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1. Rodríguez-Paradinas M, Rivera T: [Nasosinusal schwannoma]. Acta Otorrinolaringol Esp; 2010 Jul-Aug;61(4):321-3
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  • [Title] [Nasosinusal schwannoma].
  • [Transliterated title] Schwannoma nasosinusal.
  • Within this area, the most frequent location is the VIII cranial nerve (80%).
  • [MeSH-major] Neurilemmoma. Paranasal Sinus Neoplasms

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  • [Copyright] Copyright 2009 Elsevier España, S.L. All rights reserved.
  • (PMID = 19850271.001).
  • [ISSN] 1988-3013
  • [Journal-full-title] Acta otorrinolaringológica española
  • [ISO-abbreviation] Acta Otorrinolaringol Esp
  • [Language] spa
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Spain
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2. Martínez Ferreras A, Rodrigo Tapia JP, Llorente Pendás JL, Suárez Nieto C: [Endoscopic nasal surgery for pterigopalatine fossa schwannoma]. Acta Otorrinolaringol Esp; 2005 Jan;56(1):41-3
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  • [Title] [Endoscopic nasal surgery for pterigopalatine fossa schwannoma].
  • [Transliterated title] Abordaje de un schwannoma de la fosa pterigo-palatina por cirugía endoscópica nasal.
  • Schwannomas are tumors of nerve sheath origin, the lesion being derived from the Schwann cells surrounding neural tissue in most of the peripheral, cranial and autonomic nerves.
  • In the nose and paranasal sinuses these tumors are very uncommon (fewer than 4% of all schwannomas).
  • In the pterygopalatine fossa they are very rare, and arise in this case from the ophthalmic and maxillary branches of the trigeminal nerve.
  • In this exceptional site, the tumor is classically approached through a Caldwell-Luc incision.
  • In this case, we report the removal of a schwannoma of the pterygopalatine fossa by endoscopic sinus surgery.
  • [MeSH-major] Endoscopy / methods. Neurilemmoma / pathology. Neurilemmoma / surgery. Paranasal Sinus Neoplasms / pathology. Paranasal Sinus Neoplasms / surgery

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  • (PMID = 15747724.001).
  • [ISSN] 0001-6519
  • [Journal-full-title] Acta otorrinolaringológica española
  • [ISO-abbreviation] Acta Otorrinolaringol Esp
  • [Language] spa
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Spain
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3. Bhanvadia V, Santwani P: Intrascrotal extratesticular schwannoma. J Cytol; 2010 Jan;27(1):37-9
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  • [Title] Intrascrotal extratesticular schwannoma.
  • Primary nerve sheath tumors in an intrascrotal and extratesticular location are extremely rare, with only a few cases reported in the literature.
  • We report a fine needle aspiration cytology (FNAC)-diagnosed and histologically confirmed case of paratesticular schwannoma in a 32-year-old male.
  • This case is documented to highlight the rarity of paratesticular schwannoma and the role of FNAC in its pre-operative interpretation.

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  • (PMID = 21042535.001).
  • [ISSN] 0974-5165
  • [Journal-full-title] Journal of cytology
  • [ISO-abbreviation] J Cytol
  • [Language] ENG
  • [Publication-type] Journal Article
  • [Publication-country] India
  • [Other-IDs] NLM/ PMC2964850
  • [Keywords] NOTNLM ; Fine needle aspiration cytology / schwannoma / scrotum
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4. Stark AM, Buhl R, Hugo HH, Straube T, Mehdorn HM: Chronic Recurrent Subarachnoid Hemorrhage from a Trigeminal Nerve Malignant Peripheral Nerve Sheath Tumor: Case Report. Neurosurgery; 2006 Aug 01;59(2):E425
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  • [Title] Chronic Recurrent Subarachnoid Hemorrhage from a Trigeminal Nerve Malignant Peripheral Nerve Sheath Tumor: Case Report.

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  • (PMID = 28180626.001).
  • [ISSN] 1524-4040
  • [Journal-full-title] Neurosurgery
  • [ISO-abbreviation] Neurosurgery
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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5. Ohba S, Miwa T, Kawase T: Trochlear Nerve Schwannoma with Intratumoral Hemorrhage. Neurosurgery; 2006 Apr 01;58(4):E791
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  • [Title] Trochlear Nerve Schwannoma with Intratumoral Hemorrhage.

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  • (PMID = 28180533.001).
  • [ISSN] 1524-4040
  • [Journal-full-title] Neurosurgery
  • [ISO-abbreviation] Neurosurgery
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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6. Murovic JA, Gibbs IC, Chang SD, Mobley BC, Park J, Adler JR: Foraminal Nerve Sheath Tumors: Intermediate Follow-up after Cyberknife Radiosurgery. Neurosurgery; 2009 Feb 01;64(suppl_2):A33-A43
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  • [Title] Foraminal Nerve Sheath Tumors: Intermediate Follow-up after Cyberknife Radiosurgery.

