrase inhibition; (2) respiratory acidosis, and (3) metabolic acidosis. We measured ONpH with a glass pH electrode and ONPO(2) with a polarographic oxygen electrode. One of the electrodes was placed in the vitreous cavity 0.5 mm...

  19. Detection of disease-associated prion protein in the optic nerve and the adrenal gland of cattle with bovine spongiform encephalopathy by using highly sensitive immunolabeling procedures.

    Science.gov (United States)

    Okada, Hiroyuki; Iwamaru, Yoshifumi; Fukuda, Shigeo; Yokoyama, Takashi; Mohri, Shirou

    2012-04-01

    A sensitive immunohistochemical procedure, the tyramide signal amplification (TSA) system, was applied to detect the localization of immunolabeled disease-associated prion protein (PrP(Sc)) in cattle affected with bovine spongiform encephalopathy (BSE). In this procedure, immunolabeling could be visualized in the optic nerve and the adrenal medulla. In the optic nerve, the dual immunofluorescent technique showed that the granular PrP(Sc) was occasionally detected in the astrocytes, microglia, and myelin sheath adjacent to the axon. Clustered PrP(Sc) was also scattered in association with microglial cells and astrocytes of the optic nerve. In the adrenal gland, PrP(Sc) immunolabeling was confined within the sympathetic nerve fibers and endings. The results suggest that (1) PrP(Sc) might centrifugally spread within and between glial cells and/or the non-axonal (also known as ad-axonal) region of nerve fibers, rather than the axonal and/or extracellular space pathway in the optic nerve, and (2) the sympathetic innervations might be important for the trafficking of BSE agent in the adrenal glands of cattle. This study also suggests that tyramide-based immunochemical analysis should be performed to detect immunolabeled PrP(Sc) in the extracerebral tissues of BSE-affected cattle.

  20. Characterization of Optic Nerve Regeneration using transgenic Zebrafish

    Directory of Open Access Journals (Sweden)

    Heike eDiekmann

    2015-04-01

    Full Text Available In contrast to the adult mammalian central nervous system (CNS, fish are able to functionally regenerate severed axons upon injury. Although the zebrafish is a well-established model vertebrate for genetic and developmental studies, its use for anatomical studies of axon regeneration has been hampered by the paucity of appropriate tools to visualize re-growing axons in the adult CNS. On this account, we used transgenic zebrafish that express enhanced green fluorescent protein (GFP under the control of a GAP-43 promoter. In adult, naïve retinae, GFP was restricted to young retinal ganglion cells (RGCs and their axons. Within the optic nerve, these fluorescent axons congregated in a distinct strand at the nerve periphery, indicating age-related order. Upon optic nerve crush, GFP expression was markedly induced in RGC somata and intra-retinal axons at 4 to at least 14 days post injury. Moreover, individual axons were visualized in their natural environment of the optic nerve using wholemount tissue clearing and confocal microscopy. With this novel approach, regenerating axons were clearly detectable beyond the injury site as early as 2 days after injury and grew past the optic chiasm by 4 days. Regenerating axons in the entire optic nerve were labelled from 6 to at least 14 days after injury, thereby allowing detailed visualization of the complete regeneration process. Therefore, this new approach could now be used in combination with expression knockdown or pharmacological manipulations to analyze the relevance of specific proteins and signaling cascades for axonal regeneration in vivo. In addition, the RGC-specific GFP expression facilitated accurate evaluation of neurite growth in dissociated retinal cultures. This fast in vitro assay now enables the screening of compound and expression libraries. Overall, the presented methodologies provide exciting possibilities to investigate the molecular mechanisms underlying successful CNS regeneration in

  1. Acute Myeloid Leukemia Relapse Presenting as Complete Monocular Vision Loss due to Optic Nerve Involvement

    Directory of Open Access Journals (Sweden)

    Shyam A. Patel

    2016-01-01

    Full Text Available Acute myeloid leukemia (AML involvement of the central nervous system is relatively rare, and detection of leptomeningeal disease typically occurs only after a patient presents with neurological symptoms. The case herein describes a 48-year-old man with relapsed/refractory AML of the mixed lineage leukemia rearrangement subtype, who presents with monocular vision loss due to leukemic eye infiltration. MRI revealed right optic nerve sheath enhancement and restricted diffusion concerning for nerve ischemia and infarct from hypercellularity. Cerebrospinal fluid (CSF analysis showed a total WBC count of 81/mcl with 96% AML blasts. The onset and progression of visual loss were in concordance with rise in peripheral blood blast count. A low threshold for diagnosis of CSF involvement should be maintained in patients with hyperleukocytosis and high-risk cytogenetics so that prompt treatment with whole brain radiation and intrathecal chemotherapy can be delivered. This case suggests that the eye, as an immunoprivileged site, may serve as a sanctuary from which leukemic cells can resurge and contribute to relapsed disease in patients with high-risk cytogenetics.

  2. Overexpression of PDGFRA cooperates with loss of NF1 and p53 to accelerate the molecular pathogenesis of malignant peripheral nerve sheath tumors

    Science.gov (United States)

    Ki, D H; He, S; Rodig, S; Look, A T

    2017-01-01

    Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive, frequently metastatic sarcomas that are associated with neurofibromatosis type 1 (NF1), a prominent inherited genetic disease in humans. Although loss of the NF1 gene predisposes to MPNST induction, relatively long tumor latency in NF1 patients suggests that additional genetic or epigenetic abnormalities are needed for the development of these nerve sheath malignancies. To study the molecular pathways contributing to the formation of MPNSTs in NF1 patients, we used a zebrafish tumor model defined by nf1 loss in a p53-deficient background together with the overexpression of either wild-type or constitutively activated PDGFRA (platelet-derived growth factor receptor-α) under control of the sox10 neural crest-specific promoter. Here we demonstrate the accelerated onset and increased penetrance of MPNST formation in fish overexpressing both the wild-type and the mutant PDGFRA transgenes in cells of neural crest origin. Interestingly, overexpression of the wild-type PDGFRA was even more potent in promoting transformation than the mutant PDGFRA, which is important because ~78% of human MPNSTs have expression of wild-type PDGFRA, whereas only 5% harbor activating mutations of the gene encoding this receptor. Further analysis revealed the induction of cellular senescence in zebrafish embryos overexpressing mutant, but not wild-type, PDGFRA, suggesting a mechanism through which the oncogenic activity of the mutant receptor is tempered by the activation of premature cellular senescence in an NF1-deficient background. Taken together, our study suggests a model in which overexpression of wild-type PDGFRA associated with NF1 deficiency leads to aberrant activation of downstream RAS signaling and thus contributes importantly to MPNST development—a prediction supported by the ability of the kinase inhibitor sunitinib alone and in combination with the MEK inhibitor trametinib to retard MPNST progression in

  3. Overexpression of PDGFRA cooperates with loss of NF1 and p53 to accelerate the molecular pathogenesis of malignant peripheral nerve sheath tumors.

    Science.gov (United States)

    Ki, D H; He, S; Rodig, S; Look, A T

    2017-02-23

    Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive, frequently metastatic sarcomas that are associated with neurofibromatosis type 1 (NF1), a prominent inherited genetic disease in humans. Although loss of the NF1 gene predisposes to MPNST induction, relatively long tumor latency in NF1 patients suggests that additional genetic or epigenetic abnormalities are needed for the development of these nerve sheath malignancies. To study the molecular pathways contributing to the formation of MPNSTs in NF1 patients, we used a zebrafish tumor model defined by nf1 loss in a p53-deficient background together with the overexpression of either wild-type or constitutively activated PDGFRA (platelet-derived growth factor receptor-α) under control of the sox10 neural crest-specific promoter. Here we demonstrate the accelerated onset and increased penetrance of MPNST formation in fish overexpressing both the wild-type and the mutant PDGFRA transgenes in cells of neural crest origin. Interestingly, overexpression of the wild-type PDGFRA was even more potent in promoting transformation than the mutant PDGFRA, which is important because ~78% of human MPNSTs have expression of wild-type PDGFRA, whereas only 5% harbor activating mutations of the gene encoding this receptor. Further analysis revealed the induction of cellular senescence in zebrafish embryos overexpressing mutant, but not wild-type, PDGFRA, suggesting a mechanism through which the oncogenic activity of the mutant receptor is tempered by the activation of premature cellular senescence in an NF1-deficient background. Taken together, our study suggests a model in which overexpression of wild-type PDGFRA associated with NF1 deficiency leads to aberrant activation of downstream RAS signaling and thus contributes importantly to MPNST development-a prediction supported by the ability of the kinase inhibitor sunitinib alone and in combination with the MEK inhibitor trametinib to retard MPNST progression in

  4. Streptozotocin-induced diabetes, and the optic nerve blood barrier.

    Science.gov (United States)

    Alemán, R; Mompeó, B; Castaño, I

    2016-04-01

    To study the features of the endoneurial micro-vessels of the optic nerve in streptozotocin-induced diabetic animals. Optic nerves from control and streptozotocin-induced diabetic animals were studied by light and transmission electron microscopy. Patency was determined by indirect immunofluorescence albumin detection. The expression of major histocompatibility complex class II molecules was performed by direct immunofluorescence. The endoneurial vessels were counted, and the endothelial cell, the basement membrane, and the surface of the transverse section of the nerve were measured. Vessels of diabetic rats showed vessel wall thickening, preservation of pericytes, an increase in endothelial cell transcytosis, and an increased number of perivascular macrophage cells. It may be concluded that the effects of hyperglycaemia on the inner vessels of the optic nerve are more similar to the cerebral diabetic vessels than to the retinal vessels in diabetic animals. Copyright © 2016 Sociedad Española de Oftalmología. Published by Elsevier España, S.L.U. All rights reserved.

  5. Expression profiling of 519 kinase genes in matched malignant peripheral nerve sheath tumor/plexiform neurofibroma samples is discriminatory and identifies mitotic regulators BUB1B, PBK and NEK2 as overexpressed with transformation.

    Science.gov (United States)

    Stricker, Thomas P; Henriksen, Kammi J; Tonsgard, James H; Montag, Anthony G; Krausz, Thomas N; Pytel, Peter

    2013-07-01

    About 50% of all malignant peripheral nerve sheath tumors (MPNSTs) arise as neurofibromatosis type 1 associated lesions. In those patients malignant peripheral nerve sheath tumors are thought to arise through malignant transformation of a preexisting plexiform neurofibroma. The molecular changes associated with this transformation are still poorly understood. We sought to test the hypothesis that dysregulation of expression of kinases contributes to this malignant transformation. We analyzed expression of all 519 kinase genes in the human genome using the nanostring nCounter system. Twelve cases of malignant peripheral nerve sheath tumor arising in a background of preexisting plexiform neurofibroma were included. Both components were separately sampled. Statistical analysis compared global changes in expression levels as well as changes observed in the pairwise comparison of samples taken from the same surgical specimen. Immunohistochemical studies were performed on tissue array slides to confirm expression of selected proteins. The expression pattern of kinase genes can separate malignant peripheral nerve sheath tumors and preexisting plexiform neurofibromas. The majority of kinase genes is downregulated rather than overexpressed with malignant transformation. The patterns of expression changes are complex without simple recurring alteration. Pathway analysis demonstrates that differentially expressed kinases are enriched for kinases involved in the direct regulation of mitosis, and several of these show increased expression in malignant peripheral nerve sheath tumors. Immunohistochemical studies for the mitotic regulators BUB1B, PBK and NEK2 confirm higher expression levels at the protein level. These results suggest that the malignant transformation of plexiform neurofibroma is associated with distinct changes in the expression of kinase genes. The patterns of these changes are complex and heterogeneous. There is no single unifying alteration. Kinases involved

  6. CT, MRI, and (18)F-FDG PET/CT findings of malignant peripheral nerve sheath tumor of the head and neck.

    Science.gov (United States)

    Kim, Ha Youn; Hwang, Ji Young; Kim, Hyung-Jin; Kim, Yi Kyung; Cha, Jihoon; Park, Gyeong Min; Kim, Sung Tae

    2017-10-01

    Background Malignant peripheral nerve sheath tumor (MPNST) is a highly malignant tumor and rarely occurs in the head and neck. Purpose To describe the imaging features of MPNST of the head and neck. Material and Methods We retrospectively analyzed computed tomography (CT; n = 14), magnetic resonance imaging (MRI; n = 16), and (18)F-FDG PET/CT (n = 5) imaging features of 18 MPNSTs of the head and neck in 17 patients. Special attention was paid to determine the nerve of origin from which the tumor might have arisen. Results All lesions were well-defined (n = 3) or ill-defined (n = 15) masses (mean, 6.1 cm). Lesions were at various locations but most commonly the neck (n = 8), followed by the intracranial cavity (n = 3), paranasal sinus (n = 2), and orbit (n = 2). The nerve of origin was inferred for 11 lesions: seven in the neck, two in the orbit, one in the cerebellopontine angle, and one on the parietal scalp. Attenuation, signal intensity, and enhancement pattern of the lesions on CT and MRI were non-specific. Necrosis/hemorrhage/cystic change within the lesion was considered to be present on images in 13 and bone change in nine. On (18)F-FDG PET/CT images, all five lesions demonstrated various hypermetabolic foci with maximum standard uptake value (SUVmax) from 3.2 to 14.6 (mean, 7.16 ± 4.57). Conclusion MPNSTs can arise from various locations in the head and neck. Though non-specific, a mass with an ill-defined margin along the presumed course of the cranial nerves may aid the diagnosis of MPSNT in the head and neck.

  7. Nerve sheath tumor, benign neurogenic slow-growing solitary neurilemmoma of the left ulnar nerve: A case and review of literature

    Directory of Open Access Journals (Sweden)

    Martin Andra Elena

    2016-06-01

    Full Text Available This paper represent a report of a case with ulnar nerve schwannoma (neurilemmoma, benign neurogenic slow-growing, tumors originating from Schwann cells along the course of a nerve (1 (2 (3. Schwannomas are the most common tumors of the peripheral nerves which occur in the adults (0.8–2% (5. Usually they progress slowly and so they can remain painless swellings for a few years before other symptoms appear. Most of these lesions could be diagnosed clinically, are mobile in the longitudinal plane along the course of the involved nerve but not in the transverse plane (7. EMG, MRI, and ultrasonography are useful tools in the diagnosis. The definitive treatment of benign peripheral nerve schwannomatosis is complete enucleation of the tumor mass without damaging the intact nerve fascicles followed by confirmatory hystopathological examination (12. We present the case of a 62 years old right hand-dominant female who notice a slow increasing bulge over the inner aspect of her distal volar left forearm superior to the wrist, for a longer period of time not exactly specified; this was tracked and associated by pain, tingling and numbness over inner one and half fingers of her left hand in progress until the presentations. A diagnosis of soft-tissue tumor was presumed clinically. The other investigations were ultrasonography (US, nerve conduction studies (NCSs such as sensory nerve action potential (SNAP and compound muscle action potential (CMAP. In this case IRM was suggestive of a benign growth in her left ulnar nerve in the forearm region. Microsurgical techniques were used for ample enucleation of the tumor the distal volar left forearm. Subsequent histopathological examination confirmed the presumed diagnosis of a benign cellular schwannoma. At her last follow-up one month after surgery, the patient was neurological gradually improving sensory and motor function and she is highly satisfied with the results of surgery.

  8. Spatially- and temporally-controlled postnatal p53 knockdown cooperates with embryonic Schwann cell precursor Nf1 gene loss to promote malignant peripheral nerve sheath tumor formation.

    Science.gov (United States)

    Hirbe, Angela C; Dahiya, Sonika; Friedmann-Morvinski, Dinorah; Verma, Inder M; Clapp, D Wade; Gutmann, David H

    2016-02-16

    Malignant peripheral nerve sheath tumors (MPNSTs) are highly aggressive sarcomas that arise sporadically or in association with the Neurofibromatosis type 1 (NF1) cancer predisposition syndrome. In individuals with NF1, MPNSTs are hypothesized to arise from Nf1-deficient Schwann cell precursor cells following the somatic acquisition of secondary cooperating genetic mutations (e.g., p53 loss). To model this sequential genetic cooperativity, we coupled somatic lentivirus-mediated p53 knockdown in the adult right sciatic nerve with embryonic Schwann cell precursor Nf1 gene inactivation in two different Nf1 conditional knockout mouse strains. Using this approach, ~60% of mice with Periostin-Cre-mediated Nf1 gene inactivation (Periostin-Cre; Nf1(flox/flox) mice) developed tumors classified as low-grade MPNSTs following p53 knockdown (mean, 6 months). Similarly, ~70% of Nf1+/- mice with GFAP-Cre-mediated Nf1 gene inactivation (GFAP-Cre; Nf1(flox/null) mice) developed low-grade MPNSTs following p53 knockdown (mean, 3 months). In addition, wild-type and Nf1+/- mice with GFAP-Cre-mediated Nf1 loss develop MPNSTs following somatic p53 knockout with different latencies, suggesting potential influences of Nf1+/- stromal cells in MPNST pathogenesis. Collectively, this new MPNST model system permits the analysis of somatically-acquired events as well as tumor microenvironment signals that potentially cooperate with Nf1 loss in the development and progression of this deadly malignancy.

  9. Primary Gliosarcoma of the Optic Nerve: A Unique Adult Optic Pathway Glioma.

    Science.gov (United States)

    Cimino, Patrick J; Sychev, Yevgeniy V; Gonzalez-Cuyar, Luis F; Mudumbai, Raghu C; Keene, C Dirk

    2016-10-11

    A 90-year-old woman presented with 1-year history of right-sided progressive proptosis, neovascular glaucoma, blindness, and worsening ocular pain. No funduscopic examination was possible because of a corneal opacity. Head CT scan without contrast demonstrated a heterogeneous 4.1 cm (anterior-posterior) by 1.7 cm (transverse) cylindrical mass arising in the right optic nerve and extending from the retrobulbar globe to the optic canal. She underwent palliative enucleation with subtotal resection of the orbital optic nerve and tumor. Pathological examination showed effacement of the optic nerve by an infiltrative high-grade glial neoplasm with biphasic sarcomeric differentiation. Invasion into the uvea and retina was present. The neoplasm was negative for melan-A, HMB45, tyrosinase, synaptophysin, smooth muscle actin, and epithelial membrane antigen. The glioma had strongly intense, but patchy immunopositivity for glial fibrillary acidic protein. Multiple foci of neoplastic cells had pericellular reticulin staining. The overall features were diagnostic of a gliosarcoma (World Health Organization grade IV) of the optic nerve. Postoperative MRI demonstrated postsurgical changes and residual gliosarcoma with extension into the optic chiasm. The patient died 2 and a half months after her enucleation surgery at her nursing home. Autopsy was unavailable due to the caregiver wishes, making a definitive cause of death unknown. Gliosarcoma is a rare variant of glioblastoma, and this is the first documented case presenting as a primary neoplasm of the optic nerve.

  10. Experimental optic neuritis induced by the microinjection of lipopolysaccharide into the optic nerve.

    Science.gov (United States)

    Aranda, Marcos L; Dorfman, Damián; Sande, Pablo H; Rosenstein, Ruth E

    2015-04-01

    Optic neuritis (ON) is a condition involving primary inflammation, demyelination, and axonal injury in the optic nerve which leads to retinal ganglion cell (RGC) loss, and visual dysfunction. We investigated the ability of a single microinjection of bacterial lipopolysaccharide (LPS) directly into the optic nerve to induce functional and structural alterations compatible with ON. For this purpose, optic nerves from male Wistar rats remained intact or were injected with vehicle or LPS. The effect of LPS was evaluated at several time points post-injection in terms of: i) visual pathway and retinal function (visual evoked potentials (VEPs) and electroretinograms, (ERGs), respectively), ii) anterograde transport from the retina to its projection areas, iii) consensual pupil light reflex (PLR), iv) optic nerve histology, v) microglia/macrophage reactivity (by Iba-1- and ED1-immunostaining), vi) astrocyte reactivity (by glial fibrillary acid protein-immunostaining), vii) axon number (by toluidine blue staining), vii) demyelination (by myelin basic protein immunoreactivity and luxol fast blue staining), viii) optic nerve ultrastructure, and ix) RGC number (by Brn3a immunoreactivity). LPS induced a significant and persistent decrease in VEP amplitude and PLR, without changes in the ERG. In addition, LPS induced a deficit in anterograde transport, and an early inflammatory response consisting in an increased cellularity, and Iba-1 and ED1-immunoreactivity in the optic nerve, which were followed by changes in axonal density, astrocytosis, demyelination, and axon and RGC loss. These results suggest that the microinjection of LPS into the optic nerve may serve as a new experimental model of primary ON.

  11. CT and MR Manifestations of Malignant Peripheral Nerve Sheath Tumor in Spine%脊柱恶性外周神经鞘瘤的CT和MRI表现

    Institute of Scientific and Technical Information of China (English)

    郎宁; 刘晓光; 袁慧书; 谢敬霞

    2011-01-01

    Objective To explore ihe CT and MRI manifestations of malignant peripheral nerve sheath tumor ( MPNST) located in spine and to improve the preoperative diagnostic accuracy. Materials and Methods The CT (n = 10} and/or MRI( n =11) data of 12 cases of MPNST proved by pathological results were analyzed retrospectively. Results MPNST manifested as circular ( n = 2 ) or irregularly tabulated tumors ( n = 10). Three cases showed inconspicuous bone destruction and 9 cases showed adjacent vertebrae invasion, in which mainly invaded the pedicle of vertebral arch and vertebral laminae adjacent to ihe neural foramens as well as vertebral bodies adjacent to tumors. The bone destruction was mainly osteolytic destruction ( n = 8) - Coexistence of osteolytic destruction and irregular tumor bone were seen in one case. The tumors were isodensity(n =4) ,coexistence of iso/hypo-density (n =4) ,or iso/hyper-density (n = 2) on plain CT imaging. The tumors appeared as hyper-intensity on T2 WI and hypo-intensity on T1WI. There were patchy hyper intensity areas (n = 8 ) , punctu-al/stripy hypo-intensity areas (n =2) ,clustered vessel signals (n = 1 ) in tumors on T2WI. Inhomogeneous enhancement was seen on contrast-enhanced scan in all the cases- Conclusion The imaging features of tumors included enlarged neural foramens,obvious peripheral bone destruction, inhomogeneous enhancement with non enhanced necrotic area, especially connected with spinal nerve,will help to make a diagnosis of malignant peripheral nerve sheath tumor.%目的 分析脊柱恶性外周神经鞘瘤(maligrant peripheral nerve sheath tumor,MPNST)的CT和MRI表现,提高术前对该病诊断的准确性.资料与方法 回顾性分析12例经病理证实的脊柱MPNST的CT(n =10)和/或MRI(n=11)表现.结果 MPNST表现为类圆形(n=2)或不规则分叶状(n=10)肿块.3例未见明显骨质破坏,9例有邻近椎骨侵犯,主要侵犯神经孔相邻的椎弓根、椎板及相邻局部椎体,骨质破

  12. Optic nerve head biomechanics in aging and disease.

    Science.gov (United States)

    Downs, J Crawford

    2015-04-01

    This nontechnical review is focused upon educating the reader on optic nerve head biomechanics in both aging and disease along two main themes: what is known about how mechanical forces and the resulting deformations are distributed in the posterior pole and ONH (biomechanics) and what is known about how the living system responds to those deformations (mechanobiology). We focus on how ONH responds to IOP elevations as a structural system, insofar as the acute mechanical response of the lamina cribrosa is confounded with the responses of the peripapillary sclera, prelaminar neural tissues, and retrolaminar optic nerve. We discuss the biomechanical basis for IOP-driven changes in connective tissues, blood flow, and cellular responses. We use glaucoma as the primary framework to present the important aspects of ONH biomechanics in aging and disease, as ONH biomechanics, aging, and the posterior pole extracellular matrix (ECM) are thought to be centrally involved in glaucoma susceptibility, onset and progression.

  13. Optical Feedback Control and Electrical-Optical Costimulation of Peripheral Nerves.

    Science.gov (United States)

    Kapur, Sahil K; Richner, Thomas J; Brodnick, Sarah K; Williams, Justin C; Poore, Samuel O

    2016-09-01

    Optogenetics is an emerging technology that enables the expression of light-activated ion channels in mammalian cells. Neurons expressing light-activated ion channels can be depolarized using the appropriate wavelength of light. Optical stimulation of neurons could have important implications for further understanding and managing peripheral nerve deficits leading to paresis or paralysis. This study examines the utility of this technology in a feedback-controlled system and the advantages of coupling this technology with conventional electrical stimulation. The sciatic nerves of transgenic mice expressing blue light-activated ion channels (channelrhodopsin-2) were optically manipulated to generate electromyographic responses in the gastrocnemius muscle and to develop two potential applications of this technology: feedback-controlled optical stimulation using a proportional-integral controller, and simultaneous electrical-optical stimulation. The authors observed repeatable and predictable behavior of the optical controller in over 200 trials and a statistically significant decreased error when using optical feedback control as opposed to non-feedback controlled stimulation (n = 6 limbs). A second application of this technology was the amplification of electrically generated peripheral nerve signals using an optical source. Amplification of electrical activity was observed even when subthreshold electrical stimulation was used. Optical feedback control and optical amplification of subthreshold activity extend the versatility of optogenetics in peripheral nerve applications. Optical feedback control is a new application of an approach originally developed for functional electrical stimulation. Optical amplification of subthreshold electrical stimulation motivates future investigations into the optical amplification of endogenous subthreshold peripheral nerve activity (e.g., following spinal cord injury).

  14. Probabilistic Modeling of Intracranial Pressure Effects on Optic Nerve Biomechanics

    Science.gov (United States)

    Ethier, C. R.; Feola, Andrew J.; Raykin, Julia; Myers, Jerry G.; Nelson, Emily S.; Samuels, Brian C.

    2016-01-01

    Altered intracranial pressure (ICP) is involved/implicated in several ocular conditions: papilledema, glaucoma and Visual Impairment and Intracranial Pressure (VIIP) syndrome. The biomechanical effects of altered ICP on optic nerve head (ONH) tissues in these conditions are uncertain but likely important. We have quantified ICP-induced deformations of ONH tissues, using finite element (FE) and probabilistic modeling (Latin Hypercube Simulations (LHS)) to consider a range of tissue properties and relevant pressures.

  15. Optic nerve infiltration by acute lymphoblastic leukemia: MRI contribution

    Energy Technology Data Exchange (ETDEWEB)

    Soares, Maria de Fatima; Braga, Flavio Tulio [Federal University of Sao Paulo, Department of Diagnostic Imaging, Paulista School of Medicine, Sao Paulo (Brazil); Rocha, Antonio Jose da [Santa Casa de Misericordia de Sao Paulo, Servico de Diagnostico por Imagem, Sao Paulo (Brazil); Lederman, Henrique Manoel [Federal University of Sao Paulo, Division of Diagnostic Imaging in Pediatrics, Department of Diagnostic Imaging, Sao Paulo (Brazil)

    2005-08-01

    We describe the clinical presentation and imaging features of a patient with acute lymphoblastic leukemia (ALL) that was complicated by optic nerve infiltration. The clinical and diagnostic characteristics of this complication must be recognized so that optimal therapy can be started to prevent blindness. MR imaging is useful in early detection and should be performed in any leukemic patient with ocular complaints, even during remission. (orig.)

