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Sample records for nerve sheath meningioma

  1. Primary optic nerve sheath meningioma

    Energy Technology Data Exchange (ETDEWEB)

    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. Cystic change in primary paediatric optic nerve sheath meningioma.

    Science.gov (United States)

    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.

  3. A rare case of bilateral optic nerve sheath meningioma

    Directory of Open Access Journals (Sweden)

    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.

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

    Science.gov (United States)

    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.

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

    NARCIS (Netherlands)

    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

  6. 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.

  7. 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.

  8. An unusual radiological presentation of optic nerve sheath meningiom

    Directory of Open Access Journals (Sweden)

    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.

  9. 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

  10. 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

  11. Meningioma

    Science.gov (United States)

    ... brain tissue, nerves or vessels may cause serious disability. Meningiomas occur most commonly in women, and are often discovered at older ages, but ... meningioma. Female hormones. Meningiomas are more common in women, ... tumors. Obesity. A high BMI (body mass index) is an established risk ...

  12. 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.

  13. 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.

  14. 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

  15. Optic Nerve Sheath Mechanics in VIIP Syndrome

    Science.gov (United States)

    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

  16. 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...

  17. 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.

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

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    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. 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.

  20. Malignant Peripheral Nerve Sheath Tumour of the Maxilla

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    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.

  1. 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.)

  2. [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.

  3. 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.

  4. 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.

  5. File list: DNS.Neu.50.AllAg.Nerve_Sheath_Neoplasms [Chip-atlas[Archive

    Lifescience Database Archive (English)

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  1. File list: ALL.Neu.10.AllAg.Nerve_Sheath_Neoplasms [Chip-atlas[Archive

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    Lifescience Database Archive (English)

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    Lifescience Database Archive (English)

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  9. 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.

  10. 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.

  11. 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...

  12. 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.

  13. 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.

  14. [A Case of Foramen Magnum Meningioma Manifesting as Hypoglossal Nerve Palsy].

    Science.gov (United States)

    Inaka, Yasufumi; Otani, Naoki; Nishida, Sho; Ueno, Hideaki; Tomiyama, Arata; Tomura, Satoshi; Toyooka, Terushige; Wada, Kojiro; Mori, Kentaro

    2017-04-01

    We report a case of foramen magnum meningioma manifesting as hypoglossal nerve palsy. A 72-year-old woman presented with progressive hypoglossal nerve palsy and lingual atrophy on the left side. Gadolinium-enhanced T1-weighted magnetic resonance imaging revealed a heterogeneously enhanced mass lesion with dural tail sign partially extending into the hypoglossal canal. The transcondylar approach was performed to expose the hypoglossal canal and resect the tumor completely. Histological examination revealed a transitional meningioma. The postoperative course was uneventful. Hypoglossal nerve palsy improved gradually after the operation.

  15. 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

  16. 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.

  17. 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

  18. 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

  19. 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.

  20. 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.

  1. Microgravity-Driven Optic Nerve/Sheath Biomechanics Simulations

    Science.gov (United States)

    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

  2. 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.

  3. 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.

  4. 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.

  5. 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.

  6. 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.

  7. 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.

  8. 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

  9. 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.

  10. 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

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  19. 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.

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

    Directory of Open Access Journals (Sweden)

    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.

  1. 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.

  2. 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.

  3. Ectopic orbital meningioma: Fact or fiction?

    Science.gov (United States)

    Tan, Lee Teak; Stewart, Christopher M; Sheerin, Fintan; MacDonald, Brendan; Silva, Priy; Norris, Jonathan H

    2017-06-01

    Primary intraorbital ectopic meningiomas are rare and their existence remains controversial. We present a 30-year-old female with painless, non-axial proptosis and a palpable superomedial mass. The MRI demonstrated that the mass had no optic nerve sheath or sphenoid wing involvement and was initially reported to have no intracranial extension. The patient was initially thought to have an ectopic orbital meningioma. Subsequent multidisciplinary team (MDT) consultation and further specialist review of the MRI revealed a subtle dural tail connecting to an enhancing mass in the olfactory groove. Biopsy revealed a WHO Grade 1 transitional meningioma with an infiltrative pattern. We argue that some previously reported cases of ectopic meningioma may lack the requisite imaging to discover the primary disease. Our report highlights the importance of MRI in this group of patients and the role of a skull-base MDT with specialist neuroradiology input to determine the true origin and extent of these extradural orbital meningiomas.

  4. Concurrent spinal nerve root schwannoma and meningioma mimicking single-component schwannoma.

    Science.gov (United States)

    Nakamizo, Akira; Suzuki, Satoshi O; Shimogawa, Takafumi; Amano, Toshiyuki; Mizoguchi, Masahiro; Yoshimoto, Koji; Sasaki, Tomio

    2012-04-01

    We present a first case of concurrent tumors consisting of schwannoma and meningioma arising at the same spinal level in a patient without neurofibromatosis. A 49-year-old man without clinical evidence of neurofibromatosis presented with a 5-month history of right neck pain. MRI demonstrated an extradural tumor involving the right-sided C2 nerve root with a small intradural component. T1- and T2-weighted and contrast-enhanced MRI could not differentiate the intradural tumor as different from the extradural tumor. Total removal of the tumors was performed. No contiguity of the extradural tumor with the intradural tumor was seen. The intradural tumor attached strongly to the dura mater around the C2 nerve root exits. Intraoperative pathological diagnosis confirmed the extradural tumor as schwannoma and the intradural tumor as meningioma. We therefore thoroughly coagulated the dura mater adjacent to the intradural tumor and resected the dura mater around the nerve root exits together with the tumor. Pathological examination revealed that the resection edge of the extradural component consisted of a spinal nerve with thickened epineurium and was free of neoplastic cells. No schwannoma component was evident in the intradural tumor. No obvious transition thus existed between the extra- and intradural tumors. Distinguishing these tumors prior to surgery is critical for determining an optimal surgical plan, as schwannoma and meningioma require different surgical procedures. We therefore recommend a careful review of preoperative imaging with the possibility of concurrent tumors in mind. © 2011 Japanese Society of Neuropathology.

  5. 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.

  6. 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

  7. Optic Nerve Sheath Mechanics and Permeability in VIIP Syndrome

    Science.gov (United States)

    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

  8. Biomechanics of the Optic Nerve Sheath in VIIP Syndrome

    Science.gov (United States)

    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

  9. 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.

  10. 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...

  11. 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.

  12. 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%.

  13. 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.

  14. 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.

  15. 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

  16. 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.

  17. 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.)

  18. 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.

  19. 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

  20. 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.

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

    Directory of Open Access Journals (Sweden)

    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.

  2. 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.)

  3. 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.

  4. 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...

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

    Science.gov (United States)

    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

  6. 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.

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

    Energy Technology Data Exchange (ETDEWEB)

    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.

  8. 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...

  9. 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

  10. 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

  11. 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.

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

    NARCIS (Netherlands)

    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

  13. 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.

  14. 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.

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

    Directory of Open Access Journals (Sweden)

    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

  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. 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.

  18. Scalp meningioma

    Directory of Open Access Journals (Sweden)

    Singh Sunil

    2008-01-01

    Full Text Available Primary extracranial meningiomas occur very rarely. We present a rare case of extracranial meningioma of the transitional variant which was excised satisfactorily. There was no suggestion of any connection to the intracranial compartment or cranial nerves. The underlying galea was uninvolved, suggesting the true extracranial nature of this tumour. This rare diagnosis should nonetheless be kept in the differential diagnosis of scalp tumors.

  19. 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.

  20. 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.

  1. 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.

  2. 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.

  3. 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.

  4. 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...

  5. 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...

  6. 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.)

  7. 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.

  8. 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.

  9. 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

  10. 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.

  11. 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.)

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

    Energy Technology Data Exchange (ETDEWEB)

    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.

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

    Science.gov (United States)

    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.

  15. 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

  16. 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.

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

    Science.gov (United States)

    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.

  18. 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.

  19. 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.

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

    Science.gov (United States)

    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.

  1. 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.

  2. 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.

  3. 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.

  4. 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.

  5. 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.

  6. 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.

  7. 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.

  8. 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.

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

  11. 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

  12. 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.

  13. 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

  14. 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.

  15. 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.)

  16. 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

  17. 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

  18. 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

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

    Directory of Open Access Journals (Sweden)

    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.

  20. 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

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

    Directory of Open Access Journals (Sweden)

    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.

  2. 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.

  3. 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.

  4. 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.

  5. 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

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

    Directory of Open Access Journals (Sweden)

    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.

  7. 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.

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

    Science.gov (United States)

    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

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

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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.

  11. 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.

  12. 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.

  13. 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

  14. 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.

  15. 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.

  16. 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. 

  17. 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.

  18. 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

  19. 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

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

    Science.gov (United States)

    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.

  1. 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.

  2. 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...

  3. 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.

  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. 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.

  6. 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.

  7. 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.

  8. 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.

  9. 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.

  10. 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.

  11. 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.

  12. 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

  13. 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.

  14. 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.

  15. 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.

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

    Science.gov (United States)

    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.

  17. 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.

  18. 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.

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

    Science.gov (United States)

    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.

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

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    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.

  1. 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.

  2. 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...

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

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    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

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

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    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.

  5. 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

  6. 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

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

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    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.)

  8. 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

  9. 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.

  10. Meningioma Causing Visual Impairment: Outcomes and Toxicity After Intensity Modulated Radiation Therapy

    Energy Technology Data Exchange (ETDEWEB)

    Maclean, Jillian, E-mail: jillian.maclean@uclh.nhs.uk [Radiotherapy Department, University College London Hospital, London (United Kingdom); Fersht, Naomi [Radiotherapy Department, University College London Hospital, London (United Kingdom); Bremner, Fion [Neuro-Ophthalmology Department, National Hospital for Neurology and Neurosurgery, London (United Kingdom); Stacey, Chris; Sivabalasingham, Suganya [Radiotherapy Department, University College London Hospital, London (United Kingdom); Short, Susan [Radiotherapy Department, University College London Hospital, London (United Kingdom); Leeds Institute of Molecular Medicine, St James University Hospital, Leeds (United Kingdom)

    2013-03-15

    Purpose: To evaluate ophthalmologic outcomes and toxicity of intensity modulated radiation therapy (IMRT) in patients with meningiomas causing visual deficits. Methods and Materials: A prospective observational study with formal ophthalmologic and clinical assessment of 30 consecutive cases of meningioma affecting vision treated with IMRT from 2007 to 2011. Prescriptions were 50.4 Gy to mean target dose in 28 daily fractions. The median follow-up time was 28 months. Twenty-six meningiomas affected the anterior visual pathway (including 3 optic nerve sheath meningiomas); 4 were posterior to the chiasm. Results: Vision improved objectively in 12 patients (40%). Improvements were in visual field (5/16 patients), color vision (4/9 patients), acuity (1/15 patients), extraocular movements (3/11 patients), ptosis (1/5 patients), and proptosis (2/6 patients). No predictors of clinical response were found. Two patients had minor reductions in tumor dimensions on magnetic resonance imaging, 1 patient had radiological progression, and the other patients were stable. One patient experienced grade 2 keratitis, 1 patient had a minor visual field loss, and 5 patients had grade 1 dry eye. Conclusion: IMRT is an effective method for treating meningiomas causing ophthalmologic deficits, and toxicity is minimal. Thorough ophthalmologic assessment is important because clinical responses often occur in the absence of radiological change.

  11. 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.

  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. 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

  14. 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.

  15. 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

  16. 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.

  17. 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.

  18. 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

  19. 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.

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Directory of Open Access Journals (Sweden)

    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

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

    Directory of Open Access Journals (Sweden)

    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.

  3. 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

  4. Cerebrospinal fluid dissemination of anaplastic intraventricular meningioma: report of a case presenting with progressive brainstem dysfunction and multiple cranial nerve palsies.

    Science.gov (United States)

    Fujimaki, Motoki; Takanashi, Masashi; Kobayashi, Manami; Wada, Kei-ichiro; Machida, Yutaka; Kondo, Akihide; Hattori, Nobutaka; Miwa, Hideto

    2016-05-31

    It is extremely rare to see cerebrospinal fluid dissemination of intraventricular meningioma, particularly with the development of acute, progressive brainstem/cerebellar dysfunction with an absence of mass formation in the corresponding anatomical sites. An 81-year-old man was admitted because of double vision, right facial nerve palsy and truncal ataxia. Brain magnetic resonance imaging showed normal findings except for a tumor mass in the left lateral ventricle, which had been noted over 6 months previously. The patient developed hiccups, hyperventilation, and drowsiness, which worsened progressively, and did not respond to corticosteroid or intraventricular immunoglobulin therapy. Cerebrospinal fluid study revealed a mild elevation of protein, and cytology was negative. The patient died and an autopsy was performed. Postmortem investigation disclosed a malignant transformation of benign fibroid meningioma with cerebrospinal fluid dissemination of the malignant cells, diversely involving the surface of brainstem, cerebellum, and spinal cords, secondarily resulting in extensive ischemia in the brain parenchyma by vessel occlusion. If a patient with an intraventricular tumor develops acute, progressive neurological symptoms, the possibility that it is be caused by cerebrospinal fluid dissemination of tumor cells, after malignant transformation, should be considered.

  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. 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.

    Directory of Open Access Journals (Sweden)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

  8. 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

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

    Directory of Open Access Journals (Sweden)

    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.

  10. 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 ...

  11. 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

  12. 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.

  13. 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.

  14. 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.

  15. 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.

  16. 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

  17. 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.

  18. 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.

  19. 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.

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

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    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

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

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    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.

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

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    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.

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

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    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.

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

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    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.

  5. 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.

  6. [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).

  7. Sphenoid wing meningioma--an unusual cause of duro-optic calcification.

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    Patankar T

    1997-04-01

    Full Text Available Sphenoid ridge is the third commonest site of intracranial meningiomas. Although sphenoid ridge meningiomas often involve the optic canal, calcification along the optic nerve has not been reported with these tumors. We describe CT features of a calcified optic nerve in a patient with a calcified sphenoid ridge meningioma.

  8. 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.

  9. 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.

  10. 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.

  11. 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

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

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    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.)

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

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    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.

  14. Extradural en-plaque spinal meningioma with intraneural invasion.

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    Tuli, Jayshree; Drzymalski, Dan Michael; Lidov, Hart; Tuli, Sagun

    2012-01-01

    Extradural spinal meningiomas are rare. Our understanding of purely extradural spinal meningiomas is incomplete because most reports rarely differentiate purely extradural meningiomas from extradural meningiomas with an intradural component. Occasionally, reports have described involvement of the adjacent nerve root, but there has never been a description of an extradural meningioma that actually infiltrates the nerve root. A 42-year-old woman presented with progressive lower extremity weakness and numbness below T3 during the span of 4 months with imaging evidence of an extradural lesion compressing the cord from T4 through T6. Surgical resection revealed an extradural mass extending through the foramen at T5-6 and encompassing the cord and T5 root on the left. Pathologically, the lesion was a World Health Organization grade I meningioma with nerve root invasion and a concerning elevated mindbomb homolog 1 (MIB-1) of 9.4%. Purely extradural meningiomas are rare, and our case is one of the first to describe a patient with an extradural meningioma that actually infiltrates the nerve root. Extradural spinal meningiomas are usually not adherent to the dura, but only appear to be adherent or invade (as in our patient) the adjacent nerve root. They are easily mistaken preoperatively and grossly intraoperatively for malignant metastatic tumors and can change the proposed surgical treatment. The long-term prognosis remains uncertain, but our patient's last follow-up suggests a favorable prognosis. Copyright © 2012 Elsevier Inc. All rights reserved.

  15. 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.

  16. 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

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

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    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.

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

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    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 ...

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

    Directory of Open Access Journals (Sweden)

    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

  20. 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

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

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    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

  2. Extradural spinal meningioma: MRI

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    Sato, N. [Department of Diagnostic Radiology, Yale University School of Medicine, P. O. Box 20 8042, New Haven, CT 06520-8042 (United States); Sze, G. [Department of Diagnostic Radiology, Yale University School of Medicine, P. O. Box 20 8042, New Haven, CT 06520-8042 (United States)

    1997-06-01

    We report a case of extradural spinal meningioma with pathologically proven features of malignant transformation. The MRI findings of extradural spinal meningioma and differences in the findings from intradural meningiomas are discussed. (orig.). With 1 fig.

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

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    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

  4. 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

  5. Meningioma of the Posterior Skull Base

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    Biggs, Michael Thomas; Fagan, Paul A.; Sheehy, John P.R.; Bentivoglio, Peter J.; Doust, Bruce D.; Tonkin, John

    1991-01-01

    Combined intratemporal and cerebellopontine angle meningiomas are rejatively rare. There are unsolved problems with the stability of the skull and spine and the lower cranial nerves and there is a marked tendency for the tumor to involve the spinal cord. This article reports on five cases of combined intratemporal and cerebellopontine angle meningiomas. ImagesFigure 1p44-bFigure 2Figure 3Figure 4Figure 5 PMID:17170820

  6. Monocular nasal hemianopia from atypical sphenoid wing meningioma.

    Science.gov (United States)

    Stacy, Rebecca C; Jakobiec, Frederick A; Lessell, Simmons; Cestari, Dean M

    2010-06-01

    Neurogenic monocular nasal field defects respecting the vertical midline are quite uncommon. We report a case of a unilateral nasal hemianopia that was caused by compression of the left optic nerve by a sphenoid wing meningioma. Histological examination revealed that the pathology of the meningioma was consistent with that of an atypical meningioma, which carries a guarded prognosis with increased chance of recurrence. The tumor was debulked surgically, and the patient's visual field defect improved.

  7. Ancient schwannoma at the olfactory groove mimicking meningioma: A case report

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    Heo, Young Jin; Jeong, Hae Woong [Dept. of Radiology, Busan Paik Hospital, Inje University College of Medicine, Busan (Korea, Republic of)

    2015-12-15

    Schwannomas are benign slow-growing nerve sheath tumors, which can develop in any peripheral or central nerve that contains Schwann cells. Schwannomas located near the olfactory groove are extremely rare and radiological diagnosis can be difficult. Moreover, ancient schwannoma is an uncommon variant, and radiologic findings are rarely reported. Herein, we reported a surgically confirmed case of ancient schwannoma at the olfactory groove in a 44-year-old woman presenting with headache and visual disturbance. Brain magnetic resonance imaging (MRI) showed a solid and cystic extra-axial mass located in the subfrontal area mimicking an olfactory groove meningioma. Histopathologic diagnosis of ancient schwannoma was confirmed by immunohistochemical staining for S100, CD56, vimentin, and other markers. Furthermore, we described the clinical manifestations, MRI characteristics, and histopathologic findings of the case, and presented a review of related literature.

  8. 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

  9. 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.

  10. Primary Intraosseous Meningioma.

    Science.gov (United States)

    Chen, Thomas C

    2016-04-01

    Primary intraosseous meningiomas are a subtype of primary extradural meningiomas. They represent approximately two-thirds of extradural meningiomas and fewer than 2% of meningiomas overall. These tumors originate within the bones of the skull and can have a clinical presentation and radiographic differential diagnosis different from those for intradural meningiomas. Primary intraosseous meningiomas are classified based on location and histopathologic characteristics. Treatment is primarily surgical resection with wide margins if possible. Sparse literature exists regarding the use of adjuvant therapies. The literature regarding primary intraosseous meningiomas consists primarily of clinical case reports and case series. This literature is reviewed and summarized in this article.

  11. 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

  12. 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

  13. Meningioma involving Meckel's cave: transpetrosal surgical anatomy and clinical considerations.

    Science.gov (United States)

    Choo, Daniel I; Steward, David L; Pensak, Myles L

    2002-09-01

    Meningiomas originating in Meckel's cave (MC) are uncommon lesions that represent 1% of all intracranial meningiomas. Innovations in skull base surgery have enabled resection of these lesions with less morbidity, but require an intimate knowledge of both lesional pathology and regional microneuroanatomy. To review the surgical and clinical considerations involved in the management of MC meningiomas, we retrospectively reviewed data from patients who underwent transpetrosal resection of primary MC meningiomas between 1984 and 1998. Of 146 patients who underwent transpetrosal removal of meningiomas, 7 were believed to have tumors originating in MC. All 7 patients presented with trigeminal dysfunction, facial pain, and/or headache. Complete tumor removal was achieved in 5 of the 7 patients. Facial hypoesthesia or anesthesia, paralysis of cranial nerve VI, and ophthalmoplegia were among the postoperative complications encountered. Meningiomas of MC represent treatable lesions whose diagnosis requires prompt imaging of patients with trigeminal dysfunction and symptoms of facial pain and headache.

  14. 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

  15. 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.

  16. 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.

  17. [Meningioma of the optic canal. Diagnostic aspects apropos of 3 surgically treated cases].

    Science.gov (United States)

    Bazin, A; Scherpereel, B; Rousseaux, P; Bernard, M H; Peruzzi, P; Baudrillard, J C; Delattre, J F

    1988-01-01

    Three cases of meningiomas originated in the optic nerve sheath in its intra-canalicular portion, have been operated on in Reims since we dispose of the TDM; it gave us the opportunity to re-define the way how to explore an area always difficult to investigate; indeed it has been impossible for a long time to clearly isolate meningiomas in the optic canal through the classical neuro-radiologic technics. The anatomic study confirms that the optic canal orientation is fixed, constantly inclined downward and forward with an angle of minus forty degrees with regard to the horizontal line; our work establishes that it is possible to apply it to the CT scan; in this incidence, TDM perfectly allows to visualize the optic canal and the optic nerve, from the anterior clinoid process to the posterior orbital pole. This incidence seems us to be interesting, in addition to classical neuro-ophthalmic planes, for early diagnosis or supervision of tumors of the optic canal.

  18. 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.

  19. 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

  20. Meningiomas of Meckel's cave.

    Science.gov (United States)

    Delfini, R; Innocenzi, G; Ciappetta, P; Domenicucci, M; Cantore, G

    1992-12-01

    A series of 16 patients with meningiomas of Meckel's cave is reported. Trigeminal neuralgia, typical or atypical, was the initial symptom in 10 patients (62.5%). At admission, trigeminal signs and symptoms were present in 15 patients (93.7%); in 7 patients (43.7%), trigeminal dysfunction was combined with the impairment of other cranial nerves. On retrospective analysis, these patients fall into two clinical groups that differ also in prognosis. Group 1 comprises eight patients with trigeminal signs and symptoms only. These patients had small meningiomas strictly affecting Meckel's cave. Total removal of the tumor was achieved in seven of eight patients, without adjunctive postoperative neurological deficits. In this group, there were no tumor recurrences. Group 2 comprises the other eight patients in whom trigeminal dysfunction was combined with impairment of other cranial nerves. These patients had large tumors arising from Meckel's cave and secondarily invading the cavernous sinus (five patients) or extending into the posterior fossa (two patients) or largely growing into the middle fossa (one patient). Total removal was achieved in only one patient, and a worsening of the preoperative neurological status was observed in four patients; there were three cases of tumor progression. A subtemporal intradural approach (used in the past in every case) is still used for the small tumors of Group 1 with good results. Since 1985, for tumors involving the cavernous sinus, we have employed a frontotemporal craniotomy with extradural clinoidectomy and superior and lateral approach to the cavernous sinus. When the tumor extends toward the posterior fossa, we use a combined temporosuboccipital-transpetrosal approach.(ABSTRACT TRUNCATED AT 250 WORDS)

  1. Meningioma of the scapula

    Energy Technology Data Exchange (ETDEWEB)

    Llauger, Jaume; Aixut, Sonia; Canete, Noemi; Palmer, Jaume; Sola, Marta [Universitat Autonoma de Barcelona, Department of Radiology, Hospital de la Santa Creu I Sant Pau, Barcelona (Spain); Bague, Silvia [Universitat Autonoma de Barcelona, Department of Pathology, Hospital de la Santa Creu I Sant Pau, Barcelona (Spain)

    2008-02-15

    Meningiomas account for approximately 15% of all intracranial tumors and are the most common non-glial primary tumors of the central nervous system. Most meningiomas are benign neoplasms with characteristic imaging features. Primary extradural meningiomas account for only 1-2% of all meningiomas. They must be differentiated from intradural meningiomas with secondary extradural extension and/or metastases. The vast majority of extradural meningiomas are found in the skull or in the head and neck region. We report on an extremely rare case of primary extradural meningioma that was located in the scapula. The lesion was resected. Radiographic findings and pathologic features are discussed. To the best of our knowledge, this form of presentation of an extradural meningioma has not been previously described. (orig.)

  2. 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.)

  3. Intraosseous microcystic meningioma

    Energy Technology Data Exchange (ETDEWEB)

    Okamoto, Sumika; Hisaoka, Masanori; Hashimoto, Hiroshi [Dept. of Pathology and Oncology, Univ. of Occupational and Environmental Health, Yahatanishi-ku, Kitakyushu (Japan); Aoki, Takatoshi [Dept. of Radiology, Univ. of Occupational and Environmental Health, Kitakyushu (Japan); Kadoya, Chitoshi; Kobanawa, Satoshi [Dept. of Neurosurgery, Univ. of Occupational and Environmental Health, Kitakyushu (Japan)

    2000-06-01

    Extradural ectopic meningioma is a rare tumor. We report on an example of microcystic meningioma arising in the skull of an elderly woman. Radiological examination revealed a localized osteolytic lesion in the left parietal bone. At surgery, it was discovered that the tumor was located within the skull without any evidence of extraosseous extension. The light microscopic, immunohistochemical and ultrastructural features were consistent with a microcystic variant of meningioma. To our knowledge, this is the first case of an intraosseous microcystic meningioma, and we believe that this type of meningioma should be considered in the differential diagnoses of myxoid bone tumors of the calvarium. (orig.)

  4. 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.

  5. 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.

  6. 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.

  7. 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

  8. 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.

  9. 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.

  10. [{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.)

  11. 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

  12. 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.

  13. MRI imaging of tuberculum sellae meningioma

    Energy Technology Data Exchange (ETDEWEB)

    Kuchiki, Megumi; Hosoya, Takaaki; Watanabe, Nami; Nagahata, Morio; Yamaguchi, Koichi [Yamagata Univ. (Japan). School of Medicine

    1996-02-01

    We reviewed three MRI cases of suprasellar meningioma in comparison with the other sella region tumors. T1-weighted images of pre- and post contrast enhancement could clearly delineate the optic chiasm, optic nerve and pituitary gland. MRI findings of tuberculum sellae meningioma were characterized as follows. The pituitary gland was easily identified and compressed postero-inferiorly in the sella. The pituitary stalk was backward compressed by the tumor. The optic chiasm was compressed posteriorly or postero-superiorly by the tumor. It was important for its differential diagnosis to identify the displacement direction of the structures in and around the sella. (author).

  14. Meningiomas of the cerebellopontine angle.

    Science.gov (United States)

    Matthies, C; Carvalho, G; Tatagiba, M; Lima, M; Samii, M

    1996-01-01

    Meningiomas of the cerebellopontine angle (CPA) represent a clinically and surgically interesting entity. The opportunity of complete surgical excision and the incidence of impairment of nerval structures largely depend on the tumour biology that either leads to displacement of surrounding structures by an expansive type of growth or to an enveloping of nerval and vascular structures by an en plaque type of growth. As the origin and the direction of growth are very variable, the exact tumour extension in relation to the nerval structures and the tumour origin can be identified sometimes only at the time of surgery. Out of a series of 230 meningiomas of the posterior skull base operated between 1978 and 1993, data of 134 meningiomas involving the cerebellopontine angle are presented. There were 20% male and 80% female patients, age at the time of surgery ranging from 18 to 76 years, on the average 51 years. The clinical presentation was characterized by a predominant disturbance of the cranial nerves V (19%), VII (11%), VIII (67%) and the caudal cranial nerves (6%) and signs of ataxia (28%). 80% of the meningiomas were larger than 30 mm in diameter, 53% led to evident brainstem compression or dislocation and 85% extended anteriorly to the internal auditory canal. Using the lateral suboccipital approach in the majority of cases and a combined presigmoidal or combined suboccipital and subtemporal approaches in either sequence in 5%, complete tumour removal (Simpson I and II) was accomplished in 95% and subtotal tumour removal in 5%. Histologically the meningiotheliomatous type was most common (49%) followed by the mixed type (19%), fibroblastic (16%), psammomatous (7%), hemangioblastic (7%) and anaplastic (2%) types. Major post-operative complications were CSF leakage (8%) requiring surgical revision in 2% and hemorrhage (3%) requiring revision in 2%. While the majority of neurological disturbances showed signs of recovery, facial nerve paresis or paralysis was

  15. 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。

  16. 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.

  17. 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.

  18. 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.

  19. 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.

  20. Evaluation of intratumoural heterogeneity on {sup 18}F-FDG PET/CT for characterization of peripheral nerve sheath tumours in neurofibromatosis type 1

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    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.)

  1. Surgery of petroclival meningiomas. Recent surgical results and outcomes

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    Radoi Mugurel

    2015-03-01

    Full Text Available Petroclival meningiomas represent only 10% of all meningiomas located in the posterior fossa, but are some of the most formidable challenges in skull base surgery. We described our recent experience (2005-September2014 regarding the surgery of these tumors. We retrospectively analyzed surgical results and outcome in 11 cases of petroclival meningiomas. Most common symptoms in our series were headache and gait disturbance, while cranial nerves palsies represented the most common presenting signs. There were 8 females and 3 males, and the mean age was 52 years. Surgical approaches chosen for petroclival meningiomas in our series were retrosigmoid (9 patients and subtemporal transtentorial (2 patients. We achieved total tumor resection in 5 cases (45% and subtotal resection in 6 cases (55%. Overall outcome (total/subtotal resection was good in 6 cases, fair in 3 cases and poor in one case. One postoperative death occurred due to hemorrhagic midbrain infarction (9%. Complications were usually related to cranial nerve deficits: loss of hearing (2 patients, paresis of trochlear nerve (1 patient, trigeminal nerve (3 patients and facial nerve (1 patient. In 4 patients these cranial nerves deficits were transient. In one case, a patient developed postoperative hydrocephalus and needed shunt placement. Despite the fact that complications can be disastrous, we considered that an appropriate approach, combined with microsurgical techniques and a better understanding of the anatomy, greatly decrease the incidence and severity of complications and make feasible a total tumor resection.