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  • (PMID = 28175586.001).
  • [ISSN] 1524-4040
  • [Journal-full-title] Neurosurgery
  • [ISO-abbreviation] Neurosurgery
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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7. Sarica FB, Cekinmez M, Tufan K, Erdoğan B, Sen O, Altinörs MN: A rare case of massive NF1 with invasion of entire spinal axis by neurofibromas: case report. Turk Neurosurg; 2008 Jan;18(1):99-106
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  • [Title] A rare case of massive NF1 with invasion of entire spinal axis by neurofibromas: case report.
  • Also called 'von Recklinghausen disease' or 'peripheral neurofibromatosis', it comprises 90% of all neurofibromatosis (NF) cases.
  • It is characterized by multiple peripheral nerve sheath tumors of benign character called neurofibromas.
  • Surgical intervention is indicated when myelopathy and motor losses develop in the case of paraspinal neurofibromas, which are frequently localized to the cervical and lumbar regions.
  • The level of surgical intervention required should be carefully considered and should take into account neurophysiological tests of paraspinal neurofibroma cases that allow estimation of the risk that the neurofibromas will invade the complete spinal axis.
  • In the present paper, we discuss an NF1 case in which paraspinal neurofibromas were observed along the complete spinal axis of a 32-year-old male patient who arrived at the clinic reporting increasingly intense pins and needles and weakness.
  • [MeSH-minor] Adult. Disease Progression. Humans. Male. Spine

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  • (PMID = 18382989.001).
  • [ISSN] 1019-5149
  • [Journal-full-title] Turkish neurosurgery
  • [ISO-abbreviation] Turk Neurosurg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Turkey
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8. Feitsma H, Kuiper RV, Korving J, Nijman IJ, Cuppen E: Zebrafish with mutations in mismatch repair genes develop neurofibromas and other tumors. Cancer Res; 2008 Jul 1;68(13):5059-66
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  • [Title] Zebrafish with mutations in mismatch repair genes develop neurofibromas and other tumors.
  • This genetic predisposition to colon cancer is linked to heterozygous familial mutations, and loss-of-heterozygosity is necessary for tumor development.
  • In contrast, the rare cases with biallelic MMR mutations are juvenile patients with brain tumors, skin neurofibromas, and café-au-lait spots, resembling the neurofibromatosis syndrome.
  • Here, we describe the identification and characterization of novel knockout mutants of the three major MMR genes, mlh1, msh2, and msh6, in zebrafish and show that they develop tumors at low frequencies.
  • Predominantly, neurofibromas/malignant peripheral nerve sheath tumors were observed; however, a range of other tumor types was also observed.
  • Our findings indicate that zebrafish mimic distinct features of the human disease and are complementary to mouse models.
  • [MeSH-major] DNA Mismatch Repair. DNA Repair Enzymes / genetics. Neoplasms / genetics. Neurofibromatoses / genetics. Zebrafish / genetics
  • [MeSH-minor] Abdominal Neoplasms / genetics. Amino Acid Sequence. Animals. Animals, Genetically Modified. Base Sequence. Brain Neoplasms / genetics. DNA Mutational Analysis. DNA-Binding Proteins / genetics. Eye Neoplasms / genetics. Female. Hemangiosarcoma / genetics. Male. Microsatellite Instability. MutS Homolog 2 Protein / genetics. Mutation / physiology. Nerve Sheath Neoplasms / genetics


9. Karabatsou K, Kiehl TR, Wilson DM, Hendler A, Guha A: Potential Role of 18Fluorodeoxyglucose-Positron Emission Tomography/Computed Tomography in Differentiating Benign Neurofibroma from Malignant Peripheral Nerve Sheath Tumor Associated with Neurofibromatosis 1. Neurosurgery; 2009 Oct 01;65(suppl_4):A160-A170
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  • [Title] Potential Role of 18Fluorodeoxyglucose-Positron Emission Tomography/Computed Tomography in Differentiating Benign Neurofibroma from Malignant Peripheral Nerve Sheath Tumor Associated with Neurofibromatosis 1.

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  • (PMID = 28180848.001).
  • [ISSN] 1524-4040
  • [Journal-full-title] Neurosurgery
  • [ISO-abbreviation] Neurosurgery
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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10. Murovic JA, Kim DH, Kline DG: Sciatic Benign and Malignant Non-nerve and Nerve Sheath Tumors: A Series of 50 Louisiana State University Medical Center Patients 825. Neurosurgery; 2006 Aug 01;59(2):461-462
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  • [Title] Sciatic Benign and Malignant Non-nerve and Nerve Sheath Tumors: A Series of 50 Louisiana State University Medical Center Patients 825.