  16. Optic nerve and chiasm enlargement in a case of infantile Krabbe disease: quantitative comparison with 26 age-matched controls

    Energy Technology Data Exchange (ETDEWEB)

    Patel, Bhairav; Gimi, Barjor [University of Texas Southwestern Medical Center at Dallas, Department of Radiology, Dallas, TX (United States); Vachha, Behroze [University of Texas Southwestern Medical Center at Dallas, Department of Developmental Disabilities, Dallas, TX (United States); Agadi, Satish [Children' s Medical Center Dallas, Department of Neurology, Dallas, TX (United States); Koral, Korgun [University of Texas Southwestern Medical Center at Dallas, Department of Radiology, Dallas, TX (United States); Children' s Medical Center Dallas, Department of Radiology, Dallas, TX (United States)

    2008-06-15

    Hypertrophy of the optic nerves and optic chiasm is described in a 5-month-old boy with infantile Krabbe disease. Optic nerve and optic chiasm hypertrophy is a rarely described feature of Krabbe disease. The areas of the prechiasmatic optic nerves and optic chiasm were measured and compared with those of 26 age-matched controls. The areas of the prechiasmatic optic nerves and optic chiasm were 132% and 53% greater than normal, respectively. (orig.)

  17. Bilateral Traumatic Globe Luxation with Optic Nerve Transection

    Directory of Open Access Journals (Sweden)

    Levent Tok

    2014-12-01

    Full Text Available Purpose: The purpose of this study was to document clinical findings and management of a patient with bilateral globe luxation and optic nerve transection. Materials and Methods: A 25-year-old female patient was admitted to the emergency department with bilateral traumatic globe luxation following a motor vehicle accident. Results: Visual acuity testing showed no light perception. The right pupil was dilated and bilaterally did not react to light. The globes were bilaterally intact. A computed tomography scan revealed Le Fort type II fractures, bilateral optic nerve transection and disruption of all extraocular muscles. The globes of the patient were bilaterally reduced into the orbit. However, the patient developed phthisis bulbi in the right eye at month 3. Conclusion: Globe luxation presents a dramatic clinical picture, and may lead to the development of severe complications due to the concomitance of complete optic nerve dissection and multiple traumas. Even if the luxated globe is repositioned into the orbit, there is still an increased risk of the development of phthisis due to ischemia.

  18. Detection of optic nerve lesions in optic neuritis using frequency-selective fat-saturation sequences

    Energy Technology Data Exchange (ETDEWEB)

    Miller, D.H. (NMR Research Unit, National Hospital for Neurology and Neurosurgery, London (United Kingdom)); MacManus, D.G. (NMR Research Unit, National Hospital for Neurology and Neurosurgery, London (United Kingdom)); Bartlett, P.A. (St. Mary' s Hospital, London (United Kingdom)); Kapoor, R. (NMR Research Unit, National Hospital for Neurology and Neurosurgery, London (United Kingdom)); Morrissey, S.P. (NMR Research Unit, National Hospital for Neurology and Neurosurgery, London (United Kingdom)); Moseley, I.F. (NMR Research Unit, National Hospital for Neurology and Neurosurgery, London (United Kingdom))

    1993-02-01

    MRI was performed on seven patients with acute optic neuritis, using two sequences which suppress the signal from orbital fat: frequency-selective fat-saturation and inversion recovery with a short inversion time. Lesions were seen on both sequences in all the symptomatic optic nerves studied. (orig.)

  19. Remyelination of optic nerve lesions: spatial and temporal factors.

    Science.gov (United States)

    Klistorner, Alexandr; Arvind, Hemamalini; Garrick, Raymond; Yiannikas, Con; Paine, Mark; Graham, Stuart L

    2010-07-01

    Optic neuritis provides an in vivo model to study demyelination. The effects of myelin loss and recovery can be measured by the latency of the multifocal visual evoked potentials. We investigated whether the extent of initial inflammatory demyelination in optic neuritis correlates with the remyelinating capacity of the optic nerve. Forty subjects with acute unilateral optic neuritis and good visual recovery underwent multifocal visual evoked potentials testing at 1, 3, 6 and 12 months. Average latency changes were analyzed. Extensive latency delay at baseline significantly improved over time with rate of recovery slowed down after 6 months. Magnitude of latency recovery was independent of initial latency delay. Latency recovery ranged from 7 to 17 ms across the whole patient cohort (average = 11.3 (3.1) ms) despite the fact that in a number of cases the baseline latency delay was more than 35-40 ms. Optic nerve lesions tend to remyelinate at a particular rate irrespective of the size of the initial demyelinated zone with smaller lesions accomplishing recovery more completely. The extent of the initial inflammatory demyelination is probably the single most important factor determining completeness of remyelination. The time period favorable to remyelination is likely to be within the first 6 months after the attack.

  20. LHON and other optic nerve atrophies: the mitochondrial connection.

    Science.gov (United States)

    Howell, Neil

    2003-01-01

    The clinical, biochemical and genetic features of Leber's hereditary optic neuropathy (LHON) are reviewed. The etiology of LHON is complex, but the primary risk factor is a mutation in one of the seven mitochondrial genes that encode subunits of respiratory chain complex I. The pathogenesis of LHON is not yet understood, but one plausible model is that increased or altered mitochondrial ROS production renders the retinal ganglion cells vulnerable to apoptotic cell death. In addition to LHON, there are a large number of other optic nerve degenerative disorders including autosomal dominant optic atrophy, the toxic/nutritional optic neuropathies and glaucoma. A review of the recent scientific literature suggests that these disorders also involve mitochondrial dysfunction or altered mitochondrial signaling pathways in their pathogenesis. This mitochondrial link provides new avenues of experimental investigation to these major causes of loss of vision.

  1. Chemical shift selective magnetic resonance imaging of the optic nerve in patients with acute optic neuritis

    Energy Technology Data Exchange (ETDEWEB)

    Larsson, H.B.W.; Thomsen, C.; Frederiksen, J.; Henriksen, O.; Olesen, J.

    Optic neuritis is often the first manifestion of multiple sclerosis (MS). Sixteen patients with acute optic neuritis and one patient with benign intracranial hypertension (BIH) were investigated by magnetic resonance imaging, using a chemical shift selective double spin echo sequence. In 3 of the 16 patients, abnormalities were seen. In one patient with bilateral symptoms, signal hyperintensity and swelling of the right side of the chiasm were found. In another patient the optic nerve was found diffusely enlarged with only a marginally increased signal in the second echo. In the third patient an area of signal hyperintensity and swelling was seen in the left optic nerve. In the patient with BIH the subarachnoid space which surrounds the optic nerves was enlarged. Even using this refined pulse sequence, avoiding the major artefact in imaging the optic nerve, the chemical shift artefact, lesions were only shown in 3/16 (19%) of the patients with optic neuritis. Nevertheless, the presented chemical shift selective double spin echo sequence may be of great value for detection of retrobulbar lesions.

  2. Optic nerve atrophy and retinal nerve fibre layer thinning following optic neuritis: evidence that axonal loss is a substrate of MRI-detected atrophy.

    Science.gov (United States)

    Trip, S Anand; Schlottmann, Patricio G; Jones, Stephen J; Li, Wai-Yung; Garway-Heath, David F; Thompson, Alan J; Plant, Gordon T; Miller, David H

    2006-05-15

    Magnetic resonance imaging (MRI) measures of brain atrophy are often considered to be a marker of axonal loss in multiple sclerosis (MS) but evidence is limited. Optic neuritis is a common manifestation of MS and results in optic nerve atrophy. Retinal nerve fibre layer (RNFL) imaging is a non-invasive way of detecting axonal loss following optic neuritis. We hypothesise that if the optic nerve atrophy that develops following optic neuritis is contributed to by axonal loss, it will correlate with thinning of the RNFL. Twenty-five patients were studied at least 1 year after a single unilateral attack of optic neuritis without recurrence, with a selection bias towards incomplete recovery. They had MR quantification of optic nerve cross-sectional area and optic nerve lesion length, as well as optical coherence tomography (OCT) measurement of mean RNFL thickness and macular volume, quantitative visual testing, and visual evoked potentials (VEPs). Fifteen controls were also studied. Significant optic nerve atrophy (mean decrease 30% versus controls), RNFL thinning (mean decrease 33% versus controls), and macular volume loss occurred in patients' affected eyes when compared with patients' unaffected eyes and healthy controls. The optic nerve atrophy was correlated with the RNFL thinning, macular volume loss, visual acuity, visual field mean deviation, and whole field VEP amplitude but not latency. These findings suggest that axonal loss contributes to optic nerve atrophy following a single attack of optic neuritis. By inference, axonal loss due to other post-inflammatory brain lesions is likely to contribute to the global MRI measure of brain atrophy in multiple sclerosis.

  3. The value of magnetic resonance imaging in the differentiation between malignant peripheral nerve-sheath tumors and non-neurogenic malignant soft-tissue tumors

    Energy Technology Data Exchange (ETDEWEB)

    Herendael, B.H. van; Heyman, S.R.G.; Vanhoenacker, F.M.; Parizel, P.M.; Schepper, A.M. de [University Hospital of Antwerp, Department of Radiology, Edegem (Belgium); Temmerman, G. de; Bloem, J.L. [Leiden University Medical Center, Department of Radiology, Leiden (Netherlands)

    2006-10-15

    To assess the sensitivity and specificity of MRI criteria in the differentiation between malignant peripheral nerve sheath tumors (MPNST) and non-neurogenic malignant soft-tissue tumors (MSTT). MRI examinations of 105 patients with pathologically proven malignant soft-tissue lesions (35 MPNST and 70 MSTT) were retrospectively reviewed, the reviewers being unaware of the pathological diagnosis. Using a standardized protocol, the tumors were evaluated for multiple parameters regarding morphology and appearance on different sequences before and after gadolinium contrast administration (location, distribution, delineation, homogeneity, size, shape, relationship to bone and neurovascular bundle, intralesional hemorrhage, necrosis, perilesional edema, lymphangitis and signal intensities). Results were compared using a chi-square or Fisher's exact test. MRI findings suggestive of MPNST (p<0,05) were intermuscular distribution, location on the course of a large nerve, nodular morphology, and overall non-homogeneity on T1-weighted images, T2-weighted images and T1-weighted images after gadolinium contrast injection. MRI findings in favor of MSTT were intramuscular distribution, ill-delineated appearance of more than 20% of the lesion's circumference, and presence of intralesional blood vessels, perilesional edema and lymphangitis. There is no significant difference for degree and pattern of enhancement after gadolinium contrast injection, nor for presence of bone involvement or cystic or necrotic areas. MRI provides several features that contribute to the differentiation between MPNST and non-neurogenic malignant soft-tissue tumors. MRI findings suggestive of MPNST should be helpful to pathologists in the strategy for further examination. (orig.)

  4. Optic nerve diffusion tensor imaging after acute optic neuritis predicts axonal and visual outcomes.

    Science.gov (United States)

    van der Walt, Anneke; Kolbe, Scott C; Wang, Yejun E; Klistorner, Alexander; Shuey, Neil; Ahmadi, Gelareh; Paine, Mark; Marriott, Mark; Mitchell, Peter; Egan, Gary F; Butzkueven, Helmut; Kilpatrick, Trevor J

    2013-01-01

    Early markers of axonal and clinical outcomes are required for early phase testing of putative neuroprotective therapies for multiple sclerosis (MS). To assess whether early measurement of diffusion tensor imaging (DTI) parameters (axial and radial diffusivity) within the optic nerve during and after acute demyelinating optic neuritis (ON) could predict axonal (retinal nerve fibre layer thinning and multi-focal visual evoked potential amplitude reduction) or clinical (visual acuity and visual field loss) outcomes at 6 or 12 months. Thirty-seven patients presenting with acute, unilateral ON were studied at baseline, one, three, six and 12 months using optic nerve DTI, clinical and paraclinical markers of axonal injury and clinical visual dysfunction. Affected nerve axial diffusivity (AD) was reduced at baseline, 1 and 3 months. Reduced 1-month AD correlated with retinal nerve fibre layer (RNFL) thinning at 6 (R=0.38, p=0.04) and 12 months (R=0.437, p=0.008) and VEP amplitude loss at 6 (R=0.414, p=0.019) and 12 months (R=0.484, p=0.003). AD reduction at three months correlated with high contrast visual acuity at 6 (ρ = -0.519, p = 0.001) and 12 months (ρ = -0.414, p=0.011). The time-course for AD reduction for each patient was modelled using a quadratic regression. AD normalised after a median of 18 weeks and longer normalisation times were associated with more pronounced RNFL thinning and mfVEP amplitude loss at 12 months. Affected nerve radial diffusivity (RD) was unchanged until three months, after which time it remained elevated. These results demonstrate that AD reduces during acute ON. One month AD reduction correlates with the extent of axonal loss and persistent AD reduction at 3 months predicts poorer visual outcomes. This suggests that acute ON therapies that normalise optic nerve AD by 3 months could also promote axon survival and improve visual outcomes.

  5. Wilms' tumor gene 1 (WT1 silencing inhibits proliferation of malignant peripheral nerve sheath tumor sNF96.2 cell line.

    Directory of Open Access Journals (Sweden)

    Rosalba Parenti

    Full Text Available Wilms' tumor gene 1 (WT1 plays complex roles in tumorigenesis, acting as tumor suppressor gene or an oncogene depending on the cellular context. WT1 expression has been variably reported in both benign and malignant peripheral nerve sheath tumors (MPNSTs by means of immunohistochemistry. The aim of the present study was to characterize its potential pathogenetic role in these relatively uncommon malignant tumors. Firstly, immunohistochemical analyses in MPNST sNF96.2 cell line showed strong WT1 staining in nuclear and perinuclear areas of neoplastic cells. Thus, we investigated the effects of silencing WT1 by RNA interference. Through Western Blot analysis and proliferation assay we found that WT1 knockdown leads to the reduction of cell growth in a time- and dose-dependent manner. siWT1 inhibited proliferation of sNF96.2 cell lines likely by influencing cell cycle progression through a decrease in the protein levels of cyclin D1 and inhibition of Akt phosphorylation compared to the control cells. These results indicate that WT1 knockdown attenuates the biological behavior of MPNST cells by decreasing Akt activity, demonstrating that WT1 is involved in the development and progression of MPNSTs. Thus, WT1 is suggested to serve as a potential therapeutic target for MPNSTs.

  6. The Challenge of Cancer Genomics in Rare Nervous System Neoplasms: Malignant Peripheral Nerve Sheath Tumors as a Paradigm for Cross-Species Comparative Oncogenomics.

    Science.gov (United States)

    Carroll, Steven L

    2016-03-01

    Comprehensive genomic analyses of common nervous system cancers provide new insights into their pathogenesis, diagnosis, and treatment. Although analogous studies of rare nervous system tumors are needed, there are major barriers to performing such studies. Cross-species comparative oncogenomics, identifying driver mutations in mouse cancer models and validating them in human tumors, is a promising alternative. Although still in its infancy, this approach is being applied to malignant peripheral nerve sheath tumors (MPNSTs), rare Schwann cell-derived malignancies that occur sporadically, after radiotherapy, and in neurofibromatosis type 1. Studies of human neurofibromatosis type 1-associated tumors suggest that NF1 tumor suppressor loss in Schwann cells triggers cell-autonomous and intercellular changes, resulting in development of benign neurofibromas; subsequent neurofibroma-MPNST progression is caused by aberrant growth factor signaling and mutations affecting the p16(INK4A)-cyclin D1-CDK4-Rb and p19(ARF)-Mdm2-p53 cell cycle pathways. Mice with Nf1, Trp53, and/or Cdkn2a mutations that overexpress the Schwann cell mitogen neuregulin-1 or overexpress the epidermal growth factor receptor validate observations in human tumors and, to various degrees, model human tumorigenesis. Genomic analyses of MPNSTs arising in neuregulin-1 and epidermal growth factor receptor-overexpressing mice and forward genetic screens with Sleeping Beauty transposons implicate additional signaling cascades in MPNST pathogenesis. These studies confirm the utility of mouse models for MPNST driver gene discovery and provide new insights into the complexity of MPNST pathogenesis.

  7. Malignant Peripheral Nerve Sheath Tumor in the Lesser Sac Masquerading as a Gastrointestinal Stromal Tumor of the Stomach: A Case Report

    Directory of Open Access Journals (Sweden)

    Yeo

    2016-09-01

    Full Text Available A malignant peripheral nerve sheath tumor (MPNST is very rare, representing 3 - 10% of all soft tissue sarcomas. Approximately half of all such tumors are diagnosed in patients with neurofibromatosis type 1 (NF1. The extremities are involved most commonly, and the patient’s age is usually 20 - 50 years. Herein, we present a case of an MPNST that developed sporadically in an unusual location and in an unusually younger patient. A 16-year-old girl presented with dyspepsia and abdominal distension. Computed tomography (CT demonstrated a well-defined mass approximately 12 cm in diameter in the lesser sac. The mass was composed principally of a heterogeneously enhancing solid portion and exhibited some cystic changes. Clinically and radiologically, the mass appeared to be a gastrointestinal stromal tumor of the stomach. The patient underwent exploratory laparotomy and mass excision. The surgical specimen revealed a solid firm mass measuring 13 × 13 × 6 cm. Histopathological and immunohistochemical analyses identified an MPNST. The patient underwent adjuvant chemotherapy but developed local recurrence with peritoneal seeding 8 months after surgery. Despite treatment with a different chemotherapeutic regimen, the disease progressed systemically and the patient died 3 years and 5 months after surgery. The uncommon tumor location and CT findings of our present MPNST case provide valuable information in terms of future clinical diagnoses of this rare but highly malignant disease.

  8. Perineurial malignant peripheral nerve sheath tumor in the setting of multiple soft tissue perineuriomas: A rare presentation of an uncommon tumor

    Directory of Open Access Journals (Sweden)

    Bharat Rekhi

    2013-01-01

    Full Text Available Perineurioma is an uncommon soft tissue tumor with characteristic histological and immunohistochemical features. Herein, this tumor is presented within a rare clinical setting, in a 45-year-old gentleman, with multiple soft tissue swellings and a previous history of surgical excision of a thigh mass. Four years back, he developed multiple soft tissue tumor swellings for which he lately underwent multiple wide excisions, elsewhere that were reported as multiple dermatofibrosarcoma protuberans (DFSPs. Histopathological review of the thigh and axillary tumor showed a perineurioma (EMA+, S100-P-ve, CD34-ve, low MIB1, whereas the recurrent leg mass disclosed a high-grade perineurial malignant peripheral nerve sheath tumor (MPNST (EMA+, CD56+, S100-P-, high MIB1, CD34-ve, p53+ve. Within four months of post-excision, he developed additional swellings, and died of respiratory insufficiency. This case forms the first documented case of perineurial MPNST with multiple, metachronous soft tissue perineuriomas. Available literature review of perineurial MPNSTs and diagnostic implications are discussed herewith

  9. Sulindac derivatives inhibit cell growth and induce apoptosis in primary cells from malignant peripheral nerve sheath tumors of NF1-patients

    Directory of Open Access Journals (Sweden)

    Friedrich Reinhard E

    2004-05-01

    Full Text Available Abstract Background Malignant peripheral nerve sheath tumors (MPNSTs are neoplasms leading to death in most cases. Patients with Neurofibromatosis type 1 have an increased risk of developing this malignancy. The metabolites of the inactive prodrug Sulindac, Sulindac Sulfide and Sulindac Sulfone (Exisulind are new chemopreventive agents that show promising results in the treatment of different cancer types. In this study we examined the antineoplastic effect of these compounds on primary cells derived from two MPNSTs of Neurofibromatosis type 1 patients. Results Exisulind and Sulindac Sulfide showed a dramatic time- and dose-dependent growth inhibitory effect with IC50-values of 120 μM and 63 μM, respectively. The decrease in viability of the tested cells correlated with induction of apoptosis. Treatment with 500 μM Exisulind and 125 μM Sulindac Sulfide for a period of 2 days increased the rate of apoptosis 21-27-fold compared to untreated cells. Reduced expression of RAS-GTP and phosphorylated ERK1/2 was detected in treated MPNST cells. Moreover, elevated levels of phosphorylated SAPK/JNK were found after drug treatment, and low activation of cleaved caspase-3 was seen. Conclusions Our results suggest that this class of compounds may be of therapeutic benefit for Neurofibromatosis type 1 patients with MPNST.

  10. EZH2-miR-30d-KPNB1 pathway regulates malignant peripheral nerve sheath tumour cell survival and tumourigenesis.

    Science.gov (United States)

    Zhang, Pingyu; Garnett, Jeannine; Creighton, Chad J; Al Sannaa, Ghadah Abbas; Igram, Davis R; Lazar, Alexander; Liu, Xiuping; Liu, Changgong; Pollock, Raphael E

    2014-02-01

    Malignant peripheral nerve sheath tumours (MPNSTs), which develop sporadically or from neurofibromatosis, recur frequently with high metastatic potential and poor outcome. The polycomb group protein enhancer of zeste homologue 2 (EZH2) is an important regulator for various human malignancies. However, the function of EZH2 in MPNSTs is unknown. Here we report that the EZH2-miR-30d-KPNB1 signalling pathway is critical for MPNST tumour cell survival in vitro and tumourigenicity in vivo. Up-regulated EZH2 in MPNST inhibits miR-30d transcription via promoter binding activity, leading to enhanced expression of the nuclear transport receptor KPNB1 that is inhibited by miR-30d targeting of KPNB1 3' UTR region. Furthermore, inhibition of EZH2 or KPNB1, or miR-30d over-expression, induces MPNST cell apoptosis in vitro and suppresses tumourigenesis in vivo. More importantly, forced over-expression of KPNB1 rescues MPNST cell apoptosis induced by EZH2 knockdown. Immunohistochemical analyses show that EZH2 and KPNB1 over-expression is observed in human MPNST specimens and is negatively associated with miR-30d expression. Our findings identify a novel signalling pathway involved in MPNST tumourigenesis, and also suggest that EZH2-miR-30d-KPNB1 signalling represents multiple potential therapeutic targetable nodes for MPNST.

  11. [{sup 18}F]FDG PET/CT in the diagnosis of malignant peripheral nerve sheath tumours in neurofibromatosis type-1

    Energy Technology Data Exchange (ETDEWEB)

    Warbey, Victoria S.; O' Doherty, Michael J. [King' s College London, Clinical PET Centre, Guy' s and St Thomas' NHS Foundation Trust, London (United Kingdom); Ferner, Rosalie E. [King' s College London, Department of Neurology, Guy' s and St Thomas' Hospitals NHS Foundation Trust, London (United Kingdom); Dunn, Joel T. [King' s College London, Clinical PET Centre, Division of Imaging Sciences, Guy' s, King' s and St Thomas' School of Medicine, London (United Kingdom); Calonje, Eduardo [Guy' s and St Thomas' Hospitals NHS Foundation Trust, Department of Dermatopathology, London (United Kingdom)

    2009-05-15

    The detection of malignant peripheral nerve sheath tumours (MPNSTs) in patients with neurofibromatosis 1 (NF1) remains a clinical challenge. The purpose of this study was to evaluate the use of [{sup 18}F]2-fluoro-2-deoxy-d-glucose PET/CT (FDG PET/CT with early and delayed imaging) in patients with symptomatic neurofibromas, to revalidate current cut-off values for identification of malignant change within neurofibromas and to examine the relationship between SUV and tumour grade. Patients with symptomatic neurofibromas underwent FDG PET/CT imaging at 90 and 240 min. Semiquantitative analysis using maximum standardized uptake value (SUVmax) was performed and correlated with histology. In 69 patients, 85 lesions were identified for analysis, including 10 atypical neurofibromas and 21 MPNSTs. Sensitivity of FDG PET/CT in diagnosing NF1-associated MPNST was 0.97 (95% CI 0.81-0.99) and the specificity was 0.87 (CI 0.74-0.95). There was a significant difference in SUVmax between early and delayed imaging and in SUVmax between tumours identified as benign and malignant on PET/CT. There was also a significant difference in SUVmax between tumour grades. FDG PET/CT is a highly sensitive and specific imaging modality for the diagnosis of MPNST in NF1 patients. We recommend performing early (90 min) and delayed imaging at 4 h for accurate lesion characterization and using a cut-off SUVmax of 3.5 on delayed imaging to achieve maximal sensitivity. (orig.)

  12. Discovery of a small molecule targeting IRA2 deletion in budding yeast and neurofibromin loss in malignant peripheral nerve sheath tumor cells

    Science.gov (United States)

    Wood, Matthew; Rawe, Melissa; Johansson, Gunnar; Pang, Shu; Soderquist, Ryan S.; Patel, Ami V.; Nelson, Sandra; Seibel, William; Ratner, Nancy; Sanchez, Yolanda

    2011-01-01

    Malignant peripheral nerve sheath tumor (MPNST) is a life-threatening complication of neurofibromatosis type 1 (NF1). NF1 is caused by mutation in the gene encoding neurofibromin, a negative regulator of Ras signaling. There are no effective pharmacologic therapies for MPNST. To identify new therapeutic approaches targeting this dangerous malignancy, we developed assays in NF1+/+ and NF1−/ − MPNST cell lines and in budding yeast lacking the NF1 homologue IRA2 (ira2Δ). Here we describe UC1, a small molecule that targets NF1−/− cell lines and ira2Δ budding yeast. Using yeast genetics we identified NAB3 as a high-copy suppressor of UC1 sensitivity. NAB3 encodes an RNA binding protein that associates with the C-terminal domain of RNA Pol II and plays a role in the termination of non-polyadenylated RNA transcripts. Strains with deletion of IRA2 are sensitive to genetic inactivation of NAB3, suggesting an interaction between Ras signaling and Nab3-dependent transcript termination. This work identifies a lead compound and a possible target pathway for NF1-associated MPNST, and demonstrates a novel model system approach to identify and validate target pathways for cancer cells in which NF1 loss drives tumor formation. PMID:21697395

  13. Radiation-Induced Peripheral Malignant Nerve Sheath Tumor Arising from Vestibular Schwannoma after Linac-Based Stereotactic Radiation Therapy: A Case Report and Review of Literatures

    Directory of Open Access Journals (Sweden)

    Putipun Puataweepong

    2012-01-01

    Full Text Available In recent years the use of stereotactic radiation for vestibular schwannomas has increased worldwide. However, malignant transformation associated with radiation, although uncommon, has been reported in recent publications. We present a case of the 34 year-old female who had left vestibular schwannoma and who underwent surgery and postoperative stereotactic radiotherapy (SRT, hypofraction in 2005. At 6 years after SRT, the patient came with left facial palsy and severe headache. CT brain revealed progression in size with cystic and hemorrhagic changes of the preexisting tumor at left CPA with new obstructive hydrocephalus. Partial tumor removal was done, and the pathological report was malignant peripheral nerve sheath tumor (MPNST. Regarding the uncertainty of carcinogenesis risk, we should still practice radiation therapy with caution, especially in the young patient with tumor predisposition syndrome. Because of low incidence of MPNST after radiation, it should not be a major decision about giving radiotherapy. However, with the poor prognosis of MPNST, this possibility should be explained to the patient before radiation treatment option.

  14. Intracranial Pressure Influences the Behavior of the Optic Nerve Head.

    Science.gov (United States)

    Hua, Yi; Tong, Junfei; Ghate, Deepta; Kedar, Sachin; Gu, Linxia

    2017-03-01

    In this work, the biomechanical responses of the optic nerve head (ONH) to acute elevations in intracranial pressure (ICP) were systematically investigated through numerical modeling. An orthogonal experimental design was developed to quantify the influence of ten input factors that govern the anatomy and material properties of the ONH on the peak maximum principal strain (MPS) in the lamina cribrosa (LC) and postlaminar neural tissue (PLNT). Results showed that the sensitivity of ONH responses to various input factors was region-specific. In the LC, the peak MPS was most strongly dependent on the sclera thickness, LC modulus, and scleral canal size, whereas in the PLNT, the peak MPS was more sensitive to the scleral canal size, neural tissue modulus, and pia mater modulus. The enforcement of clinically relevant ICP in the retro-orbital subarachnoid space influenced the sensitivity analysis. It also induced much larger strains in the PLNT than in the LC. Moreover, acute elevation of ICP leads to dramatic strain distribution changes in the PLNT, but had minimal impact on the LC. This work could help to better understand patient-specific responses, to provide guidance on biomechanical factors resulting in optic nerve diseases, such as glaucoma, papilledema, and ischemic optic neuropathy, and to illuminate the possibilities for exploiting their potential to treat and prevent ONH diseases.