  2. 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.

  3. 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.

  4. 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

  5. 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

  6. MULTIPLE SPINAL CANAL MENINGIOMAS

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    Nandigama Pratap Kumar

    2016-10-01

    Full Text Available BACKGROUND Meningiomas of the spinal canal are common tumours with the incidence of 25 percent of all spinal cord tumours. But multiple spinal canal meningiomas are rare in compare to solitary lesions and account for 2 to 3.5% of all spinal meningiomas. Most of the reported cases are both intra cranial and spinal. Exclusive involvement of the spinal canal by multiple meningiomas are very rare. We could find only sixteen cases in the literature to the best of our knowledge. Exclusive multiple spinal canal meningiomas occurring in the first two decades of life are seldom reported in the literature. We are presenting a case of multiple spinal canal meningiomas in a young patient of 17 years, who was earlier operated for single lesion. We analysed the literature, with illustration of our case. MATERIALS AND METHODS In September 2016, we performed a literature search for multiple spinal canal meningiomas involving exclusively the spinal canal with no limitation for language and publication date. The search was conducted through http://pubmed.com, a wellknown worldwide internet medical address. To the best of our knowledge, we could find only sixteen cases of multiple meningiomas exclusively confined to the spinal canal. Exclusive multiple spinal canal meningiomas occurring in the first two decades of life are seldom reported in the literature. We are presenting a case of multiple spinal canal meningiomas in a young patient of 17 years, who was earlier operated for solitary intradural extra medullary spinal canal meningioma at D4-D6 level, again presented with spastic quadriparesis of two years duration and MRI whole spine demonstrated multiple intradural extra medullary lesions, which were excised completely and the histopathological diagnosis was transitional meningioma. RESULTS Patient recovered from his weakness and sensory symptoms gradually and bladder and bowel symptoms improved gradually over a period of two to three weeks. CONCLUSION Multiple

  7. Keyhole approach surgery for petroclival meningioma

    Institute of Scientific and Technical Information of China (English)

    ZHU Wei; MAO Ying; ZHOU Liang-fu; ZHANG Rong; CHEN Liang

    2006-01-01

    Background In China, the feasibility of keyhole approach in surgical treatment of petroclival meningioma has not been well evaluated. This report summarized our experience in 25 patients with petroclival meningioma who had been treated with keyhole approach surgery.Methods From July 2000 to July 2005, 25 patients with petroclival meningioma were subjected to resection via subtemporal, retrosigmoid or combined keyhole approaches. The extent of tumor resection was evaluated by MRI 3 months after surgery, and postoperative complications were investigated.Results The maximum diameter of tumors ranged from 2 to 7 cm (mean, 4.5 cm). Gross total resection (GTR)was achieved in 14 patients, giving a GTR rate of 56%. Subtotal resection (STR) was carried out in 8 patients and partial resection in 3. Thirteen patients kept normal neurological status, whereas others suffered from cranial nerve deficits (Ⅶ, Ⅶ, Ⅲ and lower CN). One patient died in the postoperative period.Conclusions Keyhole approach surgery, especially the combined keyhole approach is suitable for the treatment of petroclival meningioma. It provides easy and quick access to the supra- and infratentorial juxta-clival region without drilling of the petrous bone. Complications related to the approach can be minimized.

  8. Cervical spinal meningioma mimicking intramedullary spinal tumor.

    Science.gov (United States)

    Senturk, Senem; Guzel, Aslan; Guzel, Ebru; Bayrak, Aylin Hasanefendioğlu; Sav, Aydin

    2009-01-01

    Case report. To report a very unusual spinal meningioma, mimicking an intramedullary spinal tumor. Spinal meningiomas, usually associated with signs and symptoms of cord or nerve root compression, are generally encountered in women aged over 40. Radiologic diagnosis is often established by their intradural extramedullary location on magnetic resonance images. A 60-year-old woman had a 6-month history of progressive weakness in her upper extremities, difficulty in walking, and cervical pain radiating through both arms. Neurologic examination revealed motor strength deficiency in all her extremities, with extensor reflexes, clonus, and bilateral hyper-reflexiveness. A sensory deficit was present all over her body. Magnetic resonance images revealed that the spinal cord appeared expanded with an ill-defined, homogeneously contrast-enhanced, lobulated, eccentric mass at the C1-C3 level. The patient was operated with a preliminary diagnosis of an intramedullary tumor. At surgery, the mass was found to be extramedullary, and gross total resection was performed. Histopathological examination revealed a meningioma characterized by the presence of fibrous and meningothelial components. The patient was able to ambulate with a cane, and extremity strength and sensation improved 2 months after surgery. Spinal meningiomas can mimic intramedullary tumors, and should be considered in differential diagnosis of intradural tumors with atypical appearance.

  9. Management of Spinal Meningiomas.

    Science.gov (United States)

    Ravindra, Vijay M; Schmidt, Meic H

    2016-04-01

    Spinal meningiomas are the most common spinal tumors encountered in adults, and account for 6.5% of all craniospinal tumors. The treatment for these lesions is primarily surgical, but emerging modalities may include chemotherapy and radiosurgery. In this article, the current management of spinal meningiomas and the body of literature surrounding conventional treatment is reviewed and discussed. Copyright © 2016 Elsevier Inc. All rights reserved.

  10. Optic neuropathy after irradiation of meningioma.

    Science.gov (United States)

    Goldsmith, B J; Rosenthal, S A; Wara, W M; Larson, D A

    1992-10-01

    Radiation-induced optic neuropathy (RON) is a rare and catastrophic complication of currently employed radiation therapy regimens for meningiomas that have been partially resected and sampled for biopsy. Between 1972 and 1989, 49 patients received postoperative irradiation for partially resected or biopsy sampled meningiomas, with the optic nerve within the treatment field. One patient experienced RON. The latency period for this case was 23 months. A review of the literature disclosed few cases of RON after treatment for meningioma; however, 42 cases of RON have been reported after radiation therapy for other lesions. The authors constructed two approaches to predict optic nerve radiation tolerance. The first is modeled on a previous proposal for a neural tissue nominal standard dose term and enabled accurate prediction of safe treatment regimens and risk of RON. This approach compared favorably with previously employed nominal standard dose terms. The second approach, based on the linear-quadratic model, proved unsuccessful due to its failure to achieve statistical significance.

  11. Chromosomal instability in meningiomas.

    Science.gov (United States)

    van Tilborg, Angela A G; Al Allak, Bushra; Velthuizen, Sandra C J M; de Vries, Annie; Kros, Johan M; Avezaat, Cees J J; de Klein, Annelies; Beverloo, H Berna; Zwarthoff, Ellen C

    2005-04-01

    Approximately 60% of sporadic meningiomas are caused by inactivation of the NF2 tumor suppressor gene on chromosome 22. No causative gene is known for the remaining 40%. Cytogenetic analysis shows that meningiomas caused by inactivation of the NF2 gene can be divided into tumors that show monosomy 22 as the sole abnormality and tumors with a more complex karyotype. Meningiomas not caused by the NF2 gene usually have a diploid karyotype. Here we report that, besides the clonal chromosomal aberrations, the chromosome numbers in many meningiomas varied from one metaphase spread to the other, a feature that is indicative of chromosomal instability. Unexpectedly and regardless of genotype, a subgroup of tumors was observed with an average number of 44.9 chromosomes and little variation in the number of chromosomes per metaphase spread. In addition, a second subgroup was recognized with a hyperdiploid number of chromosomes (average 48.5) and considerable variation in numbers per metaphase. However, this numerical instability resulted in a clonal karyotype with chromosomal gains and losses in addition to loss of chromosome 22 only in meningiomas caused by inactivation of the NF2 gene. In cultured cells of all tumor groups, bi- and multinucleated cells were seen, as well as anaphase bridges, residual chromatid strings, multiple spindle poles, and unseparated chromatids, suggesting defects in the mitotic apparatus or kinetochore. Thus, we conclude that even a benign and slow-growing tumor like a meningioma displays chromosomal instability.

  12. Intraparenchymal papillary meningioma of brainstem: case report and literature review

    Directory of Open Access Journals (Sweden)

    Jiang Xiao-Bing

    2012-01-01

    Full Text Available Abstract Both intraparenchymal papillary meningioma and papillary meningioma with cyst formation of brainstem have never been reported. The authors present an extremely rare case of patient with intraparenchymal papillary meningioma of brainstem. A 23-year-old Chinese male presented with a 4-month history of progressive left upper limb and facial nerve palsy. Magnetic resonance imaging revealed a cystic-solid, heterogeneously enhancing mass in pons and right cerebral peduncle with no dural attachment. The tumor was totally removed via subtemporal approach. During surgery, the lesion was found to be completely intraparenchymal. Histological and immunohistochemical examinations were compatible with the diagnosis of papillary meningioma. The lesion recurred nine months after primary surgery, a second surgery followed by radiotherapy was performed. Till to now (nearly 2 years after the treatment, the patient is tumor free survival. Intraparenchymal meningioma of brainstem with cystic formation is very rare, however, it should be considered as a differential diagnosis of a brainstem neoplasm. The present case strongly recommended that postoperative radiotherapy was essential for the patients with papillary meningiomas.

  13. 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.

  14. 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.

  15. 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

  16. Large spinal meningioma with hemorrhage after selective root block in the thoraco-lumbar spine.

    Science.gov (United States)

    Lee, Heui Seung; Lee, Sang Hyung; Chung, Young Seob; Yang, Hee-Jin; Son, Young-Je; Park, Sung Bae

    2013-12-01

    Spinal meningioma accounts for 25% of all spinal cord tumors and occurs mostly in the thoracic region. Spontaneous intraspinal bleeding associated with spinal meningioma has rarely been reported. Most cases of hemorrhage associated with meningiomas are extratumoral and subarachnoid, whereas subdural and intratumoral hemorrhage cases have been reported to occur rarely. We experienced a case of a 58-year-old woman with thoracolumbar spinal meningioma accompanied by intraspinal subdural hematoma. She presented with progressively worsened back pain and newly developed weakness in the right lower extremity after a selective nerve root block in the lumbar spine. She underwent the operation and progressively showed neurological recovery during the postoperative course. We report a thoracolumbarspinal meningioma with subdural and intratumoral hemorrhage after a spinal procedure that caused a sudden neurological deterioration.

  17. Cervical Extradural Meningioma: Case Report and Literature Review

    Science.gov (United States)

    Frank, Brian L; Harrop, James S; Hanna, Amgad; Ratliff, John

    2008-01-01

    Background: Extradural lesions are most commonly metastatic neoplasms. Extradural meningioma accounts for 2.7 to 10% of spinal neoplasms and most commonly is found in the thoracic spine. Design: Case report. Findings: A 45-year-old woman presented with posterior cervicothoracic pain for 8 months following a motor vehicle crash. Magnetic resonance imaging of the cervical spine revealed an enhancing epidural mass. Computerized tomography of the chest, abdomen, and pelvis revealed no systemic disease. Due to the lesion's unusual signal characteristics and location, an open surgical biopsy was completed, which revealed a psammomatous meningioma. Surgical decompression of the spinal cord and nerve roots was then performed. The resection was subtotal due to the extension of the tumor around the vertebral artery. Conclusion: Meningiomas should be considered in the differential diagnosis of contrast-enhancing lesions in the cervical spine. PMID:18795481

  18. Chordoid meningioma: A diagnostic dilemma

    Directory of Open Access Journals (Sweden)

    Fouzia Siraj

    2015-01-01

    Full Text Available Chordoid meningioma (CM, classified as Grade II/atypical meningioma according to the World Health Organization classification, is a rare subtype, which represents only 0.5% of all meningiomas. Morphologically, it can mimic other chondroid and myxoid tumors within the brain and its vicinity thus posing a diagnostic challenge. Accurate diagnosis, therefore, assumes importance as these tumors have an aggressive clinical course and propensity to recur compared to classical meningiomas. Furthermore, the prognosis and treatment strategies vary when compared to tumors with morphological overlap. We present a case of CM in a 14-year-old girl and discuss its clinicopathological and immunohistochemical features.

  19. Microsurgical treatment of medial sphenoid ridge meningioma

    Directory of Open Access Journals (Sweden)

    Wei-qi HE

    2011-02-01

    Full Text Available Objective To explore the microsurgical technique of medial sphenoid ridge meningioma resectional therapy.Methods The clinical data were retrospectively analyzed of 29 patients(13 males and 16 females;aged 18-68 years with average of 42 years;duration of disease was 5 months to 8 years,averaged 28 months with medial sphenoidal ridge meningioma and admitted from Jan.2005 to Jan.2010.The anatomical relationship of the tumor to surrounding structures was assessed intraoperatively,the tumor was then completely resected through cutting off the tumor supplying vessels,shrinking the tumor volume and separating the tumors from adjacent vessels and nerves.All the patients were followed up for 4 months to 4 years.Results Of the 29 cases,20 got total tumor removal,7 got subtotal and 2 got partial tumor removal.Of the 20 patients with obviously preoperative visual impairment,12 were obviously relieved,6 showed no improvement and 2 got symptoms aggravation.Hemiplegia occurred in 2 cases and oculomoter nerve palsy in 6 cases.There was no death after surgery.A 6 months to 4 years follow-up showed that no recurrence was found in 27 patients with tumor resection level of Simpson I and II,2 patients with tumor resection level of Simpson III received postoperative radiotherapy or gamma knife surgery,and 1 recurred and received reoperation.Conclusions Fine intraoperative assessment of the anatomical relationship of the tumor to surrounding structures,separating and excising tumor according to the assessed result is the key of medial sphenoid ridge meningioma resection,and the tumor resection is favorable to visual rehabilitation and tumor control.

  20. Epidural Cystic Spinal Meningioma

    Science.gov (United States)

    Zhang, Ji; Chen, Zheng-he; Wang, Zi-feng; Sun, Peng; Jin, Jie-tian; Zhang, Xiang-heng; Zhao, Yi-ying; Wang, Jian; Mou, Yong-gao; Chen, Zhong-ping

    2016-01-01

    Abstract Cystic spinal meningioma (CSM) is an uncommon meningioma variant. Extradural CSMs are particularly rare and difficult to distinguish from other intraaxial tumors. This study presents a case of a 36-year-old woman with intraspinal extradual CSM at the thoracolumbar spine. She experienced persistent weakness, progressive numbness, and sensory disturbance in the right lower limb. Magnetic resonance imaging (MRI) of the patient revealed an irregular cystic mass at the thoracic 11 to lumbar 3 levels dorsally. This case was misdiagnosed as other neoplasms prior to surgery because of the atypical radiographic features and location of the tumor. Extradural CSMs should be considered in the differential diagnosis of intraspinal extradural cystic neoplasms. Complete removal of cystic wall provides an optimal outcome, rendering the lesion curable. PMID:26986119

  1. Primary extradural meningioma arising from the calvarium

    Directory of Open Access Journals (Sweden)

    N Ravi

    2013-06-01

    Full Text Available Meningiomas are the most common intracranial tumours. Meningiomas arising at other locations are termed primary extradural meningiomas (EDM and are rare. Here we report a case of EDM arising from the calvarium – a primary calvarial meningioma (PCM.

  2. Meningioma in Down Syndrome.

    Science.gov (United States)

    Yamamoto, Takahiro; Shinojima, Naoki; Todaka, Tatemi; Nishikawa, Shigeyuki; Yano, Shigetoshi; Kuratsu, Jun-ichi

    2015-09-01

    Down syndrome comprises multiple malformations and is due to trisomy of chromosome 21. There is epidemiologic evidence that individuals with Down syndrome are at decreased risk for solid tumors including brain tumors. It has been suggested that some genes expressed on the extra copy of chromosome 21 act as tumor suppressor genes and contribute to protection against tumorigenesis. We report the first case to our knowledge of a patient with Down syndrome, an 8-year-old boy, with an intracranial meningioma, in which the status of chromosome 21 was examined. The diagnosis was based on histologic examination of the surgically resected tumor. Postoperatively, the patient's neurologic status improved, and there was no tumor regrowth in the next 2 years. Fluorescence in situ hybridization for chromosome 22 confirmed high allele loss involving the neurofibromin 2 gene locus, a finding typical in meningiomas. Fluorescence in situ hybridization also revealed chromosome 21 heterogeneity in tumor cells; not only cells with trisomy 21 but also cells with disomy and monosomy 21 were present. All blood cells from the patient manifested trisomy 21. Deletion of the chromosome 21 allele may be associated with tumorigenesis of meningioma in Down syndrome. This supports the hypothesis that some genes whose expression is increased on the extra copy of chromosome 21 function as tumor suppressor genes and that they contribute to the reduced tumor incidence in individuals with Down syndrome. Copyright © 2015 Elsevier Inc. All rights reserved.

  3. Extension and origin of parasellar meningiomas. Evaluation on MRI

    Energy Technology Data Exchange (ETDEWEB)

    Hara, Yoshie; Nakamura, Mitsugu; Asada, Masahiro; Tamaki, Norihiko [Kobe Univ. (Japan). School of Medicine

    1997-06-01

    We evaluated MRI of forty patients who had histologically confirmed meningiomas around the sella turcica. Coronal section of the spin-echo/fast spin-echo T{sub 1} weighted imaging (with and without GD-DTPA enhancement) were mainly investigated. We precisely examined two points; the location of the bulk of tumor mass, the anatomical relationship between the tumor and the anterior clinoid process, superior orbital fissure, optic nerve, and the internal carotid artery (ICA). Based on MRI findings, we recognized five different tumor origins. Meningiomas originating lateral to the anterior clinoid process never extended medially into the cavernous sinus or the sella turcica, while tumors originating medial to the anterior clinoid process did not migrate laterally beyond the process. Tumors originating from the cavernous sinus tended to invade though the dura into the superior orbital fissure and involved the anterior clinoid process. Based upon the tumor origin and their extension investigated as revealed by MRI, we speculated that the anterior clinoid process and the tough dural folds attached to it (the anterior and posterior petroclinoid ligaments, and the interclinoid ligament) played important roles as barriers against the extension of meningiomas. These dural folds may be the unique part of the dura that most resists invasion of meningiomas from extracavernous regions. Meningiomas originated from the inferomedial surface of the anterior clinoid process may have been misdiagnosed as tuberculum sellae meningiomas; however, the close anatomical relation of the tumor origin and the distal carotid suggests their potential to directly invade the internal carotid artery. (K.H.)

  4. 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.

  5. [Extradural spinal meningioma: case report].

    Science.gov (United States)

    Dagain, A; Dulou, R; Lahutte, M; Dutertre, G; Pouit, B; Delmas, J-M; Camparo, P; Pernot, P

    2009-12-01

    We report a case of purely extradural spinal meningioma and discuss the potential pitfalls in differential diagnosis. Spinal meningiomas account for 20-30% of all spinal neoplasms. Epidural meningiomas are infrequent intraspinal tumors that can be easily confused with malignant neoplasms or spinal schwannomas. A 62-year-old man with a previous history of malignant disease presented with back pain and weakness of the lower limbs. Magnetic resonance imaging revealed a well-enhanced T4 intraspinal lesion. The intraoperative histological examination showed a meningioma (confirmed by postoperative examination). Opening the dura mater confirmed the purely epidural location of the lesion. The postoperative course was uneventful with no recurrence 12 months after surgery. Purely extradural spinal meningiomas can mimic metastatic tumors or schwannomas. Intraoperative histology is mandatory for optimal surgical decision making.

  6. 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.

  7. 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.

  8. 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.

  9. 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。

  10. 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.

  11. 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.

  12. Infratentorial meningioma in an 8-year-old child as first sign of neurofibromatosis type 2.

    Science.gov (United States)

    Stettner, G M; Rostasy, K M; Ludwig, H C; Merkler, D; Fahsold, R; Gärtner, J

    2007-02-01

    Meningiomas are rare intracranial tumors in pediatric patients. In contrast to meningiomas in adults, childhood ones have a poorer prognosis because of their high growth potential and tendency to recur. Meningiomas are often associated with neurofibromatosis type 2 (NF2) which is an autosomal-dominant disorder. In contrast to adults who primarily present with symptoms due to vestibular tumors, the initial symptoms in children with NF2 are subtle skin tumors, posterior capsular cataracts, or neurological signs secondary to cranial nerve(s) schwannoma excluding vestibular nerve, and/or brainstem or spinal cord compression. Here we report on the clinical, radiological, and histological findings in an 8-year-old boy who was diagnosed with an isolated infratentorial meningioma and a novel splice site mutation in the NF2 gene. The same mutation was detected in the boy's mother who suffered from hearing loss and tinnitus due to a bilateral vestibular schwannoma. Our patient demonstrates the need for molecular testing for NF2 gene mutations even in isolated childhood meningiomas although they do not fulfill the clinical criteria of NF2.

  13. Expression of Androgen Receptor in Meningiomas

    Institute of Scientific and Technical Information of China (English)

    2001-01-01

    In order to investigate the expression of androgen receptor (AR) in meningiomas and its relation to tumor proliferative potential, we examined the expression of AR and proliferating cell nuclear antigen (PCNA) by avidine-biotin complex immunohistochemistry in 39 cases of meningiomas. Of the 39 cases of meningiomas, 20(51 %) showed positive AR immunoreactivity. The AR expression positivity rates were 31 % (6/19) in benign meningiomas, 58 % (7/12) in atypical meningiomas, 87.5 % (7/8) in malignant meningiomas, respectively. In addition to the tumor cells, cells of microvascular endothelial proliferation were frequently AR positive. Malignant meningiomas had a significantly higher percentage of AR positive cells compared with atypical and benign meningiomas (P<0.05). The mean proliferating cell nuclear antigen labeling index (PCNA LI) was significantly higher in the malignant meningiomas when compared with atypical meningiomas (P<0.05) and benign meningiomas (P<0.05). AR positive meningiomas had higher PCNA LI than AR negative meningiomas (P<0.05). The expression of AR in tumor tissues was significantly related with PCNA LI. These data indicated that AR in the meningiomas was correlated with histological grade and AR might participate in the growth of these tumors and tumor angiogenesis. The measurement of AR in these tumors may indirectly represent tumor growth potential.

  14. Metastasis to a spinal meningioma.

    Science.gov (United States)

    Bansil, Rohit; Walia, Bipin S; Khan, Zahid; Abrari, Andleeb

    2017-01-01

    Metastasis of one cancer to another is rare. Here, we report a spinal meningioma that was infiltrated by metastatic deposits from another cancer. A 62-year-old male presented with a progressive spastic paraparesis. Magnetic resonance (MR) imaging of the spine suggested a well-defined intradural extramedullary (IDEM) T8 mass in the dorsal spinal canal. When excised, it proved histologically to be a meningothelial meningioma infiltrated by metastatic deposits from an adenocarcinoma. Tumor to tumor metastasis rarely occurs, and meningioma, owing to its biological character and increased vascularity, is one of the most common recipients of a metastases from other lesions.

  15. Anterior Interhemispheric Approach for Olfactory Groove Meningioma

    Directory of Open Access Journals (Sweden)

    Imam Hidayat

    2016-09-01

    Full Text Available Objective: To evaluate the surgical technique with bifrontal interhemispheric approach for total removal of tumor in olfactory groove meningioma (OGM. Methods: This study described a case of a 38-year-old woman with bilateral blindness, anosmia, and behaviour changes. Imaging studies show a tumor mass in midfrontal base. Surgery using a bifrontal interhemispheric approach was performed and total removal was achieved and postoperative computed tomography (CT scan was performed to confirm the result. Histopathological findings established a diagnosis of meningioma. Results: A coronal skin incision behind the hairline was utilized. The scalp was elevated, taking care to reserve the vascularized pericranium medial to the linea temporalis of each side, and preserving the 2 supraorbital nerves. Eight burr holes were used, with the two initial holes made on each side of the orbitotemporal region, and the other four holes at the midline. A bifrontal craniotomy was performed. The tumor was first detached from its attachment with bipolar cautery and debulked. During this step, the main tumor feeder arteries from the anterior and posterior ethmoidal artery were interrupted, and the tumor devascularized. Total tumor removal through surgical intervention was achieved and confirmed by head CT-scan postoperatively. Conclusions: This case report supports the suitability of the bifrontal interhemispheric approach for OGM resection with additional radiation therapy.

  16. Clinicopathological Analysis of 24 Cases with Malignant Peripheral Nerve Sheath Tumors%恶性周围神经鞘膜瘤24例临床病理分析

    Institute of Scientific and Technical Information of China (English)

    潘登

    2001-01-01

    [ PurpoSE] To investigate the light microscopic manifestation in malignant peripheral nerve sheath tumors (MPNSTs) and provide a way of thinking for differential diagnosis. [Methods] Twenty-four cases of MPNSTs were studied by routine stain and proved with immunohistochemistry and its morphological characters were analysed by light microscopy. [Results]According to morphological characters, MPNSTs were classified as follows:general type: 17 cases (71% ), special type: 7cases (29%). S-100 is the most sensitve marker of MPNST and myelin basic protein (MBP) is the most reliable marker. [ Conclusions ] Morphological characters of MPNSTs are various. They are easy to misdiagnosis as other tumors. Immunohistochemistrv is playing an important role in the accurate diagnosis.%[目的]探讨恶性周围神经鞘膜瘤镜下形态特点,确立鉴别诊断思路。[方法]24例恶性周围神经鞘膜瘤经常规检查及免疫标记证实,观察其HE切片镜下形态。[结果]恶性周围神经鞘膜瘤根据形态可分为普通型17例(71%)和特殊型7例(29%)。。S-100为该瘤最敏感的标记物,髓磷脂碱性蛋白为最可靠的标记物。[结论]恶性周围神经鞘膜瘤组织形态多样,变异甚大.常易错诊为其它肿瘤,免疫标记对确立正确的诊断思路有较大帮助。

  17. 11例口腔颌面部恶性外周神经鞘肿瘤临床分析%Malignant peripheral nerve sheath tumors in oral maxillofacial region:clinical analysis of 11 cases

    Institute of Scientific and Technical Information of China (English)

    郑磊; 张建国; 俞光岩; 高岩

    2010-01-01

    目的:初步探讨口腔颌面部恶性外周神经鞘肿瘤(malignant peripheral nerve sheath tumor,MPNST)的临床特点、治疗和预后.方法:对1987年11月至2008年11月诊治的11例MPNST患者的临床特点、病理资料、治疗方法和疗效进行总结和分析.结果:10例患者局部渐进性增大的肿物为其主要症状,5例患者受累神经出现支配区域麻木或疼痛.11例患者经组织病理证实,免疫组织化学染色,SMA(5/11)、S-100(7/11)、p53(5/11)、Ki-67(11/11)和vimentin(11/11)呈阳性表达.2年和5年生存率分别为30%和9.5%,平均生存时间为33.9个月.结论:口腔颌面部恶性外周神经鞘肿瘤是一种高度恶性肿瘤,预后差,易出现局部复发和远处转移,彻底的手术切除是口腔颌面部MPNST主要的治疗方法和重要的预后因素.

  18. 表现为肛周红色肿物的肛门原发性恶性外周神经鞘瘤%Malignant peripheral nerve sheath tumor manifests as a red perianal tumor

    Institute of Scientific and Technical Information of China (English)

    曾跃平; 钟定荣; 渠涛; 孙秋宁

    2015-01-01

    报告1例肛门原发性恶性外周神经鞘瘤(malignant peripheral nerve sheath tumor,MPNST).患者男,38岁.肛周红色肿物伴疼痛3个月就诊.皮肤科检查:肛周偏左侧一7 cm×1.5 cm×1 cm红色肿物,表面有糜烂、结痂.皮损组织病理检查:鳞状上皮黏膜及柱状上皮下方可见梭形细胞肿瘤,肿瘤广泛累及黏膜固有层和肠壁深肌层.瘤细胞呈旋涡状或束带状生长,细胞核质比例增大,异形性明显,病理性核分裂象易见.免疫组化:肿瘤细胞弥漫强阳性表达波形蛋白(vimentin)和S-100蛋白,Ki-67 30%阳性,不表达上皮膜抗原(EMA)、B-细胞淋巴瘤基因2蛋白(Bcl-2)、CD34、CD117、Dog-1、黑素-A (melan-A)、HMB-45、平滑肌肌动蛋白(SMA)和结蛋白(desmin).诊断:肛门原发性MPNST.