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  • (PMID = 28180642.001).
  • [ISSN] 1524-4040
  • [Journal-full-title] Neurosurgery
  • [ISO-abbreviation] Neurosurgery
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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16. Wowra B, Muacevic A, Jess-Hempen A, Hempel JM, Müller-Schunk S, Tonn JC: Outpatient gamma knife surgery for vestibular schwannoma: definition of the therapeutic profile based on a 10-year experience. J Neurosurg; 2005 Jan;102(s_supplement):114-118
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  • [Title] Outpatient gamma knife surgery for vestibular schwannoma: definition of the therapeutic profile based on a 10-year experience.
  • OBJECT: The purpose of the study was to define the therapeutic profile of outpatient gamma knife surgery (GKS) for vestibular schwannoma (VS) by using sequential tumor volumetry to quantify changes following treatment.
  • The actuarial 6-year tumor control rate after a single GKS treatment was 95%.
  • Tumor swelling was observed in 43 patients (38.7%).
  • Recurrence was significantly associated with NF2 (p < 0.003) and the reduced dose (p < 0.03) delivered to these tumors.
  • The incidence of facial nerve neuropathy was mainly determined by surgery prior to GKS (p < 0.0001).
  • Facial nerve radiation toxicity was mild and transient.
  • No permanent facial nerve toxicity was observed.
  • The risk of hearing loss was correlated with age and transient tumor swelling (p < 0.05) but not with dose parameters or NF2.

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  • (PMID = 28306422.001).
  • [ISSN] 1933-0693
  • [Journal-full-title] Journal of neurosurgery
  • [ISO-abbreviation] J. Neurosurg.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Keywords] NOTNLM ; gamma knife surgery / tumor volumetry / vestibular schwannoma
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17. Chung WY, Liu KD, Shiau CY, Wu HM, Wang LW, Guo WY, Ming-Tak Ho D, Hung-Chi Pan D: Gamma knife surgery for vestibular schwannoma: 10-year experience of 195 cases. J Neurosurg; 2005 Jan;102(s_supplement):87-97
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  • [Title] Gamma knife surgery for vestibular schwannoma: 10-year experience of 195 cases.
  • OBJECT: The authors conducted a study to determine the optimal radiation dose for vestibular schwannoma (VS) and to examine the histopathology in cases of treatment failure for better understanding of the effects of irradiation.
  • Seventy-two patients (37%) had undergone partial or total excision of their tumor prior to gamma knife surgery (GKS).
  • The mean tumor volume was 4.1 cm<sup>3</sup> (range 0.04-23.1 cm<sup>3</sup>).
  • Multiisocenter dose planning placed a prescription dose of 11 to 18.2 Gy on the 50 to 94% isodose located at the tumor margin.
  • At the latest MR imaging assessment decreased or stable tumor volume was demonstrated in 93.6% of the patients.
  • Uncontrolled tumor swelling was noted in five patients at 3.5, 17, 24, 33, and 62 months after GKS, respectively.
  • Histopathological examination of specimens in three cases (one at 62 months after GKS) revealed a long-lasting radiation effect on vessels inside the tumor.
  • A margin 12-Gy dose with homogeneous distribution is effective in preventing tumor progression, while posing no serious threat to normal cranial nerve function.

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  • (PMID = 28306447.001).
  • [ISSN] 1933-0693
  • [Journal-full-title] Journal of neurosurgery
  • [ISO-abbreviation] J. Neurosurg.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Keywords] NOTNLM ; gamma knife surgery / stereotaxy / vestibular schwannoma
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18. Inoue HK: Low-dose radiosurgery for large vestibular schwannomas: long-term results of functional preservation. J Neurosurg; 2005 Jan;102(s_supplement):111-113
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • OBJECT: The author conducted a study to assess the long-term results obtained in patients who underwent GKS for large vestibular schwannomas (> cochlear nerve functions were evaluated.
  • METHODS: Twenty consecutive large tumors in 18 patients (including two cases of neurofibromatosus Type 2 [NF2]) were followed for more than 6 years.
  • There were eight tumors that were more than 4 cm in maximum diameter.
  • Microsurgery had already been performed prior to GKS in 11 patients (nine recurrent and two residual tumors).
  • Fourteen of 15 tumors were stable or decreased in size.
  • Facial nerve function was preserved in all patients and hearing preserved in four of five patients with cochlear nerve function prior to radiosurgery.
  • CONCLUSIONS: Gamma knife surgery seems to have a place in the low-dose treatment of selected large vestibular schwannoma in patients with a reasonable chance of retaining facial function and pretreatment hearing level.