  15. Hypothalamic dysfunction without hamartomas causing gelastic seizures in optic nerve hypoplasia.

    Science.gov (United States)

    Fink, Cassandra; Borchert, Mark; Simon, Carrie Zaslow; Saper, Clifford

    2015-02-01

    This report describes gelastic seizures in patients with optic nerve hypoplasia and hypothalamic dysfunction without hypothalamic hamartoma. All participants (n = 4) from the optic nerve hypoplasia registry study at Children's Hospital Los Angeles presenting with gelastic seizures were included. The clinical and pathology characteristics include hypothalamic dysgenesis and dysfunction, but no hamartomas. Optic nerve hypoplasia is the only reported condition with gelastic seizures without hypothalamic hamartomas, suggesting that hypothalamic disorganization alone can cause gelastic seizures.

  16. Dynamic Modulation of Myelination in Response to Visual Stimuli Alters Optic Nerve Conduction Velocity

    Science.gov (United States)

    Etxeberria, Ainhoa; Hokanson, Kenton C.; Dao, Dang Q.; Mayoral, Sonia R.; Mei, Feng; Redmond, Stephanie A.; Ullian, Erik M.

    2016-01-01

    Myelin controls the time required for an action potential to travel from the neuronal soma to the axon terminal, defining the temporal manner in which information is processed within the CNS. The presence of myelin, the internodal length, and the thickness of the myelin sheath are powerful structural factors that control the velocity and fidelity of action potential transmission. Emerging evidence indicates that myelination is sensitive to environmental experience and neuronal activity. Activity-dependent modulation of myelination can dynamically alter action potential conduction properties but direct functional in vivo evidence and characterization of the underlying myelin changes is lacking. We demonstrate that in mice long-term monocular deprivation increases oligodendrogenesis in the retinogeniculate pathway but shortens myelin internode lengths without affecting other structural properties of myelinated fibers. We also demonstrate that genetically attenuating synaptic glutamate neurotransmission from retinal ganglion cells phenocopies the changes observed after monocular deprivation, suggesting that glutamate may constitute a signal for myelin length regulation. Importantly, we demonstrate that visual deprivation and shortened internodes are associated with a significant reduction in nerve conduction velocity in the optic nerve. Our results reveal the importance of sensory input in the building of myelinated fibers and suggest that this activity-dependent alteration of myelination is important for modifying the conductive properties of brain circuits in response to environmental experience. SIGNIFICANCE STATEMENT Oligodendrocyte precursor cells differentiate into mature oligodendrocytes and are capable of ensheathing axons with myelin without molecular cues from neurons. However, this default myelination process can be modulated by changes in neuronal activity. Here, we show, for the first time, that experience-dependent activity modifies the length of myelin

  17. Anterograde degeneration along the visual pathway after optic nerve injury.

    Directory of Open Access Journals (Sweden)

    Yuyi You

    Full Text Available PURPOSE: To investigate anterograde degenerative changes along the visual pathway in a rat model of optic nerve axotomy. METHODS: Optic nerve transection was performed in adult Sprague-Dawley rats. Animals were sacrificed at regular time intervals and tissues harvested. Immunoblotting followed by densitometric analysis was used to determine the phosphorylation profile of Akt in the dorsal lateral geniculate nucleus (dLGN and the primary visual cortex (V1. The neuronal cell size and cell density were measured in the dLGN and the V1 using Nissl staining. The prevalence of apoptosis was characterized by terminal deoxynucleotidyl-transferase-mediated biotin-dUTP nick end labelling (TUNEL histochemistry. Caspase-3 antibodies were also used to identify apoptotic cells. Neurons and astrocytes were detected using NeuN and glial fibrillary acidic protein (GFAP, respectively. RESULTS: An early and sustained loss of Akt phosphorylation was observed after optic nerve transection in both dLGN and V1. At week one, a decrease in the neuronal cell size (50.5±4.9 vs 60.3±5.0 µm(2, P = 0.042 and an increase of TUNEL positive cells (7.9±0.6 vs 1.4±0.5 ×10(2 cells/mm(2, P<0.001 were evident in the dLGN but not in V1. A significant decline in neuronal cell number (14.5±0.1 vs 17.4±1.3 ×10(2 cells/mm(2, P = 0.048, cell size (42.5±4.3 vs 62.1±4.7 µm(2, P = 0.001 and an increase in apoptotic cells (5.6±0.5 vs 2.0±0.4 ×10(2 cells/mm(2, P<0.001 appeared in V1 initially at one month post-transection. The changes in the visual pathway continued through two months. Both neuronal cells and GFAP-positive glial cells were affected in this anterograde degeneration along the visual pathway. CONCLUSIONS: Anterograde degeneration along the visual pathway takes place in target relay (LGN and visual cortex following the optic nerve injury. Apoptosis was observed in both neural and adjacent glial cells. Reduction of Akt phosphorylation preceded cellular and apoptotic

  18. Anterograde degeneration along the visual pathway after optic nerve injury.

    Science.gov (United States)

    You, Yuyi; Gupta, Vivek K; Graham, Stuart L; Klistorner, Alexander

    2012-01-01

    To investigate anterograde degenerative changes along the visual pathway in a rat model of optic nerve axotomy. Optic nerve transection was performed in adult Sprague-Dawley rats. Animals were sacrificed at regular time intervals and tissues harvested. Immunoblotting followed by densitometric analysis was used to determine the phosphorylation profile of Akt in the dorsal lateral geniculate nucleus (dLGN) and the primary visual cortex (V1). The neuronal cell size and cell density were measured in the dLGN and the V1 using Nissl staining. The prevalence of apoptosis was characterized by terminal deoxynucleotidyl-transferase-mediated biotin-dUTP nick end labelling (TUNEL) histochemistry. Caspase-3 antibodies were also used to identify apoptotic cells. Neurons and astrocytes were detected using NeuN and glial fibrillary acidic protein (GFAP), respectively. An early and sustained loss of Akt phosphorylation was observed after optic nerve transection in both dLGN and V1. At week one, a decrease in the neuronal cell size (50.5±4.9 vs 60.3±5.0 µm(2), P = 0.042) and an increase of TUNEL positive cells (7.9±0.6 vs 1.4±0.5 ×10(2) cells/mm(2), P<0.001) were evident in the dLGN but not in V1. A significant decline in neuronal cell number (14.5±0.1 vs 17.4±1.3 ×10(2) cells/mm(2), P = 0.048), cell size (42.5±4.3 vs 62.1±4.7 µm(2), P = 0.001) and an increase in apoptotic cells (5.6±0.5 vs 2.0±0.4 ×10(2) cells/mm(2), P<0.001) appeared in V1 initially at one month post-transection. The changes in the visual pathway continued through two months. Both neuronal cells and GFAP-positive glial cells were affected in this anterograde degeneration along the visual pathway. Anterograde degeneration along the visual pathway takes place in target relay (LGN) and visual cortex following the optic nerve injury. Apoptosis was observed in both neural and adjacent glial cells. Reduction of Akt phosphorylation preceded cellular and apoptotic changes.

  19. [Local involvement of the optic nerve by acute lymphoblastic leukemia].

    Science.gov (United States)

    Bernardczyk-Meller, Jadwiga; Stefańska, Katarzyna

    2005-01-01

    The leucemias quite commonly involve the eyes and adnexa. In some cases it causes visual complants. Both, the anterior chamber of the eye and the posterior portion of the globe may sites of acute or chronic leukemia and leucemic relapse. We report an unique case of a 14 years old leucemic patient who suffered visual loss and papilloedema, due to a unilateral local involvement within optic nerve, during second relapse of acute lymphocytic leuemia. In spite of typical treatment of main disease, the boy had died. The authors present typical ophthalmic features of the leucemia, too.

  20. ULTRASOUND AND COMPUTED TOMOGRAPHIC DIAGNOSIS OF OPTIC NERVE TUMORS

    Directory of Open Access Journals (Sweden)

    S. V. Saakyan

    2012-01-01

    Full Text Available A comprehensive examination was made in 93 patients, including 18 children, with tumors of the optic nerve (ON. Duplex ultrasound scanning was performed in 39 patients, of them there were 11 patients with ON gliomas and 28 with ON meningiomas. The specific computed tomographic and echographic signs of ON glioma and meningiomas were detected. The studies have shown that duplex ultrasound scanning and structural computed tomography of orbital sockets are highly informative complementary imaging procedures for ON tumors, which permits one to make their correct diagnosis, to specify surgical volume, and to plan adequate treatment.

  1. Retinal nerve fiber layer thickness is associated with lesion length in acute optic neuritis

    DEFF Research Database (Denmark)

    Kallenbach, K; Simonsen, Helle Juhl; Sander, B

    2010-01-01

    included 41 patients with unilateral optic neuritis and 19 healthy volunteers. All patients were evaluated and examined within 28 days of onset of symptoms. The peripapillary retinal nerve fiber layer thickness (RNFLT), an objective quantitative measure of optic nerve head edema, was measured by optical...... coherence tomography and the length and location of the inflammatory optic nerve lesion were evaluated using MRI. RESULTS: Ophthalmoscopically, 34% of the patients had papillitis. The retinal nerve fiber layer in affected eyes (mean 123.1 microm) was higher during the acute phase than that of fellow eyes...... (mean 98.1 microm, p eyes (mean 97.1 microm, p

  2. Retinal nerve fiber layer thickness is associated with lesion length in acute optic neuritis

    DEFF Research Database (Denmark)

    Kallenbach, K; Simonsen, Helle Juhl; Sander, B;

    2010-01-01

    BACKGROUND: Acute optic neuritis occurs with and without papillitis. The presence of papillitis has previously been thought to imply an anterior location of the neuritis, but imaging studies seeking to test this hypothesis have been inconclusive. METHODS: This prospective observational cohort study...... included 41 patients with unilateral optic neuritis and 19 healthy volunteers. All patients were evaluated and examined within 28 days of onset of symptoms. The peripapillary retinal nerve fiber layer thickness (RNFLT), an objective quantitative measure of optic nerve head edema, was measured by optical...... in the development of optic nerve head edema in optic neuritis....

  3. Parallel changes in structural and functional measures of optic nerve myelination after optic neuritis.

    Directory of Open Access Journals (Sweden)

    Anneke van der Walt

    Full Text Available Visual evoked potential (VEP latency prolongation and optic nerve lesion length after acute optic neuritis (ON corresponds to the degree of demyelination, while subsequent recovery of latency may represent optic nerve remyelination. We aimed to investigate the relationship between multifocal VEP (mfVEP latency and optic nerve lesion length after acute ON.Thirty acute ON patients were studied at 1, 3, 6 and 12 months using mfVEP and at 1 and 12 months with optic nerve MRI. LogMAR and low contrast visual acuity were documented. By one month, the mfVEP amplitude had recovered sufficiently for latency to be measured in 23 (76.7% patients with seven patients having no recordable mfVEP in more than 66% of segments in at least one test. Only data from these 23 patients was analysed further.Both latency and lesion length showed significant recovery during the follow-up period. Lesion length and mfVEP latency were highly correlated at 1 (r = 0.94, p = <0.0001 and 12 months (r = 0.75, p < 0.001. Both measures demonstrated a similar trend of recovery. Speed of latency recovery was faster in the early follow-up period while lesion length shortening remained relatively constant. At 1 month, latency delay was worse by 1.76 ms for additional 1mm of lesion length while at 12 months, 1mm of lesion length accounted for 1.94 ms of latency delay.A strong association between two putative measures of demyelination in early and chronic ON was found. Parallel recovery of both measures could reflect optic nerve remyelination.

  4. Parallel changes in structural and functional measures of optic nerve myelination after optic neuritis.

    Science.gov (United States)

    van der Walt, Anneke; Kolbe, Scott; Mitchell, Peter; Wang, Yejun; Butzkueven, Helmut; Egan, Gary; Yiannikas, Con; Graham, Stuart; Kilpatrick, Trevor; Klistorner, Alexander

    2015-01-01

    Visual evoked potential (VEP) latency prolongation and optic nerve lesion length after acute optic neuritis (ON) corresponds to the degree of demyelination, while subsequent recovery of latency may represent optic nerve remyelination. We aimed to investigate the relationship between multifocal VEP (mfVEP) latency and optic nerve lesion length after acute ON. Thirty acute ON patients were studied at 1, 3, 6 and 12 months using mfVEP and at 1 and 12 months with optic nerve MRI. LogMAR and low contrast visual acuity were documented. By one month, the mfVEP amplitude had recovered sufficiently for latency to be measured in 23 (76.7%) patients with seven patients having no recordable mfVEP in more than 66% of segments in at least one test. Only data from these 23 patients was analysed further. Both latency and lesion length showed significant recovery during the follow-up period. Lesion length and mfVEP latency were highly correlated at 1 (r = 0.94, p = <0.0001) and 12 months (r = 0.75, p < 0.001). Both measures demonstrated a similar trend of recovery. Speed of latency recovery was faster in the early follow-up period while lesion length shortening remained relatively constant. At 1 month, latency delay was worse by 1.76 ms for additional 1mm of lesion length while at 12 months, 1mm of lesion length accounted for 1.94 ms of latency delay. A strong association between two putative measures of demyelination in early and chronic ON was found. Parallel recovery of both measures could reflect optic nerve remyelination.

  5. Cell proliferation and apoptosis in optic nerve and brain integration centers of adult troutOncorhynchus mykiss after optic nerve injury

    Institute of Scientific and Technical Information of China (English)

    Evgeniya V Pushchina; Sachin Shukla; Anatoly A Varaksin; Dmitry K Obukhov

    2016-01-01

    Fishes have remarkable ability to effectively rebuild the structure of nerve cells and nerve ifbers after central nervous system injury. However, the underlying mechanism is poorly understood. In order to address this issue, we investigated the proliferation and apoptosis of cells in contralateral and ipsilateral optic nerves, after stab wound injury to the eye of an adult troutOncorhynchus mykiss. Heterogenous population of proliferating cells was investigated at 1 week after injury. TUNEL labeling gave a qualitative and quantita-tive assessment of apoptosis in the cells of optic nerve of trout 2 days after injury. After optic nerve injury, apoptotic response was investigated, and mass patterns of cell migration were found. The maximal con-centration of apoptotic bodies was detected in the areas of mass clumps of cells. It is probably indicative of massive cell death in the area of high phagocytic activity of macrophages/microglia. At 1 week after optic nerve injury, we observed nerve cell proliferation in the trout brain integration centers: the cerebellum and the optic tectum. In the optic tectum, proliferating cell nuclear antigen (PCNA)-immunopositive radial glia-like cells were identified. Proliferative activity of nerve cells was detected in the dorsal proliferative (matrix) area of the cerebellum and in parenchymal cells of the molecular and granular layers whereas local clusters of undifferentiated cells which formed neurogenic niches were observed in both the optic tectum and cerebellum after optic nerve injury.In vitro analysis of brain cells of trout showed that suspension cells compared with monolayer cells retain higher proliferative activity, as evidenced by PCNA immunolabeling. Phase contrast observation showed mitosis in individual cells and the formation of neurospheres which gradually increased during 1–4 days of culture. The present ifndings suggest that trout can be used as a novel model for studying neuronal regeneration.

  6. [Optic nerve pits: clinical and therapeutic review of 21 cases].

    Science.gov (United States)

    Montenegro, M; Bonnet, M

    1989-01-01

    A retrospective study of optic nerve pits in patients referred to our clinic during the last 15 years was conducted. The study included 21 eyes in 19 patients. The optic pit was associated with a serous macular detachment (SMD) in 19 eyes (90%). Various treatments of the SMD were used depending on the time period. Systemic corticosteroids were used in five patients. The SMD did not respond to this treatment. Argon laser photocoagulation was applied to the temporal side of the optic disc in five patients. Intravitreal injection of pure gas was used as an adjunct to photocoagulation treatment in eleven eyes. Pure SF 6 was used in 8 eyes, and C 3 F 8 in 3 eyes. The follow-up after treatment was over 6 months in 10 patients who underwent photocoagulation or the combination of photocoagulation and gas injection. The SMD remained unchanged in two eyes which underwent photocoagulation treatment without gas injection. In the group of patients treated by photocoagulation in association with SF 6 injection, the SMD totally reattached in one eye, decreased in 2 eyes and remained unchanged in 2 eyes. Total resorption of subretinal fluid and permanent retinal reattachment occurred in the 3 eyes treated by intravitreal injection of pure C 3 F 8 as an adjunct to photocoagulation treatment. Further clinical investigations on large series of patients are required to determine whether prolonged retinal tamponade by C 3 F 8 gas in association with photocoagulation treatment is a valuable method in the management of SMD complicating optic nerve pits.

  7. A sheath-less combined optical and impedance micro-cytometer.

    Science.gov (United States)

    Spencer, Daniel; Elliott, Gregor; Morgan, Hywel

    2014-08-21

    We describe a sheath-less micro-cytometer that measures four different parameters, namely fluorescence, large angle side scatter and dual frequency electrical impedance (electrical volume and opacity). The cytometer was benchmarked using both size and fluorescent bead standards and demonstrates excellent size accuracy (CVs ≤ 2.1%), sensitivity and dynamic range (3.5 orders of magnitude) at sample flow rates of 80 μL per minute. The cytometer was evaluated by analysing human blood, and a four part differential leukocyte assay for accurate CD4+ T-cell enumeration was demonstrated. The integration of impedance, fluorescence and side scatter into a single miniature cytometer platform provides the core information content of a classical cytometer in a highly compact, simple, portable and low cost format.

  8. The thickness of the retrobulbar portion of the optic nerve in Graves ophthalmopathy measured by ultrasound

    Directory of Open Access Journals (Sweden)

    Stefanović Ivan

    2009-01-01

    Full Text Available Introduction. The clinical diagnostic of Graves ophthalmopathy is based on the association of ocular signs and the disease of the thyroid gland. The evolution of the disease involves the development of eye globe protrusion, extraocular muscle thickening pressuring the optic nerve, which can result in its thickness. Objective. The aim of the paper is to find whether the retrobulbar optic nerve thickened and if there was a correlation between its possible thickening and the thickness of the muscles in Graves ophthalmopathy. We also wished to test the theory of compressive aetiology of such thickening using a 30-degree test. Methods. We examined 28 patients with Graves ophthalmopathy. The thickness of the retrobulbar optic nerve was measured by ultrasound on a B-scan using the Schraeder's method and by the largest thickness of the internal muscle. Results. The thickness of the retrobulbar portion of the optic nerve in the 52 analyzed eyes with signs of the disease ranged between 3.24 mm to 6.30 mm, with median of 5.13 mm, indicating that the majority of the patients had optic nerve thickening rating at this value. Forty-eight eyes had a marked retrobulbar optic nerve thickening, with the thickening over 4 mm, while in 4 eyes with signs of Graves ophthalmopathy the thickness of the optic nerve was within normal limits. We detected that 92.3% of the patients with muscular thickening also had a directly proportional thickening of the retrobulbar optic nerve. By using the 30-degree test we confirmed the diagnosis of compressive neuropathy. Conclusion. Patients with Graves ophthalmopathy and thickened muscles, also have a thickening of the retrobulbar optic nerve; the rate of the thickness directly depends on the degree of the muscular thickness. The word is of compressive neuropathy, i.e. the thickness of the optic nerve is the result of subarachnoid fluid stasis caused by the compression on the optic nerve.

  9. Visual Evoked Potential Recording in a Rat Model of Experimental Optic Nerve Demyelination.

    Science.gov (United States)

    You, Yuyi; Gupta, Vivek K; Chitranshi, Nitin; Reedman, Brittany; Klistorner, Alexander; Graham, Stuart L

    2015-07-29

    The visual evoked potential (VEP) recording is widely used in clinical practice to assess the severity of optic neuritis in its acute phase, and to monitor the disease course in the follow-up period. Changes in the VEP parameters closely correlate with pathological damage in the optic nerve. This protocol provides a detailed description about the rodent model of optic nerve microinjection, in which a partial demyelination lesion is produced in the optic nerve. VEP recording techniques are also discussed. Using skull implanted electrodes, we are able to acquire reproducible intra-session and between-session VEP traces. VEPs can be recorded on individual animals over a period of time to assess the functional changes in the optic nerve longitudinally. The optic nerve demyelination model, in conjunction with the VEP recording protocol, provides a tool to investigate the disease processes associated with demyelination and remyelination, and can potentially be employed to evaluate the effects of new remyelinating drugs or neuroprotective therapies.

  10. High Spatial Resolution Imaging Mass Spectrometry of Human Optic Nerve Lipids and Proteins

    Science.gov (United States)

    Anderson, David M. G.; Spraggins, Jeffrey M.; Rose, Kristie L.; Schey, Kevin L.

    2015-06-01

    The human optic nerve carries signals from the retina to the visual cortex of the brain. Each optic nerve is comprised of approximately one million nerve fibers that are organized into bundles of 800-1200 fibers surrounded by connective tissue and supportive glial cells. Damage to the optic nerve contributes to a number of blinding diseases including: glaucoma, neuromyelitis optica, optic neuritis, and neurofibromatosis; however, the molecular mechanisms of optic nerve damage and death are incompletely understood. Herein we present high spatial resolution MALDI imaging mass spectrometry (IMS) analysis of lipids and proteins to define the molecular anatomy of the human optic nerve. The localization of a number of lipids was observed in discrete anatomical regions corresponding to myelinated and unmyelinated nerve regions as well as to supporting connective tissue, glial cells, and blood vessels. A protein fragment from vimentin, a known intermediate filament marker for astrocytes, was observed surrounding nerved fiber bundles in the lamina cribrosa region. S100B was also found in supporting glial cell regions in the prelaminar region, and the hemoglobin alpha subunit was observed in blood vessel areas. The molecular anatomy of the optic nerve defined by MALDI IMS provides a firm foundation to study biochemical changes in blinding human diseases.

  11. Short term reproducibility of a high contrast 3-D isotropic optic nerve imaging sequence in healthy controls

    Science.gov (United States)

    Harrigan, Robert L.; Smith, Alex K.; Mawn, Louise A.; Smith, Seth A.; Landman, Bennett A.

    2016-03-01

    The optic nerve (ON) plays a crucial role in human vision transporting all visual information from the retina to the brain for higher order processing. There are many diseases that affect the ON structure such as optic neuritis, anterior ischemic optic neuropathy and multiple sclerosis. Because the ON is the sole pathway for visual information from the retina to areas of higher level processing, measures of ON damage have been shown to correlate well with visual deficits. Increased intracranial pressure has been shown to correlate with the size of the cerebrospinal fluid (CSF) surrounding the ON. These measures are generally taken at an arbitrary point along the nerve and do not account for changes along the length of the ON. We propose a high contrast and high-resolution 3-D acquired isotropic imaging sequence optimized for ON imaging. We have acquired scan-rescan data using the optimized sequence and a current standard of care protocol for 10 subjects. We show that this sequence has superior contrast-to-noise ratio to the current standard of care while achieving a factor of 11 higher resolution. We apply a previously published automatic pipeline to segment the ON and CSF sheath and measure the size of each individually. We show that these measures of ON size have lower short- term reproducibility than the population variance and the variability along the length of the nerve. We find that the proposed imaging protocol is (1) useful in detecting population differences and local changes and (2) a promising tool for investigating biomarkers related to structural changes of the ON.

  12. Ultraintense short-wavelength emission from ZnO-sheathed MgO nanorods induced by subwavelength optical resonance cavity formation: verification of previous hypothesis.

    Science.gov (United States)

    Jin, Changhyun; Kim, Hyunsu; Lee, Chongmu

    2012-03-01

    A recent paper reported that intense emissions with a range of wavelengths over a wide spectral range, from ultraviolet to infrared light, might be possible by sheathing MgO nanorods with a semiconducting material with an optimal sheath thickness. In addition, the paper hypothesized that an ultraintense short-wavelength emission could be obtained by sheathing MgO nanorods with a ~17 nm ZnO thin film in the paper. In this study, we found that the intensity ratio of the near-band edge emission to the deep level emission (I(NBE)/I(DL)) of the MgO-core/ZnO-shell nanorods with a mean shell layer thickness of 17 nm was as high as ~30, whereas the I(NBE)/I(DL) ratio of the bare-MgO nanorods was 0. This near-band edge emission intensity enhancement by sheathing the MgO nanorods with ZnO is by far more significant than that by sheathing the ZnO nanorods with other materials including MgO. This is because subwavelength optical resonance cavities form in the MgO-core/ZnO-shell nanorods with faceted surfaces, whereas they do not form in the ZnO-core/MgO (or other material)-shell nanorods with no faceted surfaces.

  13. Protein expression of BIRC5, TK1, and TOP2A in malignant peripheral nerve sheath tumours--A prognostic test after surgical resection.

    Science.gov (United States)

    Kolberg, Matthias; Høland, Maren; Lind, Guro E; Ågesen, Trude H; Skotheim, Rolf I; Hall, Kirsten Sundby; Mandahl, Nils; Smeland, Sigbjørn; Mertens, Fredrik; Davidson, Ben; Lothe, Ragnhild A

    2015-06-01

    No consensus treatment regime exists beyond surgery for malignant peripheral nerve sheath tumours (MPNST), and the purpose of the present study was to find new approaches to stratify patients with good and poor prognosis and to better guide therapeutic intervention for this aggressive soft tissue cancer. From a total of 67 MPNSTs from Scandinavian patients with and without neurofibromatosis type 1, 30 MPNSTs were investigated by genome-wide RNA expression profiling and 63 MPNSTs by immunohistochemical (IHC) analysis, and selected genes were submitted to analyses of disease-specific survival. The potential drug target genes survivin (BIRC5), thymidine kinase 1 (TK1), and topoisomerase 2-alpha (TOP2A), all encoded on chromosome arm 17q, were up-regulated in MPNST as compared to benign neurofibromas. Each of them was found to be independent prognostic markers on the gene expression level, as well as on the protein level. A prognostic profile was identified by combining the nuclear expression scores of the three proteins. For patients with completely resected tumours only 15% in the high risk group were alive after two years, as compared to 78% in the low risk group. In conclusion, we found a novel protein expression profile which identifies MPNST patients with inferior prognosis even after assumed curative surgery. The tested proteins are drug targets; therefore the expression profile may provide predictive information guiding the design of future clinical trials. Importantly, as the effect is seen on the protein level using IHC, the biomarker panel can be readily implemented in routine clinical testing.

  14. TAGLN expression is upregulated in NF1-associated malignant peripheral nerve sheath tumors by hypomethylation in its promoter and subpromoter regions.

    Science.gov (United States)

    Park, Gun-Hoo; Lee, Su-Jin; Yim, Hyunee; Han, Jae-Ho; Kim, Hyon J; Sohn, Young-Bae; Ko, Jung Min; Jeong, Seon-Yong

    2014-10-01

    Neurofibromatosis type 1 (NF1) caused by NF1 gene mutation is a commonly inherited autosomal dominant disorder. Malignant peripheral nerve sheath tumors (MPNSTs), a type of aggressive sarcoma, are a major cause of mortality in NF1 patients. The malignant transformation of benign plexiform neurofibromas (PNs) to MPNSTs is a marked peculiarity in NF1 patients, yet the pathogenesis remains poorly understood. We found that an actin-associated protein transgelin (SM22) was highly expressed in NF1-deficient MPNST tissues compared to NF1-deficient PN tissues using immunohistological staining and primary cultured MPNST cells in western blot analysis. We further found that this transgelin upregulation was caused by increased transcriptional expression of the TAGLN gene encoding transgelin. Comparison of DNA methylation values in the promoter and subpromoter regions of the TAGLN gene in three types of NF1-deficient primary-cultured cells, derived from an NF1 patient's normal phenotype, a benign PN and MPNST tissues, revealed that the TAGLN gene was hypomethylated in the MPNST cells. Next, to determine the functional role of transgelin in MPNST pathogenesis, we manipulated the TAGLN gene expression and investigated the alteration of the RAS-mitogen-activated protein kinase (MAPK) signaling pathway in the normal-phenotypic and malignant tumor cells. The downregulation of TAGLN expression in NF1-deficient MPNST tumor cells through the treatment of the small interfering RNA resulted in a decrease in the RAS activation (GTP-RAS) and the downstream ERK1/2 activation (phosphorylated ERK1/2), while the overexpression of TAGLN in normal-phenotypic NF1-deficient cells caused an increase in RAS and ERK1/2 activation. These results indicate that upregulation of transgelin caused by hypomethylation of the TAGLN gene is closely involved in tumor progression in NF1.