  19. {sup 18}F-FDG PET/CT for detection of malignant peripheral nerve sheath tumours in neurofibromatosis type 1: tumour-to-liver ratio is superior to an SUV{sub max} cut-off

    Energy Technology Data Exchange (ETDEWEB)

    Salamon, Johannes [University Medical Centre Hamburg-Eppendorf, Department of Diagnostic and Interventional Radiology, Hamburg (Germany); University Hospital Hamburg-Eppendorf, Department of Diagnostic and Interventional Radiology, Hamburg (Germany); Veldhoen, Simon [University Medical Centre Hamburg-Eppendorf, Department of Diagnostic and Interventional Radiology, Hamburg (Germany); University Medical Centre Wuerzburg, Department of Diagnostic and Interventional Radiology, Wuerzburg (Germany); Apostolova, Ivayla [Otto-von-Guericke University, Department of Radiology and Nuclear Medicine, Magdeburg (Germany); Bannas, Peter; Yamamura, Jin; Herrmann, Jochen; Adam, Gerhard; Derlin, Thorsten [University Medical Centre Hamburg-Eppendorf, Department of Diagnostic and Interventional Radiology, Hamburg (Germany); Friedrich, Reinhard E. [University Medical Centre Hamburg-Eppendorf, Department of Oral and Maxillofacial Surgery, Hamburg (Germany); Mautner, Victor F. [University Medical Centre Hamburg-Eppendorf, Department of Neurology, Hamburg (Germany)

    2014-02-15

    To evaluate the usefulness of normalising intra-tumour tracer accumulation on {sup 18}F-fluorodeoxyglucose (FDG) positron emission tomography/computed tomography (PET/CT) to reference tissue uptake for characterisation of peripheral nerve sheath tumours (PNSTs) in neurofibromatosis type 1 (NF1) compared with the established maximum standardised uptake value (SUVmax) cut-off of >3.5. Forty-nine patients underwent FDG PET/CT. Intra-tumour tracer uptake (SUVmax) was normalised to three different reference tissues (tumour-to-liver, tumour-to-muscle and tumour-to-fat ratios). Receiver operating characteristic (ROC) analyses were used out to assess the diagnostic performance. Histopathology and follow-up served as the reference standard. Intra-tumour tracer uptake correlated significantly with liver uptake (r{sub s} = 0.58, P = 0.016). On ROC analysis, the optimum threshold for tumour-to-liver ratio was >2.6 (AUC = 0.9735). Both the SUVmax cut-off value of >3.5 and a tumour-to-liver ratio >2.6 provided a sensitivity of 100 %, but specificity was significantly higher for the latter (90.3 % vs 79.8 %; P = 0.013). In patients with NF1, quantitative {sup 18}F-FDG PET imaging may identify malignant change in neurofibromas with high accuracy. Specificity could be significantly increased by using the tumour-to-liver ratio. The authors recommend further evaluation of a tumour-to-liver ratio cut-off value of >2.6 for diagnostic intervention planning. (orig.)

  20. Clinicopathologic features of dermal nerve sheath myxoma and neurothekeoma:a comparative study%真皮神经鞘黏液瘤和 Neurothekeoma 的临床病理学对比性研究

    Institute of Scientific and Technical Information of China (English)

    丁华; 汪亮亮; 许晓琳; 刘绮颖; 喻林; 王坚

    2016-01-01

    Objective To investigate the clinicopathologic characteristics and differential diagnosis of dermal nerve sheath myxoma ( DNSM) and neurothekeoma ( NTK).Methods Clinical, pathological features and immunohistochemical profiles in 9 cases of DNSM and 8 cases of NTK were comparatively studied.The literature was reviewed.Results The involved site of 9 DNSMs included the hand/fingers (n=3), ear (n=2), face, back, abdominal wall and waist (1 case each).Two of 8 NTKs arose in the forearm ( n=2 ) , one each in the nose, lip, shoulder, supraclavicular region, leg and hand.The most common presentation was a painless cutaneous nodule or plaque which grew slowly.Grossly, DNSM was often gelatinous, whereas NTK appeared relatively solid.Microscopically, both tumors were located in the dermis and/or subcutis.DNSM was composed of well-defined multiple lobules separated by fibrous septa.NTK also exhibited lobular or multinodular architecture, but was relatively ill-defined.Besides, fascicular or whorl-like arrangement was present in 3 cases of NTK.The lobules in DNSM consisted of a paucicellular proliferation of spindled, stellate and epithelioid cells forming interconnecting cords within abundant myxoid matrix.Small syncytial-like aggregates were readily noted.The constituted neoplastic cells in NTK were composed of ovoid to round epithelioid or histiocytoid cells.Scattered multinucleated giant cells were present in 2 cases.Based on the amount of myxoid matrix, 8 NTKs were further classified into cellular (5 cases), mixed (2 cases) and myxoid (1 case).By immunohistochemistry, neoplastic cells in DNSM were diffusely positive for S-100 protein, CD68, glial fibrillary acidic protein and SOX10, whereas neoplastic cells in NTK consistently expressed CD10 and microphthalmia transcription factor, with negativity for S-100 protein and SOX10.One patient each with DNSM and NTK experienced local recurrence due to incomplete excision. Conclusions Although DNSM and NTK share clinical and

  1. Meningeal tumors histologically mimicking meningioma.

    Science.gov (United States)

    Barresi, Valeria; Caffo, Maria; Branca, Giovanni; Caltabiano, Rosario; Tuccari, Giovanni

    2012-10-15

    A number of meningeal neoplastic lesions may radiologically and clinically simulate meningioma. In the present paper, we review meningeal non-meningothelial tumors which may also mimic different histotypes of meningioma at the histological examination. Awareness that these lesions exist may facilitate their recognition and correct diagnosis, which is of fundamental importance for prognosis and an appropriate therapeutic approach. Histological and immunohistochemical clues for the differential diagnosis are discussed.

  2. Dumbbell Shaped Transforaminal Paravertebral Meningioma

    Directory of Open Access Journals (Sweden)

    ismail serifoglu

    2015-03-01

    Full Text Available Dumbbell tumors are tumors of two or more regions of the spinal column. The majority of the dumbbell tumors are schwannomas. The presentation of spinal meningiomas as a dumbbell tumors are very rare. The diagnosis of Dumbbell-shaped meningiomas with imaging methods is important for preoperative accurate treatment planning and to prevent its postsurgical recurrences. [Cukurova Med J 2015; 40(Suppl 1: 38-41

  3. En plaque meningioma with angioinvasion

    Directory of Open Access Journals (Sweden)

    Basu Keya

    2010-04-01

    Full Text Available En plaque meningioma is a rare type of meningioma characterized by infiltrative nature, sheet-like growth and at times invading the bone. We report here a case of en plaque meningioma with typical grade I histomorphology along with unusual feature of angioinvasion. The patient was a 55-year-old man presenting with headache and painful proptosis of right eye. Imaging modalities revealed an en -plaque meningioma extending into the right sylvian fissure, with thickening of right temporal calvarium, greater wing of sphenoid and extension into the orbit. Magnetic resonance angiography showed medial displacement of right middle cerebral artery. The tumor was removed from the sylvian fissure and right temporal convexity. However, only subtotal removal of the intraorbital part was possible. Histology showed a meningothelial meningioma with low tumor cell proliferation, but infiltration into the bone, skeletal muscle and angioinvasion. Recognition of meningiomas en plaque is useful, as these tumors are difficult to resect completely, and are more prone to undergo recurrence or malignant change. In addition, angioinvasion seen in this tumor may have additional prognostic significance.

  4. Uncommon progression of an extradural spinal meningioma

    National Research Council Canada - National Science Library

    Ben Nsir, Atef; Boughamoura, Mohamed; Mahmoudi, Houda; Kilani, Mohamed; Hattab, Nejib

    2014-01-01

    Extradural spinal meningiomas are rare. Our understanding of purely extradural spinal meningiomas is still incomplete and they may be easily confused with malignant neoplasms, much more common in this location...

  5. Intraparenchymal Angiomatous Meningioma: A Diagnostic Dilemma.

    Science.gov (United States)

    Bansal, Divya; Diwaker, Preeti; Gogoi, Priyanka; Nazir, Wazid; Tandon, Anupama

    2015-10-01

    Meningioma arises from the arachnoid cap cells of the cerebrum. Intraparenchymal meningiomas or meningiomas without dural attachment are rare. We report a case of 40-year-old male who presented with a history of headache, dizziness and gradual loss of vision since one year. Clinicoradiological diagnosis of a high grade glioma was considered. Tumour was excised and haematoxylin and eosin stained sections revealed a tumour comprised predominantly of variable sized blood vessels showing hyalinization in a background of plump spindle cells with oval vesicular nuclei. In view of these features angiomatous meningioma was suspected. However, to confirm the diagnosis, a panel of immunohistochemical markers including vimentin, EMA and GFAP was done and a final diagnosis of angiomatous meningioma was offered. Angiomatous meningioma is a rare variant of meningioma and even much rarer in the intraparenchymal location. Angiomatous meningioma should be considered in the differential diagnosis of highly vascular intraparenchymal brain tumours.

  6. Tic douloureux in patients with incidental intracranial meningioma; MR findings and review of literature

    Directory of Open Access Journals (Sweden)

    A. Elsotouhy

    2015-06-01

    We discuss two middle aged ladies who presented with trigeminal neuralgia, MRI in both of them revealed the presence of intracranial extra-axial mass lesions with the characteristic features of meningioma causing trigeminal nerve compression and explaining the cause of neuralgia.

  7. Chordoid meningioma: a report of two cases

    Institute of Scientific and Technical Information of China (English)

    LIU Ai-jun; WANG Fu-lin; LI Xiang-hong

    2007-01-01

    @@ Although meningiomas are common tumors in adults,the chordoid variant is very rare. According to the World Health Organization Classification of Tumors of the Nervous System (Lyon, 2000),1 chordoid meningioma together with another rare variant, lymphoplasmacyterich meningioma, usually contain abundant lymphoplasmacytic infiltration, and are associated even with hematological abnormalities, especially in young adults.

  8. Hemifacial Spasm Associated with Contralateral Foramen Magnum Meningioma.

    Science.gov (United States)

    Matsuda, Masahide; Akutsu, Hiroyoshi; Yamamoto, Tetsuya; Ishikawa, Eiichi; Matsumura, Akira

    2016-05-01

    Hemifacial spasm (HFS) caused by a contralateral tumor is extremely rare, and all previously reported cases involved tumors at the cerebellopontine angle. We provide the first report to our knowledge of HFS caused by a contralateral foramen magnum meningioma, which improved after tumor removal without microvascular decompression. An 80-year-old woman presented with HFS caused by a contralateral foramen magnum meningioma. She had a 3-year history of gradual worsening of right-sided HFS. Magnetic resonance imaging showed a homogeneous gadolinium-enhancing tumor occupying the left ventrolateral portion of the foramen magnum. The loop of the right anterior inferior cerebellar artery extended into the right pontomedullary junction, compressing the root exit zone (REZ) of the right facial nerve. After tumor removal, with no attempt of confirmation of vascular compression of the right facial nerve REZ, facial spasm gradually decreased and eventually almost disappeared. Postoperative magnetic resonance imaging revealed that the loop of anterior inferior cerebellar artery had shifted away from the right facial nerve REZ. Dorsal shift of the anterior inferior cerebellar artery loop toward the facial nerve REZ along with dorsal shift of the vertebral and basilar arteries secondary to the huge contralateral mass located in the ventrolateral portion of the foramen magnum was suggested to represent the main cause of facial spasm. Although an extremely rare condition, facial spasm can develop as a result of contralateral tumors in locations other than the cerebellopontine angle. Copyright © 2016 Elsevier Inc. All rights reserved.

  9. Edema and malignancy in meningiomas Edema e malignidade em meningiomas

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    Tobias Alécio Mattei

    2005-06-01

    Full Text Available PURPOSE: In recent years there have been many attempts to define a subset of aggressive malignant meningiomas based on histopathology and imaging technologies. The purpose of this study was to evaluate the level of peritumoral edema and its volume using the imaging technologies, computer tomography and magnetic resonance imaging, and correlate these results with the histological WHO classification. Reported causes of tumoral edema and its relationships to the histological characteristics were also reviewed. METHODS: The cases of 55 patients with meningiomas who underwent surgery at the Hospital das Clinicas (Fac Med Univ Sao Paulo between September 1993 and September 1997 were reviewed. The level of edema according to the classification of Ide et al. (1995 was compared to the histological WHO classification. RESULTS: Classification of the degree of edema was: level 0 edema - 28 cases ; level I edema - 19 cases; level II edema - 8 cases. Histological classification was: benign meningioma - 43 cases; atypical meningiomas - 11 cases; malignant meningioma - 1 case. There was a significant (P = .0089 correlation between the degree of tumoral edema and the histological characteristics. CONCLUSIONS: These results suggest that the degree of edema as revealed by computer tomography and magnetic resonance imaging can be an important clinical predictive factor for the histological grade of the meningioma.OBJETIVO: Nos últimos anos têm-se descrito alguns subtipos de meningiomas de comportamento peculiarmente agressivo. Muitas tentativas têm sido feitas no intuito de estabelecer critérios imagenológicos ou histopatológicos de malignidade. O objetivo desse estudo é avaliar, através de Tomografia Computadorizada e Ressonância Nuclear Magnética o grau de edema peritumoral e seu volume, correlacionando-os com a classificação histológica da OMS. As causas relatadas de edema peritumoral e sua possível correlação histológica foram também revistos

  10. Double spinal dural sheath: a cadaveric case report

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    Loughenbury P

    2010-03-01

    Full Text Available A previously unreported variation in the anatomy of the spinal dural sheath was observed during routine cadaveric dissection, consisting of a duplication of the dural layer, with layers adherent throughout their length. The double dural sheath completely enveloped the spinal cord and nerve roots, and extended from C2 to L5: both layers were of similar thickness to a single dural sheath. Duplication of the dura mater in the form of two complete dural sheaths has not been previously observed and/or reported in a cadaveric study. However, areas of localised duplication of the ventral aspect of the dural sheath have been observed during intra-operative dissection, particularly in association with idiopathic herniation of the spinal cord. Complete duplication of the spinal dura mater is of clinical interest in spinal surgery, particularly in relation to idiopathic spinal cord herniation.

  11. Preoperative cellulose porous beads for therapeutic embolization of meningioma: provocation test and technical considerations

    Energy Technology Data Exchange (ETDEWEB)

    Kai, Yutaka; Morioka, Motohiro; Yano, Shigetoshi; Nakamura, Hideo; Makino, Keishi; Mizuno, Takamasa; Takeshima, Hideo; Kuratsu, Jun-ichi [Kumamoto University, Department of Neurosurgery, Graduate School of Medical Sciences, Kumamoto (Japan); Hamada, Jun-ichiro [Kanazawa University, Department of Neurosurgery, Graduate School of Medical Sciences, Kanazawa (Japan)

    2007-05-15

    Cellulose porous beads (CPBs) are exceptionally uniform in size and nonabsorbable and they provide highly effective tumor devascularization. The risk of cranial nerve palsy must not be overlooked when embolization with CPBs is considered in meningioma patients. We attempted to identify patients at risk of cranial nerve palsy after meningioma embolization. Prior to preoperative superselective embolization with 200 {mu}m diameter CPBs, 141 patients with meningioma underwent provocation test with lidocaine and amytal. They were divided into two groups on the basis of whether they were or were not considered eligible for embolization. We evaluated the differences between the two groups with respect to tumor anatomy, angiographic findings, and clinical presentation and recorded complications associated with the embolization of the meningioma. Of the 141 patients, 128 underwent CPB embolization (group 2); 13 were not embolized because their provocation test results were positive (group 1, n = 11) or because they showed vasospasm (n = 2). Group 1 patients had meningioma in the cavernous sinus or petroclival region. Characteristically, the feeders were of middle meningeal artery origin and exhibited a posteromedial course toward the petrous apex or cavernous sinus. In group 2 patients the middle meningeal artery was the feeder, but it lacked branches coursing posteromedially. Three of these patients experienced complications which included intratumoral hemorrhage (n = 2) and post-embolization hearing disturbance (n = 1). Patients with meningioma whose tumor-feeding arteries run posteromedially toward the petrous apex or cavernous sinus are at increased risk of post-embolization cranial nerve palsy. Appropriate protocols, including lidocaine and amytal provocation tests, may reduce the risk of complications after CPB embolization of the external carotid territory in this group of patients. (orig.)

  12. Atypical Imaging Appearances of Meningioma

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    Ahmad Soltani shirazi

    2009-01-01

    Full Text Available "nIntroduction: Meningiomas are the commonest primary non-glial intracranial tumors. The diagnosis is usually correctly established on characteristic imaging appearances. Atypical meningiomas may be difficult to diagnose because of their similarity to other brain tumors. This paper presents one case of atypical meningioma, misdiagnosed primarily as glioblastoma multiforms (GBM by radiological techniques. "nCase report: A 15-year-old girl presented with a severe intermittent generalized headache that on occasion localized to retro-orbital and vertex. Other manifestations were blurred vision, photophobia, diplopia, weakness and clumsiness of the right hand. The result of systemic and neurological examinations was normal, except for a positive right hand drift test. MRI showed a large lobulated mass with peripheral edema, central necrosis and a heterogenous enhancement at the central part of the parietal lobe inducing to subfalcian herniation. Glioblastoma multiforms (GBM was misdiagnosed for the patient on the basis of MRI appearance. Pathology evaluation was compatible with meningioma (WHO grade I to II. The patient was operated and discharged with minimal right hand weakness. Physiotherapy was recommended to improve the remaining problems. "nConclusion: Atypical meningiomas may mimic other intracranical brain lesions and may cause misdiagnosis. It is important to be aware of these features in order to avoid misdiagnosis. "n"n  

  13. 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

  14. The Significance of PTEN Expression and DNA Ploidy Analysis in Malignant Peripheral Nerve Sheath Tumors%恶性外周神经鞘瘤中PTEN表达及DNA倍体分析的意义

    Institute of Scientific and Technical Information of China (English)

    郝风云; 杨凤霞; 张彩新; 张景芳; 纪祥瑞

    2008-01-01

    目的:通过检测第10号染色体上磷酸酶和张力蛋白同源缺失基因(PTEN)在恶性外周神经鞘瘤(malignant peripheral nerve sheath tumors,MPNST)中的表达,佐以流式细胞术对MPNST进行DNA倍体分析,研究两者与其临床生物学行为之问的关系.方法:对65例良、恶性外周神经肿瘤石蜡包埋组织应用免疫组化进行PTEN的检测,兼以流式细胞术行DNA倍体分析.结果:PTEN在良、恶性外周神经肿瘤中阳性表达有显著差异(x2=17.628,P<0.001);PTEN阳性表达强度与MPNST的分化程度无相关性(rs=0.067,P:0.699).MPNST发生异倍体4例,均为低异倍体(DI值<0.90);SPF、PI值在良、恶性外周神经肿瘤中有显著差剔(x2=28.674,P<0.001;x2=23.730,P<0.001),且与MPNST病理组织学分级呈正相关(rs=0.418,P<0.01;rs=0.347,P<0.05);PTEN的阳性表达以及阳性表达强度与DNA倍体分析3项指标均无关联.结论:PTEN可以作为鉴别良、恶性外周神经肿瘤以及判断外周神经鞘瘤恶性程度高低的一个有价值的指标;异倍体的出现以及SPF、PI值的增高是判断MPNST恶性程度(演进与异质化)的重要标志.

  15. 原发椎管内恶性周围神经鞘瘤6例并文献复习%Primary spinal malignant peripheral nerve sheath tumor: report of six cases and review of literature

    Institute of Scientific and Technical Information of China (English)

    马骏; 王贵怀

    2013-01-01

    目的 探讨原发椎管内恶性周围神经鞘瘤(MPNST)的临床特点和治疗.方法 收集我院神经外科近10年来6例原发椎管内恶性周围神经鞘瘤手术患者资料,结合文献复习进行临床分析.结果 椎管内MPNST可累及脊髓任何节段,病变多发或呈丛状生长.6例患者行肿瘤全切除或近全切除术,术中肿瘤表现各异,术后病理诊断为恶性周围神经鞘瘤,部分患者术后进一步行辅助放疗.远期临床症状缓解明显,但肿瘤易复发.结论 原发于椎管内的恶性周围神经鞘瘤较为罕见,表现出恶性生物学行为,术前诊断困难,手术完全切除+术后辅助放疗治疗可能延缓疾病进展,但手术完全切除难度大,术后缺乏有效的辅助治疗方案,远期复发率高.%Objective To investigate the clinical characteristic and treatment of primary spinal malignant peripheral nerve sheath tumor ( MPNST). Methods The clinical data of 6 patients with primary spinal MPNST of our neurosurgery department in recent 10 years were analyzed with review of literature. Results The total or subtotal tumor resection were performed for each patient. The characteristics of the tumor vary from person to person. The diagnosis of MPNST was confirmed by postoperative pathology examination. Some went on for adjuvant radiotherapy. The symptoms relieved well after long-term of follow-up. But the recurrence rate of the tumor was relatively high. Conclusions Primary spinal MPNST has a low incidence. Preoperative diagnosis proves to be a challenge. Total tumor resection with adjuvant radiotherapy may be recommended. But given the malignant characteristic of the tumor total resection is difficult to achieve, and due to the lack of experience of adjuvant therapy the overall prognosis is poor.

  16. Clinical and MRI Features of Malignant Peripheral Nerve Sheath Tumor in Soft Tissue%软组织恶性外周神经鞘瘤的临床及MRI表现

    Institute of Scientific and Technical Information of China (English)

    朱丽娜; 刘艳; 明洁; 王慧丽

    2013-01-01

    Objective To analyze clinical and MRI features of malignant peripheral nerve sheath tumors in soft tissue, and to improve its preoperative diagnostic accuracy. Methods Clinical and MRI features of 14 patients with histologically diagnosed soft tissue MPNST were analyzed retrospectively. Results Clinical features of all cases were localized mass with tenderness, one cases was accompanied with neurofibromatosis I. Most of them were solitary mass located in muscular layer, circular(9/14) or irregular shape(5/14), irregular edge( 10/14), incomplete capsule(8/14) , the average maximum diameter was 10.3 cm. MR features were mainly isointense relative to muscle on T, weighted images and T2 weighted FS images, irregular necrosis zone in intratumoral and perilesion edema like zone( 10/14) were seen with inhomogeneous enhancement (10/14). Conclusion Clinical and MRI manifestations of soft tissue MPNST have certain characteristics, which have certain significance to improve the preoperative diagnostic accuracy.%目的 分析软组织恶性外周神经鞘瘤(MPNST)患者的临床及MRI表现,提高术前对其诊断的准确性.方法 回顾性分析14例经病理证实的软组织MPNST患者的临床及MRI表现. 结果 14例MPNST患者临床均表现为伴压痛或触痛的局部肿块,其中1例伴有神经纤维瘤病I型.MRI多表现为位于肌肉层(6/14)的单发(11/14)肿块,呈类圆形(9/14)或不规则形(5/14),边缘多欠光整(10/14),有不完整包膜(8/14),最大径平均10.3 cm.T1 WI、T2 WI脂肪抑制边缘以等信号为主,内部可见不规则坏死区,周围软组织可见水肿改变(10/14),增强扫描多呈明显不均匀强化(10/14).结论 软组织MPNST的临床与MRI表现有一定特征,对提高其术前诊断准确性有一定的意义.

  17. Dural tail sign in spinal meningiomas

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    Alorainy, Ibrahim A. [Department of Radiology and Diagnostic Imaging, College of Medicine and King Khalid Univerity Hospital, King Saud University, P.O. Box 9047, Riyadh 11413 (Saudi Arabia)]. E-mail: alorainy@ksu.edu.sa

    2006-12-15

    Objective: To study the association between the 'dural tail sign' and spinal meningiomas on MR imaging. Methods: Retrospective review of MR examinations of all pathologically proven spinal meningiomas from 1998 to 2005 was performed. Lesions were evaluated for size, signal intensity, enhancement pattern, and presence or absence of dural tail. The dural tail length and direction in reference to the meningioma were also evaluated. Results: Seven spinal meningiomas were identified in seven patients. One lesion was purely extradural, while the remaining were intradural extramedullary. Dural tail was present in four cases (57%) and its length ranged between 5 and 21 mm. The tail was seen cranial and caudal to the meningioma in three cases and only cranially in one. Coronal images were available in three cases and in two of these; the dural tail was clearly depicted. Conclusions: 'Dural tail sign' is as common in spinal meningiomas as in cranial meningiomas.

  18. Totally ossified metaplastic spinal meningioma.

    Science.gov (United States)

    Ju, Chang Il; Hida, Kazutoshi; Yamauchi, Tomohiro; Houkin, Kiyohiro

    2013-09-01

    A 61-year-old woman with a very rare case of totally ossified large thoracic spinal metaplastic meningioma, showing progressing myelopathy is presented. Computed tomographic images showed a large totally ossfied intradural round mass occupying the spinal canal on T9-10 level. Magnetic resonance imaging revealed a large T9-10 intradural extramedullary mass that was hypointense to spinal cord on T1- and T2-weighted sequences, partial enhancement was apparent after Gadolinium administration. The spinal cord was severely compressed and displaced toward the right at the level of T9-10. Surgical removal of the tumor was successfully accomplished via the posterior midline approach and the histological diagnosis verified an ossified metaplastic meningioma. The clinical neurological symptoms of patient were improved postoperatively. In this article we discuss the surgical and pathological aspects of rare case of spinal totally ossified metaplastic meningioma.

  19. Clinical analysis of 26 central nervous system malignant peripheral nerve sheath tumors%26例中枢神经系统恶性周围神经鞘瘤的临床分析

    Institute of Scientific and Technical Information of China (English)

    胡孟庆; 赵锦程; 任晓辉; 赵萌; 王军梅; 姜中利

    2014-01-01

    目的 探讨中枢神经系统恶性周围神经鞘瘤(MPNST)的诊断、治疗及预后.方法 回顾性总结2004年至2011年收治的26例经病理证实的颅内和椎管内MPNST,应用Log-rank法和Cox回归进行统计分析,并结合文献对本病的特点、治疗方法和预后进行分析.结果 26例中男16例,女10例.肿瘤位于颅内17例,椎管内9例.临床表现主要有疼痛、神经受累症状等.全切除14例,部分切除12例.共随访到21例,术后随访1.5 ~ 102.0个月,其中5例因肿瘤复发而死亡,7例肿瘤复发,9例无明显复发.单因素分析显示:女性、肿瘤全切、原发病变与较好预后相关(P<0.05).多因素分析显示:全切除为预后好的独立因素(P =0.006).结论 中枢神经系统MPNST临床罕见,全切除是改善预后的重要的手段,对于未全切除的病例,术后放疗值得推荐.%Objective To explore the diagnosis,comprehensive treatment strategies and prognosis for intracranial and intraspinal malignant peripheral nerve sheath tumors (MPNST).Methods The investigators summarized 26 cases with MPNST which were performed by post-operation pathology from 2004 to 2011.By Log-rank and Cox model analysis,we analyzed the clinical features,radiological manifestations,pathological features,comprehensive treatment strategies and prognosis,and relevant literatures.Results Among the 26 cases,16 cases were male,10 cases were female,17 tumors were in cranial,9 were located in intraspinal.symptoms mainly included pain and impaired dysfunction of nerves.The follow-up data of 21 patients were collected from 1.5 to 102.0 months after surgery.Among these patients,5 patients had died due to tumor recurrence,7 patients were alive with tumor recurrence and 9 patients had no recurrence.Univariate analysis revealed that female gender,gross total resection,and primary MPNST were significantly associated with favorable prognosis(P <0.05).Multivariate analysis revealed only gross total resection to be

  20. Malignant spinal meningioma in a CD-1 mouse.

    Science.gov (United States)

    Balme, Emmanuelle; Roth, Daniel Robert; Perentes, Elias

    2008-08-01

    Spontaneous meningiomas are extremely rare tumors in small laboratory animals, except in some strains of rats and in the B6C3F1 mouse. We report the case of a male CD-1 mouse in poor health condition, with severe apathy and partial paresis of hindlegs. No macroscopic lesion was noticed at necropsy. Microscopic examination revealed the presence of a malignant meningioma (approximately 3mm in diameter) at the distal lumbar level of the spinal cord, invading the vertebral canal, and bilaterally the ventral and dorsal nerve roots and the dorsal root ganglia. The tumor displayed highly cellular sheets of polygonal cells with a well-delineated, abundant, eosinophilic cytoplasm and scarce fibrovascular stroma; epithelioid and sarcomatous areas were also present. The mitotic activity was moderate. Tumor cells expressed vimentin immunoreactivity and were negative for periodic acid-Schiff (PAS), silver impregnation for reticulin, chromogranin A, glial fibrillary acidic protein (GFAP), cytokeratin (CK) and S-100 protein. The diagnosis of malignant spinal meningioma was based on the morphologic features of the neoplasm, the evidence of local invasion and the immunohistochemical results.

  1. Canine malignant peripheral nerve sheath tumor involving nerve roots of the third lumbar spinal cord segmentTumor maligno da bainha de nervo periférico envolvendo raízes nervosas do terceiro segmento medular lombar em um cão

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    Elisângela Olegário da Silva

    2012-12-01

    Full Text Available Malignant peripheral nerve sheath tumors (MPNST involving spinal nerve roots are uncommon in dogs. A nine-year old, intact, mixed-breed dog, demonstrated clinical signs of incoordination in the pelvic limbs and micturition for approximately one week. Clinical examination revealed proprioceptive deficits and bilateral patellar hyperreflexia. During exploratory celiotomy a mass was observed adhered to the lumbar vertebral segments. Medical therapy was initiated, but neurological signs were progressive, and the owner opted for euthanasia. Gross examination showed that the mass in the abdominal cavity was attached to the lumbar segments L3 and L4, causing bone lysis in L3, but showed no tumor invasion into the spinal canal. Microscopic features were characterized by prominent proliferation of ovoid and fusiform cells with poorly defined cytoplasm arranged in interlacing bundles and concentric whorls. The cells were embedded in a delicate to moderate collagenous stroma and moderate anisokariose and high mitotic activity were noted. The immunohistochemical assay showed positive staining for GFAP, S-100 protein and vimentin, and negative staining for factor VIII, ?-actin and citokeratine. The definitive diagnosis of malignant peripheral nerve sheath tumor was made on the basis of the histological and immunohistochemical findings. Tumores malignos da bainha de nervo periférico (TMBNP em raízes nervosas espinhais são incomuns em cães. Relata-se o caso de um cão, sem raça definida, nove anos de idade, não castrado, com histórico de incoordenação em membros pélvicos e retenção urinária há aproximadamente uma semana. Ao exame clínico constatou-se déficit proprioceptivo e hiperreflexia patelar bilaterais. Durante a celiotomia exploratória constatou-se uma massa intensamente vascularizada e aderida aos segmentos vertebrais lombares. Estabeleceu-se plano terapêutico e o animal foi tratado com fluidoterapia, anti-inflamatório e analg

  2. [Phase contrast MRI-based evaluation of cerebrospinal fluid circulation parameters in patients with foramen magnum meningiomas].

    Science.gov (United States)

    Kondrakhov, S V; Zakharova, N E; Fadeeva, L M; Tanyashin, S V

    Meningiomas of the foramen magnum (FMR) region account for 1.8 to 3.2% of all meningiomas. The international literature has insufficient data describing the state of cerebrospinal fluid (CSF) circulation in these patients. We studied 38 patients with FM meningiomas, aged from 35 to 79 years (mean age, 56.7 years). The mean meningioma size was 30 mm (10-60 mm). Meningiomas had the anterolateral localization in 29 patients, ventral localization in 5 patients, and dorsal localization in 4 patients. Twenty nine patients underwent surgery. All operated patients were examined before and after surgery. The CSF circulation was studied using phase contrast MRI (PC-MRI). The size and localization of FM meningiomas do not significantly affect the CFS circulation parameters. Pyramidal symptoms, sensory disorders, and XIth cranial nerve dysfunction are correlated with the CFS circulation parameters. According to the preoperative PC-MRI data, the CFS circulation parameters in all FM meningioma patients were significantly higher than their normal values. Surgery was followed by a reduction in the peak positive velocity, negative peak velocity, and range of the maximum linear velocity amplitude. Positive and negative volumes and the stroke volume did not change. Recovery dynamics of the CFS circulation parameters was similar, regardless of surgery completeness. According to the PC-MRI data, the CFS circulation parameters did not reach normal values in all groups of operated patients. The results of investigation of the CFS circulation in patients with FM meningiomas support the use of palliative surgery (partial resection, dural plasty, craniovertebral junction decompression) in the case of inoperable meningiomas.

  3. Meningiomas anteriores e antero-laterais do forame magno Anterior and lateral foramen magnum meningiomas

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    Osvaldo Inácio de Tella Jr

    2006-06-01

    Full Text Available Relatamos nossa experiência com 11 pacientes portadores de meningiomas do forame magno, oito cranioespinhais e três espinocraniais. A média de idade foi 50,8 anos, o sintoma mais comum foi cefaléia occipital com duração média de 18,6 meses. Os principais achados neurológicos foram tetraparesia e comprometimento dos nervos cranianos baixos. O tratamento foi cirúrgico, sempre com exposição da artéria vertebral em sua entrada na dura-máter da fossa posterior e ressecção de parte do côndilo occipital apenas em três casos. Ressecção total foi possível em sete pacientes e parcial nos demais, devido às aderências a vasos e nervos. O prognóstico esteve relacionado com as condições neurológicas pré-operatórias.We report our experience with 11 cases of foramen magnum meningiomas, eight originating inside the posterior fossa and three in the caudal region. The mean age of the patients was 50.8 years and the main complaint was cervical headache for at least 18.6 months and at the neurological examination, tetraparesis and deficit of the lower cranial nerves were very often observed. All patients were submitted to surgical treatment, always with exposition of the vertebral artery at the entry zone in the duramater of the posterior fossa, with partial removal of the occipital condyle in only three cases. Total resection was obtained in seven patients and partial removal in the other four due to adherences to vessels and nerves. The prognostic was related to the neurological condition before surgery.

  4. Congenital intracranial meningioma. A case report and literature review

    DEFF Research Database (Denmark)

    Madsen, C; Schrøder, H D

    1993-01-01

    A case report of congenital intracranial meningioma is presented. We describe what appears to be the first fetal meningioma of the fibroblastic subtype. The literature is reviewed, and the subtype and sex distribution of fetal meningiomas is discussed.......A case report of congenital intracranial meningioma is presented. We describe what appears to be the first fetal meningioma of the fibroblastic subtype. The literature is reviewed, and the subtype and sex distribution of fetal meningiomas is discussed....

  5. Recurrent spinal meningioma: a case report.

    Science.gov (United States)

    Choi, Hoi Jung; Paeng, Sung Hwa; Kim, Sung Tae; Jung, Yong Tae

    2012-09-01

    Meningiomas are the second most common intradural spinal tumors accounting for 25% of all spinal tumors. Being a slow growing and invariably benign tumor, it responds favorably to surgical excision. In addition, spinal meningioma has low recurrence rates. However, we experienced a case of intradural extramedullary spinal meningioma which recurred 16 years after the initial surgery on a 64-year-old woman. She presented with progressive neurological symptoms and had a surgical history of removal of thoracic spinal meningioma 16 years ago due to bilateral low leg weakness. She underwent a second operation at the same site and a pale yellowish tumor was excised, which was histopathologically confirmed as meningothelial meningioma, compared with previously transitional type. she showed neurological recovery after the operation. We, therefore, report the good results of this recurrent intradural spinal meningioma case developed after 16 years with literature review.

  6. Uncommon Progression of an Extradural Spinal Meningioma

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    Atef Ben Nsir

    2014-01-01

    Full Text Available Extradural spinal meningiomas are rare. Our understanding of purely extradural spinal meningiomas is still incomplete and they may be easily confused with malignant neoplasms, much more common in this location. We report a rare case of a purely extradural thoracic spine meningioma in a 70-year-old man, with an unusual progression. In addition we discuss the pathogenesis of these tumors and the potential pitfalls in differential diagnosis and review the relevant literature concerning their treatment and outcome.

  7. Mifepristone (RU 486) treatment of meningiomas.

    OpenAIRE

    Lamberts, S.W.; Tanghe, H L; Avezaat, C J; Braakman, R; Wijngaarde, R.; Koper, J. W.; de Jong, H

    1992-01-01

    Meningiomas are common brain tumours which are generally benign, well circumscribed and slow growing. In a minority of patients complete surgical removal is not possible and re-growth of tumour tissue is a major clinical problem. Most meningiomas contain progesterone receptors. The anti-progestational drug mifepristone (RU 486) binds to these receptors. Ten patients were treated with 12 recurrent or primary "inoperable" meningiomas, all of whom had shown recent neuroradiological and/or ophtha...

  8. Primary intraosseous meningioma: CT and MRI appearance.

    Science.gov (United States)

    Tokgoz, Nil; Oner, Yusuf A; Kaymaz, Memduh; Ucar, Murat; Yilmaz, Guldal; Tali, Turgut E

    2005-09-01

    Benign primary intraosseous meningioma presenting with osteolytic skull lesion and soft-tissue component is rare. CT and MR imaging of a patient with frontoparietal scalp swelling showed an osteolytic intracalvarial lesion with an extradural soft-tissue component. Following wide surgical resection, the histological examination revealed an intraosseous chordoid meningioma. The clinical and radiological findings of primary intraosseous meningioma are discussed and the relevant literature is reviewed.

  9. Meningioma - a review of 52 cases

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    E. Fynn

    2004-04-01

    Full Text Available Meningiomas are extra-axial neoplasms representing 15 - 20% of primary intracranial neoplasms. Incidence peaks in patients aged 40 - 60 years, and they more commonly affect women than men. Most meningiomas arise from arachnoidal cells on the inner surface of the dura and they thefore grow inward toward the brain to form bulky intradural tumour masses. Most meningiomas are benign, but ~6% are atypical or aggressive, and 1 - 2% are frankly malignant. Common locations for meningiomas include the cerebral convexity, parasagittal region, sphenoid wing, olfactory groove, tuberculum sella, posterior fossa, and cavernous sinus.

  10. Endonasal transsphenoidal removal of tuberculum sellae meningiomas: technical note.

    Science.gov (United States)

    Cook, Shon W; Smith, Zachary; Kelly, Daniel F

    2004-07-01

    Tuberculum sellae meningiomas traditionally have been removed through a transcranial approach. More recently, the sublabial transsphenoidal approach has been used to remove such tumors. Here, we describe use of the direct endonasal transsphenoidal approach for removal of suprasellar meningiomas. Three women, aged 32, 34, and 55 years, each sought treatment for visual loss and headaches. In each patient, magnetic resonance imaging (MRI) showed a suprasellar mass causing optic chiasmal and optic nerve compression (average size, 2 x 2 cm). All three patients underwent tumor removal via an endonasal approach with the operating microscope. Suprasellar exposure was facilitated by removal of the posterior planum sphenoidale. Ultrasound was used to help define tumor location before dural opening. The extent of tumor removal was verified with angled endoscopes in all patients, and with intraoperative MRI in one patient. The surgical dural and bony defects were repaired in all patients with abdominal fat, titanium mesh, and 2 to 3 days of cerebrospinal fluid lumbar drainage. Nasal packing was not used. There were no postoperative cerebrospinal fluid leaks or meningitis. One patient required a reoperation 2 weeks after surgery to reduce the size of her fat graft, which was causing optic nerve compression; within 24 hours, her vision rapidly improved. At 3 months after surgery, all three patients had normal vision, no new endocrinopathy, and no residual tumor on MRI. At 10 months after surgery, one patient had a small asymptomatic tumor regrowth seen on MRI. The endonasal approach with the operating microscope appears to be an effective minimally invasive method for removing relatively small midline tuberculum sellae meningiomas. Intraoperative ultrasound, the micro-Doppler probe, and angled endoscopes are useful adjuncts for safely and completely removing such tumors. Longer follow-up is needed to monitor for tumor recurrence in these patients.

  11. The value of VEP in the diagnosis and post-operative monitoring of meningioma.

    Science.gov (United States)

    Hidajat, Rudy R; McLay, Jan L; Goode, David H; Hidayat, J Ray

    2006-11-01

    Meningiomas are the most frequently reported primary intracranial neoplasms. At first they usually cause unilateral visual loss but eventually result in a bilateral loss of vision. Depending upon the size and location of the tumour, the ocular signs and symptoms of meningiomas may include visual field abnormalities, optic atrophy, papilledema, diplopia and proptosis. This case report highlights the value of visual evoked potentials (VEP) in a patient with unexplained bilateral optic atrophy and a progressive loss of vision over 2 years. As a result of a delayed response in the VEP recorded from the right eye, a compressive lesion of the optic nerve was suspected. That prompted the referring ophthalmologist to request a MRI scan which led to the diagnosis of meningioma. Following the subtotal removal of the suprasellar meningioma, the remaining vision in the right eye improved and the latency of the VEP returned to the normal range. However, the VEP from the blind eye (left) did not show any measurable response either pre- or postoperatively. Experience with this patient suggests that early recognition of optic nerve compression is vital to an optimal outcome and the VEP technique, which is much more cost-effective than MRI, is clinically useful for detecting such compressive lesions.

  12. Meningiomas: estudo epidemiologic e anátomo-patológico de 304 casos Meningiomas: epidemiological and pathological studies of 304 cases

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    Luiz Fernando Bleggi Torres

    1996-12-01

    Full Text Available No presente estudo, foram pesquisados os tumores do sistema nervoso central (SNC diagnosticados na cidade de Curitiba entre 1990 e 1994. Realizaram-se 2371 biópsias de SNC das quais 1340 casos eram neoplasias primárias. Dentre as neoplasias primárias de SNC 304 (22,6% eram meningiomas. Duzentos e nove meningiomas ocorreram em mulheres (68,7% e 95 ocorreram em homens (31,3%. As idades variaram de 3 a 90 anos, com média de 45,8 anos. Duzentos e oitenta meningiomas localizavam-se no cérebro, 10 na medula espinhal, 9 no cerebelo e 5 na emergência de nervos cranianos. Na classificação histológica verificou-se que 294 (96,7% eram do tipo clássico, 6 malignos (ou anaplásicos, 3 atípicos e 1 papilar. No grupo dos meningiomas clássicos 267 eram meningoteliais, 10 psamomatosos, 5 fibroblásticos, 5 microcísticos, 5 transicionais e 2 angiomatoses. Os autores concluem que as neoplasias meníngeas representaram o grupo mais freqüente de tumores primários do SNC e apresentaram características epidemiológicas e anátomo-patológicas similares às da literatura mundial, na qual é dada ênfase à dificuldade de estabelecer critérios biológicos e histológicos de malignidade neste grupo de tumores.The authors have retrospectively reviewed all tumors of central nervous system (CNS operated at the most important neurosurgery hospitals of Curitiba in a 5-year period (1990-1994 and found 304 (22.4% cases of meningioma. Age mean of the patients was 48.5 years, with a range of 3 to 90 years. A marked female preponderance (68.7% was noted. The most common tumor location was brain (n=280 and the remaining cases occurred in spinal cord (n=10, cerebellum (n=9 and cranial nerves (n=5. Histologically, there were 294 (96.7% meningiomas of the classical type, six malignant or anaplastic, three atypical and one papillary. Two hundred and sixty seven classical meningiomas were from the meningotelial subtype, ten psamomatousos, five fibroblastic, five

  13. Intradural extramedullary hemangiopericytoma of the thoracic spine infiltrating a nerve root: a case report and literature review.

    Science.gov (United States)

    Moscovici, Samuel; Ramirez-DeNoriega, Fernando; Fellig, Yakov; Rosenthal, Guy; Cohen, José E; Itshayek, Eyal

    2011-11-01

    Review the presentation and diagnosis of an intradural extramedullary hemangiopericytoma of the thoracic spine. To present a rare case of intradural, subpial hemangiopericytoma in the thoracic spine, with a brief overview of the literature. Spinal intradural extramedullary hemangiopericytoma is rare entity that radiographically mimics nerve-sheath tumors. These lesions are typically diagnosed at surgery performed due to suspicion of tumor. A 20-year-old man who presented with back pain, leg weakness, and sphincter incontinence. MR imaging demonstrated an intradural extramedullary lesion at the T9-T10 level that was isointense on T1- and T2-weighted images and homogeneously enhancing after administration of gadolinium, with cystic components seen on T2-weighted images. The preoperative diagnosis was meningioma or schwannoma. At surgery, the lesion was bluish and completely subpial, with apparent nerve root invasion. Pathological examination revealed a neoplasm adjacent to a nerve root with possible focal infiltration. Abundant reticulin fibers and widened, branching vascular channels imparting a staghorn appearance were seen. Up to five mitotic figures were counted in one high-power field. On immunostaining, the neoplastic cells were diffusely immunoreactive for CD99 and immunonegative for EMA, CD34, and S-100 protein. The pathological diagnosis was consistent with anaplastic hemangiopericytoma, WHO grade III. This is the ninth report of spinal intradural hemangiopericytoma. The location of the neoplasm supports the hypothesis that hemangiopericytoma may arise from the spinal pial capillaries.

  14. Classification system of foramen magnum meningiomas

    Science.gov (United States)

    Bruneau, Michaël; George, Bernard

    2010-01-01

    Background: Foramen magnum meningiomas (FMMs) are challenging tumors. We report a classification system based on our experience of 107 tumors. Materials and Methods: The three main algorithm criteria included the compartment of development of the tumor, its dural insertion, and its relation to the vertebral artery. Results: The compartment of development was most of the time intradural (101/107, 94.4%) and less frequently extradural (3/107, 2.8%) or both intra-extradural. (3/107, 2.8%). When developed inside the intradural compartment, FMMs were subdivided into posterior (6/104, 5.8%), lateral (57/104, 54.8%), and anterior (41/104, 39.4%), if their insertion was respectively posterior to the dentate ligament, anterior to the dentate ligament without or with extension over the midline. Anterior and lateral intradural lesions grew below (77/98, 78.6%), above (16/98, 16.3%), or on both sides (5/98, 5.1%) of the VA. Only three cases of extraduralFMMs (3/107, 2.8%) were resected by an antero-lateral approach while all the other ones (104/107, 97.2%) were removed successfully by a postero-lateral approach. Lower cranial nerves were displaced superiorly in FMM growing below the VA but their position cannot be anticipated in other situations. Conclusions: This classification system helps for defining the best surgical approach but also for anticipating the position of the lower cranial nerves and therefore for reducing the surgical morbidity. PMID:20890409

  15. Acute recurrent haemorrhage of an intracranial meningioma.

    Science.gov (United States)

    Bellut, David; Nern, Christian; Burkhardt, Jan-Karl; Könü, Dilek; Bertalanffy, Helmut; Krayenbühl, Niklaus

    2011-07-01

    Meningioma-associated haemorrhages are rare. To our knowledge this is the first report of a patient with an acute two-stage haemorrhage of a benign intracranial meningioma (World Health Organization grade I) verified by cranial CT scan and histopathological examination. Early surgery with complete tumour removal led to a good outcome for the patient. Copyright © 2011 Elsevier Ltd. All rights reserved.

  16. Meningioma y degeneración hialina

    OpenAIRE

    Iglesias Rozas, José Rafael, 1942-

    1984-01-01

    Cuatro imágenes de un meningioma y una degeneración de la membrana hialina en una paciente de 43 años. Four pictures of a meningioma and a degeneration of the hyaline membrane in a 43-year-old female patient.

  17. Peritumoral brain edema in angiomatous supratentorial meningiomas

    DEFF Research Database (Denmark)

    Nassehi, Damoun; Sørensen, Lars Peter; Dyrbye, Henrik;

    2013-01-01

    The aim of this work was to study the vascular endothelial growth factor A (VEGF-A) pathway and peritumoral brain edema (PTBE) through comparison of non-angiomatous and angiomatous meningiomas. Meningiomas are common intracranial tumors, which often have PTBE. VEGF-A is an integral part of PTBE...

  18. Osteoblastic meningioma of the fourth ventricle.

    Science.gov (United States)

    Johnson, M D; Tulipan, N; Whetsell, W O

    1989-04-01

    Meningiomas of the fourth ventricle are rare neoplasms. Only meningothelial and fibroblastic subtypes, purportedly arising from the tela choroidea, have been described. In this report we describe clinical, neuroradiological and pathological findings in a 52-year-old man with mild hydrocephalus produced by a large, calcified, osteoblastic meningioma of the fourth ventricle.

  19. Skull base meningioma. Surgical and adjuvant treatment with clinical and PET evaluation

    Energy Technology Data Exchange (ETDEWEB)

    Gudjonsson, O

    2001-05-01

    The treatment strategy for skull base meningiomas remains a controversial issue. Because of the proximity of these tumours to critical neurovascular structures, the risk for vascular damage and new cranial neuropathies postoperatively is significant. To avoid unacceptable neurological deficits the surgical treatment strategy includes different surgical approaches and a subtotal removal of these tumours in some cases. However, because the rate of recurrence and progression is significant in these patients, a demand for adjuvant treatment and better prognostic methods is called for so that treatment and follow-up can be tailored to each patient. Accordingly, we have chosen to evaluate general outcome and facial nerve function after translabyrinthine and transcochlear approaches for cerebellopontine angle (CPA) meningiomas. Furthermore, we have evaluated two adjuvant treatments, namely, irradiation by high-energy proton beams and medical treatment with interferon-alpha as well as evaluation of the treatment effect with {sup 11}C-L-methionine PET. In addition, we have evaluated a new PET tracer ({sup 76}Br-BrdU) for 'in vivo' determination of the growth potential of intracranial tumours. Conclusion: The translabyrinthine and transcochlear approaches are apparently safe surgical procedures in the treatment of CPA meningiomas. Proton beam therapy is technically feasible as suggested by the fact that only minimal side effects were observed. Moreover, none of the meningiomas treated have shown progression during a 36-month follow-up. Our results indicate that IFN-alpha can be an effective oncostatic treatment for certain patients with meningiomas. The {sup 11}C-L-methionine PET method might be used as a complement to CT or MRI in the evaluation of the effect of proton beam and IFN-alpha treatment in meningiomas. The present attempt failed to demonstrate that the PET tracer {sup 76}Br-BrdU could be used for the non-invasive characterisation of growth potential in

  20. Foramen magnum meningiomas: surgical treatment in a single public institution in a developing country

    Directory of Open Access Journals (Sweden)

    Benedicto Oscar Colli

    2014-07-01

    Full Text Available Objective: To analyze the clinical outcome of patients with foramen magnum (FM meningiomas. Method: Thirteen patients (11 Feminine / 2 Masculine with FM meningiomas operated on through lateral suboccipital approach were studied. Clinical outcome were analyzed using survival (SC and recurrence-free survival curves (RFSC. Results: All tumors were World Health Organization grade I. Total, subtotal and partial resections were acchieved in 69.2%, 23.1% and 7.7%, respectively, and SC was better for males and RFSC for females. Tumor location, extent of resection and involvement of vertebral artery/lower cranial nerves did not influence SC and RFSC. Recurrence rate was 7.7%. Operative mortality was 0. Main complications were transient (38.5% and permanent (7.7% lower cranial nerve deficits, cerebrospinal fluid fistula (30.8%, and transient and permanent respiratory difficulties in 7.7% each. Conclusions: FM meningiomas can be adequately treated in public hospitals in developing countries if a multidisciplinary team is available for managing postoperative lower cranial nerve deficits.

  1. Fibroma of tendon sheath.

    Science.gov (United States)

    Smith, P S; Pieterse, A S; McClure, J

    1982-01-01

    A series of nine cases of fibroma of tendon sheath is described including details of the ultrastructural features of two cases. The series was composed of lesions from six males and three females with a mean age of 38 yr. The most common site of involvement was the hand (including fingers) and the mean greater diameter was 19 mm. Typically the tumours were lobulated and microscopically there was a collagenous stroma with spindle and stellate cells in a moderate degree of cellularity. One recurrence was noted in the series. The lesion was distinguished from circumscribed fibromatosis, nodular fasciitis, neurofibroma, leiomyoma, scar tissue, giant cell tumour of tendon sheath (localised nodular tenosynovitis) and fibrous histiocytoma. Ultrastructural studies revealed that the large majority of cells present in the two cases studied were myofibroblasts and fibroma of tendon sheath is therefore the third instance of a benign tumour containing these cells (the other two being dermatofibroma and giant cell fibroma of the oral mucosa). Images PMID:7107956

  2. Posterior fossa meningioma (surgical experiences)

    African Journals Online (AJOL)

    Wael M. Moussa

    2012-08-27

    Aug 27, 2012 ... Morbidity included decreased level of consciousness, cranial nerve palsy and wound infection. ..... (10 cases), trigeminal nerve compression (6 cases), facial nerve .... and magnetic resonance imaging for surgical planning for menin- ... anatomy scanning in the operation of intracranial parasagittal.

  3. SPECT and PET Imaging of Meningiomas

    Directory of Open Access Journals (Sweden)

    Varvara Valotassiou

    2012-01-01

    Full Text Available Meningiomas arise from the meningothelial cells of the arachnoid membranes. They are the most common primary intracranial neoplasms and represent about 20% of all intracranial tumors. They are usually diagnosed after the third decade of life and they are more frequent in women than in men. According to the World Health Organization (WHO criteria, meningiomas can be classified into grade I meningiomas, which are benign, grade II (atypical and grade III (anaplastic meningiomas, which have a much more aggressive clinical behaviour. Computed Tomography (CT and Magnetic Resonance Imaging (MRI are routinely used in the diagnostic workup of patients with meningiomas. Molecular Nuclear Medicine Imaging with Single Photon Emission Computed Tomography (SPECT and Positron Emission Tomography (PET could provide complementary information to CT and MRI. Various SPECT and PET tracers may provide information about cellular processes and biological characteristics of meningiomas. Therefore, SPECT and PET imaging could be used for the preoperative noninvasive diagnosis and differential diagnosis of meningiomas, prediction of tumor grade and tumor recurrence, response to treatment, target volume delineation for radiation therapy planning, and distinction between residual or recurrent tumour from scar tissue.

  4. Meckel's cave meningiomas with subarachnoid hemorrhage.

    Science.gov (United States)

    Rosenberg, G A; Herz, D A; Leeds, N; Strully, K

    1975-06-01

    Two patients with Meckel's Cave meningiomas were initially hospitalized as a result of subarachnoid hemorrhage. Four-vessel angiography was necessary to exclude other causes of bleeding while demonstrating these lesions. Apoplectic presentation in both cases led to early diagnosis and successful surgical therapy. A review of the literature reveals subarachnoid hemorrhage to be a rarity in association with meningiomas. The two patients currently reported are believed to be the only examples on record of hemorrhagic meningiomas arising from the region of Meckel's Cave.

  5. A neurogenic tumor containing a low-grade malignant peripheral nerve sheath tumor (MPNST) component with loss of p16 expression and homozygous deletion of CDKN2A/p16: a case report showing progression from a neurofibroma to a high-grade MPNST.

    Science.gov (United States)

    Tajima, Shogo; Koda, Kenji

    2015-01-01

    Development of malignant peripheral nerve sheath tumors (MPNSTs) is a stepwise process that involves the alteration of many cell cycle regulators and the double inactivation of the NF1 gene. Inactivation of the TP53 gene and deletion of the CDKN2A/p16 gene are known to play an important role in the process. Herein, we present a 19-year-old man with a familial history of neurofibromatosis type 1, in whom the tumor arose from the intercostal nerve and showed 3 components: a neurofibroma, a low-grade MPNST, and a high-grade MPNST. Loss of p16 expression and homozygous deletion of the CDKN2A/p16 gene were observed in both the low-grade and the high-grade MPNST. In contrast to low-grade MPNSTs, high-grade MPNSTs generally tend to lose expression of p16 and harbor homozygous deletion of the CDKN2A/p16 gene. Loss of p16 expression and homozygous deletion of the CDKN2A/p16 gene in low-grade MPNST in our case might be related to its progression to high-grade MPNST. To the best of our knowledge, this is the first study correlating the p16 expression status and CDKN2A/p16 gene alteration in low-grade MPNSTs.

  6. Reparação microcirúrgica de nervo periférico por meio de sutura, cola de fibrina ou bainha de BioFill® em ratos Wistar Microsurgical repair of peripheral nerve by means of suture, fibrin glue or BioFill® sheath in Wistar rats

    Directory of Open Access Journals (Sweden)

    M.F.P. Torres

    2003-10-01

    Full Text Available Com o objetivo de comparar três técnicas de neuroanastomose, foram utilizados 54 ratos fêmeas, distribuídos em três grupos de 18 animais, cujos nervos ciáticos direitos foram transeccionados e, em seguida, tratados mediante o emprego das seguintes técnicas: três pontos de sutura epineural, um ponto de sutura epineural associado à bainha de BioFill® e um ponto de sutura epineural com cola de fibrina. A presença do material de sutura foi responsável pelo mau direcionamento das fibras nervosas e formação de neuroma na área da anastomose devido à colisão das fibras nervosas em regeneração com a reação granulomatosa ao fio de sutura. Alterações como degeneração Walleriana, formação de neuroma e proliferação de tecido fibroso foram observadas nos três tratamentos, sendo mais evidentes no grupo que recebeu três pontos de sutura epineural. Os resultados histológicos superiores obtidos com o emprego da bainha de BioFill® e da cola de fibrina comprovaram a eficiência dessas duas técnicas na reparação de nervos periféricos lesados. O uso da bainha de BioFill® oferece vantagens como disponibilidade, simplicidade técnica e baixo custo, tornando-a a melhor alternativa na reparação microcirúrgica do nervo periférico.Aiming to compare three neuroanastomosis techniques, 54 female rats were divided into three groups of 18 animals each. The right-side ciatic nerves of these animals were trans-sectioned and, immediately, treated by means of the following techniques: three plain epineural suture stitches, one epineural suture stitch together with a BioFill® sheath, and one perineural suture stitch with fibrine glue. Analysis of the results was based on histopathological aspects of the anastomotic area. It was noted that the presence of the suture material was responsible for the poor directioning of the nerve fibers and formation of a neurome in the anastomosis area, due to the collision of the regenerating nervous fibers

  7. Dusty Sheaths in Magnetized Plasmas

    Institute of Scientific and Technical Information of China (English)

    Yu. I. Chutov; O. Yu. Kravchenko; S. Masuzaki; A. Sagara; R. D. Smirnov; Yu. Tomita

    2004-01-01

    Parameters of self-consistent magnetized dusty sheaths are investigated using computer simulations of a temporal evolution of one-dimensional slab plasma with dust particles. The evolution is caused by a collection of electrons and ions by both a wall (electrode) and dust particles, which are initially immersed into plasma and distributed in front of the electrode. Obtained results show the existence of oscillations of a self-consistent potential in magnetized dusty sheaths including boundary potentials. Dust particles weaken magnetized sheaths and create additional sheaths close to a boundary of dust particles. The magnetic field does not influence on the dust particle charge.