  15. Nerve Sheath Tumors in Neurofibromatosis Type 1: Assessment of Whole-Body Metabolic Tumor Burden Using F-18-FDG PET/CT.

    Directory of Open Access Journals (Sweden)

    Johannes Salamon

    Full Text Available To determine the metabolically active whole-body tumor volume (WB-MTV on F-18-fluorodeoxyglucose positron emission tomography/computed tomography (F-18-FDG PET/CT in individuals with neurofibromatosis type 1 (NF1 using a three-dimensional (3D segmentation and computerized volumetry technique, and to compare PET WB-MTV between patients with benign and malignant peripheral nerve sheath tumors (PNSTs.Thirty-six NF1 patients (18 patients with malignant PNSTs and 18 age- and sex-matched controls with benign PNSTs were examined by F-18-FDG PET/CT. WB-MTV, whole-body total lesion glycolysis (WB-TLG and a set of semi-quantitative imaging-based parameters were analyzed both on a per-patient and a per-lesion basis.On a per-lesion basis, malignant PNSTs demonstrated both a significantly higher MTV and TLG than benign PNSTs (p < 0.0001. On a per-patient basis, WB-MTV and WB-TLG were significantly higher in patients with malignant PNSTs compared to patients with benign PNSTs (p < 0.001. ROC analysis showed that MTV and TLG could be used to differentiate between benign and malignant tumors.WB-MTV and WB-TLG may identify malignant change and may have the potential to provide a basis for investigating molecular biomarkers that correlate with metabolically active disease manifestations. Further evaluation will determine the potential clinical impact of these PET-based parameters in NF1.

  16. Evaluation of intratumoural heterogeneity on {sup 18}F-FDG PET/CT for characterization of peripheral nerve sheath tumours in neurofibromatosis type 1

    Energy Technology Data Exchange (ETDEWEB)

    Salamon, Johannes; Derlin, Thorsten; Bannas, Peter; Busch, Jasmin D.; Herrmann, Jochen; Adam, Gerhard [University Medical Center Hamburg-Eppendorf, Department of Diagnostic and Interventional Radiology, Hamburg (Germany); Bockhorn, Maximilian [University Medical Center Hamburg-Eppendorf, Department of General, Visceral and Thoracic Surgery, Hamburg (Germany); Hagel, Christian [University Medical Center Hamburg-Eppendorf, Institute of Neuropathology, Hamburg (Germany); Friedrich, Reinhard E. [University Medical Center Hamburg-Eppendorf, Department of Oral and Maxillofacial Surgery, Hamburg (Germany); Mautner, Victor F. [University Medical Center Hamburg-Eppendorf, Department of Neurology, Hamburg (Germany)

    2013-05-15

    The aim of the study was to evaluate the potential usefulness of intratumoural tracer uptake heterogeneity on {sup 18}F-fluorodeoxyglucose (FDG) positron emission tomography (PET)/CT as compared to a cut-off maximum standardized uptake value (SUV{sub max}) for characterization of peripheral nerve sheath tumours (PNSTs) in neurofibromatosis type 1 (NF1). Fifty patients suffering from NF1 were examined by {sup 18}F-FDG PET/CT. Intralesional tracer uptake was analysed qualitatively and semi-quantitatively by measuring the mean and maximum SUV. Uptake heterogeneity was graded qualitatively using a three-point scale and semi-quantitatively by calculating an SUV-based heterogeneity index (HI{sub SUV}). Cohen's {kappa} was used to determine inter- and intra-rater agreement. Histopathological evaluation and clinical as well as radiological follow-up examinations served as the reference standards. A highly significant correlation between the degree of intratumoural uptake heterogeneity on {sup 18}F-FDG PET and malignant transformation of PNSTs was observed (p < 0.0001). Semi-quantitative HI{sub SUV} was significantly higher in malignant PNSTs (MPNSTs) than in benign tumours (p = 0.0002). Both intralesional heterogeneity and SUV{sub max} could be used to identify malignant tumours with a sensitivity of 100 %. Cohen's {kappa} was 0.86 for inter-rater agreement and 0.88 for intra-rater agreement on heterogeneity. MPNSTs in patients with NF1 demonstrate considerable intratumoural uptake heterogeneity on {sup 18}F-FDG PET/CT. Assessment of tumour heterogeneity is highly reproducible. Both tumour heterogeneity and a cut-off SUV{sub max} may be used to sensitively identify malignant PNSTs, but the specificity is higher for the latter. A combination of both methods leads to a non-significant improvement in diagnostic performance. (orig.)

  17. How the Build Up of Aqueous Humor Can Damage the Optic Nerve

    Science.gov (United States)

    ... Aqueous Humor Can Damage the Optic Nerve How the Build Up of Aqueous Humor Can Damage the Optic Nerve Most, but not all, forms of ... name. Phone (office) Sign Up Join us in the fight to end brain and eye disease 3 ...

  18. Optic nerve vascular compression in a patient with a tuberculum sellae meningioma.

    Science.gov (United States)

    Mizrahi, Cezar José; Moscovici, Samuel; Dotan, Shlomo; Spektor, Sergey

    2015-01-01

    Background. Optic nerve vascular compression in patients with suprasellar tumor is a known entity but is rarely described in the literature. Case Description. We present a unique, well-documented case of optic nerve strangulation by the A1 segment of the anterior cerebral artery in a patient with a tuberculum sellae meningioma. The patient presented with pronounced progressive visual deterioration. Following surgery, there was immediate resolution of her visual deficit. Conclusion. Vascular strangulation of the optic nerve should be considered when facing progressive and/or severe visual field deterioration in patients with tumors proximal to the optic apparatus.

  19. Optic nerve diffusion tensor imaging after acute optic neuritis predicts axonal and visual outcomes.

    Directory of Open Access Journals (Sweden)

    Anneke van der Walt

    Full Text Available BACKGROUND: Early markers of axonal and clinical outcomes are required for early phase testing of putative neuroprotective therapies for multiple sclerosis (MS. OBJECTIVES: To assess whether early measurement of diffusion tensor imaging (DTI parameters (axial and radial diffusivity within the optic nerve during and after acute demyelinating optic neuritis (ON could predict axonal (retinal nerve fibre layer thinning and multi-focal visual evoked potential amplitude reduction or clinical (visual acuity and visual field loss outcomes at 6 or 12 months. METHODS: Thirty-seven patients presenting with acute, unilateral ON were studied at baseline, one, three, six and 12 months using optic nerve DTI, clinical and paraclinical markers of axonal injury and clinical visual dysfunction. RESULTS: Affected nerve axial diffusivity (AD was reduced at baseline, 1 and 3 months. Reduced 1-month AD correlated with retinal nerve fibre layer (RNFL thinning at 6 (R=0.38, p=0.04 and 12 months (R=0.437, p=0.008 and VEP amplitude loss at 6 (R=0.414, p=0.019 and 12 months (R=0.484, p=0.003. AD reduction at three months correlated with high contrast visual acuity at 6 (ρ = -0.519, p = 0.001 and 12 months (ρ = -0.414, p=0.011. The time-course for AD reduction for each patient was modelled using a quadratic regression. AD normalised after a median of 18 weeks and longer normalisation times were associated with more pronounced RNFL thinning and mfVEP amplitude loss at 12 months. Affected nerve radial diffusivity (RD was unchanged until three months, after which time it remained elevated. CONCLUSIONS: These results demonstrate that AD reduces during acute ON. One month AD reduction correlates with the extent of axonal loss and persistent AD reduction at 3 months predicts poorer visual outcomes. This suggests that acute ON therapies that normalise optic nerve AD by 3 months could also promote axon survival and improve visual outcomes.

  20. Automated interpretation of optic nerve images: a data mining framework for glaucoma diagnostic support.

    Science.gov (United States)

    Abidi, Syed S R; Artes, Paul H; Yun, Sanjan; Yu, Jin

    2007-01-01

    Confocal Scanning Laser Tomography (CSLT) techniques capture high-quality images of the optic disc (the retinal region where the optic nerve exits the eye) that are used in the diagnosis and monitoring of glaucoma. We present a hybrid framework, combining image processing and data mining methods, to support the interpretation of CSLT optic nerve images. Our framework features (a) Zernike moment methods to derive shape information from optic disc images; (b) classification of optic disc images, based on shape information, to distinguish between healthy and glaucomatous optic discs. We apply Multi Layer Perceptrons, Support Vector Machines and Bayesian Networks for feature sub-set selection and image classification; and (c) clustering of optic disc images, based on shape information, using Self-Organizing Maps to visualize sub-types of glaucomatous optic disc damage. Our framework offers an automated and objective analysis of optic nerve images that can potentially support both diagnosis and monitoring of glaucoma.

  1. Systemic and ocular findings in 100 patients with optic nerve hypoplasia.

    Science.gov (United States)

    Garcia, M Lourdes; Ty, Edna B; Taban, Mehryar; David Rothner, A; Rogers, Douglas; Traboulsi, Elias I

    2006-11-01

    To describe associated ocular, neurologic, and systemic findings in a population of children with optic nerve hypoplasia, a retrospective chart review of 100 patients with optic nerve hypoplasia for the presence of neurologic, radiologic, and endocrine abnormalities was performed. Neuroimaging and endocrine studies were obtained in 65 cases. Visual acuity and associated ocular, neurologic, endocrine, systemic, and structural brain abnormalities were recorded. Seventy-five percent had bilateral optic nerve hypoplasia. Conditions previously associated with optic nerve hypoplasia and present in our patients include premature birth in 21%, fetal alcohol syndrome in 9%, maternal diabetes in 6%, and endocrine abnormalities in 6%. Developmental delay was present in 32%, cerebral palsy in 13%, and seizures in 12%. Of those imaged, 60% had an abnormal study. Neuroimaging showed abnormalities in ventricles or white- or gray-matter development in 29 patients, septo-optic dysplasia in 10, hydrocephalus in 10, and corpus callosum abnormalities in 8. There was an associated clinical neurologic abnormality in 57% of patients with bilateral optic nerve hypoplasia and in 32% of patients with unilateral optic nerve hypoplasia. Patients with unilateral and bilateral optic nerve hypoplasia frequently have a wide range and common occurrence of concomitant neurologic, endocrine, and systemic abnormalities.

  2. Optic nerve magnetisation transfer ratio after acute optic neuritis predicts axonal and visual outcomes.

    Science.gov (United States)

    Wang, Yejun; van der Walt, Anneke; Paine, Mark; Klistorner, Alexander; Butzkueven, Helmut; Egan, Gary F; Kilpatrick, Trevor J; Kolbe, Scott C

    2012-01-01

    Magnetisation transfer ratio (MTR) can reveal the degree of proton exchange between free water and macromolecules and was suggested to be pathological informative. We aimed to investigate changes in optic nerve MTR over 12 months following acute optic neuritis (ON) and to determine whether MTR measurements can predict clinical and paraclinical outcomes at 6 and 12 months. Thirty-seven patients with acute ON were studied within 2 weeks of presentation and at 1, 3, 6 and 12 months. Assessments included optic nerve MTR, retinal nerve fibre layer (RNFL) thickness, multifocal visual evoked potential (mfVEP) amplitude and latency and high (100%) and low (2.5%) contrast letter acuity. Eleven healthy controls were scanned twice four weeks apart for comparison with patients. Patient unaffected optic nerve MTR did not significantly differ from controls at any time-point. Compared to the unaffected nerve, affected optic nerve MTR was significantly reduced at 3 months (mean percentage interocular difference = -9.24%, p = 0.01), 6 months (mean = -12.48%, p<0.0001) and 12 months (mean = -7.61%, p = 0.003). Greater reduction in MTR at 3 months in patients was associated with subsequent loss of high contrast letter acuity at 6 (ρ = 0.60, p = 0.0003) and 12 (ρ = 0.44, p = 0.009) months, low contrast letter acuity at 6 (ρ = 0.35, p = 0.047) months, and RNFL thinning at 12 (ρ = 0.35, p = 0.044) months. Stratification of individual patient MTR time courses based on flux over 12 months (stable, putative remyelination and putative degeneration) predicted RNFL thinning at 12 months (F(2,32) = 3.59, p = 0.02). In conclusion, these findings indicate that MTR flux after acute ON is predictive of axonal degeneration and visual disability outcomes.

  3. Optic nerve magnetisation transfer ratio after acute optic neuritis predicts axonal and visual outcomes.

    Directory of Open Access Journals (Sweden)

    Yejun Wang

    Full Text Available Magnetisation transfer ratio (MTR can reveal the degree of proton exchange between free water and macromolecules and was suggested to be pathological informative. We aimed to investigate changes in optic nerve MTR over 12 months following acute optic neuritis (ON and to determine whether MTR measurements can predict clinical and paraclinical outcomes at 6 and 12 months. Thirty-seven patients with acute ON were studied within 2 weeks of presentation and at 1, 3, 6 and 12 months. Assessments included optic nerve MTR, retinal nerve fibre layer (RNFL thickness, multifocal visual evoked potential (mfVEP amplitude and latency and high (100% and low (2.5% contrast letter acuity. Eleven healthy controls were scanned twice four weeks apart for comparison with patients. Patient unaffected optic nerve MTR did not significantly differ from controls at any time-point. Compared to the unaffected nerve, affected optic nerve MTR was significantly reduced at 3 months (mean percentage interocular difference = -9.24%, p = 0.01, 6 months (mean = -12.48%, p<0.0001 and 12 months (mean = -7.61%, p = 0.003. Greater reduction in MTR at 3 months in patients was associated with subsequent loss of high contrast letter acuity at 6 (ρ = 0.60, p = 0.0003 and 12 (ρ = 0.44, p = 0.009 months, low contrast letter acuity at 6 (ρ = 0.35, p = 0.047 months, and RNFL thinning at 12 (ρ = 0.35, p = 0.044 months. Stratification of individual patient MTR time courses based on flux over 12 months (stable, putative remyelination and putative degeneration predicted RNFL thinning at 12 months (F(2,32 = 3.59, p = 0.02. In conclusion, these findings indicate that MTR flux after acute ON is predictive of axonal degeneration and visual disability outcomes.

  4. Differentiating Mild Papilledema and Buried Optic Nerve Head Drusen Using Spectral Domain Optical Coherence Tomography

    Science.gov (United States)

    Kulkarni, Kaushal M.; Pasol, Joshua; Rosa, Potyra R.; Lam, Byron L.

    2013-01-01

    Purpose To evaluate the clinical utility of spectral domain optical coherence tomography (SD-OCT) in differentiating mild papilledema from buried optic nerve head drusen (ONHD). Design Comparative case series. Participants 16 eyes of 9 patients with ultrasound-proven buried ONHD, 12 eyes of 6 patients with less than or equal to Frisén grade 2 papilledema due to idiopathic intracranial hypertension. 2 normal fellow eyes of patients with buried ONHD were included. Methods A raster scan on the optic nerve and retinal nerve fiber layer (RNFL) thickness analysis was performed on each eye using SD-OCT. Eight eyes underwent enhanced depth imaging SD-OCT. Images were assessed qualitatively and quantitatively to identify differentiating features between buried ONHD and papilledema. Five clinicians trained with a tutorial and masked to the underlying diagnosis reviewed the SD-OCT images of each eye independently to determine the diagnosis. Main outcome measures Differences in RNFL thickness in each quadrant between the two groups, and diagnostic accuracy of five independent clinicians based on the SD-OCT images alone. Results We found no statistically significant difference in RNFL thickness between buried ONHD and papilledema in any of the four quadrants. Diagnostic accuracy among the readers was low and ranged from 50–64%. The kappa coefficient of agreement among the readers was 0.35 (95% Confidence interval: 0.19, 0.54). Conclusions SD-OCT is not clinically reliable in differentiating buried ONHD and mild papilledema. PMID:24321144

  5. Ischemic injury leads to extracellular matrix alterations in retina and optic nerve

    Science.gov (United States)

    Reinhard, Jacqueline; Renner, Marina; Wiemann, Susanne; Shakoor, Daniel A.; Stute, Gesa; Dick, H. Burkhard; Faissner, Andreas; Joachim, Stephanie C.

    2017-01-01

    Retinal ischemia occurs in a variety of eye diseases. Restrained blood flow induces retinal damage, which leads to progressive optic nerve degeneration and vision loss. Previous studies indicate that extracellular matrix (ECM) constituents play an important role in complex tissues, such as retina and optic nerve. They have great impact on de- and regeneration processes and represent major candidates of central nervous system glial scar formation. Nevertheless, the importance of the ECM during ischemic retina and optic nerve neurodegeneration is not fully understood yet. In this study, we analyzed remodeling of the extracellular glycoproteins fibronectin, laminin, tenascin-C and tenascin-R and the chondroitin sulfate proteoglycans (CSPGs) aggrecan, brevican and phosphacan/RPTPβ/ζ in retinae and optic nerves of an ischemia/reperfusion rat model via quantitative real-time PCR, immunohistochemistry and Western blot. A variety of ECM constituents were dysregulated in the retina and optic nerve after ischemia. Regarding fibronectin, significantly elevated mRNA and protein levels were observed in the retina following ischemia, while laminin and tenascin-C showed enhanced immunoreactivity in the optic nerve after ischemia. Interestingly, CSPGs displayed significantly increased expression levels in the optic nerve. Our study demonstrates a dynamic expression of ECM molecules following retinal ischemia, which strengthens their regulatory role during neurodegeneration. PMID:28262779

  6. Size of the intracranial optic nerve and optic tract in neonates at term-equivalent age at magnetic resonance imaging

    Energy Technology Data Exchange (ETDEWEB)

    Oyama, Jun; Mori, Kouichi [Tsuchiura Kyodo General Hospital, Department of Radiology, Tsuchiura, Ibaraki (Japan); Imamura, Masatoshi [Tsuchiura Kyodo General Hospital, Department of Neonatology, Tsuchiura, Ibaraki (Japan); Mizushima, Yukiko [Tsuchiura Kyodo General Hospital, Department of Ophthalmology, Tsuchiura, Ibaraki (Japan); Tateishi, Ukihide [Tokyo Medical and Dental University, Departments of Diagnostic Radiology and Nuclear Medicine, Tokyo (Japan)

    2016-04-15

    The expected MRI-based dimensions of the intracranial optic nerve and optic tract in neonates are unknown. To evaluate the sizes of the intracranial optic nerve and optic tract in neonates at term-equivalent age using MRI. We retrospectively analyzed brain MRI examinations in 62 infants (28 boys) without intracranial abnormalities. The images were obtained in infants at term-equivalent age with a 1.5-tesla MRI scanner. We measured the widths and heights of the intracranial optic nerve and optic tract and calculated the cross-sectional areas using the formula for an ellipse. The means ± standard deviation of the width, height and cross-sectional area of the intracranial optic nerve were 2.7 ± 0.2 mm, 1.7 ± 0.2 mm and 3.5 ± 0.5 mm{sup 2}, respectively. The width, height and cross-sectional area of the optic tract were 1.5 ± 0.1 mm, 1.6 ± 0.1 mm and 2.0 ± 0.2 mm{sup 2}, respectively. Using univariate and multivariate analyses, we found that postmenstrual age showed independent intermediate positive correlations with the width (r = 0.48, P < 0.01) and cross-sectional area (r = 0.40, P < 0.01) of the intracranial optic nerve. The lower bounds of the 95% prediction intervals for the width and cross-sectional area of the intracranial optic nerve were 0.07 x (postmenstrual age in weeks) - 0.46 mm, and 0.17 x (postmenstrual age in weeks) - 4.0 mm{sup 2}, respectively. We identified the sizes of the intracranial optic nerve and optic tract in neonates at term-equivalent age. The postmenstrual age at MRI independently positively correlated with the sizes. (orig.)

  7. Malignant Peripheral Nerve Sheath Tumor in Palate: Case Report%腭部恶性周围神经鞘膜瘤1例报告

    Institute of Scientific and Technical Information of China (English)

    王开; 候光宇

    2011-01-01

    腭部恶性周围神经鞘膜瘤(palatal malignant peripheral nerve sheath tumor,MPNST)是一种少见的具有神经分化潜能的恶性肿瘤,其发生大多与神经干及神经纤维瘤有关.由于形态学瘤谱范围广、免疫表型异质性大,发病率较低,是软组织肿瘤中最难诊断的恶性肿瘤之一.我科2010-09收治1例,现报告如下.1 临床资料1.1 病史及临床资料患者男性,61岁,2010-09 因“右上后牙区内侧牙龈肿痛2月余,加重2周”入院.现病史:患者2个月前发现其右侧上颌后牙内侧牙龈上,出现一米粒大小的肿物,伴有疼痛肿胀,偶有局部麻木感,曾于当地医院就诊.给予“头孢类抗生素”药物口服及静脉点滴约2周余具.体药名不详,自觉疼痛及肿胀稍有缓解.2周前右上后牙医再次明显肿起,并伴有两颗后牙松动脱落,且疼痛感逐渐加重,肿物快速增大,遂来我院就诊.门诊取病理活检,病理报告显示:(右上牙龈)分化差恶性肿瘤,结合免疫组织化学考虑为非上皮源性,类型难定,建议手术切除再做进一步明确诊断.

  8. Imaging apperances of malignant peripheral nerve sheath tumor%外周恶性神经鞘膜瘤的影像学表现

    Institute of Scientific and Technical Information of China (English)

    倪恩珍; 王亚非

    2012-01-01

    目的:总结分析外周恶性神经鞘膜瘤的影像学表现,提高鉴别诊断能力.方法:回顾性分析11例经手术或病理证实的恶性神经鞘膜瘤的影像学表现,并与手术病理对照分析,11例中行CT检查3例,行MRI检查4例,CT和MRI均检查4例.结果:CT平扫表现为等、低混合密度软组织肿块影,形态不规则,病灶边缘较光整2例,边缘毛糙5例.CT增强扫描呈不均匀强化,坏死区不强化,网格样强化2例,不均匀强化5例.T1WI呈等、略低信号,内见局灶性长T1信号影;T2 WI序列以略高信号为主,内见局灶性长T2信号,周围软组织见片状浸润高信号;MRI增强扫描呈不均匀强化.结论:恶性外周神经鞘膜瘤的CT和MRI表现具有一定的特征性.%Objective:To review the imaging appearances of malignant peripheral nerve sheath tumors (MPNST) in order to improve its diagnostic accuracy. Methods: The imaging appearances of 11 cases with surgery and pathology proved MPNST were retrospectively studied and correlated with surgery and pathology. Of the 11 patients,3 patients had CT,4 patients had MRI and 4 patients had both of the two. Results: The CT appearances included iso/hypo-attenuated soft tissue mass with irregular contour,2 lesions had relatively smooth margin,and 5 lesions were ill-defined. Heterogeneous enhancement was assessed with network-like enhancement in 2 patients and heterogeneous enhancement in 5 patients. The MRI appearances showed iso-/slight hypo-intensities with focal long T1 signal intensity on T1WI, mainly slight high signal intensity with focal long T2 signal areas on T2 WI,hyper-intense infiltration of the adjacent soft tissue could also be revealed. Heterogeneous enhancement was showed after Gadolinium administration. Conclusion: Certain CT and MRI characteristics of MPNSTs could be revealed.

  9. Molecular characterization of permanent cell lines from primary, metastatic and recurrent malignant peripheral nerve sheath tumors (MPNST) with underlying neurofibromatosis-1.

    Science.gov (United States)

    Fang, Yuqiang; Elahi, Abul; Denley, Ryan C; Rao, Pulivarthi H; Brennan, Murray F; Jhanwar, Suresh C

    2009-04-01

    Malignant peripheral nerve sheath tumors (MPNSTs) develop in patients with underlying NF1, and usually arise as a result of malignant transformation of a pre-existing plexiform neurofibroma. The clonal cytogenetic abnormalities reported in primary MPNST include complex karyotypes with chromosome numbers in the triploid or tetraploid range with recurrent abnormalities of several chromosomes including losses or imbalances. As a prelude to cell biological, pharmacological, and functional studies to investigate pathways and gene(s) associated with multistep tumorigenesis, which includes progression, metastasis and resistance to therapy in MPNST, detailed molecular cytogenetic and genetic analyses of cell lines from primary, metastatic and recurrent MPNST with underlying NF1 disorder have been performed. The clonal cytogenetic abnormalities detected in the primary tumor cell line were similar to those observed in primary cultures of this tumor. Due to the complexity of the rearrangements seen by G-banded karyotype analysis, further characterization of the clonal abnormalities in these three cell lines was performed by molecular cytogenetic techniques, including CGH and SKY. CGH analysis detected recurrent deletions of 9p, 12q21-q32, complete losses of the X-chromosome, and gains of the chromosomal segment 17q25 in all three cell lines. SKY analysis detected extensive clonal abnormalities in these cell lines. The nature and the alterations of the cell cycle regulators, particularly those associated with G1-S checkpoints and known to be deregulated in MPNST, were studied. These cell cycle regulators included those associated with Rb1-cyclin D1 and the p53 pathways. The findings are consistent with the argument that an imbalance between the cyclin activators of CDKs and inhibitory proteins such as p16 result in uncontrollable proliferation in the cell lines, associated with progression of the disease. LOH and expression of the p53 gene in metastatic and recurrent cell

  10. NF1 deficiency causes Bcl-xL upregulation in Schwann cells derived from neurofibromatosis type 1-associated malignant peripheral nerve sheath tumors.