  8. Stereotactic radiosurgery for intracranial meningiomas

    Energy Technology Data Exchange (ETDEWEB)

    Kida, Yoshihisa; Kobayashi, Tatsuya; Tanaka, Takayuki; Oyama, Hirofumi; Iwakoshi, Takayasu (Komaki City Hospital, Hokkaido (Japan))

    1994-07-01

    Stereotactic radiosurgery for intracranial meningiomas was attempted using a 201-source cobalt gamma knife. Forty patients bearing 42 tumors were involved in this study. Their ages ranged from 30 to 91 years, with an average of 55.1 years. The most frequent sites of origin were the parasellar and petroclival regions. The mean tumor diameter was 27.2 mm and the marginal tumor dose of radiosurgery ranged from 10 to 20 Gy, depending on tumor location and size. Serial imaging studies with MRI were obtained in all 40 cases, in which minor tumor shrinkage was demonstrated in 7.9%, 40.0% and 53.3% at 6, 12 and 18 months after radiosurgery respectively. Only two tumors became enlarged after the treatment. Obvious low signal intensity on MRI, indicating central tumor necrosis, was found in 32% at 12 months and 40% at 18 months. Four large tumors over 40 mm in mean diameter were treated by staged radiosurgery with intervals of 1.5 to 7 months. A similar good response was able to be obtained in all 4 cases, even though they were treated with a marginal dose less than 12 Gy. Symptomatic edema occurred in 5 cases (12.5%) within 12 months and required corticosteroid therapy and hyperosmotic diuresis. In conclusion stereotactic radiosurgery has proved to be an effective and relatively safe method for the treatment of intracranial meningiomas. (author).

  9. Meningioma with hemorrhagic onset: Two case reports

    Directory of Open Access Journals (Sweden)

    Mukul Vij

    2012-01-01

    Full Text Available Haemorrhage is a rare complication of meningiomas that can occur spontaneously, after embolization, stereotactic radiation and perioperatively. Our first case was a 16 year old male, admitted with spastic quadriparesis, and retention of urine. Magnetic Resonance Imaging (MRI revealed anteriorly placed cervical intradural extramedullary mass. Patient underwent emergency surgery following sudden worsening of neurological symptoms and intratumoral bleed was noted peroperatively. Tumor was labeled as angiomatous meningioma with hemorrhage. The second case was of a 45 year female who presented with history of sudden onset weakness in right upper and lower limb followed by unconsciousness. MRI revealed heterogeneous lesion in left parasagittal area with intratumoral bleed. Left frontal craniotomy with tumour decompression was performed. Tumour was labelled as meningothelial meningioma with haemorrhage. Meningiomas with hemorrhagic onset remain rare, and pathophysiology is still incompletely understood. Prevention and outcome of intratumoral haemorrhage highly depends on early diagnosis and adequate treatment.

  10. Spontaneous Regression of an Incidental Spinal Meningioma

    Directory of Open Access Journals (Sweden)

    Ali Yilmaz

    2015-12-01

    Full Text Available AIM: The regression of meningioma has been reported in literature before. In spite of the fact that the regression may be involved by hemorrhage, calcification or some drugs withdrawal, it is rarely observed spontaneously. CASE REPORT: We report a 17 year old man with a cervical meningioma which was incidentally detected. In his cervical MRI an extradural, cranio-caudal contrast enchanced lesion at C2-C3 levels of the cervical spinal cord was detected. Despite the slight compression towards the spinal cord, he had no symptoms and refused any kind of surgical approach. The meningioma was followed by control MRI and it spontaneously regressed within six months. There were no signs of hemorrhage or calcification. CONCLUSION: Although it is a rare condition, the clinicians should consider that meningiomas especially incidentally diagnosed may be regressed spontaneously.

  11. Spontaneous Regression of an Incidental Spinal Meningioma.

    Science.gov (United States)

    Yilmaz, Ali; Kizilay, Zahir; Sair, Ahmet; Avcil, Mucahit; Ozkul, Ayca

    2016-03-15

    The regression of meningioma has been reported in literature before. In spite of the fact that the regression may be involved by hemorrhage, calcification or some drugs withdrawal, it is rarely observed spontaneously. We report a 17 year old man with a cervical meningioma which was incidentally detected. In his cervical MRI an extradural, cranio-caudal contrast enchanced lesion at C2-C3 levels of the cervical spinal cord was detected. Despite the slight compression towards the spinal cord, he had no symptoms and refused any kind of surgical approach. The meningioma was followed by control MRI and it spontaneously regressed within six months. There were no signs of hemorrhage or calcification. Although it is a rare condition, the clinicians should consider that meningiomas especially incidentally diagnosed may be regressed spontaneously.

  12. Spontaneous Regression of an Incidental Spinal Meningioma

    National Research Council Canada - National Science Library

    Yilmaz, Ali; Kizilay, Zahir; Sair, Ahmet; Avcil, Mucahit; Ozkul, Ayca

    2015-01-01

    AIM: The regression of meningioma has been reported in literature before. In spite of the fact that the regression may be involved by hemorrhage, calcification or some drugs withdrawal, it is rarely observed spontaneously. CASE REPORT...

  13. Primary intraosseous meningiomas of the skull.

    Science.gov (United States)

    Changhong, L; Naiyin, C; Yuehuan, G; Lianzhong, Z

    1997-07-01

    The plain film, computed tomography (CT) and angiographic findings in 10 patients with primary intraosseous meningioma were reviewed and the differential diagnosis considered. In nine patients with benign primary intraosseous meningioma, the radiological findings revealed intraosseous expansile growth. In one patient with the malignant form of the tumour, osteolytic growth was evident on plain film and CT. In eight patients, the tumour tissue was hyperdense (65-85 HU) on the unenhanced CT images and striking enhancement was shown in seven. In five patients angiography showed that the external carotid artery fed the tumour, while in one the vasculature was normal. Benign primary intraosseous meningioma showed expansile growth and malignant tumour showed osteolytic growth. If a combination of the plain film, angiogram and CT findings is considered, a diagnosis of the benign tumour can be made and a diagnosis of benign meningioma can be suggested.

  14. Atypical imaging appearances of intracranial meningiomas

    Energy Technology Data Exchange (ETDEWEB)

    O' Leary, S. [Radiology Department, Derriford Hospital, Plymouth (United Kingdom); Adams, W.M. [Radiology Department, Derriford Hospital, Plymouth (United Kingdom); Parrish, R.W. [Radiology Department, Derriford Hospital, Plymouth (United Kingdom); Mukonoweshuro, W. [Radiology Department, Derriford Hospital, Plymouth (United Kingdom)]. E-mail: William.mukonoweshuro@phnt.swest.nhs.uk

    2007-01-15

    Meningiomas are the commonest primary, non-glial intracranial tumours. The diagnosis is often correctly predicted from characteristic imaging appearances. This paper presents some examples of atypical imaging appearances that may cause diagnostic confusion.

  15. Prostate carcinoma mimicking a sphenoid wing meningioma

    Directory of Open Access Journals (Sweden)

    Lucas H. Bradley

    2015-01-01

    Conclusion: We conclude that dural-based metastatic lesions may mimic meningiomas, warranting thorough pre-operative work-up to exclude the possibility of metastasis. In certain cases, identification of widespread disease might preclude surgery and favor palliation, instead.

  16. Extradural spinal meningioma: Revisiting a rare entity

    Directory of Open Access Journals (Sweden)

    Guruprasad Bettaswamy

    2016-01-01

    Full Text Available Spinal meningiomas are mostly intradural in location although at times these are associated with some extradural extensions. Purely extradural spinal meningiomas (EDSMs are however, extremely rare and when present, may cause diagnostic dilemma preoperatively. Only seven cases of pure EDSM have been reported till date. In this paper, we describe two cases of EDSM affecting the cervical spine and present their clinical profiles, radiological findings, operative management, and follow-up data, along with a review of the literature.

  17. Atypical Growth Pattern of an Intraparenchymal Meningioma

    Science.gov (United States)

    Chen, Xiaoxi

    2016-01-01

    Meningiomas are the most common primary nonneuroglial extra-axial neoplasms, which commonly present as spherical or oval masses with a dural attachment. Meningiomas without dural attachment are rare and, according to their locations, are classified into 5 varieties, including intraventricular, deep Sylvain fissure, pineal region, intraparenchymal, or subcortical meningiomas. To the best of our knowledge, intraparenchymal meningioma with cerebriform pattern has never been reported. In this paper, we report a 34-year-old Chinese male patient who presented with paroxysmal headaches and progressive loss of vision for 10 months and blindness for 2 weeks. A thorough physical examination revealed loss of bilateral direct and indirect light reflex. No other relevant medical history and neurologic deficits were noted. Computed tomography and magnetic resonance imaging scans showed an irregular mass with a unique cerebriform pattern and extensive peritumoral edema in the parietal-occipital-temporal region of the right cerebral hemisphere. The initial diagnosis was lymphoma. Intraoperatively, the tumor was completely buried in a sulcus in the parietal-occipital-temporal region without connecting to the dura. The histological diagnosis was intracranial meningioma based on pathological examination. Therefore, when an unusual cerebriform growth pattern of a tumor is encountered, an intraparenchymal meningioma should be considered as a differential diagnosis. PMID:27752384

  18. Direct Comparison of Gamma Knife Radiosurgery and Microsurgery for Small Size Meningiomas.

    Science.gov (United States)

    Bir, Shyamal C; Patra, Devi Prasad; Maiti, Tanmoy Kumar; Bollam, Papireddy; Minagar, Alireza; Nanda, Anil

    2017-05-01

    Patients with small (<3 cm) intracranial meningiomas can be either observed or treated. Treatment can be either radiosurgery or microsurgery if and when tumor progression occurs. We compared local tumor growth control and recurrence-free survival (RFS) of microsurgical resection and radiosurgery in small intracranial meningiomas and identified predictors of unfavorable outcome. A retrospective review (2005-2016) was performed of 90 consecutive patients with intracranial meningiomas who underwent either microsurgery (n = 31) or Gamma Knife radiosurgery (GKRS) (n = 59). The study population was evaluated clinically and radiographically after treatment. GKRS in meningiomas showed a significantly higher percentage of local control of tumor growth compared with microsurgery (P = 0.02) 5 and 10 years (P = 0.003) after treatment. The median RFS was also significantly higher in the GKRS group compared with the microsurgery group (P = 0.04). There was no difference in RFS between Simpson grade I resection and GKRS (P = 0.69). In univariate analysis, RFS after procedures was significantly affected by tumor involvement of cranial nerves, presence of comorbidities, and preoperative Karnofsky performance scale score ≤70. In multivariate analysis, only preoperative Karnofsky performance scale score ≤70 was a predictor of unfavorable outcome. GKRS offers a high rate of tumor control and longer RFS that is comparable to Simpson grade I resection. Subtotal resection is not a good choice for small meningiomas. The treatment procedure should be tailored according to the presence of comorbidities and the maximum benefit for the patient. Copyright © 2017 Elsevier Inc. All rights reserved.

  19. Predictors of severe complications in intracranial meningioma surgery

    DEFF Research Database (Denmark)

    Bartek, Jiri; Sjåvik, Kristin; Förander, Petter

    2015-01-01

    OBJECTIVE: To investigate predictors of complications after intracranial meningioma resection using a standardized reporting system for adverse events. METHODS: A retrospective review was conducted in a Scandinavian population-based cohort of 979 adult operations for intracranial meningioma perfo...

  20. [Psychopathology and neuro-ophthalmology in meningioma of the olfactory groove].

    Science.gov (United States)

    Lange, E; Bräunig, K P; Fötzsch, R

    1982-01-01

    In 14 patients with a surgically nad histopathologically verified meningioma of the olfactory nerve groove (OGM) (12 women, 2 men), clinical findings and diagnosis were analysed, especially with respect to neuropathology and psychopathology. The apodictic theorem that an OGM leads to a Foster-Kennedy syndrome as well as to a psychopathological orbital brain syndrome cannot be maintained. The Foster-Kennedy syndrome does not occur in a characteristically high incidence, and psychopathologically an orbital brain syndrome can only be diagnosed in case of an anterior location of the OGM. The more the OGM shows starting point or growth development to or from the anterior chiasmal angle, the more is resembles that psychopathological syndrome that we have found and described for the meningioma of the Tuberculus sellae.

  1. Ossified thoracic spinal meningioma: report of two cases

    Energy Technology Data Exchange (ETDEWEB)

    Chung, Seung Eun; Lee, Sang Ho; Park, Hyeon Seon; Kim, Dae Yong [Wooridul Spine Hospital, Seoul (Korea, Republic of); Kim, Tae Hong [Inje University College of Medicine, Seoul (Korea, Republic of)

    2004-10-15

    Spinal meningioma is one of relatively common spinal tumors with an incidence of 25-46%. However, ossified spinal meningioma is known to be extremely rare and its incidence of all the meningiomas is about 0.7-5.5%. Until now, there has been no report of ossified meningioma in Korea. We experienced two cases of this rare lesion, and we report on the CT and MRI findings along with a review of the related literature.

  2. Childhood atypical meningioma with perineural spread: MR findings

    Energy Technology Data Exchange (ETDEWEB)

    Wei, Feng-Yu.; Wong, Alex Mun-Ching; Wong, Ho-Fai; Ng, Shu-Hang [Chang Gung Memorial Hospital, Department of Diagnostic Radiology, Kwei-Shan, Tao-Yuan (Taiwan); Wu, Chieh-Tsai [Chang Gung Memorial Hospital, Department of Neurosurgery, Kwei-Shan, Tao-Yuan (Taiwan); Lin, Kuang-Lin [Chang Gung Memorial Hospital, Division of Pediatric Neurology, Kwei-Shan, Tao-Yuan (Taiwan)

    2005-09-01

    Meningiomas are uncommon in children. When they occur, they are frequently associated with neurofibromatosis type 2. Childhood meningiomas are generally large and commonly associated with cyst formation and an unusual location. Perineural tumor spread, occasionally associated with head and neck malignancies, is very rare in meningiomas. We present the MR findings of an atypical meningioma with perineural spread in a 4.5-year-old girl. (orig.)

  3. En plaque meningioma in thoracic spine: case report

    Energy Technology Data Exchange (ETDEWEB)

    Park, Hyeon Seon; Kang, Ho Yeong; Yoon, Deug Hee; Jo, Byung June; Lee, Sang Ho; Choi, Young Geun [Wooridul Spine Hospital, Seoul (Korea, Republic of); Lee, Sang Yeun [Puchon Daesung Hospital, Puchon (Korea, Republic of)

    2005-07-15

    Spinal en plaque meningioma is rarely found in the spinal canal, although lateral sphenoid wing meningioma displays a propensity for growth en plaque. We encountered a case of completely circumferential spinal en plaque meningioma, which is an even rarer condition. Herein, we report the CT MRI findings along with a review of the related literature.

  4. Case Report: Trigeminal Neuralgia Caused by a Minute Meningioma with Hyperostosed Suprameatal Tubercle

    Directory of Open Access Journals (Sweden)

    Yukitomo Ishi

    2015-08-01

    Full Text Available Cerebellopontine angle tumors might occasionally provoke trigeminal neuralgia but are usually large enough to be diagnosed radiographically. We present a case of trigeminal neuralgia caused by a very small meningioma covering the suprameatal tubercle that displayed hyperostosis at the entrance of Meckel's cave and was not obvious on routine magnetic resonance (MR images. A 72-year-old woman with intractable trigeminal neuralgia in the left V3 territory was referred to our institution. Preoperative imaging studies revealed that the left trigeminal nerve was medially distorted at the entrance of Meckel's cave by a laterally seated bone bulge covered by a minute enhanced lesion. Trigeminal nerve decompression surgery was performed via a retrosigmoid intradural suprameatal approach. We found a small meningioma that had compressed and flattened the trigeminal nerve root at the entrance of Meckel's cave, which was grossly and totally removed by suprameatal tubercle resection. There was no vascular compression of the trigeminal nerve root. The trigeminal neuralgia ceased completely after the operation. Accurate preoperative determination of the causative pathologies is essential to achieve adequate surgical results after microvascular decompression for neurovascular compression syndrome. Because conventional MR sequences are inadequate for the precise interpretation of complex neurovascular anatomy in the cerebellopontine angle and such small tumors can be overlooked on routine MR studies, high-resolution thin-slice MR examinations and careful radiological interpretations are required for correct diagnosis and treatment.

  5. Case Report: Trigeminal Neuralgia Caused by a Minute Meningioma with Hyperostosed Suprameatal Tubercle.

    Science.gov (United States)

    Ishi, Yukitomo; Asaoka, Katsuyuki; Sugiyama, Taku; Yokoyama, Yuka; Yamazaki, Kazuyoshi; Echizenya, Sumire; Itamoto, Koji; Echizenya, Kohei

    2015-01-01

    Cerebellopontine angle tumors might occasionally provoke trigeminal neuralgia but are usually large enough to be diagnosed radiographically. We present a case of trigeminal neuralgia caused by a very small meningioma covering the suprameatal tubercle that displayed hyperostosis at the entrance of Meckel's cave and was not obvious on routine magnetic resonance (MR) images. A 72-year-old woman with intractable trigeminal neuralgia in the left V3 territory was referred to our institution. Preoperative imaging studies revealed that the left trigeminal nerve was medially distorted at the entrance of Meckel's cave by a laterally seated bone bulge covered by a minute enhanced lesion. Trigeminal nerve decompression surgery was performed via a retrosigmoid intradural suprameatal approach. We found a small meningioma that had compressed and flattened the trigeminal nerve root at the entrance of Meckel's cave, which was grossly and totally removed by suprameatal tubercle resection. There was no vascular compression of the trigeminal nerve root. The trigeminal neuralgia ceased completely after the operation. Accurate preoperative determination of the causative pathologies is essential to achieve adequate surgical results after microvascular decompression for neurovascular compression syndrome. Because conventional MR sequences are inadequate for the precise interpretation of complex neurovascular anatomy in the cerebellopontine angle and such small tumors can be overlooked on routine MR studies, high-resolution thin-slice MR examinations and careful radiological interpretations are required for correct diagnosis and treatment.

  6. Ossified spinal meningiomas: Clinical and surgical features.

    Science.gov (United States)

    Alafaci, Concetta; Grasso, Giovanni; Granata, Francesca; Salpietro, Francesco M; Tomasello, Francesco

    2016-03-01

    Meningiomas constitute 25% of primary spinal tumors and predominantly involve the thoracic spinal cord. Although calcifications are commonly seen in intracranial meningiomas, gross calcifications are observed in only 1-5% of all spinal meningiomas. We report the clinical findings, surgical strategy and histological features of 9 patients with ossified spinal meningiomas (OSMs). Clinical and surgical features of 9 patients with ossified spinal meningiomas were retrospectively reviewed. There were 8 women and 1 man with a mean age of 59 years. In 7 patients, the lesions were localized in the thoracic segment of the spine while in 2 patients in the lower cervical segment. All patients presented with weakness of the lower limbs and hypoesthesia below the site level of the lesion. Only 2 patients presented with urinary incontinence. Gross-total resection of the tumor was achieved in 6 patients while in 3 a subtotal removal of the meningioma was obtained. In all patients the postoperative course was uneventful. Six patients presented with a significant neurological improvement while in 3 patients a mild improvement was observed. Microscopically, all tumors showed typical histological pattern of ossified meningioma. OSMs are amenable to surgery if the complete removal can be achieved. Because of their hard-rock consistency complete resection can be challenging. In difficult cases, subtotal removal can be advised and follow-up imaging is mandatory. Overall, the risk of long-term recurrence of the lesions is low, and a good clinical outcome after total or subtotal removal can be expected. Copyright © 2016 Elsevier B.V. All rights reserved.

  7. Endoscopic endonasal approach for a tuberculum sellae meningioma.

    Science.gov (United States)

    Fernandez-Miranda, Juan C; Pinheiro-Neto, Carlos D; Gardner, Paul A; Snyderman, Carl H

    2012-01-01

    The authors present the technical and anatomical nuances needed to perform an endoscopic endonasal removal of a tuberculum sellae meningioma. The patient is a 47-year-old female with headaches and an incidental finding of a small tuberculum sellae meningioma with no vascular encasement, no optic canal invasion, but mild inferior to superior compression of the cisternal segment of the left optic nerve. Neuroophthalmology assessment revealed no visual defects. Treatment options included clinical observation with imaging follow-up studies, radiosurgery, and resection. The patient elected to undergo surgical removal and an endonasal endoscopic approach was the preferred surgical option. Preoperative radiological studies showed the presence of an osseous ring between the left middle and anterior clinoids, the so-called carotico-clinoidal ring. The surgical implications of this finding and its management are illustrated. The surgical anatomy of the suprasellar region is reviewed, including concepts such as the chiasmatic sulcus and limbus sphenoidale, medial and lateral optico-carotid recesses, and the paraclinoidal and supraclinoidal segments of the internal carotid artery. Emphasis is made in the importance of exposing the distal dural ring of the internal carotid artery and the precanalicular segment of the optic nerve for adequate intradural dissection. The endonasal route allows for early coagulation of the tumor meningeal supply and extensive resection of dural attachments, and importantly, provides an inferior to superior access to the infrachiasmatic region that facilitates complete tumor removal without any manipulation of the optic nerve. The lateral limit of dural removal is formed by the distal dural ring, which is gently coagulated after the tumor is resected. A 45° scope is used to inspect for any residual tumor, in particular at the entrance of the optic nerve into the optic canal and at the most anterior margin of the exposure (limbus sphenoidale). The

  8. Electromagnetic induction between axons and their schwann cell myelin-protein sheaths.

    Science.gov (United States)

    Goodman, G; Bercovich, D

    2013-12-01

    Two concepts have long dominated vertebrate nerve electrophysiology: (a) Schwann cell-formed myelin sheaths separated by minute non-myelinated nodal gaps and spiraling around axons of peripheral motor nerves reduce current leakage during propagation of trains of axon action potentials; (b) "jumping" by action potentials between successive nodes greatly increases signal conduction velocity. Long-held and more recent assumptions and issues underlying those concepts have been obscured by research emphasis on axon-sheath biochemical symbiosis and nerve regeneration. We hypothesize: mutual electromagnetic induction in the axon-glial sheath association, is fundamental in signal conduction in peripheral and central myelinated axons, explains the g-ratio and is relevant to animal navigation.

  9. Coexistence of cervico-thoracic extradural en-plaque meningioma with multiple intracranial meningiomas.

    Science.gov (United States)

    Kale, Aydemir; Akyol, Cetin; Keskin, Emrah; Aydoğmuş, Evren; Aydın, Hasan Ali; Barut, Figen; Gül, Sanser; Kalaycı, Murat

    2014-01-01

    Meningioma is one of the most common tumors in the spinal cord. Extradural and en-plaque variety of meningioma occur less frequently. A 47-year-old woman is presented with radiculopathy signs. Magnetic resonance imaging revealed a lesion from C6 through T3 vertebral levels compressing the cord both anteriorly and posteriorly. Subtotally excision was performed and histopathologic signs showed transitional type of meningioma (WHO Grade 1). Post operatively, she had good neurological recovery. Intraoperative findings point out that the en-plaque meningioma was pure extradural. Twelve cases of pure extradural en-plaque meningioma have been reported in the literature. Besides, to the best our knowledge coexistence of "en plaque" spinal epidural meningioma with meningiomas in cranial cavity has not been reported. Complete resection is mandatory to prevent recurrence. Moreover, it is considerably difficult to remove the parts of tumor over anterior of the dura without complication. Copyright © 2014 Polish Neurological Society. Published by Elsevier Urban & Partner Sp. z o.o. All rights reserved.

  10. Dorsal spinal epidural psammomatous meningioma in an adult male

    Directory of Open Access Journals (Sweden)

    Sharad Pandey

    2016-01-01

    Full Text Available Meningiomas are benign in nature and arise from the arachnoid cells. They are mostly situated in the intracranial compartment, whereas spinal meningiomas are rare. Approximately, in 10% of cases, an extradural component is seen but an exclusively extradural meningioma is quite uncommon. However, WHO Grade II (atypical and Grade III (anaplastic tumors can behave aggressively. We reported a case of purely extradural psammomatous meningioma in an adult male affecting the dorsal spine although uncommon meningiomas should be included in the differential diagnosis of extradural intraspinal masses.

  11. Sheath Structures of Strongly Electronegative Plasmas

    Institute of Scientific and Technical Information of China (English)

    段萍; 王正汹; 王文春; 刘金远; 刘悦; 王晓钢

    2005-01-01

    The sheath structures of strongly electronegative plasmas are investigated on basis of the accurate Bohm criterion obtained by Sagdeev potential. It is found that the presheath transition between the bulk plasma and the sheath almost does not exist there, and that distributions of electrons, negative and positive ions in the sheath form a pure positive ion sheath near the boundary of the electrode. Furthermore, the density distribution of space net charge has a peak near the sheath edge, the spatial potential within the sheath falls faster, and the sheath thickness becomes thinner.

  12. An unusual case of clear cell meningioma

    Directory of Open Access Journals (Sweden)

    Deb Prabal

    2009-01-01

    Full Text Available Clear-cell meningioma (CCM is an uncommon, aggressive variant of meningioma, usually affecting younger females and having predilection for infratentorial locations. We present a rare case of recurrent supratentorial CCM in a 58-year-old male. Ten years back, he had an intra-axial tumor in the left occipital lobe, which was managed by surgical excision and radiotherapy. Currently, the patient presented with sudden severe headache along with speech and vision disturbances. Neuroimaging revealed an extra-axial parietooccipital tumor, with intratumoural bleed. Histopathology of both tumors showed features of CCM, immunopositive for epithelial membrane antigen (EMA and vimentin. This case illustrates multiple unusual features of a rare variant of meningioma in the form of affection of an adult age group, supratentorial location, recurrence, and intratumoral bleed. It also highlights the importance of incorporating immunohistochemistry in the diagnostic workup, to exclude CCM mimics, each having distinctive biological behavior, and prognostic outcome, and warranting different therapeutic protocols.

  13. Cervical spinal extradural meningioma: Case report.

    Science.gov (United States)

    Benzagmout, M; Azzal, F; Bitar, A; El Faïz Chaoui, M; Van Effenterre, R

    2010-10-01

    Extradural meningiomas account for approximately 7% of all spinal meningiomas and are most commonly located in the thoracic spine. Although rare, they should be included in the differential diagnosis of an extradural contrast-enhancing mass. Prognosis depends greatly on the extent of surgical resection. If considered safe, complete resection should be attempted to decrease the risk of recurrence. We report a case of spinal epidural meningioma diagnosed in an elderly woman complaining of right cervicobrachial pain of several years duration, associated with diffuse paresthesia and permanent numbness of her right index finger. The surgical removal of the tumor was incomplete because of the deep lateral extension of the lesion. Copyright © 2010 Elsevier Masson SAS. All rights reserved.

  14. Pediatric spinal clear cell meningioma. Case report.

    Science.gov (United States)

    Colen, Chaim B; Rayes, Mahmoud; McClendon, Jamal; Rabah, Raja; Ham, Steven D

    2009-01-01

    In this report the authors describe a unique case of spinal clear cell meningioma in a 13-year-old girl. Clear cell meningiomas (CCMs) are not uncommon. To the authors' knowledge, 14 cases of pediatric CCM occurring in the spinal canal have been reported. Factors lending resistance to meningioma initiation and invasion are analyzed. This 13-year-old girl presented with pain radiating down her left leg. Admission MR imaging showed an inhomogeneous enhancing intradural-extramedullary mass at the L4-5 level. Resection revealed a CCM, and radiotherapy was subsequently administered. Postoperatively there has been no recurrence in > 2 years. In this paper the authors report a case of CCM and provide a comprehensive literature review on this disease. Current recommendations for its management are still debatable, especially in the pediatric population, and the authors propose an algorithm for its treatment and surveillance.

  15. Spinal subdural hematoma following meningioma removal operation.

    Science.gov (United States)

    Jun, Hyo Sub; Oh, Jae Keun; Park, Young Seok; Song, Joon Ho

    2014-03-01

    Although blood contamination of cerebrospinal fluid (CSF) after an intracranial operation can occur, the development of a symptomatic spinal hematoma after craniotomy has been anecdotally reported and it is uncommon reported after a supratentorial meningioma removal operation. We report a case of spinal subdural hematoma following a supratentorial meningioma removal operation and discuss the mechanism of spinal subdural hematoma (SSDH) development. A 54-year-old woman presented with lumbago and radicular pain on both legs 4 days after a right parietooccipital craniotomy for meningioma removal. Only the straight leg raising sign was positive on neurologic examination but the magnetic resonance imaging (MRI) demonstrated a lumbosacral spinal subdural hematoma. The patient received serial lumbar tapping, after which her symptoms showed improvement.

  16. Primary pulmonary meningioma: A case report.

    Science.gov (United States)

    Huang, Shucheng; Chen, Li; Mao, Yuping; Tong, Hongwei

    2017-05-01

    Primary extracranial meningiomas are rare outside the head and neck region. A 44-year-old female patient had chest pain for more than 1 year. Preoperative chest computed tomography (CT) scan revealed a nodule in the right lower lobe, 1.8 cm in diameter. Tumor tissues were examined by immunohistochemistry for vimentin and S-100. Histopathologically, the tumor was characterized by whorled nests of spindle-shaped cells accompanied by psammoma bodies. Immunohistochemistry demonstrated tumor cell positivity for vimentin and S-100. This case was diagnosed as a primary pulmonary meningioma. The tumor was removed by a thoracoscopic pulmonary wedge resection. Postoperative cranial and spinal CT scan did not show any intracranial or spinal mass. No recurrence of the tumor was reported at the time of writing up this case report. A primary pulmonary meningioma should be considered in the differential diagnosis workup of pulmonary nodules.

  17. Radiosurgical planning of meningiomas: compromises with conformity.

    Science.gov (United States)

    Rowe, Jeremy G; Walton, Lee; Vaughan, Paul; Malik, Irfan; Radatz, Matthias; Kemeny, Andras

    2004-01-01

    The radiosurgical planning of meningiomas frequently necessitates compromises between irradiating tumour and risking damage to adjacent structures. In selected cases, we resolved this by excluding part of the tumour from the prescription isodose volume. Most of these compromises or 'suboptimal' plans achieved growth control. Growth control could not be related to conformity indices or to various measures of the radiation dose received by the meningioma. Examining recurrences, 75% arose from dura outside the original treatment field. These findings are discussed in terms of dose prescription protocols and the use of conformity indices in planning. The importance of the dural origin of meningiomas is well established in surgical practice, as reflected by Simpson's grades, but may be equally significant in radiosurgical practice.

  18. Giant velum interpositum meningioma in a child.

    Science.gov (United States)

    Moiyadi, Aliasgar V; Shetty, Prakash

    2012-07-01

    Intraventricular meningiomas are rare, but are relatively more often seen in children. Large size at presentation often obscures anatomical details. A particular subset of such tumors arising from the velum interpositum pose a significant surgical challenge. Thorough preoperative imaging, especially with respect to the course of the deep venous structures, provides useful evidence as to the origin. Preservation of venous anatomy at surgery is vital. We describe a 3-year-old girl with a giant velum interpositum meningioma that was completely excised with excellent outcome. This is probably the youngest such case reported.

  19. Meningiomas of the velum interpositum: surgical considerations.

    Science.gov (United States)

    Lozier, Alan P; Bruce, Jeffrey N

    2003-07-15

    Meningiomas of the third ventricle are a rare subtype of pineal region tumor that arise from the posterior portion of the velum interpositum, the double layer of pia mater that forms the roof of the third ventricle. The authors review the literature concerning these meningiomas and present a case in which the lesion was resected via the supracerebellar-infratentorial approach. The relationship of the tumor to the deep venous system and the splenium of the corpus callosum guides the selection of the most advantageous surgical approach. Posterior displacement of the internal cerebral veins demonstrated on preoperative imaging provides a strong rationale for use of the supracerebellar-infratentorial approach.

  20. Giant velum interpositum meningioma in a child

    Directory of Open Access Journals (Sweden)

    Aliasgar V Moiyadi

    2012-01-01

    Full Text Available Intraventricular meningiomas are rare, but are relatively more often seen in children. Large size at presentation often obscures anatomical details. A particular subset of such tumors arising from the velum interpositum pose a significant surgical challenge. Thorough preoperative imaging, especially with respect to the course of the deep venous structures, provides useful evidence as to the origin. Preservation of venous anatomy at surgery is vital. We describe a 3-year-old girl with a giant velum interpositum meningioma that was completely excised with excellent outcome. This is probably the youngest such case reported.

  1. Progesterone and peripheral nerve regeneration

    Institute of Scientific and Technical Information of China (English)

    Fei Fan; Haichao Li; Yuwei Wang; Yanglin Zheng; Lianjun Jia; Zhihui Wang

    2006-01-01

    OBJECTIVE: To explore the effect of progesterone on peripheral nerve regeneration.DATA SOURCES: An online search of Medline and OVID databases was under taken to identify articles about progesterone and peripheral nerve regeneration published in English between January 1990 and June 2004 by using the keywords of "peripheral nerve, injury, progesterone, regeneration".STUDY SELECTION: The data were primarily screened, those correlated with progesterone and peripheral nerve regeneration were involved, and their original articles were further searched, the repetitive studies or reviews were excluded.DATA EXTRACTION: Totally 59 articles about progesterone and peripheral nerve regeneration were collected, and 26 of them were involved, the other 33 excluded ones were the repetitive studies or reviews.DATA SYNTHESIS: Recent researches found that certain amount of progesterone could be synthetized in peripheral nervous system, and the expression of progesterone receptor could be found in sensory neurons and Schwann cells. After combined with the receptor, endogenous and exogenous progesterone can accelerate the formation of peripheral nerve myelin sheath, also promote the axonal regeneration.CONCLUSION: Progesterone plays a role in protecting neurons, increasing the sensitivity of nerve tissue to nerve growth factor, and accelerating regeneration of nerve in peripheral nerve regeneration, which provides theoretical references for the treatment of demyelinated disease and nerve injury, as well as the prevention of neuroma, especially that the in vivo level of progesterone should be considered for the elderly people accompanied by neuropathy and patients with congenital luteal phase defect, which is of positive significance in guiding the treatment.

  2. Diagnosis and Differential Diagnosis of Rhabdoid Meningioma:One Case Report and Literature Review

    Institute of Scientific and Technical Information of China (English)

    Huixia Han; Ping Du; Yan Zhang; Yongjian Deng

    2009-01-01

    @@ Introduction Rhabdoid meningioma (RM) is a special type of meningioma. The pathologic features of RM include sheets of rhabdoid tumor cells with or without the findings typical of conventional meningiomas.

  3. Discrimination between spinal extradural meningioma and both intra and extradural meningioma: Case Report.

    Science.gov (United States)

    Ito, Kenyu; Imagama, Shiro; Ando, Kei; Kobayashi, Kazuyoshi; Hida, Tetsuro; Tsushima, Mikito; Matsumoto, Akiyuki; Morozumi, Masayoshi; Tanaka, Satoshi; Nishida, Yoshihiro; Ishiguro, Naoki

    2017-02-01

    Meningiomas are common spinal tumor and mostly located at intradura. Recurrence rate after surgery for extradural meningioma was higher than intradural meningioma. A patient with intra and extradural spinal meningioma was treated and discussed its features and clinical management. A 41-year-old woman noted numbness of bilateral legs, gait disturbance, and mild bladder disturbance for over the two-month period. Magnetic resonance imaging revealed an epidural mass at T8-9 involving the dura mater from the left side. Intraoperatively, ultrasonography showed extradural tumor suppress the dura from the left side and no subarachnoid space. Therefore, it was difficult to diagnose the tumor was located at only extradural or both intra and extradural. The extradural tumor was resected as much as possible, then ultrasonography was performed for the second time. The spinal cord was decompressed and subarachnoid space was appeared with intradural tumor. The dura mater was opened in the midline for intradural exploration, and the intradural tumor appeared beside the spinal cord. The dura mater with attachment to the tumor was rescected. To prevent a recurrence, ultrasonography after removing extradural tumor is recommended to detect intra dural tumor and invasion of tumor into dura mater. In such case, removing only extradural meningioma is not enough, and gross total resection including intradural meningioma and dura mater is required.

  4. [Cytogenetic aberrations in histologically benign infiltratively growing sphenoid wing meningiomas].

    Science.gov (United States)

    Korshunov, A G; Cherekaev, V A; Bekiashev, A Kh; Sycheva, R V

    2007-01-01

    Meningiomas of the sphenoid wing (SW) frequently show an invasive pattern of growth and cause destruction of the adjacent structures. As a result, the rate of recurrent SW meningiomas is as high as 30%. Cytogenetic investigations showed no aberrations specific to invasively growing meningiomas. During this study, the authors evaluated 10 invasive and 5 non-invasive SW meningiomas via comparative genome hybridization (CGH) (matrix CGH), by using the gene chips of GenoSensor Array micromatrixes. The mean number of aberrations in the tumor cells was much greater in case of invasive meningiomas (67.4 versus 40.5 in case of non-invasive SW meningiomas. Furthermore, in invasive SW meningiomas, there were frequently losses in loci 1p, 6q, and 14q and gains in loci 15q and 10, which had been predetermined as molecular markers of stepwise progression of meningioma. Thus, the presence of a complex cytogenetic profile and progression-associated chromosome aberrations in benign SW meningiomas is linked with the increase of their invasive potential. Due to the fact that there are no well-defined adjuvant therapy regimens for recurring meningiomas at present, the revealed genomic aberrations may become potential targets for searching for drugs and a therapeutic intervention in future.

  5. Signals of apoptotic pathways in several types of meningioma.

    Science.gov (United States)

    Sabbatini, Maurizio; Comi, Cristoforo; Chiocchetti, Annalisa; Piffanelli, Valentina; Car, Pier Giorgio; Dianzani, Umberto; Monaco, Francesco; Cannas, Mario

    2011-03-01

    Meningiomas are intracranial tumour derived from meningothelial cells, which aggressive behaviour has been frequently associated to cell apoptosis. In this paper activation of several factors involved in apoptosis has been investigated on biopsies of primary, non recurrent meningiomas. Benign (meningotheliomatous, transitional, fibrous, angiomatous), atypical and anaplastic meningiomas were analysed by immunohistochemistry and western blot, to visualize the occurring of different apoptotic pathways and their association with clinical grading. Apoptotic cell have been detected by a double colorimetric staining for TUNEL and caspase-3 active form. Apoptotic signal positive cells have been detected in all type of meningiomas analysed, with exception of meningotheliomatous meningiomas. Differences have been found in the activation of apoptotic pathways between several types of grade I meningiomas and among benign, anaplastic and atypical meningiomas. An intense expression of several apoptotic inhibitor occurred in grade I meningiomas. The correlation among expression of apoptotic and inhibitory factors and cell proliferation index may suggest that in grade I meningiomas apoptosis may be related to mechanisms involved into tumor cells surviving. Instead in grade II and III meningiomas the same correlation seems indicate an high turnover of tumor cells that might be useful as index of cell proliferation and tumor mass growth.

  6. 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.

  7. Chitosan conduits combined with nerve growth factor microspheres repair facial nerve defects

    Institute of Scientific and Technical Information of China (English)

    Huawei Liu; Weisheng Wen; Min Hu; Wenting Bi; Lijie Chen; Sanxia Liu; Peng Chen; Xinying Tan

    2013-01-01

    Microspheres containing nerve growth factor for sustained release were prepared by a compound method, and implanted into chitosan conduits to repair 10-mm defects on the right buccal branches of the facial nerve in rabbits. In addition, chitosan conduits combined with nerve growth factor or normal saline, as wel as autologous nerve, were used as controls. At 90 days post-surgery, the muscular atrophy on the right upper lip was more evident in the nerve growth factor and normal sa-line groups than in the nerve growth factor-microspheres and autologous nerve groups. Electro-physiological analysis revealed that the nerve conduction velocity and amplitude were significantly higher in the nerve growth factor-microspheres and autologous nerve groups than in the nerve growth factor and normal saline groups. Moreover, histological observation il ustrated that the di-ameter, number, alignment and myelin sheath thickness of myelinated nerves derived from rabbits were higher in the nerve growth factor-microspheres and autologous nerve groups than in the nerve growth factor and normal saline groups. These findings indicate that chitosan nerve conduits com-bined with microspheres for sustained release of nerve growth factor can significantly improve facial nerve defect repair in rabbits.

  8. Meningiomas intraosseos: relato de 2 casos

    Directory of Open Access Journals (Sweden)

    Gilberto Machado de Almeida

    1975-09-01

    Full Text Available Meningiomas primários da calota craniana são raros. Na literatura de língua inglesa apenas 12 casos foram relatados. São apresentados 2 casos e discutidos aspectos etiológicos, clínicos e radiológicos.

  9. Meningioma of Foramen Magnum Causing Drop Attacks

    Science.gov (United States)

    Mahore, Amit; Mavani, Sandip; Rangarajan, Vithal; Patil, Manoj; Sathe, Prashant; Kawale, Juhi; Tikeykar, Vishakha

    2015-01-01

    A 52-year-old female presented with frequent episodes of falls without loss of consciousness. These episodes lasted for brief period followed by full neurological recovery. Magnetic resonance imaging (MRI) of the brain showed foramen magnum meningioma encasing left vertebral artery. The patient had dramatic improvement after excision of the tumor. PMID:25793133

  10. Meningioma of Foramen Magnum Causing Drop Attacks

    Directory of Open Access Journals (Sweden)

    Amit Mahore

    2015-01-01

    Full Text Available A 52-year-old female presented with frequent episodes of falls without loss of consciousness. These episodes lasted for brief period followed by full neurological recovery. Magnetic resonance imaging (MRI of the brain showed foramen magnum meningioma encasing left vertebral artery. The patient had dramatic improvement after excision of the tumor.

  11. Carbon dioxide laser surgery of basal meningiomas.

    Science.gov (United States)

    Bartal, A D; Heilbronn, Y D; Avram, J; Razon, N

    1982-02-01

    This preliminary report summarizes our results with laser surgery in patients with basal meningiomas (8 basilar and 1 intraventricular extending also into the third ventricle). Illustrative preoperative and postoperative CT scans are included. The advantages and limitations of this surgical technique are discussed briefly.

  12. Paraclinoid aneurysm concealed by sphenoid wing meningioma.

    Science.gov (United States)

    Petrecca, Kevin; Sirhan, Denis

    2009-02-01

    The coexistence of brain tumours and aneurysms is rare. In all previously reported cases the aneurysm was detectable by angiography. We report here a case in which a paraclinoid internal carotid artery aneurysm was coexistent and concealed from angiographic detection by an adjacent parasellar meningioma.

  13. Clinical and pathological features of intracranial meningiomas in children: A retrospective analysis

    Institute of Scientific and Technical Information of China (English)

    Kun Yang; Lisheng He; Linsun Dai; Jian Chen; Zhixiong Lin

    2006-01-01

    nerve damage in 4cases(27%,4/15),instability of gait and incoordination in 2 cases,conscious disturbance in 3 cases,nystagmus in 2 cases and lump under scalp in 1 case.②Imaging examination:Most of tumor CT plain scans were slightly high-density imaging. Most enhanced scanning appeared well-distributed enhancement and clear boundary.MRI examination observed the lumor appeared equal or long T1 or longer T2 abnormal mixed singles with clear boundary.Two cases had cystic lesion of tumor and 8 cases had peritumor edema.Digital substraction angiography(DSA)observed that theirs obvious tumor staining and displecement of corresponding brain vessels due to compression in 2 cases.③Preoperative misdiagnosis:Six children(40%,6/15)were misdiagnosed to have other diseases due to the untypical symptoms,including diseases of gastrointestinal tract in 3 cases.eye diseases in 2 cases.and encephalitis in 1 case.Most cases were diagnosed after CT or MRI scanning.④Sites and sizes of tumors:Tumor located at supratentodal parts in 13 cases(87%.13,15),including cerebral convexity in 7 cases(46.7%.7/15),intraventdcle in 2 cases(13.3%,2/15),carebral parenchyma in 2 cases(13.3%,2/15),parafalx and parasagittal sinus in 2 cases respectively.Tumor located at infratentodal part in 2 cases(13.3%,2/15),which at cerebellar hemisphere and cerebellopontine angle respectively.The maximal diameter of tumor was 1.3-8.0 cm with an average of 4.6 cm.⑤Pathological types: The pathoiogical examination reported that the meningioma was benign in 11 cases(73.3%,11/15)and malignant in 4 cases(26.7%,4/15).Among the benign meningiomas,it was syncytial in 5 cases.fibrous in 4 cases,and transitional in 2 cases.⑥Treatments:All the menigiomas of children were resected.and 14 cases(93.3%,14/15)had the tumor resected totally (Simpson grades Ⅰ-Ⅲ),including Simpson grade Ⅰ resection in 7 cases,grade Ⅱ in 4 Cases,and grade Ⅲ in 3 cases.One case only underwent subtotal resection(Simpson Ⅳ),this Case had

  14. 脊柱原发恶性外周神经鞘膜瘤的诊断与治疗%Management of primary spinal intraosseous malignant peripheral nerve sheath tumor

    Institute of Scientific and Technical Information of China (English)

    祝斌; 刘晓光; 刘忠军; 姜亮; 韦峰; 马庆军; 党耕町

    2010-01-01

    目的:探讨脊柱原发恶性外周神经鞘膜瘤(malignant peripheral nerve sheat tumor,MPNST)的诊断和治疗方法.方法:1998年1月~2008年1月我科收治3例表现为椎体内病变的脊柱MPNST患者,均为女性,年龄分别为24、41、42岁.从出现症状到临床确诊的时间1~12个月,平均5个月.累及胸椎1例,腰椎2例.术前均行CT引导下病灶穿刺活检明确诊断为MPNST.均采用肿瘤包膜外分离、分块切除术.结果:3例患者术中平均出血4000ml,平均手术时间482min,2例患者肿物完全切除,1例患者肿物次全切除.术后症状均缓解.分别于术后3、4、30个月复发,2例复发后未再行手术治疗,其中1例行序贯放疗,分别于术后5个月、11个月死亡:1例复发后3次行肿瘤刮除术.末次术后行肿瘤疫苗治疗,末次术后随访20个月无复发.结论:脊柱原发MPNST罕见,影像学表现缺乏特异性,病灶穿刺取活组织行组织学及免疫组化检查是术前诊断的主要方法.手术可以缓解症状,但易复发,放疗对控制肿瘤复发效果不确切.

  15. Uptake of AV-1451 in meningiomas.

    Science.gov (United States)

    Bruinsma, Tyler J; Johnson, Derek R; Fang, Ping; Senjem, Matthew; Josephs, Keith A; Whitwell, Jennifer L; Boeve, Bradley F; Pandey, Mukesh K; Kantarci, Kejal; Jones, David T; Vemuri, Prashanthi; Murray, Melissa; Graff-Radford, Jonathan; Schwarz, Christopher G; Knopman, David S; Petersen, Ronald C; Jack, Clifford R; Lowe, Val J

    2017-09-08

    AV-1451 is an imaging agent labeled with the positron-emitting radiolabel Fluorine-18. 18F-AV-1451 binds paired helical filament tau (PHF-tau), a pathology related to Alzheimer's disease. In our study of AV-1451 uptake in the brains of cognitively normal subjects, we noted a case of a meningioma with visually significant uptake of AV-1451. We initiated the present retrospective study to further examine cases of meningioma that underwent AV-1451 imaging. We searched the patient records of 650 patients who had undergone AV-1451 at our institution for the keyword "meningioma" to identify potential cases. PET/CT and MRI results were visually reviewed and semi-quantitative analysis of PET was performed. A paired student's t test was run between background and tumor standard uptake values. Fisher's exact test was used to examine the association between AV-1451 uptake and presence of calcifications on CT. We identified 12 cases of meningioma, 58% (7/12) of which demonstrated uptake greater than background using both visual analysis and tumor-to-normal cortex ratios (T/N + 1.90 ± 0.83). The paired student's t test revealed no statistically significant difference between background and tumor standard uptake values (p = 0.09); however, cases with a T/N ratio greater than one showed statistically higher uptake in tumor tissue (p = 0.01). A significant association was noted between AV-1451 uptake and presence of calcifications (p = 0.01). AV-1451 PET imaging should be reviewed concurrently with anatomic imaging to prevent misleading interpretations of PHF-tau distribution due to meningiomas.

  16. Revisiting the plasma sheath - dust in plasma sheath

    CERN Document Server

    Das, G C; Bora, M P

    2015-01-01

    In this work, we have considered the formation of warm plasma sheath in the vicinity of a wall in a plasma with considerable presence of dust particles. As an example, we have used the parameters relevant in case of lunar plasma sheath, though the results obtained in this work could be applied to any other physical situation such as laboratory plasma. In the ion-acoustic time scale, we neglect the dust dynamics. The dust particles affect the sheath dynamics by affecting the Poisson equation which determines the plasma potential in the sheath region. We have assumed the current to a dust particle to be balanced throughout the analysis. This makes the grain potential dependent on plasma potential, which is then incorporated into the Poisson equation. The resultant numerical model becomes an initial value problem, which is described by a 1-D integro-differential equation, which is then solved self-consistently by incorporating the change in plasma potential caused by inclusion of the dust potential in the Poisso...

  17. 5-Aminolevulinic acid fluorescence-guided surgery for spinal meningioma.

    Science.gov (United States)

    Muroi, Carl; Fandino, Javier; Coluccia, Daniel; Berkmann, Sven; Fathi, Ali-Reza; Landolt, Hans

    2013-01-01

    Fluorescence-guided surgery for cranial meningioma has been reported to be useful. There are no reports about spinal cases using this technique. We report on a meningioma of the cervical spine for which fluorescence-guided surgery was used. A 78-year-old female patient with a meningioma located in the cervical spine underwent surgery using fluorescence guidance. After complete removal of the meningioma and removal and coagulation of the dural attachment (equal to Simpson grade II resection), a fluorescence-positive remnant could be identified and successfully removed. The remnant was found to harbor meningioma tissue on histological examination. Fluorescence-guided microsurgery is helpful in achieving a total resection of spinal meningiomas, and might therefore reduce the risk of recurrence. Copyright © 2013 Elsevier Inc. All rights reserved.

  18. Atypical meningioma and extensive calvarium defects in neurofibromatosis type 1

    Energy Technology Data Exchange (ETDEWEB)

    Simsek, Enver [Department of Paediatrics, Duzce Medical Faculty, Abant Izzet Baysal University, Konuralp-Duzce (Turkey); Yavuz, Cevdet [Department of Neurosurgery, Duzce Medical Faculty, Abant Izzet Baysal University, Konuralp-Duzce (Turkey); Ustundag, Nil [Department of Pathology, Abant Izzet Baysal University School of Medicine, Konuralp-Duzce (Turkey)

    2003-08-01

    A 9-year-old girl with neurofibromatosis type 1 (NF1) presented with a massive atypical meningioma and calvarial defect. Skull radiographs and cranial CT showed an extensive lytic bone lesion at the vertex. MRI demonstrated a large mass invading the calvarium and sagittal sinus. The histopathological and immunohistochemical diagnosis of the resected mass was atypical meningioma. To our knowledge, this is the first case of NF1 associated with atypical meningioma and massive calvarial defect in a child. (orig.)

  19. Paediatric intraventricular meningiomas. A report of two cases

    Directory of Open Access Journals (Sweden)

    Sushma Ramraje

    2012-02-01

    Full Text Available Meningiomas are rare in children comprising less than 3% of paediatric brain tumours and only 1.5-1.8% of all intracranial neoplasms. Intraventricular meningiomas (IVM account for 0.5-5% of all meningiomas. They arise in the ventricles from arachnoid cap cells contained within the choroid plexus, the tela choroidea, or the velum interpositum. Paediatric tumours also show an association with neurofibromatosis type 2 and previous radiation exposure.We present two cases of intraventricular meningiomas, both in children. The age and site of the lesion in the two cases are uncommon. Excision of the lesions caused a total subsidence of the symptoms with no reported recurrences.

  20. Preoperative subtyping of meningiomas by perfusion MR imaging

    Energy Technology Data Exchange (ETDEWEB)

    Zhang, Hao [University Medical Center Groningen, University of Groningen (Netherlands); Shanghai Jiaotong University affiliated First People' s Hospital, Department of Radiology, Shanghai (China); Department of Radiology, University of Groningen (Netherlands); Roediger, Lars A.; Oudkerk, Matthijs [University Medical Center Groningen, University of Groningen (Netherlands); Department of Radiology, University of Groningen (Netherlands); Shen, Tianzhen [Fudan University Huashan Hospital, Department of Radiology, Shanghai (China); Miao, Jingtao [Shanghai Jiaotong University affiliated First People' s Hospital, Department of Radiology, Shanghai (China)

    2008-10-15

    This paper aims to evaluate the value of perfusion magnetic resonance (MR) imaging in the preoperative subtyping of meningiomas by analyzing the relative cerebral blood volume (rCBV) of three benign subtypes and anaplastic meningiomas separately. Thirty-seven meningiomas with peritumoral edema (15 meningothelial, ten fibrous, four angiomatous, and eight anaplastic) underwent perfusion MR imaging by using a gradient echo echo-planar sequence. The maximal rCBV (compared with contralateral normal white matter) in both tumoral parenchyma and peritumoral edema of each tumor was measured. The mean rCBVs of each two histological subtypes were compared using one-way analysis of variance and least significant difference tests. A p value less than 0.05 indicated a statistically significant difference. The mean rCBV of meningothelial, fibrous, angiomatous, and anaplastic meningiomas in tumoral parenchyma were 6.93{+-}3.75, 5.61{+-}4.03, 11.86{+-}1.93, and 5.89{+-}3.85, respectively, and in the peritumoral edema 0.87{+-}0.62, 1.38{+-}1.44, 0.87{+-}0.30, and 3.28{+-}1.39, respectively. The mean rCBV in tumoral parenchyma of angiomatous meningiomas and in the peritumoral edema of anaplastic meningiomas were statistically different (p<0.05) from the other types of meningiomas. Perfusion MR imaging can provide useful functional information on meningiomas and help in the preoperative diagnosis of some subtypes of meningiomas. (orig.)

  1. Stereological estimation of nuclear mean volume in invasive meningiomas

    DEFF Research Database (Denmark)

    Madsen, C; Schrøder, H D

    1996-01-01

    A stereological estimation of nuclear mean volume in bone and brain invasive meningiomas was made. For comparison the nuclear mean volume of benign meningiomas was estimated. The aim was to investigate whether this method could discriminate between these groups. We found that the nuclear mean...... volume in the bone and brain invasive meningiomas was larger than in the benign tumors. The difference was significant and moreover it was seen that there was no overlap between the two groups. In the bone invasive meningiomas the nuclear mean volume appeared to be larger inside than outside the bone...

  2. [Primary osteolytic intraosseous meningioma of the occipital bone].

    Science.gov (United States)

    Bernal-García, Luis Miguel; Cabezudo-Artero, José Manuel; Marcelo-Zamorano, María Bella; Fernández-Alarcón, Luis; Gilete-Tejero, Ignacio

    2015-01-01

    Primary intraosseous meningiomas are considered extradural meningiomas when no dural attachment is present. Most of them arise from the cranial bones and can present either as an osteoblastic or an osteolytic lesion. Osteolytic intraosseous meningiomas are the rarest and very few cases have been reported. Given that many of these may develop signs of malignancy, early histological confirmation is important in order to ensure appropriate treatment. The recommended therapy is surgery, with complete resection whenever possible. We present the case of a large primary intraosseous osteolytic meningioma within the occipital bone, which was completely excised five years ago, currently presenting no signs of recurrence.

  3. Cavernous Hemangioma of the Skull and Meningioma: Association or Coincidence?

    Directory of Open Access Journals (Sweden)

    M. Kilani

    2015-01-01

    Full Text Available Intraosseous cavernous hemangiomas of the skull are rare. Meningiomas are quite frequently encountered in a neurosurgical practice. The association between these two entities is nevertheless very uncommon. The authors present a case of a 72-year-old woman suffering from headache. The MRI showed a parietal meningioma with adjacent thick bone. The meningioma and the bone were removed. The histological examination confirmed the diagnosis of meningioma and revealed a cavernoma of the skull. The relationship between the lesions suggests more than a coincidental association. Several hypotheses are proposed to explain common causal connections.

  4. Loss of SUFU Function in Familial Multiple Meningioma

    Science.gov (United States)

    Aavikko, Mervi; Li, Song-Ping; Saarinen, Silva; Alhopuro, Pia; Kaasinen, Eevi; Morgunova, Ekaterina; Li, Yilong; Vesanen, Kari; Smith, Miriam J.; Evans, D. Gareth R.; Pöyhönen, Minna; Kiuru, Anne; Auvinen, Anssi; Aaltonen, Lauri A.; Taipale, Jussi; Vahteristo, Pia

    2012-01-01

    Meningiomas are the most common primary tumors of the CNS and account for up to 30% of all CNS tumors. An increased risk of meningiomas has been associated with certain tumor-susceptibility syndromes, especially neurofibromatosis type II, but no gene defects predisposing to isolated familial meningiomas have thus far been identified. Here, we report on a family of five meningioma-affected siblings, four of whom have multiple tumors. No NF2 mutations were identified in the germline or tumors. We combined genome-wide linkage analysis and exome sequencing, and we identified in suppressor of fused homolog (Drosophila), SUFU, a c.367C>T (p.Arg123Cys) mutation segregating with the meningiomas in the family. The variation was not present in healthy controls, and all seven meningiomas analyzed displayed loss of the wild-type allele according to the classic two-hit model for tumor-suppressor genes. In silico modeling predicted the variant to affect the tertiary structure of the protein, and functional analyses showed that the activity of the altered SUFU was significantly reduced and therefore led to dysregulated hedgehog (Hh) signaling. SUFU is a known tumor-suppressor gene previously associated with childhood medulloblastoma predisposition. Our genetic and functional analyses indicate that germline mutations in SUFU also predispose to meningiomas, particularly to multiple meningiomas. It is possible that other genic mutations resulting in aberrant activation of the Hh pathway might underlie meningioma predisposition in families with an unknown etiology. PMID:22958902

  5. Analysis of subtotal resection strategy in treating petroclival meningiomas via retrosigmoid approach

    Directory of Open Access Journals (Sweden)

    Qing-hai YAO

    2016-12-01

    Full Text Available  Objective To explore optimal resection goals of petroclival meningiomas resected via retrosigmoid approach for improving quality life of patients after operation. Methods A total of 39 cases with petroclival meningiomas who underwent microsurgical removal via retrosigmoid approach from January 2006 to December 2013 in our hospital were retrospectively analyzed. Results Gross total resection was performed in 11 patients (28.21% , and subtotal resection was performed in 28 patients (71.79% . The occurrence rate of new cranial nerve injury or aggravated original cranial nerve injury in gross total resection group was significantly higher than that in subtotal resection group [6 patients (6/11 vs 4 patients (14.29%, Fisher exact probability: P = 0.017]. Postoperative Karnofsky Performance Status (KPS score in gross total resection group was significantly lower than that in subtotal resection group [(72.00 ± 9.19 score vs (82.69 ± 10.41 score; t = -2.844, P = 0.007]. There was no significant difference between 2 groups on the recurrence and/or progression rate after operation (Fisher exact probability: P = 0.545. There was no significant difference of the recurrence and/or progression rate between with or without gamma knife radiosurgery (GKRS after operation in subtotal resection group (Fisher exact probability: P = 0.529.  Conclusions Subtotal resection via retrosigmoid approach with or without gamma knife radiosurgery is reasonable and feasible strategy in the treatment of petroclival meningiomas. DOI: 10.3969/j.issn.1672-6731.2016.12.010

  6. Nerve Regeneration in the Peripheral Nervous System versus the Central Nervous System and the Relevance to Speech and Hearing after Nerve Injuries

    Science.gov (United States)

    Gordon, Tessa; Gordon, Karen

    2010-01-01

    Schwann cells normally form myelin sheaths around axons in the peripheral nervous system (PNS) and support nerve regeneration after nerve injury. In contrast, nerve regeneration in the central nervous system (CNS) is not supported by the myelinating cells known as oligodendrocytes. We have found that: 1) low frequency electrical stimulation can be…

  7. Nerve Regeneration in the Peripheral Nervous System versus the Central Nervous System and the Relevance to Speech and Hearing after Nerve Injuries

    Science.gov (United States)

    Gordon, Tessa; Gordon, Karen

    2010-01-01

    Schwann cells normally form myelin sheaths around axons in the peripheral nervous system (PNS) and support nerve regeneration after nerve injury. In contrast, nerve regeneration in the central nervous system (CNS) is not supported by the myelinating cells known as oligodendrocytes. We have found that: 1) low frequency electrical stimulation can be…

  8. Concurrent spinal schwannoma and meningioma mimicking a single cervical dumbbell-shaped tumor: case report.

    Science.gov (United States)

    Oichi, Takeshi; Chikuda, Hirotaka; Morikawa, Teppei; Mori, Harushi; Kitamura, Daisuke; Higuchi, Junya; Taniguchi, Yuki; Matsubayashi, Yoshitaka; Oshima, Yasushi; Tanaka, Sakae

    2015-12-01

    Dumbbell-shaped tumors consisting of 2 different tumors are extremely rare. Herein, the authors present a case of concurrent spinal schwannoma and meningioma mimicking a single cervical dumbbell-shaped tumor. A 64-year-old man presented with a 5-year history of gradually exacerbating left occipital pain without clinical evidence of neurofibromatosis. Magnetic resonance imaging showed an extradural tumor along the left C-2 nerve root with a small intradural component. The tumor was approached via a C-1 hemilaminectomy. The intradural tumor was resected together with the extradural tumor after opening the dura mater. The intradural tumor was attached to the dura mater around the exit point of the C-2 nerve root. Intraoperative biopsy revealed that the extradural tumor was a schwannoma and that the intradural tumor was a meningioma. The dura mater adjacent to the tumor was then coagulated and resected. Postoperative pathological examination confirmed the same diagnoses with no evidence of continuity between the intra- and extradural components. The patient's postoperative clinical course was uneventful. Clinicians should be aware that cervical dumbbell-shaped tumors can consist of 2 different tumors.