    Science.gov (United States)

    Park, Ho-Jin; Lee, Su-Jin; Sohn, Young Bae; Jin, Hyun-Seok; Han, Jae-Ho; Kim, Young-Bae; Yim, Hyunee; Jeong, Seon-Yong

    2013-02-01

    Since the bi-allelic inactivation of both neurofibromin 1 (NF1) gene alleles (NF1(-/-)) in Schwann cells (SCs) is common in both benign plexiform neurofibromas (PNs) and malignant peripheral nerve sheath tumors (MPNSTs) in patients with neurofibromatosis type 1 (NF1), other genetic alterations in SCs may be required for tumor progression of PNs to MPNSTs. We found that the anti-apoptotic Bcl-xL protein is upregulated in MPNST tissues compared to PN tissues from patients with NF1 by immunohistological staining. In addition, we investigated whether Bcl-xL is upregulated in SCs derived from MPNSTs and found a significantly higher Bcl-xL expression level in sNF96.2 MPNST-derived SCs compared to normal human SCs (HSCs). We also discovered that the increased Bcl-xL expression caused an increase in drug resistance to doxorubicin in MPNST-derived SCs. Manipulation of NF1 gene expression levels by treatment with small interfering RNA (siRNA) and overexpression of the neurofibromin GAP-related domain (NF1-GRD) demonstrated that upregulated Bcl-xL expression in MPNST-derived SCs was caused by NF1 deficiency. Treatment with the Erk1/2 inhibitor, PD98059, resulted in a slight increase in Bcl-xL levels in neurofibromin-depleted normal HSCs, indicating that Bcl-xL upregulation in MPNST-derived SCs is mediated by activated Erk1/2, which is a Ras downstream protein regulated by neurofibromin. As the reduction of Bcl-xL expression restored sensitivity to doxorubicin-induced apoptosis in sNF96.2 cells, we examined the effect of the small molecule Bcl-xL inhibitor ABT-737 on sNF96.2 cells. A very low dose of ABT-737 combined with doxorubicin synergistically enhanced sensitivity to doxorubicin-induced apoptosis in sNF96.2 cells, suggesting that ABT-737 and doxorubicin may be a good combination to effectively treat NF1-associated MPNSTs with minimal side-effects. Collectively, our results suggest that upregulation of Bcl-xL in MPNST-derived SCs may be caused by the NF1 deficiency

  11. Efficacy of nerve growth factor on the treatment of optic nerve contusion Evaluation with visual evoked potential

    Institute of Scientific and Technical Information of China (English)

    2007-01-01

    BACKGROUND: Pattern- visual evoked potential (PVEP) can reflect the functional status of retinal ganglial cells (RGC) and visual cortex, and is an objective examination for visual pathway function. It is a unique method for objectively examining the optic nerve function of optic ganglion cells.OBJECTIVE: To observe the effects of nerve growth factor (NGF) on PVEF in the treatment of optic nerve contusion, evaluate the clinical efficacy of NGF, and make an efficacy comparison with vitamin B12.DESIGN: A randomly grouping, controlled observation.SETTING: Department of Ophthalmology, Tangshan Gongren Hospital Affiliated to Hebei Medical University.PARTICIPANTS: Forty patients with optic nerve contusion caused by eye trauma, who received the treatment in the Tangshan Worker Hospital Affiliated to Hebei Medical University between January 2006 and June 2007, were recruited in this study. The involved 40 patients, including 34 males and 6 females,were aged 14 - 59 years. They were confirmed to have optic nerve contusion by ophthalmologic consultation combined with history of disease and orbital CT examination. Informed consents of treatments and detected items were obtained from all the patients. The patients were randomly divided into 2 groups with 20 in each:NGF group and vitamin B12 group.METHODS: Conservative treatment was used in the two groups. In addition, patients in the NGF group were intramuscularly injected with NGF solution 18 μg/time, once a day. Those in the vitamin B12 group were injected by the same method with common vitamin B12 of 500 μg combined with vitamin B1 of 100 mg, once a day.MAIN OUTCOME MEASURES: PVEP examination was conducted in all the patients before, one and two weeks after treatment, and latency and amplitude at P100 were detected.RESULTS: Forty patients with optic nerve contusion participated in the final analysis. Before treatment,significant differences in the latency and amplitude at P100 were not found in patients between two groups

  12. Case of acute optic nerve compression caused by tuberculum sellae meningioma with optic canal involvement

    Directory of Open Access Journals (Sweden)

    Chai Y

    2012-05-01

    Full Text Available Yuzhu Chai1, Hiroko Yamazaki1, Akihide Kondo2, Toshiyuki Oshitari3, Shuichi Yamamoto31Department of Ophthalmology, Kohnodai Hospital, National Center for Global Health and Medicine, Chiba, 2Department of Neurosurgery, Juntendo University, School of Medicine, Tokyo, 3Department of Ophthalmology and Visual Science, Chiba University Graduate School of Medicine, Chiba, JapanAbstract: We present detailed ophthalmic findings in a case of tuberculum sellae meningioma with acute visual symptoms due to optic canal involvement. A 62-year-old Japanese woman reported a 1-week history of headaches and blurred vision in her left eye. Her visual acuity was 0.3 in the left eye with no ophthalmoscopic abnormalities. A relative afferent pupillary defect and inferior temporal field defect were found in the left eye. Pattern visual evoked potentials were undetectable in the left eye. Enhanced magnetic resonance imaging showed a 9 mm intracranial lesion around the left optic nerve anterior to the chiasm. She was diagnosed with granulomatous inflammation because of the increased cell counts and protein concentration in the cerebrospinal fluid. She was treated with steroid pulse therapy, and her visual acuity and visual field defect improved to normal in 3 weeks. However, 16 months after the onset, she suffered from headaches again and had a complete loss of vision in her left eye. There was no response to steroid pulse therapy. Enhanced magnetic resonance imaging revealed that the lesion had extended into the left optic canal, and emergency tumor removal surgery was carried out. The histopathological diagnosis was meningioma. One month after the surgery, her left visual acuity improved to 1.2, and her visual field was almost normal. Pattern visual evoked potentials were present but had a prolonged P100 latency of 170 ms. A thinning of the ganglion cell complex was detected by optical coherence tomography. Ophthalmologists should be aware that a small tuberculum

  13. Integrins in Trabecular Meshwork and Optic Nerve Head: Possible Association with the Pathogenesis of Glaucoma

    Directory of Open Access Journals (Sweden)

    Yisheng Zhong

    2013-01-01

    Full Text Available Integrins are a family of membrane-spanning proteins that are important receptors for cell adhesion to extracellular matrix proteins. They also provide connections between the extracellular environment and intracellular cytoskeletons and are responsible for activation of many intracellular signaling pathways. In vitro and in vivo data strongly indicate that integrin-mediated signaling events can modulate the organization of the actin cytoskeleton in trabecular meshwork (TM cells and are associated with astrocyte migration and microglia activation of the optic nerve head in patients with primary open angle glaucoma. Consequently, increase in resistance in the TM outflow pathways and remodeling of the optic nerve head occur, which in turn increases intraocular pressure (IOP, adds additional mechanical stress and strain to optic nerve axons, and accelerates damage of axons initially caused by optic nerve head remodeling. Integrins appear to be ideal candidates for translating physical stress and strain into cellular responses known to occur in glaucomatous optic neuropathy.

  14. A model of the mammalian optic nerve fibre based on experimental data.

    Science.gov (United States)

    Oozeer, M; Veraart, C; Legat, V; Delbeke, J

    2006-08-01

    Several experimental data about membrane dynamics and pharmacological sensitivities of optic nerve axons have been published. The present work summarizes these data and computer simulations have been used to develop a model of the mammalian optic nerve fibre. The ionic currents description were derived from existing membrane models and particularly from a model of the somatic retinal ganglion cell (RGC) impulse generation. However, original equations had to be modified to match experimental data, which suggests that in RGCs, axonal and somatic ion channel expression are different. The new model is consistent with recent experimental results about optic nerve axonal excitability.

  15. Pit-like changes of the optic nerve head in open-angle glaucoma.

    Science.gov (United States)

    Radius, R. L.; Maumenee, A. E.; Green, W. R.

    1978-01-01

    Six patients with open-angle glaucoma and acquired pit-like changes in the optic nerve head are presented. In 1 patient evolution of the pit-like defect is documented. In all 6 patients progression of associated visual field deficits is described. It is suggested that such pit-like changes in selected patients with glaucoma may not represent congenital lesions but rather local, progressive nerve head disease, occurring particularly in response to raised intraocular pressure. The management of patients with optic nerve head pitting and the pathogenesis of glaucomatous optic neuropathy are discussed with respect to this observation. Images PMID:666988

  16. Assessment of Optic Nerve Impairment in Patients with Neuromyelitis Optica by MR Diffusion Tensor Imaging.

    Directory of Open Access Journals (Sweden)

    Zhiye Chen

    Full Text Available Diffusion tensor imaging (DTI has been used for the evaluation of the white matter integrity. In this study, we evaluated optic nerve impairment in patients with neuromyelitis optica (NMO using DTI.Optic nerve DTI were performed on 28 NMO patients and 38 normal controls. Fractional anisotropy (FA values were measured in the anterior, middle, and posterior parts of the intraorbital optic nerve segment. For the posterior intraorbital optic nerve, FA values of BI (0.20±0.07, MI (0.24±0.16, and NA (0.25±0.14 decreased significantly compared with that of NC (0.43±0.07 (P<0.05, and ROC analysis demonstrated that the area under the curve (AUC measurements for BI vs. NC, MI vs. NC, NA vs. NC, and NMO (including BI, MI, and NA vs. NC were 0.99, 0.93, 0.88, and 0.96, respectively. The corresponding diagnostic sensitivities of ROC analysis were 100%, 80%, 80%, and 91%; and the specificities were 93%, 97%, 91%, and 93%.Decreased FA value in the intraorbital optic nerve, especially in the posterior part of the nerve, was demonstrated as a characteristic MR feature for NMO-related optic nerve impairment.

  17. Investigation of multiple optical and biometric properties of optic nerve head (Conference Presentation)

    Science.gov (United States)

    Hong, Young-Joo; Chan, Aaron C.; Kasaragod, Deepa K.; Makita, Shuichi; Miura, Masahiro; Yasuno, Yoshiaki

    2017-02-01

    Glaucoma is a group of eye diseases which results in optic nerve damage and vision loss. Optical coherence tomography (OCT) has been widely used to investigate geometric risk factor of glaucoma. However, material properties of ONH are also important to understand intra-ocular pressure related stress. We developed Jones-matrix based multifunctional posterior eye OCT (JM-OCT), which uses 1-μm band swept-source with a 100-kHz A-line rate. It provides three different optical properties, attenuation coefficient (AC), local birefringence (LB), and optical coherence angiography (OCA). We investigated the utility those properties for the investigation of normal ONH cases. 3 mm x 3 mm area around ONH was scanned for each eye, and biometric parameters were measured in hospital. Statistical analyses were performed with the mean values of above parameters at the regions of prelamina, lamina cribrosa, peripapillary sclera, and peripapillary nerve fiber layer, and biometric parameters of age, axial eye length, refractive error, and intraocular pressure. In qualitative observation, the lamina cribrosa generally shows more hyper signals in AC, LB, and OCA than prelamina. In t-test, AC, LB, and OCA showed significant difference (p eye length is positively correlated with LB and AC in lamina cribrosa. And these LB and AC are also negatively correlated with the refractive error. Age was found to be negatively correlated with OCA in lamina cribrosa.

  18. Diffusion MR Imaging of Postoperative Bilateral Acute Ischemic Optic Neuropathy

    Energy Technology Data Exchange (ETDEWEB)

    Park, Ju Young; Lee, In Ho; Song, Chang June [Chungnam National University Hospital, Daejeon (Korea, Republic of); Hwang, Hee Youn [Eulji University Hospital, Daejeon(Korea, Republic of)

    2012-03-15

    A 57-year-old woman experienced bilateral acute ischemic optic neuropathy after spine surgery. Routine MR imaging sequence, T2-weighted image, showed subtle high signal intensity on bilateral optic nerves. A contrast-enhanced T1 weighted image showed enhancement along the bilateral optic nerve sheath. Moreover, diffusion-weighted image (DWI) and an apparent diffusion coefficient map showed markedly restricted diffusion on bilateral optic nerves. Although MR findings of T2-weighted and contrast enhanced T1-weighted images may be nonspecific, the DWI finding of cytotoxic edema of bilateral optic nerves will be helpful for the diagnosis of acute ischemic optic neuropathy after spine surgery.

  19. Method for optic nerve intensity characterization in mice from Mn2+ enhanced MR images

    Science.gov (United States)

    Xiao, D.; Ribic, J.; Butzkueven, H.; Fang, K.; Kilpatrick, T. J.; Egan, G. F.; Salvado, O.

    2009-02-01

    Investigating whether manganese transport is impaired in the optic nerve of small animal model is a new approach for evaluating optic neuritis. One needs to quantify signal intensity enhancement due to Mn2+ after intra-orbital injection, along the optic nerve from MR images. Quantification is very challenging as the optic nerve (ON) is not straight, its location does not correspond to standard slice orientation, the noise is substantial, and the signal is subject to inhomogeneity from the coil sensitivity. In this paper, we propose a semi-automatic method whereby 1) the retina point and the start of the chiasm in a mouse brain MR image are defined manually in a 3D visualization environment, 2) optic nerve in reformatted slices perpendicular to the optic nerve segment is semi-manually selected, 3) an automatic algorithm extracts the intensities along the optic nerve while correcting for intensity inhomogeneity, and 4) a model for the Mn2+ diffusion with a exponential decay function is fitted to the intensity profile. Results for the study of experimental autoimmune encephalomyelitis (EAE) are reported whereby statistically significant differences were found between the EAE and the control group.

  20. Network analysis of human glaucomatous optic nerve head astrocytes

    Directory of Open Access Journals (Sweden)

    Bhattacharya Sanjoy K

    2009-05-01

    Full Text Available Abstract Background Astrocyte activation is a characteristic response to injury in the central nervous system, and can be either neurotoxic or neuroprotective, while the regulation of both roles remains elusive. Methods To decipher the regulatory elements controlling astrocyte-mediated neurotoxicity in glaucoma, we conducted a systems-level functional analysis of gene expression, proteomic and genetic data associated with reactive optic nerve head astrocytes (ONHAs. Results Our reconstruction of the molecular interactions affected by glaucoma revealed multi-domain biological networks controlling activation of ONHAs at the level of intercellular stimuli, intracellular signaling and core effectors. The analysis revealed that synergistic action of the transcription factors AP-1, vitamin D receptor and Nuclear Factor-kappaB in cross-activation of multiple pathways, including inflammatory cytokines, complement, clusterin, ephrins, and multiple metabolic pathways. We found that the products of over two thirds of genes linked to glaucoma by genetic analysis can be functionally interconnected into one epistatic network via experimentally-validated interactions. Finally, we built and analyzed an integrative disease pathology network from a combined set of genes revealed in genetic studies, genes differentially expressed in glaucoma and closely connected genes/proteins in the interactome. Conclusion Our results suggest several key biological network modules that are involved in regulating neurotoxicity of reactive astrocytes in glaucoma, and comprise potential targets for cell-based therapy.

  1. Practical Considerations for Optic Nerve Estimation in Telemedicine

    Energy Technology Data Exchange (ETDEWEB)

    Karnowski, Thomas Paul [ORNL; Aykac, Deniz [ORNL; Chaum, Edward [ORNL; Giancardo, Luca [ORNL; Li, Yaquin [University of Tennessee, Knoxville (UTK); Tobin Jr, Kenneth William [ORNL

    2009-01-01

    The projected increase in diabetes in the United States and worldwide has created a need for broad-based, inexpensive screening for diabetic retinopathy (DR), an eye disease which can lead to vision impairment. A telemedicine network with retina cameras and automated quality control, physiological feature location, and lesion / anomaly detection is a low-cost way of achieving broad-based screening. In this work we report on the effect of quality estimation on an optic nerve (ON) detection method with a confidence metric. We report on an improvement of the fusion technique using a data set from an ophthalmologists practice then show the results of the method as a function of image quality on a set of images from an on-line telemedicine network collected in Spring 2009 and another broad-based screening program. We show that the fusion method, combined with quality estimation processing, can improve detection performance and also provide a method for utilizing a physician-in-the-loop for images that may exceed the capabilities of automated processing.

  2. Dark rearing maintains tyrosine hydroxylase expression in retinal amacrine cells following optic nerve transection

    Institute of Scientific and Technical Information of China (English)

    Wei Wan; Zhenghai Liu; Xiaosheng Wang; Xuegang Luo

    2012-01-01

    The present study examined changes in retinal tyrosine hydroxylase (TH) expression in rats having undergone optic nerve transection and housed under a normal day/night cycle or in the dark. The aim was to investigate the effects of amacrine cells on axonal regeneration in retinal ganglion cells and on the synapses that transmit visual signals. The results revealed that retinal TH expression gradually decreased following optic nerve transection in rats housed under a normal day/night cycle, reaching a minimum at 5 days. In contrast, retinal TH expression decreased to a minimum at 1 day following optic nerve transection in dark reared rats, gradually increasing afterward and reaching a normal level at 5-7 days. The number of TH-positive synaptic particles correlated with the TH levels, indicating that dark rearing can help maintain TH expression during the synaptic degeneration stage (5-7 days after optic nerve injury) in retinal amacrine cells.

  3. Calpain inhibitor attenuated optic nerve damage in acute optic neuritis in rats

    Science.gov (United States)

    Das, Arabinda; Guyton, M. Kelly; Smith, Amena; Wallace, Gerald; McDowell, Misty L.; Matzelle, Denise D.; Ray, Swapan K.; Banik, Naren L.

    2012-01-01

    Optic neuritis (ON), which is an acute inflammatory autoimmune demyelinating disease of the central nervous system (CNS), often occurs in multiple sclerosis (MS). ON is an early diagnostic sign in most MS patients caused by damage to the optic nerve leading to visual dysfunction. Various features of both MS and ON can be studied following induction of experimental autoimmune encephalomyelitis (EAE), an animal model of MS, in Lewis rats. Inflammation and cell death in the optic nerve, with subsequent damage to the retinal ganglion cells in the retina, are thought to correlate with visual dysfunction. Thus, characterizing the pathophysiological changes that lead to visual dysfunction in EAE animals may help develop novel targets for therapeutic intervention. We treated EAE animals with and without the calpain inhibitor calpeptin (CP). Our studies demonstrated that the Ca2+-activated neutral protease calpain was upregulated in the optic nerve following induction of EAE at the onset of clinical signs (OCS) of the disease and these changes were attenuated following treatment with CP. These reductions correlated with decreases in inflammation (cytokines, iNOS, COX-2, NF-κB), and microgliosis (i.e. activated microglia). We observed that calpain inhibition reduced astrogliosis (reactive astroglia) and expression of aquaporin 4 (AQP4). The balance of Th1/Th2 cytokine production and also expression of the Th1-related CCR5 and CXCR3 chemokine receptors influence many pathological processes and play both causative and protective roles in neuron damage. Our data indicated that CP suppressed cytokine imbalances. Also, Bax:Bcl-2 ratio, production of tBid, PARP-1, expression and activities of calpain and caspases, and internucleosomal DNA fragmentation were attenuated after treatment with CP. Our results demonstrated that CP decreased demyelination [loss of myelin basic protein (MBP)] and axonal damage [increase in dephosphorylated neurofilament protein (de-NFP), and also

  4. Prenatal determinants of optic nerve hypoplasia: Review of suggested correlates and future focus

    OpenAIRE

    Garcia-Filion, Pamela; Borchert, Mark

    2013-01-01

    Optic nerve hypoplasia (ONH), a congenital malformation characterized by an underdeveloped optic nerve, is a seemingly epidemic cause of childhood blindness and visual impairment with associated lifelong morbidity. While the prenatal determinants of ONH are unknown, early case reports have led to a longstanding speculation that risky health behaviors (e.g. recreational drugs, alcohol) are a likely culprit. There has yet to be a systematic review of the epidemiology of ONH to assess the common...

  5. Optic nerve oxygen tension in pigs and the effect of carbonic anhydrase inhibitors

    DEFF Research Database (Denmark)

    Stefánsson, E; Jensen, P K; Eysteinsson, T

    1999-01-01

    To evaluate how the oxygen tension of the optic nerve (ONP(O)2) is affected by the administration of the carbonic anhydrase inhibitors dorzolamide and acetazolamide and by alterations in oxygen and carbon dioxide in the breathing mixture.......To evaluate how the oxygen tension of the optic nerve (ONP(O)2) is affected by the administration of the carbonic anhydrase inhibitors dorzolamide and acetazolamide and by alterations in oxygen and carbon dioxide in the breathing mixture....

  6. Intermediate filaments of zebrafish retinal and optic nerve astrocytes and Müller glia: differential distribution of cytokeratin and GFAP

    OpenAIRE

    Mosier Amanda L; Koke Joseph R; García Dana M

    2010-01-01

    Abstract Background Optic nerve regeneration (ONR) following injury is a model for central nervous system regeneration. In zebrafish, ONR is rapid - neurites cross the lesion and enter the optic tectum within 7 days; in mammals regeneration does not take place unless astrocytic reactivity is suppressed. Glial fibrillary acidic protein (GFAP) is used as a marker for retinal and optic nerve astrocytes in both fish and mammals, even though it has long been known that astrocytes of optic nerves i...

  7. Morphological and electrophysiological characterization of the adult Siberian hamster optic nerve.

    Science.gov (United States)

    James, Emma L; Peacock, Veronique A H; Ebling, Francis J P; Brown, Angus M

    2010-12-01

    Electrophysiological recordings and transmission electron microscopy were used to characterize the compound action potential (CAP) and morphology, respectively, of the optic nerve in the Siberian hamster. The CAP was polyphasic in nature, comprising four separate but overlapping peaks, thereby implying that four sub-populations of axons defined by conduction velocity are present in the nerve. The histological analysis of nerves from four animals revealed a cross-sectional area of 128,171 μm(2) containing 78,109 axons. All of the axons were myelinated, and measurements of axon surface area revealed values ranging from 0.09 to 9.92 μm(2), although 68.3% were 2.5 μm(2)) were selectively distributed throughout the nerve. We conclude that the CAP recorded from hamster optic nerve displays four distinct peaks; however, morphological analysis failed to reveal a similar distribution of axon sizes.

  8. Complete avulsion of the globe and the optic nerve: a case report.

    Science.gov (United States)

    Ajike, S O; Oladigbolu, K K; Ogbeifun, J O; Samaila, E; Omisakin, O O; Ajike, B A

    2014-01-01

    Blunt trauma to the globe may cause rupture or avulsion of the globe with its attendant consequences. Traumatic avulsion of the globe and optic nerve are rare because of the protection offered by the bony socket and the resistance of the globe due to its pressure and the thickness of the nerve tissues. However, there are a few documented cases of avulsion of the globe and optic nerve in the literature. We report a case of traumatic avulsion of the left globe in a 38-year-old female Nigerian. Primary enucleation of the avulsed globe with insertion of orbital prosthesis was done.

  9. Optic nerve head and fibre layer imaging for diagnosing glaucoma

    Science.gov (United States)

    Michelessi, Manuele; Lucenteforte, Ersilia; Oddone, Francesco; Brazzelli, Miriam; Parravano, Mariacristina; Franchi, Sara; Ng, Sueko M; Virgili, Gianni

    2016-01-01

    Background The diagnosis of glaucoma is traditionally based on the finding of optic nerve head (ONH) damage assessed subjectively by ophthalmoscopy or photography or by corresponding damage to the visual field assessed by automated perimetry, or both. Diagnostic assessments are usually required when ophthalmologists or primary eye care professionals find elevated intraocular pressure (IOP) or a suspect appearance of the ONH. Imaging tests such as confocal scanning laser ophthalmoscopy (HRT), optical coherence tomography (OCT) and scanning laser polarimetry (SLP, as used by the GDx instrument), provide an objective measure of the structural changes of retinal nerve fibre layer (RNFL) thickness and ONH parameters occurring in glaucoma. Objectives To determine the diagnostic accuracy of HRT, OCT and GDx for diagnosing manifest glaucoma by detecting ONH and RNFL damage. Search methods We searched several databases for this review. The most recent searches were on 19 February 2015. Selection criteria We included prospective and retrospective cohort studies and case-control studies that evaluated the accuracy of OCT, HRT or the GDx for diagnosing glaucoma. We excluded population-based screening studies, since we planned to consider studies on self-referred people or participants in whom a risk factor for glaucoma had already been identified in primary care, such as elevated IOP or a family history of glaucoma. We only considered recent commercial versions of the tests: spectral domain OCT, HRT III and GDx VCC or ECC. Data collection and analysis We adopted standard Cochrane methods. We fitted a hierarchical summary ROC (HSROC) model using the METADAS macro in SAS software. After studies were selected, we decided to use 2 × 2 data at 0.95 specificity or closer in meta-analyses, since this was the most commonly-reported level. Main results We included 106 studies in this review, which analysed 16,260 eyes (8353 cases, 7907 controls) in total. Forty studies (5574

  10. The Protective Role of Mecobalamin Following Optic Nerve Crush in Adult Rats

    Institute of Scientific and Technical Information of China (English)

    Xiangmei Kong; Xinghuai Sun; Jinjun Zhang

    2004-01-01

    Purpose: To evaluate the potential for Mecobalamin as a neuroprotective agent in optic nerve crush injury.Methods: Twenty-four adult Sprague-Dawley rats were randomly divided into four groups.One group acted as normal controls, while in the other three groups the right eye was subjected to optic nerve crush injury. Of the three crush injury groups one group received no treatment, while the other two groups received intramuscular injections of VitaminB12 or Mecobalamin (10μg) immediately after crush injury and then every two days. All the rats were sacrificed one month post-treatment, and the eyes attached with optic nerves were removed for histology. The morphological changes of optic nerve axons and retinal ganglion cells (RGCs) were assessed under light microscope (LM) and transmission electromicroscope (TEM). The numbers of axons and RGCs were counted.Results: In this study we demonstrate the potential for Mecobalamin as a neuroprotective agent following optic nerve crush injury. We show here that the axons of optic nerves were loose in structure or destroyed. The mitochondria of the RGCs was swollen, and the Nissel body was less evident after the crush injury. Moreover, the number of axons and RGCs was significantly reduced (P < 0.001). However, these changes were less dramatic after the Mecobalamin-treatment. More axons and RGCs were remained in the group than those in the untreated injury group (P = 0.010 and 0.003 respectively), and those in the VitaminB12-treated group (P=0.037 and 0.035 respectively). More significantly, there were newly formed axons found in the Mecobalamin-treated group.Conclusions: Optic nerve crush injury in rats causes the loss of the axons and RGCs but this may be ameliorated by treatment with Mecobalamin.

  11. Neuromyelitis optica IgG in the cerebrospinal fluid induces astrocytopathy in optic nerve

    DEFF Research Database (Denmark)

    Soelberg, Kerstin; Lillevang, Søren Thue; Mørch, Marlene

    (anti-CD59a). A total of five mice received AQP4-IgG + C + anti-CD59a, four mice received normal-IgG + C + anti-CD59a, four mice received AQP4-IgG+ C and one normal-IgG + C. Mice were killed four days later. The optic nerves were isolated and fixed in paraformaldehyde. Paraffin embedded optic nerves...... was coincident with deposition of complement. Histopathological lesions were markedly enhanced with extensive/long-segment astrocytopathy of optic nerve and optic chiasm involvement in AQP4- IgG+ C + anti-CD59a treated mice. Such pathology was not seen in mice receiving normal human IgG, C and anti-CD59a...

  12. Optical coherence tomography in papilledema and pseudopapilledema with and without optic nerve head drusen

    Directory of Open Access Journals (Sweden)

    Shikha Talwar Bassi

    2014-01-01

    Full Text Available Aim: To compare the spectral domain optical coherence tomography (SD-OCT findings of the optic disc and the peripapillary retina of patients with a true papilledema and pseudopapilledema with and without optic nerve head drusen (ONHD. Study Design: Retrospective Case Control Study. Subjects and Methods: Peripapillary retinal nerve fiber layer (PPRNFL thickness as depicted by SD-OCT of 94 eyes of 66 patients with papilledema (30 eyes, pseudopapiledema (31 eyes, and normal controls (33 eyes was analyzed. The mean RNFL thickness, total retinal thickness (TRT at a superior and inferior edge of the disc and the quadrant wise topography of increased RNFL were compared in all three groups. Sensitivity, specificity, and area under the receiver operating characteristic curve (AROC were calculated for all the parameters. Results: The median RNFL thickness was 185.4 (129.5-349.3 μm, 122.3 (109-156.3 μm and 91.62 ± 7 μm in papilledema, pseudopapilledema, and controls, respectively. Papilledema group had thicker PPRNFL in all quadrants except temporal quadrant. TRT was thicker in papilledema and pseudopapilledema compared to controls. ONHD could be directly visualized as high reflective clumps in the sub-retinal space or the RNFL in 30 eyes. Increased RNFL thickness in all four quadrants was noted 43.3% in papilledema and 9.7% in pseudopapilledema. Normal RNFL thickness in all four quadrants was noted in 0% in papilledema and 32.3% in pseudopapilledema. Nasal RNFL had the highest AROC (0.792 indicating high diagnostic ability to differentiate papilledema from pseudopapilledema. Conclusion: SD-OCT can be used as a tool to differentiate between papilledema and pseudopapilledema.

  13. Vitro Culture and Immunohistochemical Identification of Astrocytes of Infantile Optic Nerve

    Institute of Scientific and Technical Information of China (English)

    Jianliang Zheng; Yuqing Lan; Jie Zhang; Yan Guo; Yan Luo

    2000-01-01

    Objective: To culture astrocytes of optic nerve and to establish the cell lines for further study of healing process after optic nerve trauma.Methods: Optic nerve astrocytes of infantile and adults with sudden death were cultured by tissue inoculation or tissue digestion with 0. 25% Trypsin and 0. 06% EDTA. The second and fourth passage cells were stained with HE and anti- GFAP, S-100 protein, Vimentin, and CD34 antibodies.Results: The trypsinized astrocytes of infantile optic nerve reached confluence in 7 days,but the astrocytes of adults weren't successfully cultured. The cultured cells were in polygonal shape with processes; the cytoplasm was abundant and pink; the cells had light-blue nuclei. These cells were positive in GFAP, S-100 protein and vimentin staining, and negative in CD34 staining.Conclusions: The results showed that astrocytes of infantile optic nerve can be successfully cultured and trypsinization is a better method than tissue inoculation. The culture of infantile astrocytes is easier than that of adult astrocytes. Immunohistochemistry were used to determine the source and type of those cultured cells.

  14. Malignant Peripheral Nerve Sheath Tumor: One Case Report and Literature Review%颌面部恶性外周神经鞘瘤1例报道及文献回顾

    Institute of Scientific and Technical Information of China (English)

    张素欣; 裴超; 李天客; 张鑫; 段玉芹

    2016-01-01

    恶性外周神经鞘瘤(malignant peripheral nerve sheath tumor,MPNST)是一种罕见的、来源于外周神经施万细胞或显示外周神经鞘分化特征的软组织高度恶性肿瘤.发生于颌面部内的MPNST病例少有报道,在诊断和治疗上仍然存在困难,该病具有高度侵袭性和远处转移倾向,预后不理想.手术切除是最为有效的治疗方法,术后局部复发率高,术后辅助放疗亦能有效降低复发率,化疗的效果尚不确切.

  15. Optic nerve involvement in childhood onset systemic lupus erythematosus: Three cases and a review of the literature.

    Science.gov (United States)

    Suri, D; Abujam, B; Gupta, A; Rawat, A; Saikia, B; Walker Minz, R; Gupta, V; Bansal, R; Kaushik, S; Singh, S

    2016-01-01

    The ocular system can be affected in systemic lupus erythematosus (SLE) in one third of patients. However, optic nerve involvement is relatively uncommon, but is more so in pediatric SLE patients, where it can occur in 1% of cases. We report three children with SLE who presented with optic nerve involvement. Two children had optic neuritis, with optic neuritis being the first manifestation in one child. The third child had ischaemic optic neuropathy secondary to antiphospholipid syndrome. A careful work up for SLE should be performed in every child with optic nerve disease. Prompt diagnosis and early treatment results in a better prognosis.

  16. Effect of Maixuekang capsule therapy on optic nerve function, blood coagulation function and cytokines in diabetic optic neuropathy

    Institute of Scientific and Technical Information of China (English)

    Ya-Li Hao

    2016-01-01

    Objective:To analyze the effect of Maixuekang capsule therapy on optic nerve function, blood coagulation function and cytokines in diabetic optic neuropathy.Methods: A total of 55 patients (82 eyes) with diabetic optic neuropathy treated in our hospital between December 2013 and December 2015 were selected, and according to different therapeutic methods, they were divided into observation group (n=38) (49 eyes) who received Maixuekang therapy and control group (n=17) (33 eyes) who received compound vitamin therapy. Differences in optic nerve function, blood coagulation function and cytokine content were compared between two groups after 3 months of treatment.Results:After 3 months of treatment, optic nerve function indexes MS, RNFL thickness and AP100 levels of observation group were higher than those of control group while MD and LP100 levels were lower than those of control group; blood coagulation indexes WBV, PV and FBG levels were lower than those of control group while TT, PT and APTT levels were higher than those of control group; thrombelastogram parameters R value and K value levels were higher than those of control group while α angle, MA and CI levels were lower than those of control group; oxidative stress indexes ROS, MDA and CAT content in serum were lower than those of control group while SOD content was higher than that of control group.Conclusions:Maixuekang capsule can significantly optimize the optic nerve function in patients with DON, which is specifically directly related to its anticoagulation and anti-oxidative stress effect.

  17. The effects of tumor location on diagnostic criteria for canine malignant peripheral nerve sheath tumors (MPNSTs) and the markers for distinction between canine MPNSTs and canine perivascular wall tumors.

    Science.gov (United States)

    Suzuki, S; Uchida, K; Nakayama, H

    2014-07-01

    Canine malignant peripheral nerve sheath tumors (MPNSTs) occur not only in the peripheral nervous system (PNS) but also in soft tissue and various organs (non-PNS). The most important diagnostic criterion is proof of peripheral nerve sheath origin. This is difficult in non-PNS MPNSTs, and its differential diagnosis is challenging. Canine perivascular wall tumors (PWTs) also commonly arise in soft tissue. Their histopathological features are quite similar to those of canine MPNSTs, making their differential diagnosis challenging. To elucidate whether the morphological features are applicable to diagnose non-PNS MPNSTs and to demonstrate useful markers for distinction between canine MPNSTs and PWTs, the authors examined 30 canine MPNSTs and 31 PWTs immunohistochemically for S100, nestin, NGFR, Olig2, claudin-1, CD57, PRX, α-SMA, desmin, and calponin. Among canine MPNSTs, the PNS tumors displayed significantly higher S100 and Olig2 expression than the non-PNS tumors. The expression levels of the other markers did not differ significantly, suggesting that the same morphological diagnostic criteria are applicable regardless of their location. The PWT cells displayed significantly weaker immunoreactivity than MPNSTs to markers used except α-SMA and desmin. Cluster analysis sorted most canine MPNSTs and PWTs into 2 distinctly different clusters, whereas 3 MPNSTs and 6 PWTs were assigned to the opposing cluster. These 3 MPNSTs were negative for almost all markers, while these 6 PWTs were positive for only neuronal markers. In particular, NGFR and Olig2 were almost negative in the rest of PWT cases. These findings suggest that NGFR and Olig2 are useful to distinguish these 2 tumors.

  18. CT and MRI features of malignant peripheral nerve sheath tumor of children%儿童恶性外周神经鞘瘤的CT和MRI表现

    Institute of Scientific and Technical Information of China (English)

    刘志敏; 宋蕾; 高军; 于彤; 尹光恒; 蒋玲; 彭芸; 胡克非

    2015-01-01

    目的:分析儿童恶性外周神经鞘瘤(MPNST)的CT和MRI特点,提高对本病的认识。方法回顾性分析16例经手术病理证实的MPNST的CT和MRI表现。男8例、女8例;年龄0.3~11.0岁,中位年龄2.5岁。16例均进行了CT平扫,其中8例行CT增强扫描,3例进行了MR检查。2名高年资儿童影像医师共同对图像进行分析,并协商达成一致意见。结果16例瘤灶位于颈部3例、腰背部5例、腹盆腔3例、足部2例、左侧锁骨下1例、右纵隔1例、右侧眼眶1例。15例呈肿块型,1例呈弥漫生长。CT表现:15例肿块型平扫呈实性为主(8例)或囊实性(7例)。增强扫描实性成分呈中度以上强化、渐进延迟强化,囊性成分未见明显强化。1例弥漫生长病变位于左侧颈部,与周围组织分界不清。MRI表现:2例肿块型MRI表现为T1WI呈等低信号,T2WI呈等高信号,T2WI囊性成分明显高信号,增强后有明显不均匀强化。1例弥漫生长病变MRI表现:T1WI呈稍低信号,T2WI呈等稍高信号,并明显强化。16例均呈不同程度侵袭性生长,11例伴多脏器转移及复发,4例合并神经纤维瘤病Ⅰ型,并脊柱侧弯。结论恶性外周神经鞘瘤CT及MRI表现具有一定特征改变,并能显示其侵袭性表现及多脏器转移表现,有利于明确诊断并协助临床医生制定治疗方案。%Objective To analyze CT and MR features of malignant peripheral nerve sheath tumor (MPNST)of children. Methods Sixteen patients with histologically proven MPNST were retrospectively reviewed.There were 8 male and 8 female, ages from 0.3 to 11.0 years, and median age was 2.5 years. Sixteen cases were performed with CT plain scan, and eight cases with CT enhancement scans, and three with MR examination. The imaging data were analyzed by two highly experienced doctors and obtained agreements after mutual consultation. Results Among 16 cases, 3 cases were located at neck, 5

  19. Repeatability and reproducibility of optic nerve head perfusion measurements using optical coherence tomography angiography

    Science.gov (United States)

    Chen, Chieh-Li; Bojikian, Karine D.; Xin, Chen; Wen, Joanne C.; Gupta, Divakar; Zhang, Qinqin; Mudumbai, Raghu C.; Johnstone, Murray A.; Chen, Philip P.; Wang, Ruikang K.

    2016-06-01

    Optical coherence tomography angiography (OCTA) has increasingly become a clinically useful technique in ophthalmic imaging. We evaluate the repeatability and reproducibility of blood perfusion in the optic nerve head (ONH) measured using optical microangiography (OMAG)-based OCTA. Ten eyes from 10 healthy volunteers are recruited and scanned three times with a 68-kHz Cirrus HD-OCT 5000-based OMAG prototype system (Carl Zeiss Meditec Inc., Dublin, California) centered at the ONH involving two separate visits within six weeks. Vascular images are generated with OMAG processing by detecting the differences in OCT signals between consecutive B-scans acquired at the same retina location. ONH perfusion is quantified as flux, vessel area density, and normalized flux within the ONH for the prelaminar, lamina cribrosa, and the full ONH. Coefficient of variation (CV) and intraclass correlation coefficient (ICC) are used to evaluate intravisit and intervisit repeatability, and interobserver reproducibility. ONH perfusion measurements show high repeatability [CV≤3.7% (intravisit) and ≤5.2% (intervisit)] and interobserver reproducibility (ICC≤0.966) in all three layers by three metrics. OCTA provides a noninvasive method to visualize and quantify ONH perfusion in human eyes with excellent repeatability and reproducibility, which may add additional insight into ONH perfusion in clinical practice.

  20. Axonal protection by short-term hyperglycemia with involvement of autophagy in TNF-induced optic nerve degeneration

    Directory of Open Access Journals (Sweden)

    Kana eSase

    2015-10-01

    Full Text Available Previous reports showed that short-term hyperglycemia protects optic nerve axons in a rat experimental hypertensive glaucoma model. In this study, we investigated whether short-term hyperglycemia prevents tumor necrosis factor (TNF-induced optic nerve degeneration in rats and examined the role of autophagy in this axon change process. In phosphate-buffered saline-treated rat eyes, no significant difference in axon number between the normoglycemic (NG and streptozotocin-induced hyperglycemic (HG groups was seen at 2weeks. Substantial degenerative changes in the axons were noted 2 weeks after intravitreal injection of TNF in the NG group. However, the HG group showed significant protective effects on axons against TNF-induced optic nerve degeneration compared with the NG group. This protective effect was significantly inhibited by 3-methyladenine, an autophagy inhibitor. Immunoblot analysis showed that the LC3-II level in the optic nerve was increased in the HG group compared with the NG group. Increased p62 protein levels in the optic nerve after TNF injection was observed in the NG group, and this increase was inhibited in the HG group. Electron microscopy showed that autophagosomes were increased in optic nerve axons in the HG group. Immunohistochemical study showed that LC3 was colocalized with nerve fibers in the retina and optic nerve in both the NG and HG groups. Short-term hyperglycemia protects axons against TNF-induced optic nerve degeneration. This axonal-protective effect may be associated with autophagy machinery.

  1. Optic nerve, superior colliculus, visual thalamus, and primary visual cortex of the northern elephant seal (Mirounga angustirostris) and California sea lion (Zalophus californianus).

    Science.gov (United States)

    Turner, Emily C; Sawyer, Eva K; Kaas, Jon H

    2017-02-11

    The northern elephant seal (Mirounga angustirostris) and California sea lion (Zalophus californianus) are members of a diverse clade of carnivorous mammals known as pinnipeds. Pinnipeds are notable for their large, ape-sized brains, yet little is known about their central nervous system. Both the northern elephant seal and California sea lion spend most of their lives at sea, but each also spends time on land to breed and give birth. These unique coastal niches may be reflected in specific evolutionary adaptations to their sensory systems. Here, we report on components of the visual pathway in these two species. We found evidence for two classes of myelinated fibers within the pinniped optic nerve, those with thick myelin sheaths (elephant seal: 9%, sea lion: 7%) and thin myelin sheaths (elephant seal: 91%, sea lion: 93%). In order to investigate the architecture of the lateral geniculate nucleus, superior colliculus, and primary visual cortex, we processed brain sections from seal and sea lion pups for Nissl substance, cytochrome oxidase, and vesicular glutamate transporters. As in other carnivores, the dorsal lateral geniculate nucleus consisted of three main layers, A, A1, and C, while each superior colliculus similarly consisted of seven distinct layers. The sea lion visual cortex is located at the posterior side of cortex between the upper and lower banks of the postlateral sulcus, while the elephant seal visual cortex extends far more anteriorly along the dorsal surface and medial wall. These results are relevant to comparative studies related to the evolution of large brains.

  2. Imaging retinal nerve fiber bundles using optical coherence tomography with adaptive optics.

    Science.gov (United States)

    Kocaoglu, Omer P; Cense, Barry; Jonnal, Ravi S; Wang, Qiang; Lee, Sangyeol; Gao, Weihua; Miller, Donald T

    2011-08-15

    Early detection of axonal tissue loss in retinal nerve fiber layer (RNFL) is critical for effective treatment and management of diseases such as glaucoma. This study aims to evaluate the capability of ultrahigh-resolution optical coherence tomography with adaptive optics (UHR-AO-OCT) for imaging the RNFL axonal bundles (RNFBs) with 3×3×3μm(3) resolution in the eye. We used a research-grade UHR-AO-OCT system to acquire 3°×3° volumes in four normal subjects and one subject with an arcuate retinal nerve fiber layer defect (n=5; 29-62years). Cross section (B-scans) and en face (C-scan) slices extracted from the volumes were used to assess visibility and size distribution of individual RNFBs. In one subject, we reimaged the same RNFBs twice over a 7month interval and compared bundle width and thickness between the two imaging sessions. Lastly we compared images of an arcuate RNFL defect acquired with UHR-AO-OCT and commercial OCT (Heidelberg Spectralis). Individual RNFBs were distinguishable in all subjects at 3° retinal eccentricity in both cross-sectional and en face views (width: 30-50μm, thickness: 10-15μm). At 6° retinal eccentricity, RNFBs were distinguishable in three of the five subjects in both views (width: 30-45μm, thickness: 20-40μm). Width and thickness RNFB measurements taken 7months apart were strongly correlated (p<0.0005). Mean difference and standard deviation of the differences between the two measurement sessions were -0.1±4.0μm (width) and 0.3±1.5μm (thickness). UHR-AO-OCT outperformed commercial OCT in terms of clarity of the microscopic retina. To our knowledge, these are the first measurements of RNFB cross section reported in the living human eye.

  3. Comparison of optic area measurement using fundus photography and optical coherence tomography between optic nerve head drusen and control subjects.

    Science.gov (United States)

    Flores-Rodríguez, Patricia; Gili, Pablo; Martín-Ríos, María Dolores; Grifol-Clar, Eulalia

    2013-03-01

    To compare optic disc area measurement between optic nerve head drusen (ONHD) and control subjects using fundus photography, time-domain optical coherence tomography (TD-OCT) and spectral-domain optical coherence tomography (SD-OCT). We also made a comparison between each of the three techniques. We performed our study on 66 eyes (66 patients) with ONHD and 70 healthy control subjects (70 controls) with colour ocular fundus photography at 20º (Zeiss FF 450 IR plus), TD-OCT (Stratus OCT) with the Fast Optic Disc protocol and SD-OCT (Cirrus OCT) with the Optic Disc Cube 200 × 200 protocol for measurement of the optic disc area. The measurements were made by two observers and in each measurement a correction of the image magnification factor was performed. Measurement comparison using the Student's t-test/Mann-Whitney U test, the intraclass correlation coefficient, Pearson/Spearman rank correlation coefficient and the Bland-Altman plot was performed in the statistical analysis. Mean and standard deviation (SD) of the optic disc area in ONHD and in controls was 2.38 (0.54) mm(2) and 2.54 (0.42) mm(2), respectively with fundus photography; 2.01 (0.56) mm(2) and 1.66 (0.37) mm(2), respectively with TD-OCT, and 2.03 (0.49) mm(2) and 1.75 (0.38) mm(2), respectively with SD-OCT. In ONHD and controls, repeatability of optic disc area measurement was excellent with fundus photography and optical coherence tomography (TD-OCT and SD-OCT), but with a low degree of agreement between both techniques. Optic disc area measurement is smaller in ONHD compared to healthy subjects with fundus photography, unlike time-domain and spectral-domain optical coherence tomography in which the reverse is true. Both techniques offer good repeatability, but a low degree of correlation and agreement, which means that optic disc area measurement is not interchangeable or comparable between techniques. Ophthalmic & Physiological Optics © 2013 The College of Optometrists.

  4. Upregulation of leukemia inhibitory factor (LIF during the early stage of optic nerve regeneration in zebrafish.

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    Kazuhiro Ogai

    Full Text Available Fish retinal ganglion cells (RGCs can regenerate their axons after optic nerve injury, whereas mammalian RGCs normally fail to do so. Interleukin 6 (IL-6-type cytokines are involved in cell differentiation, proliferation, survival, and axon regrowth; thus, they may play a role in the regeneration of zebrafish RGCs after injury. In this study, we assessed the expression of IL-6-type cytokines and found that one of them, leukemia inhibitory factor (LIF, is upregulated in zebrafish RGCs at 3 days post-injury (dpi. We then demonstrated the activation of signal transducer and activator of transcription 3 (STAT3, a downstream target of LIF, at 3-5 dpi. To determine the function of LIF, we performed a LIF knockdown experiment using LIF-specific antisense morpholino oligonucleotides (LIF MOs. LIF MOs, which were introduced into zebrafish RGCs via a severed optic nerve, reduced the expression of LIF and abrogated the activation of STAT3 in RGCs after injury. These results suggest that upregulated LIF drives Janus kinase (Jak/STAT3 signaling in zebrafish RGCs after nerve injury. In addition, the LIF knockdown impaired axon sprouting in retinal explant culture in vitro; reduced the expression of a regeneration-associated molecule, growth-associated protein 43 (GAP-43; and delayed functional recovery after optic nerve injury in vivo. In this study, we comprehensively demonstrate the beneficial role of LIF in optic nerve regeneration and functional recovery in adult zebrafish.

  5. Characteristics of Optic Nerve Damage Induced by Chronic Intraocular Hypertension in Rat

    Institute of Scientific and Technical Information of China (English)

    Jiantao Wang; Jian Ge; A.A. Sadun; T.T. Lam

    2004-01-01

    Purpose:To set up the Sharma's chronic intraocular hypertension model and investigate the intraocular pressure (lOP) as well as the optic nerve damage of this model in rat.Methods:The operations of the chronic intraocular hypertension model were performed as described by Sharma in 60 male Lewis albino rats. IOP was measured using the TonoPen XL immediately after surgery and then at 5 day, 2 week or 4 week intervals. Cresyl violet staining of whole-mounted retinas was used to label retinal ganglion cells (RGCs),then RGCs were counted. Paraphenylenediamine (PPD) staining was performed in the semi-thin cross sections of optic nerve of rat, in order to know whether the axons of optic nerve were degenerated or not. Results:There were 47 rats with higher IOP after the episcleral veins cauterized in 60rats. The ratio of elevated IOP was 78.3%. The IOPs were stable in 4 weeks. After cresyl violet staining, the RGCs loss was 11.0% and 11.3% was found in the central and peripheral retina respectively after 2 weeks of increased IOP. After 4 weeks of increased lOP, the loss of RGCs was 17% for the central retina and 24.6% for the peripheral retina. In the retinas without higher IOP, there was no loss of RGCs. PPD staining showed that optic nerve of rat with about 5.3% damage of axons located at the superior temporal region. Region of affected optic nerve 1 mm posterior to the globe by light microscope showed evidence of damaged axons with axonal swelling and myelin debris.Conclusion:Sharma's chronic intraocular hypertension model is a reproducible and effective glaucoma model, which mimics human glaucoma with chronically elevation IOP and induced RGCs loss and damage of optic nerve. Eye Science 2004;20:25-29.

  6. Characterization of the ionization degree evolution of the PF-400J plasma sheath by means of time resolved optical spectroscopy

    Science.gov (United States)

    Avaria, G.; Cuadrado, O.; Moreno, J.; Pavez, C.; Soto, L.

    2016-05-01

    Spectral measurements in the visible range of the plasma sheath ionization degree evolution on the plasma focus device PF-400J are presented. The measurements were done with temporal and spatial resolution in a plasma focus device of low stored energy: PF-400J (176-539 J, 880 nF, 20-35 kV, quarter period ∼ 300ns) [1]. An ICCD was attached to a 0.5 m focal length visible spectrometer, which enabled the acquisition of time resolved spectrum with 20 ns integration time throughout the whole current pulse evolution. The spatial resolution was attained using a set of lenses which allowed the focusing of a small volume of the plasma sheath in different positions of the inter-electrode space. Discharges were carried out in mixtures of Hydrogen with gases in different proportions: 5% Neon, 5% Krypton and 2% Nitrogen. Discharges using Neon as an impurity showed no ionization of the gas, just a very low intensity emission of Ne I at times much larger than the maximum current. Nitrogen, on the other hand, showed a high ionization reaching N V (N 4+) at the end of the axial phase, with a distinctive evolution of the ionization degree as the plasma sheath moved towards the end of the electrodes. A mixed result was found when using Krypton, since the ionization degree only reached levels around Kr II/III, even though it has an ionization potential lower than Neon.

  7. Peripapillary subretinal neovascularization and serous macular detachment. Association with congenital optic nerve pits.

    Science.gov (United States)

    Borodic, G E; Gragoudas, E S; Edward, W O; Brockhurst, R J

    1984-02-01

    Congenital anomalous disc changes were associated with acquired macular detachment and peripapillary choroidal neovascularization in two cases. The anomalous disc changes resembled optic nerve pits. In one case, the peripapillary choroidal neovascularization was treated with argon laser photocoagulation, with subsequent reattachment of the macula and considerable improvement in the visual acuity. Although the pathogenesis of macular detachment occurring with optic nerve pits is usually not disclosed by fluorescein angiography, leakage from choroidal neovascularization can occur with this congenital defect and may contribute to the formation of a neurosensory macular detachment. If found, choroidal neovascularization may represent a remedial cause for visual loss in a condition with an otherwise poor prognosis.

  8. Distribution of pressure-induced fast axonal transport abnormalities in primate optic nerve. An autoradiographic study.

    Science.gov (United States)

    Radius, R L

    1981-07-01

    The distribution of transport abnormalities in primate optic nerve from eyes subjected to five hours of pressure elevation (perfusion pressure of 35 mm Hg) was studied. Tissue autoradiography and electron microscopy were used to localize regions of the lamina cribrosa with increased transport interruption. A preferential involvement by this transport abnormality involved the superior, temporal, and inferior portions, to the exclusion of the nasal portion, of the optic nerve head. This observation supports the hypothesis that transport interruption seen in this model may be pertinent to the study of clinical glaucomatous neuropathy.

  9. Role of inflammation in the relationship between OM and regeneration of optic nerves

    Directory of Open Access Journals (Sweden)

    Lin-Kun Ma

    2017-07-01

    Full Text Available Oncomodulin(OMis known by us progressively as a Calcium binding protein. Recently, OM has been found that it is secreted by inflammatory cells(neutrophilic granulocyte, and a signal which can promote cell growth between innate immunity and neurons, and a key to regenerate the damaged optical nerves by activating inflammation. The function of promoting the regeneration progress of axons has become a hot issue in recent years. This article summarized the mechanism of OM and the relationship between inflammation-induced OM and optic nerve regeneration research and progress were reviewed.

  10. [The results of direct electrostimulation of the involved optic nerves in neurosurgical patients].

    Science.gov (United States)

    Khil'ko, V A; Gaĭdar, B V; Kondrat'eva, M I; Nikol'skaia, I M; Usanov, E I

    1989-01-01

    The article generalizes experience in the restoration of vision by direct stimulation of damaged optic nerves after operations for pathological conditions of the chiasmal-sellar region (tumors of the chiasmal-sellar region, optochiasmic arachnoiditis, damage of the optic nerve in the bone canal) in 111 patients. The therapeutic effect was favourable in two thirds of the patients. Indications are determined for the use of the method in various types, duration, and severity of the disease. The use of the method with due regard for the determined indications increases the reliability, degree, and rate of restoration of vision in patients who underwent operation.

  11. Optic nerve head analysis of superior segmental optic hypoplasia using Heidelberg retina tomography

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    Atsushi Miki

    2010-10-01

    , especially in the nasal superior sector. Approximately half of the eyes with SSOH were classified as abnormal using indices developed for detecting glaucoma, but the sectorial analysis revealed that the affected sectors were different from those of glaucoma. Optic nerve head measurements using the HRT may be useful in evaluating the optic disc characteristics in eyes with SSOH.Keywords: superior segmental optic hypoplasia, Heidelberg retina tomography

  12. Drusen of the optic nerve head in CT imaging; Druzy tarczy nerwu wzrokowego w obrazie tomografii komputerowej

    Energy Technology Data Exchange (ETDEWEB)

    Kabula, S.; Stefaniak, E.; Burzynska-Makuch, M.; Boron, Z. [Akademia Medyczna, Bydgoszcz (Poland)

    1994-12-31

    The optic nerve head drusen are non-cell formations, which are almost always calcified. They have a characteristic feature in CT examination, what can be helpful in differentiation from calcifications of the other origin, located in the posterior globe. Authors present cases of the optic nerve head drusen with typical feature in CT examination. (author). 4 refs, 1 fig.

  13. A study of degeneration, scar formation and regeneration after section of the optic nerve in the frog, Rana pipiens.

    OpenAIRE

    Scott, T. M.; Foote, J

    1981-01-01

    Degeneration, scar formation and regeneration have been studied after section of the optic nerve in the frog. In the normal optic nerve two types of macroglial cell were identified: astrocytes and oligodendroglia. Degeneration after injury proceeded rapidly in comparison with mammals but did not lead to the production of a dense scar. Before much scarring had developed, regenerating axons penetrated the lesioned area.

  14. Treating benign optic nerve tumors with a 3-D conformal plan

    Energy Technology Data Exchange (ETDEWEB)

    Millunchick, Cheryl Hope, E-mail: mordechaimillunchick@gmail.com [Rush University Medical Center, Radiation Oncology, Chicago, IL (United States)

    2013-07-01

    A 68 year old male patient presented for radiation therapy for treatment of a benign tumor, a glioma of his left optic nerve. The radiation oncologist intended to prescribe 52.2 Gy to the planning target volume, while maintaining a maximum of 54 Gy to the optic nerves and the optic chiasm and a maximum of 40–45 Gy to the globes in order to minimize the possibility of damaging the optic system, which is especially important as this is a benign tumor. The dosimetrist devised a conformal non-coplanar three-dimensional plan with a slightly weighted forward planning component. This plan was created in approximately 15 minutes after the critical organs and the targets were delineated and resulted in an extremely conformal and homogenous plan, treating the target while sparing the nearby critical structures. This approach can also be extended to other tumors in the brain - benign or malignant.