  9. Relationship between myelin sheath diameter and internodal length in axons of the anterior medullary velum of the adult rat.

    Science.gov (United States)

    Ibrahim, M; Butt, A M; Berry, M

    1995-11-01

    Relations between myelin sheath diameters and internodal lengths were measured in whole mounts of osmium stained intact anterior medullary velum (AMV) from glutaraldehyde perfused adult rats. The AMV is a sheet of CNS tissue which roofs the IVth ventricle and contains fascicles of myelinated fibres which arise mainly from the nucleus of the IVth cranial nerve. These fibers displayed a broad range of myelin sheath external diameters and internodal lengths, from 4 microns. Our results indicated that small and large calibre fibres may have different myelin sheath diameter-internodal length interrelations.

  10. Nerve conduction and electromyography studies.

    Science.gov (United States)

    Kane, N M; Oware, A

    2012-07-01

    Nerve conduction studies (NCS) and electromyography (EMG), often shortened to 'EMGs', are a useful adjunct to clinical examination of the peripheral nervous system and striated skeletal muscle. NCS provide an efficient and rapid method of quantifying nerve conduction velocity (CV) and the amplitude of both sensory nerve action potentials (SNAPs) and compound motor action potentials (cMAPs). The CV reflects speed of propagation of action potentials, by saltatory conduction, along large myelinated axons in a peripheral nerve. The amplitude of SNAPs is in part determined by the number of axons in a sensory nerve, whilst amplitude of cMAPs reflects integrated function of the motor axons, neuromuscular junction and striated muscle. Repetitive nerve stimulation (RNS) can identify defects of neuromuscular junction (NMJ) transmission, pre- or post-synaptic. Needle EMG examination can detect myopathic changes in muscle and signs of denervation. Combinations of these procedures can establish if motor and/or sensory nerve cell bodies or peripheral nerves are damaged (e.g. motor neuronopathy, sensory ganglionopathy or neuropathy), and also indicate if the primary target is the axon or the myelin sheath (i.e. axonal or demyelinating neuropathies). The distribution of nerve damage can be determined as either generalised, multifocal (mononeuropathy multiplex) or focal. The latter often due to compression at the common entrapment sites (such as the carpal tunnel, Guyon's canal, cubital tunnel, radial groove, fibular head and tarsal tunnel, to name but a few of the reported hundred or so 'entrapment neuropathies').

  11. Expression of nestin and Ki-67 in malignant peripheral nerve sheath tumors and its significance%巢蛋白和Ki-67在恶性外周神经鞘膜瘤中的表达及其意义

    Institute of Scientific and Technical Information of China (English)

    陆珊; 王华; 王玉萍; 杨郁; 钟延丰; 郑杰

    2012-01-01

    Objective To explore the expression of nestin and Ki-67 in malignant peripheral nerve sheath tumors (MPNST) and its significance in the differential diagnosis. Methods Immunohistochemical technique (SP) was used to detect the expression of nestin and Ki-67 in 42 cases of MPNST and 24 cases of benign peripheral nerve tumor.Results Total expression of nestin was found in 95.2 %(40/42) of MPNST.Strong expression of nestin was detected more frequent in MPNST compared to benign peripheral nerve tumors [40.5 %(17/42) versus 4.2 %(1/24),x2 =8.403,P =0.004].Ki-67 labeling index in MPNST varied from 1%-70 %.However,greater than 3 % labeling index of Ki-67 staining was observed in 64.3 %(27 / 42) of MPNST while none of the 24 benign tumors had nuclear staining exceeding 3 %. The higher Ki-67 labeling index showed significant differences between the two groups (x2 =23.518,P =0.000).Conclusion Nestin and Ki-67 are useful markers in distinguishing MPNST from benign tumors.%目的 探讨恶性外周神经鞘膜瘤(MPNST)中巢蛋白和细胞增殖核抗原Ki-67的表达情况及其意义.方法 采用免疫组织化学SP法检测42例MPNST中巢蛋白和Ki-67的蛋白表达.24例外周神经良性肿瘤作为对照.结果 MPNST中巢蛋白阳性表达率为95.2%(40/42),其中巢蛋白强阳性表达率为40.5%(17/42),明显高于外周神经良性肿瘤的4.2%(1/24),差异有统计学意义(x2=8.403,P=0.004);MPNST中Ki-67标记指数介于1%~70%之间,其中>3%的占64.3%(27/42),24例外周神经良性肿瘤中Ki-67标记指数均<3%,两者比较差异有统计学意义(x2=23.518,P=0.000).结论 联合检测巢蛋白和Ki-67免疫标志物有助于MPNST的诊断.

  12. Molecular biological determinations of meningioma progression and recurrence.

    Directory of Open Access Journals (Sweden)

    Stefan Linsler

    Full Text Available Meningiomas are tumors that arise from the coverings of the brain or spinal cord. 5% of the cases turn into malignant forms with aggressive clinical behavior and increased risk of tumor recurrence. One hundred and five patients with meningiomas were operated by open surgery. To investigate predictors of meningioma recurrence in total 124 samples of 105 patients were investigated by iFISH. Dual-probe hybridization was performed to access chromosomal alterations of chromosomes 1p-, 9p- and 22q. Additionally, methylation of TIMP3 and p16 was analyzed with MS-PCR. Of the 105 investigated tumors 59.1% (62/105 were WHO grade I, 33.3% (35/105 were WHO grade II and 7.7% (8/105 were anaplastic meningiomas (grade III, respectively. The histopathological data correlates with the recurrence rate of the investigated meningiomas. Hypermethylation of TIMP3 was detected in 13.3% of all meningiomas: 10.9% in WHO grade I meningiomas, 25.0% in grade II and 14.3% in grade III meningiomas, respectively. No correlation of TIMP3 hypermethylation with tumor recurrence or WHO grade (p = 0.2 was observed. Interestingly, deletion of 1p36 emerged as a significant predictor of shorter overall survival (log rank test, p<0.001, whereas TIMP3 promoter methylation had no significant effect on overall survival (log rank test, p = 0.799. The results of the current study support the finding that the deletion of chromosome 1p is an independent marker of meningioma recurrence and progression (p = 0.0097. Therefore the measurement of genetic aberrations in meningiomas allows in a combined histological approach a more precise assessment of the prognosis of meningiomas than histopathology alone.

  13. Tentorium schwannoma mimicking meningioma: an unusual location.

    Science.gov (United States)

    Calişaneller, Tarkan; Ozen, Ozlem; Altinörs, Nur; Caner, Hakan

    2008-07-01

    A 60-year-old female was admitted to our clinic complaining of a long-lasting headache. Cranial magnetic resonance imagining examination of the patient revealed a 22x24 mm extra-axial, well-demarcated, mass lesion based on the left tentorium. The patient underwent a craniotomy and the tumor was totally excised with the adjacent tentorium. The histopathological examination of the tumor complied with the diagnosis of schwannoma. The rest of the clinical course was uneventful and the patient was released from the hospital without any neurological deficit. Intracranial schwannomas can rarely originate from atypical dural locations and radiological techniques are not always helpful in distinguishing tentorial schwannoma from tentorial meningioma. We presented a patient with a tentorium schwannoma mimicking meningioma and discussed the current literature.

  14. Olfactory groove meningiomas: approaches and complications.

    Science.gov (United States)

    Aguiar, Paulo Henrique Pires de; Tahara, Adriana; Almeida, Antonio Nogueira; Simm, Renata; Silva, Arnaldo Neves da; Maldaun, Marcos Vinicius Calfatt; Panagopoulos, Alexandros Theodoros; Zicarelli, Carlos Alexandre; Silva, Pedro Gabriel

    2009-09-01

    Olfactory groove meningiomas (OGM) account for 4.5% of all intracranial meningiomas. We report 21 patients with OGMs. Tumors were operated on using three surgical approaches: bifrontal (7 patients), fronto-pterional (11 patients) and fronto-orbital (3 patients). Total tumor removal (Simpson Grade 1) was achieved in 13 patients and Simpson II in 8 patients. Perioperative mortality was 4.76%. The average size of the OGM was 4.3+/-1.1cm. The overall recurrence rate was 19%. We preferred to use the pterional approach, which provides quick access to the tumor with less brain exposure. It also allows complete drainage of cisternal cerebrospinal fluid, providing a good level of brain relaxation during surgery. However, for long, thin tumors, hemostasis can be difficult using this approach.

  15. Epidural Cystic Spinal Meningioma: A Case Report.

    Science.gov (United States)

    Zhang, Ji; Chen, Zheng-he; Wang, Zi-feng; Sun, Peng; Jin, Jie-tian; Zhang, Xiang-heng; Zhao, Yi-ying; Wang, Jian; Mou, Yong-gao; Chen, Zhong-ping

    2016-03-01

    Cystic spinal meningioma (CSM) is an uncommon meningioma variant. Extradural CSMs are particularly rare and difficult to distinguish from other intraaxial tumors. This study presents a case of a 36-year-old woman with intraspinal extradual CSM at the thoracolumbar spine. She experienced persistent weakness, progressive numbness, and sensory disturbance in the right lower limb. Magnetic resonance imaging (MRI) of the patient revealed an irregular cystic mass at the thoracic 11 to lumbar 3 levels dorsally. This case was misdiagnosed as other neoplasms prior to surgery because of the atypical radiographic features and location of the tumor. Extradural CSMs should be considered in the differential diagnosis of intraspinal extradural cystic neoplasms. Complete removal of cystic wall provides an optimal outcome, rendering the lesion curable.

  16. Treatment recommendations for primary extradural meningiomas.

    Science.gov (United States)

    Mattox, Austin; Hughes, Betsy; Oleson, James; Reardon, David; McLendon, Roger; Adamson, Cory

    2011-01-01

    Primary extradural meningiomas (PEMs) represent about 2% of all meningiomas and are often encountered by non-neurosurgeons. These lesions typically present as enlarging, painless, benign masses that can be surgically cured. Imaging is critical for defining involvement of adjacent structures; however, diagnosis depends on classic histologic patterns. Treatment for benign PEMs (WHO I) consists of resection with wide margins, whereas adjuvant therapy after resection of atypical (WHO II) or malignant (WHO III) PEMs should be considered. By using the collective experience from our comprehensive cancer center, including neuro-oncologists, neuroradiologists, and neurosurgeons, in addition to a complete literature review, the authors have established treatment guidelines not previously reported. This manuscript describes key features of these challenging tumors to aid in diagnosis, presents the largest published review of all reported PEMs (n = 163), and provides salient treatment guidelines to surgeons unfamiliar with these challenging tumors.

  17. Primary atypical sacral meningioma- not always benign

    Energy Technology Data Exchange (ETDEWEB)

    Bhadra, A.K.; Casey, A.T.H.; Saifuddin, A.; Briggs, T.W. [Royal National Orthopaedic Hospital, Stanmore, London (United Kingdom)

    2007-06-15

    We present a case of an atypical recurrent meningioma of the sacrum with pulmonary metastasis in a 31-year-old man. He presented with deep-seated buttock pain and urinary hesitancy for 3 months. MRI revealed a lesion occupying the central and left side of the sacral canal at the S1-S2 level. Surgical excision of the lesion via a posterior approach was undertaken, and the patient became symptom-free post-operatively. Histology confirmed atypical meningioma. Eight months later he re-presented with similar symptoms, and MRI confirmed local recurrence. The patient underwent left hemisacrectomy. Six months later he again presented with low back pain and MRI confirmed a second local recurrence. A CT scan of the chest showed multiple lung metastases. The patient died of a severe chest infection 18 months later. (orig.)

  18. Fourth ventricle meningiomas: a rare entity.

    Science.gov (United States)

    Pichierri, Angelo; Ruggeri, Andrea; Morselli, Carlotta; Delfini, Roberto

    2011-08-01

    Fourth ventricle meningiomas (FVMs) are rare, often misdiagnosed, lesions. To the best of our knowledge, 47 cases have been reported in the literature: we describe our series of three cases treated at our Institution, focusing on some diagnostic tips and intraoperative features of these tumours. Our three patients have a history of headache. Gait disturbances, vomiting and/or diplopia complicated the clinical picture before the referral at our Department. The operations were uneventful, and the patients fully recovered from neurological symptoms. They are free of recurrence at a median follow-up of 19 years. FVMs are rare lesions, which are difficult to differentiate preoperatively from the much more common ependymomas. A preoperative distinction would be extremely advantageous: indeed, although both tumours share similar radiological and clinical patterns, they clearly differ as to surgical difficulty and outcome. In fact, meningiomas are comparatively easier to remove, granting better clinical results.

  19. The far-lateral approach for foramen magnum meningiomas.

    Science.gov (United States)

    Flores, Bruno C; Boudreaux, Benjamin P; Klinger, Daniel R; Mickey, Bruce E; Barnett, Samuel L

    2013-12-01

    Foramen magnum meningiomas (FMMs) are slow growing, most often intradural and extramedullary tumors that pose significant challenges to the skull base neurosurgeon. The indolent clinical course of FMMs and their insidious onset of symptoms are important factors that contribute to delayed diagnosis and relative large size at the time of presentation. Symptoms are often produced by compression of surrounding structures (such as the medulla oblongata, upper cervical spinal cord, lower cranial nerves, and vertebral artery) within a critically confined space. Since the initial pathological description of a FMM in 1872, various surgical approaches have been described with the aim of achieving radical tumor resection. The surgical treatment of FMMs has evolved considerably over the last 4 decades due to the progress in microsurgical techniques and development of a multitude of skull base approaches. Posterior and posterolateral FMMs can be safely resected via a standard midline suboccipital approach. However, controversy still exits regarding the optimal management of anterior or anterolateral lesions. Independently of technical variations and the degree of bone removal, all modern surgical approaches to the lower clivus and anterior foramen magnum derive from the posterolateral (or far-lateral) craniotomy originally described by Roberto Heros and Bernard George. This paper is a review of the surgical management of FMMs, with emphasis on the far-lateral approach and its variations. Clinical presentation, imaging findings, important neuroanatomical correlations, recurrence rates, and outcomes are discussed.

  20. Stereotactic radiotherapy of meningiomas. Symptomatology, acute and late toxicity

    Energy Technology Data Exchange (ETDEWEB)

    Henzel, M.; Gross, M.W.; Failing, T.; Strassmann, G.; Engenhart-Cabillic, R. [Dept. of Radiation Oncology, Univ. of Gisssen (Germany); Dept. of Radiation Oncology, Marburg Univ. (Germany); Hamm, K.; Surber, G.; Kleinert, G. [Dept. of Stereotactic Neurosurgery and Radiosurgery, Helios Klinikum Erfurt (Germany)

    2006-07-15

    Background and purpose: stereotactic radiosurgery (SRS) is well established in the treatment of skull base meningiomas, but this therapy approach is limited to small tumors only. The fractionated stereotactic radiotherapy (SRT) offers an alternative treatment option. This study aims at local control, symptomatology, and toxicity. Patients and methods: between 1997-2003, 224 patients were treated with SRT (n= 183), hypofractionated SRT (n = 30), and SRS (n = 11). 95/224 were treated with SRT/SRS alone. 129/224 patients underwent previous operations. Freedom from progression and overall survival, toxicity, and symptomatology were evaluated systematically. Additionally, tumor volume (TV) shrinkage was analyzed three-dimensionally within the planning system. Results: the median follow-up was 36 months (range, 12-100 months). Overall survival and freedom from progression for 5 years were 92.9% and 96.9%. Quantitative TV reduction was 26.2% and 30.3% 12 and 18 months after SRT/SRS (p < 0.0001). 95.9% of the patients improved their symptoms or were stable. Clinically significant acute toxicity (CTC III ) was rarely seen (2.5%). Clinically significant late morbidity (III -IV ) or new cranial nerve palsies did not occur. Conclusion: SRT offers an additional treatment option of high efficacy with only few side effects. In the case of large tumor size (> 4 ml) and adjacent critical structures (< 2 mm), SRT is highly recommended. (orig.)

  1. Giant velum interpositum meningioma in a child

    OpenAIRE

    Aliasgar V Moiyadi; Prakash Shetty

    2012-01-01

    Intraventricular meningiomas are rare, but are relatively more often seen in children. Large size at presentation often obscures anatomical details. A particular subset of such tumors arising from the velum interpositum pose a significant surgical challenge. Thorough preoperative imaging, especially with respect to the course of the deep venous structures, provides useful evidence as to the origin. Preservation of venous anatomy at surgery is vital. We describe a 3-year-old girl with a giant ...

  2. Intradural extramedullary tuberculoma mimicking en plaque meningioma

    Directory of Open Access Journals (Sweden)

    Ozek Erdinc

    2009-01-01

    Full Text Available In this paper we report an 18 year old woman with an intradural extramedullary tuberculoma mimicking en plaque meningioma located in the thoracic region. The patient was operated via thoracic laminoplasty and tumor was totally resected. On the follow-up examination the magnetic resonance imaging demonstrated the total excision of the tumor. Here we describe a case of intradural extramedullary tuberculoma of the spinal cord as a complication of tuberculosis meningitis in a previously healthy young female.

  3. Meningioma in pregnancy: a rare case report

    Directory of Open Access Journals (Sweden)

    Raji Chinniah

    2016-04-01

    Full Text Available Central nervous system tumor in pregnancy is rare. We report a case of such a tumor diagnosed in the third trimester of pregnancy. Patient presented with signs of increased intracranial tension and MRI showed a meningioma. Caesarean section along with craniotomy and tumor excision done successfully in the same sitting. Mother and the baby recovered well. [Int J Reprod Contracept Obstet Gynecol 2016; 5(4.000: 1270-1272

  4. Meningioma in pregnancy: a rare case report

    OpenAIRE

    2016-01-01

    Central nervous system tumor in pregnancy is rare. We report a case of such a tumor diagnosed in the third trimester of pregnancy. Patient presented with signs of increased intracranial tension and MRI showed a meningioma. Caesarean section along with craniotomy and tumor excision done successfully in the same sitting. Mother and the baby recovered well. [Int J Reprod Contracept Obstet Gynecol 2016; 5(4.000): 1270-1272

  5. Foramen Magnum Meningioma: Dysphagia of Atypical Etiology

    Science.gov (United States)

    Tsang, Matthew W.; Mobley, Bret C.; Cheng, Walter W.

    2007-01-01

    INTRODUCTION We present a case of a foramen magnum meningioma that highlights the importance of the neurologic exam when evaluating a patient with dysphagia. A 58-year-old woman presented with an 18-month history of progressive dysphagia, chronic cough and 30-pound weight loss. Prior gastroenterologic and laryngologic workup was unrevealing. Results Her neurologic examination revealed an absent gag reflex, decreased sensation to light touch on bilateral distal extremities, hyperreflexia, and tandem gait instability. Repeat esophagogastroduodenoscopy was normal, whereas laryngoscopy and video fluoroscopy revealed marked hypopharyngeal dysfunction. Brain magnetic resonance imaging demonstrated a 3.1 × 2.7 × 2.9 cm foramen magnum mass consistent with meningioma. The patient underwent neurosurgical resection of her mass with near complete resolution of her neurologic symptoms. Pathology confirmed diagnosis of a WHO grade I meningothelial meningioma. Conclusion CNS pathology is an uncommon but impressive cause of dysphagia. Our case demonstrates the importance of a thorough neurologic survey when evaluating such a patient. PMID:18080720

  6. Extensive Growth of an Anaplastic Meningioma

    Directory of Open Access Journals (Sweden)

    Hajrullah Ahmeti

    2013-01-01

    Full Text Available We present the case of a 30-year-old male patient with an almost complete destruction of the calvarial bone through an anaplastic meningioma diagnosed in line with dizziness. Neuroimaging revealed an extensive growing, contrast enhancing lesion expanding at the supra- and infratentorial convexity, infiltrating and destroying large parts of the skull, and infiltrating the skin. Due to progressive ataxia and dysarthria with proven tumor growth in the posterior fossa in the continuing course, parts of the tumor were resected. A surgical procedure with the aim of complete tumor resection in a curative manner was not possible. Six months after the first operation, due to a new tumor progression, most extensive tumor resection was performed. Due to the aggressive and destructive growth with a high rate of recurrence and tendency of metastases, anaplastic meningiomas can be termed as malignant tumors. The extrinsic growth masks the tumor until they reach a size, which makes these tumors almost unresectable. In the best case scenarios, the five-year survival is about 50%. With the presented case, we would like to show the aggressive behavior of anaplastic meningiomas in a very illustrative way. Chemotherapy, radiotherapy, and surgery reach their limits in this tumor entity.

  7. Chemically extracted acellular allogeneic nerve graft combined with ciliary neurotrophic factor promotes sciatic nerve repair

    Institute of Scientific and Technical Information of China (English)

    Yanru Zhang; Hui Zhang; Kaka Katiella; Wenhua Huang

    2014-01-01

    A chemically extracted acellular allogeneic nerve graft can reduce postoperative immune re-jection, similar to an autologous nerve graft, and can guide neural regeneration. However, it remains poorly understood whether a chemically extracted acellular allogeneic nerve graft combined with neurotrophic factors provides a good local environment for neural regenera-tion. This study investigated the repair of injured rat sciatic nerve using a chemically extracted acellular allogeneic nerve graft combined with ciliary neurotrophic factor. An autologous nerve anastomosis group and a chemical acellular allogeneic nerve bridging group were prepared as controls. At 8 weeks after repair, sciatic functional index, evoked potential amplitude of the soleus muscle, triceps wet weight recovery rate, total number of myelinated nerve fibers and myelin sheath thickness were measured. For these indices, values in the three groups showed the autologous nerve anastomosis group > chemically extracted acellular nerve graft + ciliary neurotrophic factor group > chemical acellular allogeneic nerve bridging group. These results suggest that chemically extracted acellular nerve grafts combined with ciliary neurotrophic factor can repair sciatic nerve defects, and that this repair is inferior to autologous nerve anasto-mosis, but superior to chemically extracted acellular allogeneic nerve bridging alone.

  8. Ultrasound-guided rectus sheath block in children with umbilical hernia: Case series

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    Abdul Hamid Alsaeed

    2013-01-01

    Full Text Available Background: Umbilical hernia repair, a common day-case surgery procedure in children, is associated with a significant postoperative pain. The most popular peripheral nerve blocks used in umbilical hernia repair are rectus sheath infiltration and caudal block. The rectus sheath block may offer improved pain relief following umbilical hernia repair with no undesired effects such as lower limb motor weakness or urinary retention seen with caudal block which might delay discharge from the hospital. Ultrasound guidance of peripheral nerve blocks has reduced the number of complications and improved the quality of blocks.The aim of this case series is to assess the post rectus sheath block pain relief in pediatric patients coming for umbilical surgery. Methods: Twenty two (22 children (age range: 1.5-8 years scheduled for umbilical hernia repair were included in the study. Following the induction of general anesthesia, the ultrasonographic anatomy of the umbilical region was studied with a 5-16 MHz 50 mm linear probe. An ultrasound-guided posterior rectus sheath block of both rectus abdominis muscles (RMs was performed (total of 44 punctures. An in-plain technique using Stimuplex A insulated facet tip needle 22G 50mm. Surgical conditions, intraoperative hemodynamic parameters, and postoperative analgesia by means of the modified CHEOPS scale were evaluated. Results: ultrasonograghic visualization of the posterior sheath was possible in all patients. The ultrasound guided rectus sheath blockade provided sufficient analgesia in all children with no need for additional analgesia except for one patient who postoperatively required morphine 0.1 mg/kg intravenously. There were no complications. Conclusions: Ultrasound guidance enables performances of an effective rectus sheath block for umbilical hernia. Use of the Stimuplex A insulated facet tip needle 22G 50mm provides easy, less traumatic skin and rectus muscle penetration and satisfactory needle

  9. Ultrasound-guided rectus sheath block in children with umbilical hernia: Case series.

    Science.gov (United States)

    Alsaeed, Abdul Hamid; Thallaj, Ahmed; Khalil, Nancy; Almutaq, Nada; Aljazaeri, Ayman

    2013-10-01

    Umbilical hernia repair, a common day-case surgery procedure in children, is associated with a significant postoperative pain. The most popular peripheral nerve blocks used in umbilical hernia repair are rectus sheath infiltration and caudal block. The rectus sheath block may offer improved pain relief following umbilical hernia repair with no undesired effects such as lower limb motor weakness or urinary retention seen with caudal block which might delay discharge from the hospital. Ultrasound guidance of peripheral nerve blocks has reduced the number of complications and improved the quality of blocks. The aim of this case series is to assess the post rectus sheath block pain relief in pediatric patients coming for umbilical surgery. Twenty two (22) children (age range: 1.5-8 years) scheduled for umbilical hernia repair were included in the study. Following the induction of general anesthesia, the ultrasonographic anatomy of the umbilical region was studied with a 5-16 MHz 50 mm linear probe. An ultrasound-guided posterior rectus sheath block of both rectus abdominis muscles (RMs) was performed (total of 44 punctures). An in-plain technique using Stimuplex A insulated facet tip needle 22G 50mm. Surgical conditions, intraoperative hemodynamic parameters, and postoperative analgesia by means of the modified CHEOPS scale were evaluated. ultrasonograghic visualization of the posterior sheath was possible in all patients. The ultrasound guided rectus sheath blockade provided sufficient analgesia in all children with no need for additional analgesia except for one patient who postoperatively required morphine 0.1 mg/kg intravenously. There were no complications. Ultrasound guidance enables performances of an effective rectus sheath block for umbilical hernia. Use of the Stimuplex A insulated facet tip needle 22G 50mm provides easy, less traumatic skin and rectus muscle penetration and satisfactory needle visualiza.

  10. Spontaneous cerebrospinal fluid rhinorrhea in a patient with tentorial meningioma

    Institute of Scientific and Technical Information of China (English)

    GUNA Jing-yu; TONG Xiao-jie; WEI Xue-zhong

    2007-01-01

    @@ Spontaneous cerebrospinal fluid (CSF) rhinorrhea is rarely found, especially in patients with brain tumors.Similarly, reports of tentorial meningioma coexisting with acquired Chiari Ⅰ malformation with hydromyelia are also few. No doubt, one patient with cerebrospinal fluid rhinorrhea, tentorial meningioma and Chiari Ⅰ malformation with hydromyelia is hardly ever found. We reported one case of this rare condition.

  11. Uptake of /sup 131/I by a papillary meningioma

    Energy Technology Data Exchange (ETDEWEB)

    Preisman, R.A. (Rees-Stealy Medical Clinic, San Diego, CA); Halpern, S.E.; Shishido, R.; Waltz, T.; Callipari, F.; Reit, R.

    1977-08-01

    Significant uptake of /sup 131/I commonly occurs in thyroid malignancies, especially if the remaining normal thyroid tissue is removed prior to scanning. We report a case showing marked uptake of /sup 131/I by a papillary meningioma of the thoracic spinal cord. To our knowledge, this radionuclide has not been previously shown to accumulate in a meningioma.