  15. Atypical clinical presentation and long-term survival in a patient with optic nerve medulloepithelioma: a case report

    Directory of Open Access Journals (Sweden)

    Pastora-Salvador Natalia

    2012-05-01

    Full Text Available Abstract Introduction Medulloepithelioma is a rare congenital tumor of the primitive medullary neuroepithelium. A significant proportion of patients with medulloepithelioma arising from the optic nerve die from intracranial spread or cerebral metastasis. Because it has no known distinct clinical features and because of its low frequency, this tumor presents within the first two to six years of life and is usually misdiagnosed clinically as a different type of optic nerve tumor. Here, we describe a new and atypical case of medulloepithelioma of the optic nerve in a 12-year-old boy. To the best of our knowledge, he is the oldest reported patient to present with this disease and, now as an adult, has the longest documented period of disease-free survival. Case presentation A 12-year-old Caucasian boy with headache and unilateral amaurosis was referred for a presumed optic nerve glioma to our hospital. A computed tomography scan showed optic nerve enlargement, and fundoscopy showed a whitish mass at the optic disc. Our patient had been followed at his local hospital for four years for an 'optic disc cyst' with no change or progression. He experienced mild progressive visual impairment during that period. He was admitted for resection, and a histopathological analysis revealed a medulloepithelioma of the optic nerve. Supplemental orbital radiotherapy was performed. He remained disease-free for 25 years. Conclusions Medulloepithelioma of the optic nerve can clinically mimic more common pediatric tumors, such as optic glioma, meningioma, or retinoblastoma. Thus, medulloepithelioma should be included in the differential diagnoses of pediatric optic nerve lesions. Fundoscopy in these patients may provide relevant information for diagnosis. Anterior optic nerve medulloepitheliomas may behave differently from and have a better prognosis than medulloepitheliomas that have a more posterior location. Our case report illustrates that long-term survival can be

  16. A3 Adenosine receptors mediate oligodendrocyte death and ischemic damage to optic nerve.

    Science.gov (United States)

    González-Fernández, Estíbaliz; Sánchez-Gómez, María Victoria; Pérez-Samartín, Alberto; Arellano, Rogelio O; Matute, Carlos

    2014-02-01

    Adenosine receptor activation is involved in myelination and in apoptotic pathways linked to neurodegenerative diseases. In this study, we investigated the effects of adenosine receptor activation in the viability of oligodendrocytes of the rat optic nerve. Selective activation of A3 receptors in pure cultures of oligodendrocytes caused concentration-dependent apoptotic and necrotic death which was preceded by oxidative stress and mitochondrial membrane depolarization. Oligodendrocyte apoptosis induced by A3 receptor activation was caspase-dependent and caspase-independent. In addition to dissociated cultures, incubation of optic nerves ex vivo with adenosine and the A3 receptor agonist 2-CI-IB-MECA(1-[2-Chloro-6-[[(3-iodophenyl)methyl]amino]-9H-purin-9-yl]-1-deoxy-N-methyl-b-D-ribofuranuronamide)-induced caspase-3 activation, oligodendrocyte damage, and myelin loss, effects which were prevented by the presence of caffeine and the A3 receptor antagonist MRS 1220 (N-[9-Chloro-2-(2-furanyl)[1,2,4]-triazolo [1,5-c]quinazolin-5-yl]benzene acetamide). Finally, ischemia-induced injury and functional loss to the optic nerve was attenuated by blocking A3 receptors. Together, these results indicate that adenosine may trigger oligodendrocyte death via activation of A3 receptors and suggest that this mechanism contributes to optic nerve and white matter ischemic damage.

  17. Clinical study on intravitreal injection of cannabinoid HU-211 for optic nerve damage in glaucoma rats

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    Hui-Feng Liu

    2014-09-01

    Full Text Available AIM: To evaluate the protective effects of intravitreal injection of cannabinoid HU-211 for optic nerve in glaucoma rats, providing the experimental evidence in the treatment of glaucoma optic nerve injury.METHODS: Glaucoma rats(18 eyeswere modeled by electric coagulation sclera surface vein and randomized into 3 groups, group A received intravitreal injection of 1mg/0.1mL cannabinoid HU-211 every other day respectively; group B was given intravitreal injection of 0.1mL water every other day, group C was high intraocular pressure(IOPgroup, 6 eyes were randomly selected for blank control group(group D. IOP was observed every day. The rats were sacrificed after treatment 4wk, froze retina section, HE stain. The density fluctuation of retinal ganglion cell(RGCneurons assessment the optic nerve of rat model with chronic high IOP glaucoma were measured.RESULTS: The apoptosis and damage degree of RGC in group B was obviously higher than that in group A, with statistically significant difference(PP >0.05.CONCLUSION: Intravitreal injection of cannabinoid HU-211 shows obvious protective effect on optic nerve in glaucom rat models.

  18. How the Build Up of Aqueous Humor Can Damage the Optic Nerve

    Science.gov (United States)

    ... Twitter Google Plus Email Print this page How the Build Up of Aqueous Humor Can Damage the Optic Nerve Most, but not all, forms of ... name. Phone (office) Sign Up Join us in the fight to end brain and eye disease 3 ...

  19. A Unique Way of Learning: Teaching Young Children with Optic Nerve Hypoplasia

    Science.gov (United States)

    Mendiola, Rosalinda; Bahar, Cheryl; Brody, Jill; Slott, Gayle L.

    2005-01-01

    This booklet was inspired by the need of educators and therapists of preschool students who are blind and visually impaired to share their observations of children with Optic Nerve Hypoplasia (ONH) and the therapies found to be helpful when working with these children. The work done at the Blind Childrens Center is very rewarding, and these…

  20. Pulsatile enophthalmos, severe esotropia, kinked optic nerve and visual loss in neurofibromatosis type-1

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    Virender Sachdeva

    2015-01-01

    Full Text Available Neurofibromatosis Type I if associated with aplasia of greater wing of sphenoid may be associated with a pulsatile exophthalmos. However, very rarely it may be associated with a pulsatile enophthalmos. This clinical image describes a rare presentation with pulsatile enophthalmos, esotropia and kinking of the optic nerve due to neurofibomatosis type I.

  1. Automated Axon Counting in Rodent Optic Nerve Sections with AxonJ

    Science.gov (United States)

    Zarei, Kasra; Scheetz, Todd E.; Christopher, Mark; Miller, Kathy; Hedberg-Buenz, Adam; Tandon, Anamika; Anderson, Michael G.; Fingert, John H.; Abràmoff, Michael David

    2016-05-01

    We have developed a publicly available tool, AxonJ, which quantifies the axons in optic nerve sections of rodents stained with paraphenylenediamine (PPD). In this study, we compare AxonJ’s performance to human experts on 100x and 40x images of optic nerve sections obtained from multiple strains of mice, including mice with defects relevant to glaucoma. AxonJ produced reliable axon counts with high sensitivity of 0.959 and high precision of 0.907, high repeatability of 0.95 when compared to a gold-standard of manual assessments and high correlation of 0.882 to the glaucoma damage staging of a previously published dataset. AxonJ allows analyses that are quantitative, consistent, fully-automated, parameter-free, and rapid on whole optic nerve sections at 40x. As a freely available ImageJ plugin that requires no highly specialized equipment to utilize, AxonJ represents a powerful new community resource augmenting studies of the optic nerve using mice.

  2. MRI of optic nerve and postchiasmal visual pathways and visual evoked potentials in secondary progressive multiple sclerosis

    Energy Technology Data Exchange (ETDEWEB)

    Davies, M.B.; Hawkins, C.P. [School of Postgraduate Medicine, Keele Univ. (United Kingdom)]|[Department of Neurology and Neurophysiology, Royal Infirmary, Stoke-on-Trent (United Kingdom); Williams, R. [MRI Unit Cornwall House, Stoke-on-Trent (United Kingdom); Haq, N. [Department of Neurology, North Staffordshire Hospital, Stoke-on-Trent (United Kingdom); Pelosi, L. [Department of Neurology and Neurophysiology, Royal Infirmary, Stoke-on-Trent (United Kingdom)

    1998-12-01

    We studied the relationship between abnormalities shown by MRI and functional disturbances in the visual pathway as assessed by the visual evoked potential (VEP) in 25 patients with established multiple sclerosis (MS); only 4 of whom had a history of acute optic neuritis. Optic nerve MRI was abnormal in 19 (76 %) and is thus useful in detecting subclinical disease. Optic nerve total lesion length and area on the STIR sequence was found to correlate significantly with prolongation of the VEP latency. This may reflect a predominantly demyelinating rather than inflammatory origin for the signal change in the optic nerve. (orig.) With 5 figs., 1 tab., 25 refs.

  3. Radiological evolution and delayed resolution of an optic nerve tuberculoma: Challenges in diagnosis and treatment

    Directory of Open Access Journals (Sweden)

    Ajith Sivadasan

    2013-01-01

    Full Text Available Optic nerve tuberculomas are rarely reported and their natural history, prognosis, and duration of required treatment remain unclear. A 40-year-old immunocompetent male presented with complete loss of vision in his right eye, which had evolved over 6 weeks. He had optic atrophy on examination. Initial imaging showed right optic nerve swelling and thickening suggesting an infiltrative inflammatory optic neuropathy (infectious or noninfectious. Serial imaging revealed appearance of ring enhancement with a necrotic centre. Biopsy and culture of the coexistent parietal lobe lesion revealed Mycobacterium tuberculosis. Persistent optic nerve granuloma with evidence of radiological improvement was noted at 18 months follow-up with antituberculous therapy (ATT. Visual recovery could not be achieved. The salient features in this case include the clinical presentation initially mimicking an infiltrative or compressive optic neuropathy, rapid radiological evolution into a tuberculoma, subtle paradoxical radiological worsening after initiation of ATT and persistence of granuloma on follow up scan. The challenges involved in early diagnosis and during the treatment course will be discussed.

  4. Retinal glutamate transporter changes in experimental glaucoma and after optic nerve transection in the rat.

    Science.gov (United States)

    Martin, Keith R G; Levkovitch-Verbin, Hana; Valenta, Danielle; Baumrind, Lisa; Pease, Mary Ellen; Quigley, Harry A

    2002-07-01

    High levels of glutamate can be toxic to retinal ganglion cells. Effective buffering of extracellular glutamate by retinal glutamate transporters is therefore important. This study was conducted to investigate whether glutamate transporter changes occur with two models of optic nerve injury in the rat. Glaucoma was induced in one eye of 35 adult Wistar rats by translimbal diode laser treatment to the trabecular meshwork. Twenty-five more rats underwent unilateral optic nerve transection. Two glutamate transporters, GLAST (EAAT-1) and GLT-1 (EAAT-2), were studied by immunohistochemistry and quantitative Western blot analysis. Treated and control eyes were compared 3 days and 1, 4, and 6 weeks after injury. Optic nerve damage was assessed semiquantitatively in epoxy-embedded optic nerve cross sections. Trabecular laser treatment resulted in moderate intraocular pressure (IOP) elevation in all animals. After 1 to 6 weeks of experimental glaucoma, all treated eyes had significant optic nerve damage. Glutamate transporter changes were not detected by immunohistochemistry. Western blot analysis demonstrated significantly reduced GLT-1 in glaucomatous eyes compared with control eyes at 3 days (29.3% +/- 6.7%, P = 0.01), 1 week (55.5% +/- 13.6%, P = 0.02), 4 weeks (27.2% +/- 10.1%, P = 0.05), and 6 weeks (38.1% +/- 7.9%, P = 0.01; mean reduction +/- SEM, paired t-tests, n = 5 animals per group, four duplicate Western blot analyses per eye). The magnitude of the reduction in GLT-1 correlated significantly with mean IOP in the glaucomatous eye (r(2) = 0.31, P = 0.01, linear regression). GLAST was significantly reduced (33.8% +/- 8.1%, mean +/- SEM) after 4 weeks of elevated IOP (P = 0.01, paired t-test, n = 5 animals per group). In contrast to glaucoma, optic nerve transection resulted in an increase in GLT-1 compared with the control eye (P = 0.01, paired t-test, n = 15 animals). There was no significant change in GLAST after transection. GLT-1 and GLAST were significantly

  5. Jak/Stat Signaling Stimulates Zebrafish Optic Nerve Regeneration and Overcomes the Inhibitory Actions of Socs3 and Sfpq

    Science.gov (United States)

    Elsaeidi, Fairouz; Bemben, Michael A.; Zhao, Xiao-Feng

    2014-01-01

    The regenerative failure of mammalian optic axons is partly mediated by Socs3-dependent inhibition of Jak/Stat signaling (Smith et al., 2009, 2011). Whether Jak/Stat signaling is part of the normal regenerative response observed in animals that exhibit an intrinsic capacity for optic nerve regeneration, such as zebrafish, remains unknown. Nor is it known whether the repression of regenerative inhibitors, such as Socs3, contributes to the robust regenerative response of zebrafish to optic nerve damage. Here we report that Jak/Stat signaling stimulates optic nerve regeneration in zebrafish. We found that IL-6 family cytokines, acting via Gp130-coupled receptors, stimulate Jak/Stat3 signaling in retinal ganglion cells after optic nerve injury. Among these cytokines, we found that CNTF, IL-11, and Clcf1/Crlf1a can stimulate optic axon regrowth. Surprisingly, optic nerve injury stimulated the expression of Socs3 and Sfpq (splicing factor, proline/glutamine rich) that attenuate optic nerve regeneration. These proteins were induced in a Jak/Stat-dependent manner, stimulated each other's expression and suppressed the expression of regeneration-associated genes. In vivo, the injury-dependent induction of Socs3 and Sfpq inhibits optic nerve regeneration but does not block it. We identified a robust induction of multiple cytokine genes in zebrafish retinal ganglion cells that may contribute to their ability to overcome these inhibitory factors. These studies not only identified mechanisms underlying optic nerve regeneration in fish but also suggest new molecular targets for enhancing optic nerve regeneration in mammals. PMID:24523552

  6. Decreased retinal nerve fibre layer thickness detected by optical coherence tomography in patients with ethambutol‐induced optic neuropathy

    Science.gov (United States)

    Chai, Samantha J; Foroozan, Rod

    2007-01-01

    Background It is difficult to assess the degree of optic nerve damage in patients with ethambutol‐induced optic neuropathy, especially just after the onset of visual loss, when the optic disc typically looks normal. Aim To evaluate changes in retinal nerve fibre layer thickness (RNFLT) using optical coherence tomography (OCT) in patients with optic neuropathy within 3 months of cessation of ethambutol treatment. Design A retrospective observational case series from a single neuro‐ophthalmology practice. Methods 8 patients with a history of ethambutol‐induced optic neuropathy were examined within 3 months after stopping ethambutol treatment. All patients underwent a neuro‐ophthalmologic examination, including visual acuity, colour vision, visual fields and funduscopy. OCT was performed on both eyes of each patient using the retinal nerve fibre layer analysis protocol. Results The interval between cessation of ethambutol treatment and the initial visit ranged from 1 week to 3 months. All patients had visual deficits characteristic of ethambutol‐induced optic neuropathy at their initial visit, and the follow‐up examination was performed within 12 months. Compared with the initial RNFLT, there was a statistically significant decrease in the mean RNFLT of the temporal, superior and nasal quadrants (p = 0.009, 0.019 and 0.025, respectively), with the greatest decrease in the temporal quadrant (mean decrease 26.5 μm). Conclusions A decrease in RNFLT is observed in all quadrants in patients with ethambutol‐induced optic neuropathy who have recently discontinued the medication. This decrease is most pronounced in the temporal quadrant of the optic disc. PMID:17215265

  7. Real time imaging of peripheral nerve vasculature using optical coherence angiography

    Science.gov (United States)

    Vasudevan, Srikanth; Kumsa, Doe; Takmakov, Pavel; Welle, Cristin G.; Hammer, Daniel X.

    2016-03-01

    The peripheral nervous system (PNS) carries bidirectional information between the central nervous system and distal organs. PNS stimulation has been widely used in medical devices for therapeutic indications, such as bladder control and seizure cessation. Investigational uses of PNS stimulation include providing sensory feedback for improved control of prosthetic limbs. While nerve safety has been well documented for stimulation parameters used in marketed devices, novel PNS stimulation devices may require alternative stimulation paradigms to achieve maximum therapeutic benefit. Improved testing paradigms to assess the safety of stimulation will expedite the development process for novel PNS stimulation devices. The objective of this research is to assess peripheral nerve vascular changes in real-time with optical coherence angiography (OCA). A 1300-nm OCA system was used to image vasculature changes in the rat sciatic nerve in the region around a surface contacting single electrode. Nerves and vasculature were imaged without stimulation for 180 minutes to quantify resting blood vessel diameter. Walking track analysis was used to assess motor function before and 6 days following experiments. There was no significant change in vessel diameter between baseline and other time points in all animals. Motor function tests indicated the experiments did not impair functionality. We also evaluated the capabilities to image the nerve during electrical stimulation in a pilot study. Combining OCA with established nerve assessment methods can be used to study the effects of electrical stimulation safety on neural and vascular tissue in the periphery.

  8. A study of the magnetic field distribution in an Ag-sheathed Bi2223 tape using scanning Hall sensor and magneto-optical techniques

    Science.gov (United States)

    Kawano, K.; Abell, J. S.; Ohtake, A.; Oota, A.

    2000-09-01

    Using both magneto-optical (MO) and scanning Hall sensor techniques, magnetic field distributions have been observed in a superconducting Ag-sheathed Bi2223 monofilamentary tape in the presence of an external magnetic field. Application of the inversion scheme to the MO contrast has allowed the two-dimensional current distribution to be determined. The Hall sensor measurements indicate that the current distribution in the core depends on the applied external field, and the current flows mainly at the edge of the core in a high external field. The magnetic line profiles across the width of the tape have been analysed by a numerical calculation by modelling the current loops based on the two-dimensional current distribution from the MO image. The analysis shows that an increase in the external field limits and narrows the current flow region from the whole of the core to the edge.

  9. A study of the magnetic field distribution in an Ag-sheathed Bi2223 tape using scanning Hall sensor and magneto-optical techniques

    Energy Technology Data Exchange (ETDEWEB)

    Kawano, K. [School of Metallurgy and Materials, University of Birmingham, Birmingham B15 2TT (United Kingdom). E-mail: k.kawano at bham.ac.uk; Abell, J.S. [School of Metallurgy and Materials, University of Birmingham, Birmingham B15 2TT (United Kingdom); Ohtake, A.; Oota, A. [Department of Electrical and Electronic Engineering, Toyohashi University of Technology, Tempaku-cho, Toyohashi, Aichi 441-8580 (Japan)

    2000-09-01

    Using both magneto-optical (MO) and scanning Hall sensor techniques, magnetic field distributions have been observed in a superconducting Ag-sheathed Bi2223 monofilamentary tape in the presence of an external magnetic field. Application of the inversion scheme to the MO contrast has allowed the two-dimensional current distribution to be determined. The Hall sensor measurements indicate that the current distribution in the core depends on the applied external field, and the current flows mainly at the edge of the core in a high external field. The magnetic line profiles across the width of the tape have been analysed by a numerical calculation by modelling the current loops based on the two-dimensional current distribution from the MO image. The analysis shows that an increase in the external field limits and narrows the current flow region from the whole of the core to the edge. (author)

  10. The role of OCT in the differential diagnosis between buried optic nerve drusen and papilloedema.

    Science.gov (United States)

    Rebolleda, G; Muñoz-Negrete, F J

    2016-09-01

    Buried optic nerve head drusen are one of the most common causes of pseudo-papilloedema. In this review, we have chronologically addressed several useful traits in the differential diagnosis of a true papilloedema, using the different features of optical coherence tomography (OCT). The specificity of these features has improved at the same time as the improvement in penetration capability and resolution of newer OCT devices. Spectral domain OCT, and more specifically the enhanced depth imaging (EDI) technology, represents a turning point in directly visualise drusen, to quantify their size and to recognise their impact on neighbouring structures inside the optic nerve head. Copyright © 2016 Sociedad Española de Oftalmología. Published by Elsevier España, S.L.U. All rights reserved.

  11. Optic nerve fast axonal transport abnormalities in primates. Occurrence after short posterior ciliary artery occlusion.

    Science.gov (United States)

    Radius, R L

    1980-11-01

    Fast axonal transport abnormalities in primate (Aotus trivirgatus) optic nerve were studied in ten eyes at various intervals after occlusion of the lateral short posterior ciliary circulation. Evidence of focal axonal ischemia, as indicated by swelling of mitochondria and dissolution of cytoplasmic detail, was noted as early as one hour after occlusion. Accumulation of mitochondria, microvesicles, and dense bodies, indicating focal interruption of axonal transport mechanisms, was noted in eyes examined at 2, 4, and 6 hours. This accumulation of organelles was limited to the region of the lamina cribrosa. Nerve head abnormalities were not seen in two eyes studied at two weeks.

  12. Effect of SIRT1 regulating cholesterol synthesis in repairing retinal ganglion cells after optic nerve injury in rats

    Directory of Open Access Journals (Sweden)

    Yan Zhang

    2014-10-01

    Full Text Available AIM: To investigate the repair mechanism associated with cholesterol synthesis regulated by silent information regulator 1(SIRT1in rat model of optic nerve damage. METHODS: Preparation of optic nerve damage in 70 rats was randomly divided into normal group(10 rats, resveratrol treatment group(experimental group 30 ratsand PBS buffer control group(30 rats. The experimental group and control group was further divided into 3 subgroups(each group 10 rats, respectively. After 7, 14, 21d injected resveratrol or PBS, optic nerve injury were observed, then the rats were sacrificed. Retina was segregated; the surviving retinal ganglion cell(RGCswas counted. Dissection of optic nerve, cholesterol content of them were tested; RT-PCR was used to detect mRNA expression of SIRT1, SREBP2 and HMGCR; Western blot assay was used to test the protein expression levels of SIRT1, cholesterol regulatory element binding protein 2(SREBP2and HMGCR. RESULTS: The numbers of RGCs and cholesterol levels of rat model with optic nerve injury decreased significantly(PPPPCONCLUSION: Up-regulating the expression of SIRT1, SREBP2 and down-regulating HMGCR by resveratrol could repair the injury of optic nerve through promoting the synthesis of cholesterol in neurons and retinal ganglion cells in the repair process. SIRT1 may be as a promising new target for treatment on optic nerve damage.

  13. “Well Digging” Subcraniotomy Strategy with Navigation for Optic Nerve Decompression in Frontoorbital Fibrous Dysplasia: Preliminary Experience

    Science.gov (United States)

    Lu, Yunhe; Yang, Junyi; Wu, Yingzhi; Pan, Sida; Lu, Jiawen

    2016-01-01

    Background: During the past decades, surgical intervention has been the primary treatment modality for frontoorbital fibrous dysplasia involving optic nerve. However, controversy has surrounded the role of optic nerve decompression in a number of ways. Herein, we describe 3 patients with frontoorbital fibrous dysplasia involving optic nerve, who underwent a “well digging” subcraniotomy strategy with navigation for intraorbital unit optic nerve decompression. Methods: From 2013 to 2015, 3 patients with frontoorbital fibrous dysplasia were investigated in a retrospective manner. They underwent unilateral intraorbital optic nerve decompression with the help of “well digging” strategy and navigation. The key procedures comprise preoperative software simulation, frontoorbital subcraniotomy (like digging a well), expanding cone-shaped surgical field, intraorbital unit optic nerve decompression with navigation, correcting frontal-orbital dystopias, and deformities. Results: Both at the immediate postoperative period and during the 3–12 months follow-up, 2 cases showed improvement of visual acuity in the affected eye and 1 case showed no deterioration. Other ocular examinations including eye movement were stable. Subsequent reconstruction yielded a satisfactory cosmetic result. No postoperative complications happened. Conclusions: In our philosophy, surgical management should be tailored to each patient, which is based on the most possible potential etiology. We consider that the intraorbital optic nerve decompression may be more feasible and safer with the help of “well digging” strategy and navigation, especially for those with exophthalmos, orbital volume decreasing, and nonacute visual loss. PMID:27975011

  14. Visually evoked hemodynamical response and assessment of neurovascular coupling in the optic nerve and retina.

    Science.gov (United States)

    Riva, Charles E; Logean, Eric; Falsini, Benedetto

    2005-03-01

    The retina and optic nerve are both optically accessible parts of the central nervous system. They represent, therefore, highly valuable tissues for studies of the intrinsic physiological mechanism postulated more than 100 years ago by Roy and Sherrington, by which neural activity is coupled to blood flow and metabolism. This article describes a series of animal and human studies that explored the changes in hemodynamics and oxygenation in the retina and optic nerve in response to increased neural activity, as well as the mechanisms underlying these changes. It starts with a brief review of techniques used to assess changes in neural activity, hemodynamics, metabolism and tissue concentration of various potential mediators and modulators of the coupling. We then review: (a) the characteristics of the flicker-induced hemodynamical response in different regions of the eye, starting with the optic nerve, the region predominantly studied; (b) the effect of varying the stimulus parameters, such as modulation depth, frequency, luminance, color ratio, area of stimulation, site of measurement and others, on this response; (c) data on activity-induced intrinsic reflectance and functional magnetic resonance imaging signals from the optic nerve and retina. The data undeniably demonstrate that visual stimulation is a powerful modulator of retinal and optic nerve blood flow. Exploring the relationship between vasoactivity and metabolic changes on one side and corresponding neural activity changes on the other confirms the existence of a neurovascular/neurometabolic coupling in the neural tissue of the eye fundus and reveals that the mechanism underlying this coupling is complex and multi-factorial. The importance of fully exploiting the potential of the activity-induced vascular changes in the assessment of the pathophysiology of ocular diseases motivated studies aimed at identifying potential mediators and modulators of the functional hyperemia, as well as conditions

  15. [Difficulties of diagnosis in a case of optic nerve drusen].

    Science.gov (United States)

    Munteanu, M; Munteanu, G

    2000-01-01

    This paper presents a particullary case of optic nervue drusen. Ophthalmoscopic features (superficial drusen in one eye and deep drusen in another), clinical association with anisometropia and ambliopia, wrong interpretation of PEV and tomodensitometry mod to diagnostic confusion, supplementary investigations, prolonged and expensive treatment.

  16. Melanocytoma of the optic nerve head - a diagnostic dilemma

    Directory of Open Access Journals (Sweden)

    Zalilawati Mohmad

    2011-08-01

    Full Text Available The clinical features, autofluorescence, Bscan ultrasonography, optical coherence tomography and fluorescein angiography of the lesion were described. Multiple investigation modalities are needed to confirm the benign nature of the lesion. Careful evaluation and follow-up is crucial to avoid misdiagnosis and erroneous management.

  17. A twenty-eight channel coil array for improved optic nerve imaging

    Science.gov (United States)

    Merrill, Robb Phillip

    The purpose of this work was to design and construct a radio-frequency coil optimized for imaging the Optic Nerve (ON) on a Siemens 3T magnetic resonance imaging (MRI) scanner. The specific goals were to optimize signal sensitivity from the orbit to the optic chiasm and improve SNR over designs currently in use. The constructed coil features two fiberglass formers that can slide over each other to accommodate any arbitrary head size, while maintaining close coupling near the eyes and around the head in general. This design eliminates the air void regions that occur between the coil elements and the forehead when smaller heads are imaged in one-piece, nonadjustable coil formers. The 28 coil elements were placed using a soccer-ball pattern layout to maximize head coverage. rSNR profiles from phantom imaging studies show that the ON coil provides approximately 55% greater rSNR at the region of the optic chiasm and approximately 400% near the orbits compared to the 12-channel commercial coil. The improved rSNR in the optic nerve region allows performance of high resolution DTI, which provides a qualitative measurement for evaluating optic neuritis. Images from volunteer and patient studies with the ON coil reveal plaques that correspond well with the patient disease history of chronic bilateral optic neuritis. Correspondence of image findings with patient disease histories demonstrates that optic neuritis can be visualized and detected in patients using 3T MRI with advanced imaging coils, providing improved patient care.

  18. Application of tissue clearing and light sheet fluorescence microscopy to assess optic nerve regeneration in unsectioned tissues.