  12. Neurocognition in individuals with incidentally-identified meningioma.

    Science.gov (United States)

    Butts, Alissa M; Weigand, Stephen; Brown, Paul D; Petersen, Ronald C; Jack, Clifford R; Machulda, Mary M; Cerhan, Jane H

    2017-08-01

    Meningiomas are primary intracranial tumors that are often asymptomatic. To our knowledge, no study has attempted to describe neurocognitive function in patients with incidentally-discovered meningioma. We utilized the Mayo Clinic Study of Aging (MCSA), which is a population-based sample of Olmsted County, Minnesota residents that includes neuropsychological testing and brain MRI approximately every 15 months. Using a text search of radiologists' notes of 2402 MCSA individuals (mean age 77 years, scanned between 2004 and 2014) we identified 48 eligible subjects (2%) who had at least one meningioma. Most meningiomas were small (90% age, sex, and education. Cognitive domains assessed included memory, attention-executive function, language, and visuospatial. More women (67%) had a meningioma than men (33%). Groups did not differ on prevalence of Mild Cognitive Impairment (Meningioma = 19%, Controls = 13%). Across cognitive domains, we observed similar performance for the two groups (p's ≥ 0.21). Subtle differences emerged in memory and language domains (p = 0.05 and p = 0.11) when we divided the Meningioma group by tumor location, wherein the small group with an infratentorial tumor performed more poorly than controls globally as well as on select memory and language measures. Our findings suggest that small meningiomas are generally cognitively benign, but that may change as the tumor evolves, and might be impacted by other factors such as meningioma location.

  13. Preoperative subtyping of meningiomas by perfusion MR imaging

    NARCIS (Netherlands)

    Zhang, Hao; Roediger, Lars A.; Shen, Tianzhen; Miao, Jingtao; Oudkerk, Matthijs

    2008-01-01

    Introduction This paper aims to evaluate the value of perfusion magnetic resonance (MR) imaging in the preoperative subtyping of meningiomas by analyzing the relative cerebral blood volume (rCBV) of three benign subtypes and anaplastic meningiomas separately. Materials and methods Thirty-seven menin

  14. Perfusion MR imaging for differentiation of benign and malignant meningiomas

    NARCIS (Netherlands)

    Zhang, Hao; Rodiger, Lars A.; Shen, Tianzhen; Miao, Jingtao; Oudkerk, Matthijs

    2008-01-01

    Introduction Our purpose was to determine whether perfusion MR imaging can be used to differentiate benign and malignant meningiomas on the basis of the differences in perfusion of tumor parenchyma and/or peritumoral edema. Methods A total of 33 patients with preoperative meningiomas (25 benign and

  15. 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

  16. Exophytic intramedullary meningioma of the cervical spinal cord.

    Science.gov (United States)

    Sahni, D; Harrop, J S; Kalfas, I H; Vaccaro, A R; Weingarten, D

    2008-10-01

    Intramedullary spinal cord neoplasms are relatively uncommon. The most common intramedullary tumors are astrocytomas and ependymomas. Meningiomas can occur as an intradural tumor; however, they are typically in the extramedullary compartment. A 42-year-old male presented with progressive sensory loss in the upper extremities and lower extremity weakness. Pre-operative imaging suggested an intramedullary cervical lesion. To treat the progressive neurological abnormality, surgical resection was planned. At surgery, it was noted that the tumor originated in the cervical spinal cord and extended into the extramedullary region. Histology confirmed the lesion to be a meningioma. This meningioma variant has not previously been described. Spinal meningiomas may occur in locations other than intradural, extramedullary locations, and should be included in the differential diagnosis of intramedullary lesions. Intramedullary meningiomas can be successfully treated with surgery.

  17. Spontaneous regression together with increased calcification of incidental meningioma

    Directory of Open Access Journals (Sweden)

    Kengo Hirota

    2014-01-01

    Full Text Available Background: Regression of meningioma has been reported after hemorrhage or hormonal withdrawal. However, meningioma regression is rarely observed spontaneously. Case Description: A right falx meningioma was incidentally diagnosed and was followed at every one-year by magnetic resonance imaging (MRI for over 7 years. The tumor, with a maximum diameter of 4 cm, showed a slightly high density and was enhanced on computed tomography (CT, and a high intensity with a low-intensity core on T2 MRI, with significant edema. The meningioma gradually shrank together with a decrease of edema and increase of calcification. The initial volume, 25.5 cm 3 , regressed linearly to less than half, 9.9 cm 3 . Conclusion: Here, we report a case of an incidentally diagnosed meningioma that regressed spontaneously. The pattern of the regression was similar to that following gamma knife radiosurgery.

  18. Midkine expression in 52 human meningiomas A correlation analysis

    Institute of Scientific and Technical Information of China (English)

    Xinjun Li; Xiangguo Xia

    2008-01-01

    BACKGROUND: Several studies have shown that midkine directly participates in tumor cell growth and invasion, as well as the regulation of angiogenesis.OBJECTIVE: To investigate midkine expression in meningioma tissue in relation to angiogenesis, invasion, peritumoral edema, and clinicopathology.DESIGN, TIME AND SETTING: The present clinical, case-controlled, neuropathological study was performed at the Laboratory of Molecular Organism, People's Hospital of Deyang City between May 2007 and April 2008.MATERIALS: Fifty-two meningioma tissues were classified by WHO tumor classification of the central nervous system, comprising 40 grade Ⅰ meningioma, five grade Ⅱ meningioma, and seven grade Ⅲ meningioma. Ten normal, human cerebral maters were selected from cerebral trauma patients.METHODS: Midkine protein expression and mean microvessel density were detected using immunohistochemical techniques. Simultaneously, all data were statistically analyzed.MAIN OUTCOME MEASURES: Midkine expression and microvessel density in meningiomas and normal cerebral maters.RESULTS: The positive midkine expression rate was 64% in the meningioma tissues. However, midkine expression was not detected in normal cerebral mater tissue. The mean microvessel density was 82.0±22.7 in the meningiomas, and 25.8±6.2 in the normal cerebral mater tissues. There was significant difference in midkine expression and mean microvessel density between meningioma tissues and human cerebral maters (P 0.05). However, it closely correlated with patient clinical condition, pathological grade, invasion, and peritumoral edema (r =0.378 5, 0.741 2, 0.651 8, 0.614 2, P < 0.05).CONCLUSION: Midkinc protein was overexpressed in meningiomas and correlated to tumor angiogenesis, invasion, peritumoral edema, and clinicopathology.

  19. Nerve sheath myxoma of the dorsal paravertebral space

    Directory of Open Access Journals (Sweden)

    Melih Malkoc

    2014-01-01

    CONCLUSION: Although the most presented case of NMS are dermal tumors, it may also be found extremely rare locations. We conclude that, the definitive treatment of NSM is surgical excision with safe margins even when it is possible.

  20. Nerve biopsy

    Science.gov (United States)

    Biopsy - nerve ... A nerve biopsy is most often done on a nerve in the ankle, forearm, or along a rib. The health care ... feel a prick and a mild sting. The biopsy site may be sore for a few days ...

  1. Combination of acellular nerve graft and schwann cells-like cells for rat sciatic nerve regeneration.

    Science.gov (United States)

    Gao, Songtao; Zheng, Yan; Cai, Qiqing; Deng, Zhansheng; Yao, Weitao; Wang, Jiaqiang; Wang, Xin; Zhang, Peng

    2014-01-01

    To investigate the effect of tissue engineering nerve on repair of rat sciatic nerve defect. Forty-five rats with defective sciatic nerve were randomly divided into three groups. Rats in group A were repaired by acellular nerve grafts only. Rats in group B were repaired by tissue engineering nerve. In group C, rats were repaired by autogenous nerve grafts. After six and twelve weeks, sciatic nerve functional index (SFI), neural electrophysiology (NEP), histological and transmission electron microscope observation, recovery ratio of wet weight of gastrocnemius muscle, regenerated myelinated nerve fibers number, nerve fiber diameter, and thickness of the myelin sheath were measured to assess the effect. After six and twelve weeks, the recovery ratio of SFI and wet weight of gastrocnemius muscle, NEP, and the result of regenerated myelinated nerve fibers in groups B and C were superior to that of group A (P 0.05). The tissue engineering nerve composed of acellular allogenic nerve scaffold and Schwann cells-like cells can effectively repair the nerve defect in rats and its effect was similar to that of the autogenous nerve grafts.

  2. New technique for withdrawing broken sheath

    Directory of Open Access Journals (Sweden)

    Süleyman Cagan Efe

    2016-03-01

    Full Text Available A sheath that is broken inside vessel is a rare complication, and intravascular fragments from broken sheaths are retrieving transcutaneously by techniques including the loop snare catheter, basket catheter, and grasping/biopsy forceps. We reported a less common type of broken central venous sheath in location and a successful unique technique for retrieving it from subclavian vein by using noncompliant balloon from 40 year old female patient.

  3. Vertebral Artery Transposition Via an Extreme-Lateral Approach for Anterior Foramen Magnum Meningioma or Craniocervical Junction Tumors.

    Science.gov (United States)

    Park, Hun Ho; Lee, Kyu-Sung; Hong, Chang-Ki

    2016-04-01

    Vertebral artery (VA) transposition in the extreme-lateral transcondylar approach can minimize the manipulation of the low cranial nerves and the brain stem. The authors describe the surgical technique of VA transposition. From March 2000 to December 2014, 28 of 48 patients underwent VA transposition for anterior foramen magnum meningioma (16 patients) and craniocervical junction (CCJ) tumors (12 patients). Tumor was resected via an extreme-lateral approach with partial condylectomy to expose the anterior portion of the brain stem. For intradural tumors, the VA was mobilized caudomedially after circumferential dural incision around the VA at the level of the foramen magnum. For extradural tumors involving the CCJ, VA was transposed medially from the transverse foramen of C1 without any dural incision. Gross total resection was achieved in 26 of 28 patients (92.9%) with VA transposition. Histologically, meningioma and schwannoma were most common. The origin of the tumors was foramen magnum (57.1%), C1 nerve root (17.9%), clivus (10.7%), jugular foramen (7.1%), posterior skull base (3.6%), and hypoglossal canal (3.6%). VA transposition was performed intradurally in 19 patients (67.9%) and extradurally in 9 patients (32.1%). Surgical morbidity was 17.9% including 4 patients with hypoglossal nerve palsy and 1 patient with quadriparesis. The mean follow-up duration after surgery was 4.2 years (range, 0.1-14.8 years). VA transposition can provide a wide surgical window for anterior foramen magnum meningioma or tumors involving the CCJ with the least manipulation of the neuraxis. Copyright © 2016 Elsevier Inc. All rights reserved.

  4. Endoscopic-Assisted Posterior Intradural Petrous Apicectomy in Petroclival Meningiomas: A Clinical Series and Assessment of Perioperative Morbidity.

    Science.gov (United States)

    Tatagiba, Marcos; Rigante, Luigi; Mesquita Filho, Paulo; Ebner, Florian H; Roser, Florian

    2015-12-01

    To describe the clinical feasibility and outcome of the endoscopic-assisted posterior intradural petrous apicectomy approach (PIPA) for petroclival meningiomas extending into the supratentorial space. From 2005-2013, 29 patients with a petroclival meningioma underwent tumor removal through a PIPA approach. The approach consists of a retrosigmoid approach, intradural anterior resection of the petrous apex and microsurgical removal of the tumor, followed by endoscopic-assisted visualization and removal of tumor parts in the middle fossa or anterior to the brainstem. Patients consisted of 7 men and 22 women; the mean age of patients was 52.7 years. In 24 patients, surgery was performed with the patient in a semisitting position; in 5 patients, surgery was performed with the patient in a supine position. A total resection was achieved in 19 patients (66%). A Karnofsky performance scale score >60% was recorded in 27 patients (93%), with surgical complications that involved a cerebrospinal fluid leak in 3 patients, bleeding in the surgical cavity in 2 patients, and pneumocephalus in 1 patient. The most frequent postoperative neurologic deficit was facial palsy (34%), which disappeared or improved consistently in all but 1 patient, who required a cranial nerve VII-cranial nerve XII anastomosis. For petroclival meningiomas extending into the middle fossa, the endoscopic-assisted PIPA approach is safe and straightforward. The principal advantages of the PIPA approach are familiarity with the retrosigmoid route; the absence of temporal lobe retraction; and early control of the cranial nerves, vessels, and brainstem. However, careful patient selection regarding tumor extension is fundamental to obtaining optimal outcomes. Copyright © 2015 Elsevier Inc. All rights reserved.

  5. Genomic and Expression Profiling of Benign and Malignant Nerve Sheath Profiling of Benign and Malignant Nerve Sheath

    Science.gov (United States)

    2007-05-01

    genes that will serve as molecular markers for progression of neurofibroma to MPNST , and to identify potential therapeutic targets. miRNA expression...profiling was performed on 6 cases of MPNSTs , and 7 cases of synovial sarcomas. By using unsupervised hierarchical clustering most tumors were grouped... MPNSTs and synovial sarcoma (SS). To develop a cell line model for MPNSTs , global gene expression profiles for cell lines established from 3 primary

  6. Nanofibrous nerve conduit-enhanced peripheral nerve regeneration.

    Science.gov (United States)

    Jiang, Xu; Mi, Ruifa; Hoke, Ahmet; Chew, Sing Yian

    2014-05-01

    Fibre structures represent a potential class of materials for the formation of synthetic nerve conduits due to their biomimicking architecture. Although the advantages of fibres in enhancing nerve regeneration have been demonstrated, in vivo evaluation of fibre size effect on nerve regeneration remains limited. In this study, we analyzed the effects of fibre diameter of electrospun conduits on peripheral nerve regeneration across a 15-mm critical defect gap in a rat sciatic nerve injury model. By using an electrospinning technique, fibrous conduits comprised of aligned electrospun poly (ε-caprolactone) (PCL) microfibers (981 ± 83 nm, Microfiber) or nanofibers (251 ± 32 nm, Nanofiber) were obtained. At three months post implantation, axons regenerated across the defect gap in all animals that received fibrous conduits. In contrast, complete nerve regeneration was not observed in the control group that received empty, non-porous PCL film conduits (Film). Nanofiber conduits resulted in significantly higher total number of myelinated axons and thicker myelin sheaths compared to Microfiber and Film conduits. Retrograde labeling revealed a significant increase in number of regenerated dorsal root ganglion sensory neurons in the presence of Nanofiber conduits (1.93 ± 0.71 × 10(3) vs. 0.98 ± 0.30 × 10(3) in Microfiber, p regeneration. These results could provide useful insights for future nerve guide designs.

  7. Theory of sheath in a collisional multi-component plasma

    Indian Academy of Sciences (India)

    M K Mahanta; K S Goswami

    2001-04-01

    The aim of this brief report is to study the behaviour of sheath structure in a multicomponent plasma with dust-neutral collisions. The plasma consists of electrons, ions, micron size negatively charged dust particles and neutrals. The sheath-edge potential and sheath width are calculated for collisionally dominated sheath. Comparison of collisionless and collisionally dominated sheath are made.

  8. Tendon sheath fibroma in the thigh.

    Science.gov (United States)

    Moretti, Vincent M; Ashana, Adedayo O; de la Cruz, Michael; Lackman, Richard D

    2012-04-01

    Tendon sheath fibromas are rare, benign soft tissue tumors that are predominantly found in the fingers, hands, and wrists of young adult men. This article describes a tendon sheath fibroma that developed in the thigh of a 70-year-old man, the only known tendon sheath fibroma to form in this location. Similar to tendon sheath fibromas that develop elsewhere, our patient's lesion presented as a painless, slow-growing soft tissue nodule. Physical examination revealed a firm, nontender mass with no other associated signs or symptoms. Although the imaging appearance of tendon sheath fibromas varies, our patient's lesion appeared dark on T1- and bright on T2-weighted magnetic resonance imaging. It was well marginated and enhanced with contrast.Histologically, tendon sheath fibromas are composed of dense fibrocollagenous stromas with scattered spindle-shaped fibroblasts and narrow slit-like vascular spaces. Most tendon sheath fibromas can be successfully removed by marginal excision, although 24% of lesions recur. No lesions have metastasized. Our patient's tendon sheath fibroma was removed by marginal excision, and the patient remained disease free 35 months postoperatively. Despite its rarity, tendon sheath fibroma should be included in the differential diagnosis of a thigh mass on physical examination or imaging, especially if it is painless, nontender, benign appearing, and present in men.

  9. Molecular and genetic profiles of radiographically defined de novo meningiomas.

    Science.gov (United States)

    Kitamura, Yohei; Sasaki, Hikaru; Yoshida, Kazunari

    2012-05-01

    With the exception of radiation-induced tumors, benign meningiomas that are known to have developed within a defined time period are extremely rare. We have genetically characterized two cases of radiographically defined de novo, sporadic meningiomas--a 5-cm, left parasagittal tumor in a 61-year-old male and a 2.3-cm, right falx tumor in a 53-year-old female. Neither tumor was observed during MRIs performed for unrelated complaints 49 and 28 months before surgery, respectively. Both tumors were totally resected, and histopathological examination revealed WHO grade I meningiomas. In both cases, the MIB-1 staining indices were high for grade I meningioma (5.6% for case 1 and 9.1% for case 2), and abnormal accumulation of p53 were observed by immunohistochemistry. The two tumors shared losses of chromosome arms 1p and 7p by comparative genomic hybridization. The tumor suppressor merlin, product of the NF2 gene, was not detected in either tumor. These abnormalities found in common in both of the de novo meningiomas likely to play significant roles in the pathogenesis and/or rapid development of meningiomas. Moreover, taken together with previous studies, our findings indicate that the combined loss of 1p and 7p may play a critical role in the tumorigenesis of de novo, aggressive meningiomas.

  10. Treatment of an atypical metastatic meningioma: a case report

    Directory of Open Access Journals (Sweden)

    Conca R

    2013-04-01

    Full Text Available Meningiomas are common intracranial tumors which usually pursue a benign course. Extracranial metastases from meningiomas are very rare and the lung is the most common site. We report a 27 year old girl with an intracranial atypical meningioma with pulmonary metastasis which had been misdiagnosed as lung sequestration upon chest CT examination. She underwent subtotal surgical resection of the meningioma in 2006 and surgical removal of the lung metastasis in 2009. Then, the patient developed pleural, lung, periesophageal and diaphragmatic nodal metastases, despite only subtle increase of the intracranial residual meningioma. Therefore, she was treated by Doxil (pegylated liposomal doxorubicin and bevacizumab with a progression of disease after three cycles and then with hydroxyurea for two months with a dimensional increase of metastatic lesions. After adding sorafenib to hydroxyurea the patient showed a further progression disease and finally died for respiratory insufficiency. Metastatic meningiomas have been rarely reported. Hydroxyurea is one of the most used drug in recurrent and metastatic meningiomas, despite modest results are obtained. Further chemotherapy strategies or biological agent must be investigated in clinical trials.

  11. The integrin inhibitor cilengitide affects meningioma cell motility and invasion.

    Science.gov (United States)

    Wilisch-Neumann, Annette; Kliese, Nadine; Pachow, Doreen; Schneider, Thomas; Warnke, Jan-Peter; Braunsdorf, Werner Ek; Böhmer, Frank-Dietmar; Hass, Peter; Pasemann, Diana; Helbing, Cornelia; Kirches, Elmar; Mawrin, Christian

    2013-10-01

    Meningiomas are frequent intracranial or spinal neoplasms, which recur frequently and can show aggressive clinical behaviour. We elucidated the impact of the integrin inhibitor cilengitide on migration, proliferation, and radiosensitization of meningioma cells. We analyzed integrin expression in tissue microarrays of human meningiomas and the antimeningioma properties of cilengitide in cell cultures, subcutaneous and intracranial nude mouse models by measuring tumor volumes and survival times. αvβ5 was the predominantly expressed integrin heterodimer in meningiomas, whereas αvβ3 was mainly detected in tumor blood vessels. Application of up to 100 μg/mL cilengitide resulted in only mildly reduced proliferation/survival of meningioma cell lines. Effects on cell survival could be enhanced by irradiation. One μg/mL cilengitide was sufficient to significantly inhibit meningioma cell migration and invasion in vitro. A daily dosage of 75 mg/kg did neither affect tumor volumes nor overall survival (P = 0.813, log-rank test), but suppressed brain invasion in a significant fraction of treated animals. A combination of 75 mg/kg cilengitide daily and irradiation (2 × 5 Gy) led to a 67% reduction of MRI-estimated tumor volumes in the intracranial model (P meningiomas, although brain invasion may be reduced because of the strong antimigratory properties of the drug. The combination with radiotherapy warrants further attention. ©2013 AACR.

  12. Collision Tumor Composed of Meningioma and Cavernoma

    Science.gov (United States)

    Weigel, Jens; Neher, Markus; Schrey, Michael; Wünsch, Peter H.; Steiner, Hans-Herbert

    2017-01-01

    A true collision tumor is a rare entity composed of two histologically distinct neoplasms coinciding in the same organ. This paper reports a unique case of cerebral collision tumor consisting of two benign components. On the first hand, meningioma which is usually a benign lesion arising from the meningothelial cell in the arachnoidal membrane. On the other, cerebral cavernoma which is a well-circumscribed, benign vascular hamartoma within the brain. To our knowledge, there is no previously documented case of cerebral collision tumor consisting of two benign components. A 56-year-old Caucasian male suffered in 2002 from an atypical meningioma WHO II° located in the left lateral ventricle. Three years after the tumor extirpation, the patient suffered from a hematoma in the fourth ventricle due to a recurrently haemorrhaged cavernoma. In 2008, a recurrence of the tumor in the left lateral ventricle was discovered. Additionally, another tumor located in the quadrigeminal lamina was detected. After surgical resection of the tumor in the left lateral ventricle, the pathological examination confirmed the diagnosis of a collision tumor consisting of components of a meningioma WHO II° and a cavernoma. Postoperatively, no adjuvant treatment was needed and no tumor recurrence is discovered up to the present. A possible explanation for the collision of those two different tumors may be migration of tumor cells mediated by the cerebrospinal fluid. After 5-years of follow-up, there is no sign of any tumor recurrence; therefore, surgical tumor removal without adjuvant therapy seems to be the treatment of choice. PMID:28061500

  13. Sheath Criterion for a Collisional Electronegative Plasma Sheath in an Applied Magnetic Field

    Institute of Scientific and Technical Information of China (English)

    邹秀; 刘惠平; 邱明辉; 孙骁航

    2011-01-01

    The sheath criterion for a collisional electronegative plasma sheath in an applied magnetic field is investigated.It is assumed that the system consists of hot electrons,hot negative ions and cold positive ions.The effect of an applied magnetic field on the sheath criterion is discussed.The results reveal that the magnetic field has effects on both the upper and lower limits,which cause the range of the ion Mach number to increase.In addition,the numerical calculations of the electronegative plasma sheath are carried out to demonstrate the effects of sheath criterion on the characteristics of the sheath.%The sheath criterion for a collisioned electronegative plasma sheath in an applied magnetic Geld is investigated. It is assumed that the system consists of hot electrons, hot negative ions and cold positive ions. The effect of an applied magnetic Reid on the sheath criterion is discussed. The results reveal that the magnetic field has effects on both the upper and lower limits, which cause the range of the ion Mach number to increase. In addition, the numerical calculations of the electronegative plasma sheath are carried out to demonstrate the effects of sheath criterion on the characteristics of the sheath.

  14. Meningeal fibroma: a rare meningioma mimic.

    Science.gov (United States)

    Kakkar, Aanchal; Sharma, Mehar C; Goyal, Nishant; Sarkar, Chitra; Suri, Vaishali; Garg, Ajay; Kale, Shashank S; Suri, Ashish

    2014-08-01

    Meningeal fibromas are rare intracranial tumors that mimic meningiomas radiologically as well as histologically. The authors report 2 cases of meningeal fibroma with detailed clinical, radiological, histopathological, and immunohistochemical features, and discuss the differential diagnosis of this entity. Knowledge of this rare tumor is essential for pathologists to be able distinguish it from more common meningeal tumors, especially in younger patients. This knowledge is also essential for neurosurgeons, as incomplete resection may lead to tumor recurrence, and such patients require close follow-up.

  15. BENIGN INTRACRANIAL HYPOTENSION WITH INCIDENTAL PARASAGITTAL MENINGIOMA

    Directory of Open Access Journals (Sweden)

    Sukumar

    2015-12-01

    Full Text Available Benign intracranial hypotension, otherwise called as idiopathic intracranial hypotension is caused by CSF leaks due to disruption in spinal meninges. It is most commonly caused by disruption and leak at cervical and thoracic spine. Imaging is sometimes the most important key to the diagnosis of idiopathic intracranial hypotension, which helps in appropriate treatment of the patient. Here, we are presenting a case of benign intracranial hypotension associated with incidental parasagittal meningioma. The presence of benign intracranial hypotension was confirmed by taking MR myelogram.

  16. Histopathological analysis of 177 cases with operated intracranial meningioma

    Directory of Open Access Journals (Sweden)

    Guner Menekse

    2013-02-01

    Full Text Available Purpose: Aim of this study is to evaluate histopathologic properties of patients with intracranial meningioma who were operated due to intracranial mass. Method: 177 cases diagnosed with intracranial meningioma were retrospectively examined in our clinic between the years of 2007-2012 and their age, sex, tumor localization, and histopathologic properties were analyzed. Results: 74.6% of 177 intracranial meningioma cases were females, 25.4% were males and the ratio of Male/Female was found 3.4/10. The age interval of the cases was between 12-105 years old. It was most frequently seen at the interval of 51-60. In histopathologic examination, 163 cases (92.1% were noted as WHO Grade I, 11 cases (6.2% were WHO Grade II, and 3 cases (1.7% were WHO Grade III. While meningotelial transitional, and psammomatous type was the most frequently seen in WHO Grade I, atypical meningioma was only dominant in Grade II. In WHO Grade II and III cases, dominance of the male sex was present. While 88.1% of the cases had supratentorial localization, 11.9% had infratentorial localization, 1 case had lateral ventricle localization. In 7 cases (3.9% of the series, multiple meningiomas were determined. Conclusion: In a series of 177 cases, intracranial meningiomas were seen between 51-60 years old and its most frequent localization was convexity. While Grade I cases were seen in the female sex, dominance of the male sex was present in Grade II and III cases. Additionally, all of multiple meningiomas detected were female cases. Meningotelial meningiomas constitute the most commonly seen subtype among Grade I cases. Key Words: Intracranial meningioma, histopathological classification, clinical study [Cukurova Med J 2013; 38(1.000: 41-59

  17. Platelet-rich plasma-induced bone marrow mesenchymal stem cells versus autologous nerve grafting for sciatic nerve repair

    Institute of Scientific and Technical Information of China (English)

    Changsuo Xia; Yajuan Li; Wen Cao; Zhaohua Yu

    2010-01-01

    Autologous nerve grafting is the gold standard of peripheral nerve repair.We previously showed that autologous platelet-rich plasma(PRP)contains high concentrations of growth factors and can induce in vitro cultured bone marrow mesenchymal stem cells(BMSCs)to differentiate into Schwann cells.Here we used PRP-induced BMSCs combined with chemically extracted acellular nerves to repair sciatic nerve defects and compared the effect with autologous nerve grafting.The BMSCs and chemically extracted acellular nerve promoted target muscle wet weight restoration,motor nerve conduction velocity,and axonal and myelin sheath regeneration,with similar effectiveness to autologous nerve grafting.This finding suggests that PRP induced BMSCs can be used to repair peripheral nerve defects.

  18. Imaging diagnosis--hyperostosis associated with meningioma in a dog.

    Science.gov (United States)

    Mercier, Miyu; Heller, Heidi L Barnes; Bischoff, Matthew G; Looper, Jayme; Bacmeister, Cynthia X

    2007-01-01

    A 5-year-old neutered male Beagle mix dog had a 5-day history of generalized tonic-clonic seizures. Before the seizures, the dog had a 1-2-month history of progressive right hemiparesis. In computed tomography images, a presumed extraaxial mass with hyperostosis and destruction of the skull covering the mass were identified. Surgical excision was performed and the histopathologic diagnosis was meningioma. Hyperostosis is frequently associated with feline meningioma, but this report documents that hyperostosis may also occur secondary to meningioma in the dog.

  19. Endoscopic Endonasal Approach for Removal of Tuberculum Sellae Meningiomas.

    Science.gov (United States)

    Ditzel Filho, Leo F S; Prevedello, Daniel M; Jamshidi, Ali O; Dolci, Ricardo L; Kerr, Edward E; Campbell, Raewyn; Otto, Bradley A; Carrau, Ricardo L; Kassam, Amin

    2015-07-01

    Tuberculum sellae meningiomas are challenging lesions; their critical location and often insidious growth rate enables significant distortion of the superjacent optic apparatus before the patient notices any visual impairment. This article describes the technical nuances, selection criteria and complication avoidance strategies for the endonasal resection of tuberculum sellae meningiomas. A stepwise description of the surgical technique is presented; indications, adjuvant technologies, pitfalls and the relevant anatomy are also reviewed. Tuberculum sellae meningiomas may be safely and effectively resected through the endonasal route; invasion of the optic canals does not represent a limitation.

  20. Metastatic meningioma: positron emission tomography CT imaging findings.

    LENUS (Irish Health Repository)

    Brennan, C

    2010-12-01

    The imaging findings of a case of metastasing meningioma are described. The case illustrates a number of rare and interesting features. The patient presented with haemoptysis 22 years after the initial resection of an intracranial meningioma. CT demonstrated heterogeneous masses with avid peripheral enhancement without central enhancement. Blood supply to the larger lesion was partially from small feeding vessels from the inferior pulmonary vein. These findings correlate with a previously published case in which there was avid uptake of fluoro-18-deoxyglucose peripherally with lesser uptake centrally. The diagnosis of metastasing meningioma was confirmed on percutaneous lung tissue biopsy.