    Science.gov (United States)

    Luo, Xueting; Yungher, Benjamin; Park, Kevin K

    2014-01-01

    Optic nerve crush injury, as a model to study central nervous system (CNS) injury, is widely used to assess potential therapeutic strategies, aimed at promoting axon regeneration and neuronal survival. Traditional methods to evaluate optic nerve regeneration rely on histological sectioning. However, tissue sectioning results in inevitable loss of three-dimensional (3D) information, such as axonal trajectories and terminations. Here we describe a protocol for whole-tissue assessment of optic nerve regeneration in adult mice without the need for histological sectioning.

  19. Magnetic resonance diffusion tensor imaging (MRDTI) of the optic nerve and optic radiations at 3T in children with neurofibromatosis type I (NF-1)

    Energy Technology Data Exchange (ETDEWEB)

    Filippi, Christopher G.; Nickerson, Joshua P. [University of Vermont School of Medicine-FAHC, Department of Radiology, Burlington, VT (United States); Bos, Aaron [University of Vermont School of Medicine, Burlington, VT (United States); Salmela, Michael B. [University of Minnesota School of Medicine, Department of Radiology, Minneapolis, MN (United States); Koski, Chris J. [James Madison University, Department of Political Sciences, Harrisonburg, VA (United States); Cauley, Keith A. [University of Massachusetts Memorial Medical Center, Department of Radiology, Worcester, MA (United States)

    2012-02-15

    Optic pathway glioma (OPG) is a characteristic hallmark of neurofibromatosis type I (NF-I). To evaluate the feasibility of magnetic resonance diffusion tensor imaging (MRDTI) at 3T to detect abnormalities of the optic nerves and optic radiations in children with NF-I. 3-T MRDTI was prospectively performed in 9 children with NF-I (7 boys, 2 girls, average age 7.8 years, range 3-17 years) and 44 controls (25 boys, 19 girls, average age 8.1 years, range 3-17 years). Fractional anisotropy (FA) and mean diffusivity were determined by region-of-interest analysis for the optic nerves and radiations. Statistical analysis compared controls to NF-I patients. Two NF-I patients had bilateral optic nerve gliomas, three had chiasmatic gliomas and four had unidentified neurofibromatosis objects (UNOs) along the optic nerve pathways. All NF-I patients had statistically significant decreases in FA and elevations in mean diffusivity in the optic nerves and radiations compared to age-matched controls. MRDTI can evaluate the optic pathways in children with NF-I. Statistically significant abnormalities were detected in the diffusion tensor metrics of the optic nerves and radiations in children with NF-I compared to age-matched controls. (orig.)

  20. [Case of ruptured carotid-ophthalmic aneurysm splitting the optic nerve].

    Science.gov (United States)

    Sato, Taku; Sasaki, Tatsuya; Sakuma, Jun; Suzuki, Kyouichi; Matsumoto, Masato; Sato, Masanori; Itakura, Takeshi; Kodama, Namio

    2009-04-01

    A rare case of ruptured carotid-ophthalmic aneurysm splitting the optic nerve was reported. A 52-year-old man presented with a sudden severe headache and bilateral visual deterioration. His right visual acuity was hand motion and the left was 2.0. His left visual field revealed a partial defect of the temporal visual field. Three-dimensional CT angiography revealed an ophthalmic aneurysm of 9 mm projecting superior-medially, The operation was performed to preserve the visual function as much as possible. The C2 portion aneurysm splitting the right optic nerve was visible. Before aneurysm clipping, the right anterior clinoid process and optic canal were drilled out to reduce the tension of the optic nerve. Intraoperative monitoring of visual evoked potential (VEP) under propofol anesthesia was performed to prevent further visual disturbance. Electroretinogram (ERG) was introduced to ascertain the arrival of the light stimulus at the retina even when the VEP could not be recorded. The right ERG was recorded, but the right VEP was flat. Both ERG and VEP were reproducible on the left side. Six months after the operation, the right visual acuity had improved to 0.08 and the visual field revealed nasal hemianopsia. The left visual acuity was unchanged while the visual field had improved to upper temporal quadrant hemianopsia. Various attempts to preserve the visual function were discussed.

  1. Aggressive orbital optic nerve meningioma with benign microscopic features: a case report.

    Science.gov (United States)

    Amoli, F Asadi; Mehrabani, P Mansouri; Tari, A Sadeghi

    2007-12-01

    Primary optic nerve meningiomas occur at lower ages than meningiomas arising from the coverings of the brain and spinal cord. Here we report the case of a 20-year-old female with an aggressive orbital meningioma referred to the Ophthalmology Department of the Farabi Hospital in Tehran. The patient had a history of orbital meningioma from 10 years ago and several surgical resections due to tumor recurrence during these 10 years. On admission, the patient had a large orbital mass and severe proptosis. MRI images revealed a large left orbital mass with optic nerve involvement and extension to the left maxillary sinus, pterygoid fossa and the dura in the floor of the anterior fossa. Fine-needle aspiration cytology of the mass confirmed tumor recurrence. The patient first received radiotherapy due to the inoperable mass, and the tumor was resected 1.5 month later. Microscopic study showed meningotheliomatous meningioma with extensive involvement of the optic nerve and invasion of the optic disc, sclera and choroid. The interesting aspect of this case was the aggressive behavior of the tumor with intraocular invasion, despite its benign histopathological features, which led to wide exenteration of the eye together with resection of the upper and lower lids.

  2. Assessment of the optic nerve head parameters using Heidelberg retinal tomography III in preterm children.

    Directory of Open Access Journals (Sweden)

    Salem Alshaarawi

    Full Text Available BACKGROUND: Variations in optic nerve head morphology and abnormal retinal vascular pattern have been described in preterm children using digital image analysis of fundus photograph, optical coherence tomograph and serial funduscopy. We aimed to compare the optic nerve head parameters in preterm and term Malay children using Heidelberg Retinal Tomograph III. DESIGN: A cross sectional study. METHODOLOGY/PRINCIPAL FINDINGS: Thirty-two preterm Malay children who were born at up to 32 weeks postconception, and 32 term Malay children aged 8-16 years old were recruited into this cross sectional study, which was conducted in the Hospital Universiti Sains Malaysia, Malaysia from January to December 2011. Their optic nerves were scanned and analyzed using a Heidelberg Retinal Tomography (HRT III (Heidelberg Engineering, Germany. Preterm children showed an increased rim volume (SD (0.56 (0.26 vs 0.44 (0.18 mm(3, respectively, smaller cup shape (SD (0.18 (0.07 vs 0.25 (0.06 mm, respectively, increased height variation contour (SD (0.44 (0.14 vs 0.35 (0.08 mm, respectively, and increased cup depth (SD (0.24 (0.11 vs 0.17 (0.05 mm(3, respectively when compared to their normal peers (p0.05 in our study. CONCLUSIONS/SIGNIFICANCE: Preterm children exhibit different characteristics of optic nerve head parameters with HRT III analysis. Increased cup depth in preterm children suggests a need for close observation and monitoring. It may raise suspicion of pediatric glaucoma when proper documentation of intraocular pressure and clinical funduscopy are unsuccessful in uncooperative children.

  3. Communication through Plasma Sheaths

    CERN Document Server

    Korotkevich, A O; Zakharov, V E

    2007-01-01

    We wish to transmit messages to and from a hypersonic vehicle around which a plasma sheath has formed. For long distance transmission, the signal carrying these messages must be necessarily low frequency, typically 2 GHz, to which the plasma sheath is opaque. The idea is to use the plasma properties to make the plasma sheath appear transparent.

  4. Clinicopalhologic report on one case of palatal maligmint peripheral nerve sheath tumor%腭部恶性周围神经鞘膜瘤1例临床病理报告

    Institute of Scientific and Technical Information of China (English)

    王晶; 钟鸣; 王兆元; 魏振辉; 王岩

    2010-01-01

    @@ 恶性周围神经鞘膜瘤(malignant peripheral nerve sheath tumor,MPNST)是罕见的施万细胞或神经嵴多潜能细胞来源的梭形细胞肉瘤[1].根据世界卫生组织(WHO)2000年发表的神经系统肿瘤分类标准,MPNST是指起源于周围神经的,或显示出神经鞘膜分化的恶性肿瘤[2].它用于指那些曾被称为恶性神经鞘瘤、神经源性肉瘤和神经纤维肉瘤的肿瘤[1].MPNST为一种比较少见的恶性肿瘤,普通人群的发病率为0.001%,占所有软组织肉瘤的3%~10%[2].发生于口腔组织极为罕见.现将我院经病理明确诊断的1例MPNST进行分析,并结合文献复习报道如下.

  5. One Case of Retroperitoneal Malignant Peripheral Nerve Sheath Tumor and Literature Review%腹膜后恶性周围神经鞘膜瘤一例并文献复习

    Institute of Scientific and Technical Information of China (English)

    胡蓉; 龙丽娟; 元霄梅; 张淼; 朱广卿

    2013-01-01

    [Objective]To explore the diagnosis and treatment of malignant peripheral nerve sheath tumor (MPNST).[Methods] The auxiliary examinations and treatment of one case of MPNST were reported.The clinical results combined with literatures were analyzed retrospectively.[Results] After admission,CT,MRI and tumor biopsy were performed for the definite diagnosis.All patients received surgery,radiochemotherapy and high-intensity focused ultrasound(HIFU).Clinical efficacy was satisfactory.[Conclusion] HIFU combined with chemotherapy and radiotherapy for the treatment of MPNST is safe and effective.%[目的]探讨恶性周围神经鞘膜瘤(MPNST)诊断及治疗方法.[方法]报告本院1例MPNST的辅助检查及治疗情况,并结合文献资料进行回顾分析.[结果]入院后行CT、MRI、肿瘤活检等检查明确诊断,行手术治疗、放化疗及高能聚焦超声(HIFU)治疗,临床效果达部分缓解(PR).[结论]HIFU联合化疗及放疗治疗MPNST安全有效.

  6. Expression and Significance of MMP-9 and TIMP-1 in Malignant Peripheral Nerve Sheath Tumours%MMP-9及TIMP-1在恶性外周神经鞘膜瘤中的表达及意义

    Institute of Scientific and Technical Information of China (English)

    齐云飞; 牟英君; 裴丽霞

    2007-01-01

    目的 探讨恶性外周神经鞘膜瘤(malignant peripheral nerve sheath tumours,MPNST)中基质金属蛋白酶-9(matrix metalloproteinase-9,MMP-9)及组织金属蛋白酶抑制剂-1(tissue inhibitor of metalloproteinase-1,TIMP-1)的表达与病理分级、转移及预后的关系.方法 采用免疫组化S-P法检测MPNST中MMP-9及TIMP-1表达.结果 共检测了58例MPNST,其中MMP-9阳性表达率为89.7%(52/58),TIMP-1阳性表达率是60.3%(35/58).MMP-9蛋白酶的表达与病理学分级、转移率呈正相关,与术后生存率呈负相关;而TIMP-1则相反.结论 MMP-9、TIMP-1与MPNST病理学分级、转移及术后生存期有关,可作为判断恶性外周神经鞘膜瘤恶性程度及预后的可靠指标,为其治疗提供参考价值.

  7. Male left chest wall primary malignant peripheral nerve sheath tumor in 1 case%男性左胸壁原发恶性外周神经鞘瘤1例

    Institute of Scientific and Technical Information of China (English)

    王一博; 熊伟

    2015-01-01

    In this paper,the author retrospectively analyzes the clinical manifestation,auxiliary examination results,treatment procedure and prognosis of 1 case of malignant peripheral nerve sheath tumor(MPNST),and refers to the related literature at home and abroad,to increase the understanding of MPNST.MPNST is rare in clinic,the incidence rate is low,the clinical manifestations have no specific,the early diagnosis is difficulty.Increasing the understanding of the disease is contribute to the early detection, early diagnosis,early treatment of this disease.%目的:本文回顾性分析1例恶性外周神经鞘瘤(MPNST)的临床表现、辅助检查结果、治疗经过及预后,并参考了国内外相关文献资料,以增加对MPNST的认识。MPNST在临床上较罕见,发病率低,临床表现无特异性,早期诊断困难。因此,增加对该疾病的认识,有助于该疾病的早发现、早诊断、早治疗。

  8. Sensitivity of malignant peripheral nerve sheath tumor cells to TRAIL is augmented by loss of NF1 through modulation of MYC/MAD and is potentiated by curcumin through induction of ROS.

    Directory of Open Access Journals (Sweden)

    David E Reuss

    Full Text Available Malignant peripheral nerve sheath tumor (MPNST is a rare aggressive form of sarcoma often associated with the tumor syndrome neurofibromatosis type 1 (NF1. We investigated the effects of tumor necrosis factor-related apoptosis inducing ligand (TRAIL on NF1 associated MPNST and determinants of TRAIL sensitivity. MPNST cell lines with complete neurofibromin deficiency were sensitive to apoptotic cell death induced by TRAIL whereas MPNST cells with retained neurofibromin expression or normal human Schwann cells were resistant. Increased sensitivity to TRAIL was associated with overexpression of death receptors, especially DR5. Re-expression of the GAP related domain of neurofibromin (NF1-GRD suppressed DR5 expression and decreased sensitivity to TRAIL. We show that death receptor expression and TRAIL sensitivity critically depend on c-MYC and that c-MYC amounts are increased by MEK/ERK and PI3K/AKT signalling pathways which are suppressed by neurofibromin. Furthermore PI3K/AKT signalling strongly suppresses the MYC-antagonist MAD1 which significantly contributes to TRAIL sensitivity. Re-expression of the NF1-GRD decreased c-MYC and increased MAD1 amounts suggesting that neurofibromin influences TRAIL sensitivity at least in part by modulating the MYC/MAX/MAD network. The phytochemical curcumin further increased the sensitivity of neurofibromin deficient MPNST cells to TRAIL. This was presumably mediated by ROS, as it correlated with increased ROS production, was blocked by N-acetylcysteine and mimicked by exogenous ROS.

  9. Expression of MMP-2 and TIMP-2 in malignant peripheral nerve sheath tumors%恶性外周神经鞘膜瘤中MMP-2和TIMP-2的表达

    Institute of Scientific and Technical Information of China (English)

    鲁华东; 吕长虹

    2005-01-01

    目的探讨恶性外周神经鞘膜瘤(malignant peripheral nerve sheath tumors,MPNST)中基质金属蛋白酶-2(matrix metalloproteinase-2,MMP-2)及其相应的组织金属蛋白酶抑制剂-2(tissue inhibitor of metalloproteinase-2,TIMP-2)蛋白表达与病理分级、转移及预后的关系.方法采用免疫组化S-P法检测MPNST中MMP-2及TIMP-2表达,并行回顾性随访.结果 58例MPNST中MMP-2阳性表达51例,阳性表达率是87.9%,TIMP-2阳性表达36例,阳性表达率是62.1%. MMP-2蛋白表达与病理学分级、远处转移率呈正相关,与术后生存率呈负相关;而TIMP-2则相反.结论 MMP-2、TIMP-2与MPNST病理学分级、远处转移及术后生存期有关,可作为判断肿瘤恶性程度及预后的有用的参考指标.

  10. Comparison of Snellen acuity, VER acuity, and Arden grating scores in macular and optic nerve diseases.

    Science.gov (United States)

    Skalka, H W

    1980-01-01

    Patients with various macular and optic nerve abnormalities underwent Snellen acuity, transient VER acuity, and Arden grating testing. Snellen acuity was the coarsest of the 3 evaluations, generally falling after Arden scores and VER acuity had already undergone significant degradation. The Arden gratings appeared to be the most sensitive of the 3 tests, equalling VER performance in optic nerve diseases and surpassing it in macular diseases. Variations in results between the different tests are generally understandable if one considers the functions tested by each and the anatomical derangements caused by the diseases in question. The Arden grating test appears to be an excellent and sensitive screening test for central visual disturbances. Images PMID:7356929

  11. Report on the National Eye Institute Audacious Goals Initiative: Regenerating the Optic Nerve.

    Science.gov (United States)

    Goldberg, Jeffrey L; Guido, William

    2016-03-01

    The National Eye Institute (NEI) hosted a workshop on November 19, 2014, as part of the Audacious Goals Initiative (AGI), an NEI-led effort to rapidly expand therapies for eye diseases through coordinated research funding. The central audacious goal aims to demonstrate by 2025 the restoration of usable vision in humans through the regeneration of neurons and neural connections in the eye and visual system. This workshop focused on identifying promising strategies for optic nerve regeneration. Its principal objective was to solicit input on future AGI-related funding announcements, and specifically to ask, where are we now in our scientific progress, and what progress should we reach for in the coming years? A full report was generated as a white paper posted on the NEI Web site; this report summarizes the discussion and outcomes from the meeting and serves as guidance for future funding of research that focuses on optic nerve regeneration.

  12. The Effect of Optic Disc Center Displacement on Retinal Nerve Fiber Layer Measurement Determined by Spectral Domain Optical Coherence Tomography

    Science.gov (United States)

    Uhm, Ki Bang; Sung, Kyung Rim; Kang, Min Ho; Cho, Hee Yoon; Seong, Mincheol

    2016-01-01

    Purpose To investigate the effect of optic disc center displacement on retinal nerve fiber layer (RNFL) measurement determined by spectral domain optical coherence tomography (SD-OCT). Methods The optic disc center was manipulated at 1-pixel intervals in horizontal, vertical, and diagonal directions. According to the manipulated optic disc center location, the RNFL thickness data were resampled: (1) at a 3.46-mm diameter circle; and (2) between a 2.5-mm diameter circle and 5.4-mm square. Error was calculated between the original and resampled RNFL measurements. The tolerable error threshold of the optic disc center displacement was determined by considering test-retest variability of SD-OCT. The unreliable zone was defined as an area with 10% or more variability. Results The maximum tolerable error thresholds of optic disc center displacement on the RNFL thickness map were distributed from 0.042 to 0.09 mm in 8 directions. The threshold shape was vertically elongated. Clinically important unreliable zones were located: (1) at superior and inferior region in the vertical displacement; (2) at inferotemporal region in the horizontal displacement, and (3) at superotemporal or inferotemporal region in the diagonal displacement. The unreliable zone pattern and threshold limit varied according to the direction of optic disc displacement. Conclusions Optic disc center displacement had a considerable impact on whole RNFL thickness measurements. Understanding the effect of optic disc center displacement could contribute to reliable RNFL measurements. PMID:27783663

  13. The lens has a specific influence on optic nerve and tectum development in the blind cavefish Astyanax.

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    Soares, Daphne; Yamamoto, Yoshiyuki; Strickler, Allen G; Jeffery, William R

    2004-01-01

    We used the teleost Astyanaxmexicanus to examine the role of the lens in optic nerve and tectum development. This speciesis unusually suited for studies of nervous system development and evolution because of its two extant forms: an eyed surface dwelling (surface fish) and several blind cave dwelling (cavefish) forms. Cavefish embryos initially form eye primordia, but the lens eventually dies by apoptosis, then the retina ceases to grow, and finally the degenerating eyes sink into the orbits. Transplantation of an embryonic surface fish lens into a cavefish optic cup restores eye development. We show here that retinal nerve fibers are formed and project to the optic tectum in cavefish embryos. In adult cavefish that have completed lens degeneration, however, the number of retinal axons in the optic nerve is substantially reduced compared to surface fish. The presumptive brain domains of embryonic cavefish are not altered relative to surface fish based on expression of the regional marker genes Pax6, Pax2.1, and engrailed2. In contrast, the adult cavefish brain is elongated, the optic tectum is diminished in volume, and the number of tectal neurons is reduced relative to surface fish. Unilateral transplantation of an embryonic surface fish lens into a cavefish optic cup increases the size of the optic nerve, the number of retinotectal projections from the restored eye, and the volume and neuronal content of the contralateral optic tectum. The results suggest that the lens has a specific influence on optic nerve and tectum development during eye growth in Astyanax.

  14. [STUDY EFFECT OF ASSOSIATED PATHOLOGY ON THE DEVELOPMENT OF OPTIC NERVE ATROPHY].

    Science.gov (United States)

    Vasyuta, V A

    2015-01-01

    Studied effect of assosiated pathology on the development of optic nerve atrophy (ONA). The basis of population study was comparison some factors in the groups with ONA and without ONA (analysis case-control) and evaluation of relative risk. Analysis showed that the risk of development of ONA increased in coronary heart disease, hypertension, diabetes, atherosclerosis. A functional-organizational model of care for patients with ONA was proposed.

  15. Protective effects of cerebrolysin in a rat model of optic nerve crush.

    Science.gov (United States)

    Huang, Tzu-Lun; Huang, Sun-Ping; Chang, Chung-Hsing; Lin, Kung-Hung; Sheu, Min-Muh; Tsai, Rong-Kung

    2014-07-01

    To investigate the effects of cerebrolysin (Cbl) on optic nerves (ON) and retinal ganglion cells (RGC) in a rat model of ON crush. Rats received intravitreal injection of Cbl (n = 20), intra-ON injection of Cbl (n = 20), intraperitoneal injection (IPI) of Cbl (n = 20), or phosphate buffered saline (PBS; n = 20) every day for 2 weeks after ON crush injury. At 3 weeks post-trauma, RGC density was counted by retrograde labeling with FluoroGold and visual function was assessed by flash visual-evoked potentials. Activities of microglia after insults were quantified by immunohistochemical analysis of the presence of ED1 in the optic nerve. At 3 weeks postcrush, the densities of RGCs in the Cbl-IVI group (1125 ± 166/mm(2)) and in the Cbl-IPI treatment group (1328 ± 119/mm(2)) were significantly higher than those in the PBS group (641 ± 214/mm(2)). The flash visual-evoked potential measurements showed that latency of the P1 wave was significantly shorter in the Cbl-IVI- and Cbl-IPI-treated groups (105 ± 4 ms and 118 ± 26 ms, respectively) than in the PBS-treated group (170 ± 20 ms). However, only Cbl IPI treatment resulted in a significant decrease in the number of ED1-positive cells at the lesion sites of the ON (5 ± 2 cells/vs. 30 ± 4 cells/high-power field in control eyes). Treatment with intra-ON injection of Cbl was harmful to the optic nerve in the crush model. Systemic administration of Cbl had neuroprotective effects on RGC survival and visual function in the optic nerve crush model.

  16. Dominant inheritance of retinal ganglion cell resistance to optic nerve crush in mice

    Directory of Open Access Journals (Sweden)

    Schlamp Cassandra L

    2007-03-01

    Full Text Available Abstract Background Several neurodegenerative diseases are influenced by complex genetics that affect an individual's susceptibility, disease severity, and rate of progression. One such disease is glaucoma, a chronic neurodegenerative condition of the eye that targets and stimulates apoptosis of CNS neurons called retinal ganglion cells. Since ganglion cell death is intrinsic, it is reasonable that the genes that control this process may contribute to the complex genetics that affect ganglion cell susceptibility to disease. To determine if genetic background influences susceptibility to optic nerve damage, leading to ganglion cell death, we performed optic nerve crush on 15 different inbred lines of mice and measured ganglion cell loss. Resistant and susceptible strains were used in a reciprocal breeding strategy to examine the inheritance pattern of the resistance phenotype. Because earlier studies had implicated Bax as a susceptibility allele for ganglion cell death in the chronic neurodegenerative disease glaucoma, we conducted allelic segregation analysis and mRNA quantification to assess this gene as a candidate for the cell death phenotype. Results Inbred lines showed varying levels of susceptibility to optic nerve crush. DBA/2J mice were most resistant and BALB/cByJ mice were most susceptible. F1 mice from these lines inherited the DBA/2J phenotype, while N2 backcross mice exhibited the BALB/cByJ phenotype. F2 mice exhibited an intermediate phenotype. A Wright Formula calculation suggested as few as 2 dominant loci were linked to the resistance phenotype, which was corroborated by a Punnett Square analysis of the distribution of the mean phenotype in each cross. The levels of latent Bax mRNA were the same in both lines, and Bax alleles did not segregate with phenotype in N2 and F2 mice. Conclusion Inbred mice show different levels of resistance to optic nerve crush. The resistance phenotype is heritable in a dominant fashion involving

  17. Protective effects of cerebrolysin in a rat model of optic nerve crush

    Directory of Open Access Journals (Sweden)

    Tzu-Lun Huang

    2014-07-01

    Full Text Available To investigate the effects of cerebrolysin (Cbl on optic nerves (ON and retinal ganglion cells (RGC in a rat model of ON crush. Rats received intravitreal injection of Cbl (n = 20, intra-ON injection of Cbl (n = 20, intraperitoneal injection (IPI of Cbl (n = 20, or phosphate buffered saline (PBS; n = 20 every day for 2 weeks after ON crush injury. At 3 weeks post-trauma, RGC density was counted by retrograde labeling with FluoroGold and visual function was assessed by flash visual-evoked potentials. Activities of microglia after insults were quantified by immunohistochemical analysis of the presence of ED1 in the optic nerve. At 3 weeks postcrush, the densities of RGCs in the Cbl-IVI group (1125 ± 166/mm2 and in the Cbl-IPI treatment group (1328 ± 119/mm2 were significantly higher than those in the PBS group (641 ± 214/mm2. The flash visual-evoked potential measurements showed that latency of the P1 wave was significantly shorter in the Cbl-IVI- and Cbl-IPI-treated groups (105 ± 4 ms and 118 ± 26 ms, respectively than in the PBS-treated group (170 ± 20 ms. However, only Cbl IPI treatment resulted in a significant decrease in the number of ED1-positive cells at the lesion sites of the ON (5 ± 2 cells/vs. 30 ± 4 cells/high-power field in control eyes. Treatment with intra-ON injection of Cbl was harmful to the optic nerve in the crush model. Systemic administration of Cbl had neuroprotective effects on RGC survival and visual function in the optic nerve crush model.

  18. Mutant human myocilin induces strain specific differences in ocular hypertension and optic nerve damage in mice.

    Science.gov (United States)

    McDowell, Colleen M; Luan, Tomi; Zhang, Zhang; Putliwala, Tasneem; Wordinger, Robert J; Millar, J Cameron; John, Simon W M; Pang, Iok-Hou; Clark, Abbot F

    2012-07-01

    Elevated intraocular pressure (IOP) is a causative risk factor for the development and progression of glaucoma. Glaucomatous mutations in myocilin (MYOC) damage the trabecular meshwork and elevate IOP in humans and in mice. Animal models of glaucoma are important to discover and better understand molecular pathogenic pathways and to test new glaucoma therapeutics. Although a number of different animal models of glaucoma have been developed and characterized, there are no true models of human primary open angle glaucoma (POAG). The overall goal of this work is to develop the first inducible mouse model of POAG using a human POAG relevant transgene (i.e. mutant MYOC) expression in mouse eyes to elevate IOP and cause pressure-induced damage to the optic nerve. Four mouse strains (A/J, BALB/cJ, C57BL/6J, and C3H/HeJ) were used in this study. Ad5.MYOC.Y437H (5 × 10(7) pfu) was injected intravitreally into one eye, with the uninjected contralateral eye serving as the control eye. Conscious IOP measurements were taken using a TonoLab rebound tonometer. Optic nerve damage was determined by scoring PPD stained optic nerve cross sections. Retinal ganglion cell and superior colliculus damage was assessed by Nissl stain cell counts. Intravitreal administration of viral vector Ad5.MYOC.Y437H caused a prolonged, reproducible, and statistically significant IOP elevation in BALB/cJ, A/J, and C57BL/6J mice. IOPs increased to approximately 25 mm Hg for 8 weeks (p death or superior colliculus damage at the 8-week time point in any of the strains tested. These results demonstrate strain dependent responses to Ad5.MYOC.Y437H-induced ocular hypertension and pressure-induced optic nerve damage. Copyright © 2012 Elsevier Ltd. All rights reserved.

  19. In vivo quantitative evaluation of the rat retinal nerve fiber layer with optical coherence tomography.

    Science.gov (United States)

    Nagata, Atsushi; Higashide, Tomomi; Ohkubo, Shinji; Takeda, Hisashi; Sugiyama, Kazuhisa

    2009-06-01

    To determine whether optical coherence tomography (OCT) is useful for quantitative evaluation of the thickness of the rat retinal