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  • (PMID = 28306427.001).
  • [ISSN] 1933-0693
  • [Journal-full-title] Journal of neurosurgery
  • [ISO-abbreviation] J. Neurosurg.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Keywords] NOTNLM ; facial function / gamma knife surgery / outcome / vestibular schwannoma
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19. Tlachacova D, Schmitt M, Novotny J Jr, Novotny J, Majali M, Liscak R: A comparison of the gamma knife model C and the Automatic Positioning System with Leksell model B. J Neurosurg; 2005 Jan;102(s_supplement):25-28
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  • Data were analyzed in patients in whom the following diagnoses had been made: vestibular schwannoma, pituitary adenoma, meningioma, solitary metastasis, and other benign and malignant solitary tumors.

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  • (PMID = 28306471.001).
  • [ISSN] 1933-0693
  • [Journal-full-title] Journal of neurosurgery
  • [ISO-abbreviation] J. Neurosurg.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Keywords] NOTNLM ; conformity index / extracranial exposure / staff exposure / stereotactic radiosurgery
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20. Plotkin SR, Singh M, Cai W, O'Donnell C, Esparza S, Smith MJ, Harris GJ, Muzikansky A, Bredella MA, Kassarjian A: Whole-body MRI evaluation of tumor burden in the neurofibromatosis tumor suppressor syndromes. J Clin Oncol; 2009 May 20;27(15_suppl):2074
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Whole-body MRI evaluation of tumor burden in the neurofibromatosis tumor suppressor syndromes.
  • : 2074 Background: Neurofibromatosis 1 (NF1), NF2, and schwannomatosis are a group of related genetic disorders in which affected individuals share the predisposition to develop multiple neurofibromas and schwannomas.
  • The prevalence of internal tumors is not known because current estimates are based on regional MRI scans that may not detect occult tumors.
  • A rapid and sensitive method to detect internal tumors is highly desirable since they can cause neurologic dysfunction, compress vital structures, or transform into malignant tumors.
  • The number and type of tumors (discrete vs. plexiform) were identified by a board-certified radiologist and tumor volume was calculated using semi-automated analysis.
  • Sixty-one percent of subjects had ≥1 internal tumor.
  • The median number of tumors in affected individuals was 5 (range, 1 to 63 tumors).
  • Overall, the legs harbored the greatest number of tumors (33%), followed by the pelvis (18%), thorax (15%), abdomen (12%), arms (10%), and head/neck (7%).
  • Only 40% of internal tumors were classified as plexiform yet these tumors contributed 78% of the tumor burden by volume.
  • CONCLUSIONS: WBMRI scan is a powerful tool to evaluate the number, size, and distribution of internal tumors in patients with neurofibromatosis.
  • In addition, WBMRI may prove useful in identifying individual patients at high risk for complications (such as neurologic dysfunction or malignant transformation) due to heavy internal tumor burden and in determining the efficacy of antitumor drugs in this unique patient population.

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  • (PMID = 27964382.001).
  • [ISSN] 1527-7755
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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21. Overdiek A, Winner U, Mayatepek E, Rosenbaum T: Schwann cells from human neurofibromas show increased proliferation rates under the influence of progesterone. Pediatr Res; 2008 Jul;64(1):40-3
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  • [Title] Schwann cells from human neurofibromas show increased proliferation rates under the influence of progesterone.
  • Neurofibromatosis type 1 (NF1) is a hereditary disease caused by mutations of the NF1 gene at 17q11.2.
  • Loss of the NF1 gene product in Schwann cells leads to the development of benign nerve sheath tumors.
  • These neurofibromas may occur at any time but tend to arise during periods of hormonal imbalance, suggesting that hormones influence neurofibroma growth.
  • We chose specific medium conditions for selective proliferation of NF (+/-) and NF (-/-) cells from human neurofibromas.
  • Genetic characterization was not performed, but former works have shown that under the conditions used (+/-) and (-/-) cells can be selected.
  • We could demonstrate that Schwann cells from human neurofibromas express progesterone receptors.
  • These cells show elevated proliferation rates (highest in NF(-/-) cells) under progesterone, whereas normal human Schwann cells were not affected.
  • These data suggest that progesterone plays an important role in the development of neurofibromas in NF1.
  • [MeSH-minor] Gene Expression Regulation, Neoplastic. Genes, Neurofibromatosis 1. Humans. Time Factors. Tumor Cells, Cultured

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  • (PMID = 18360307.001).
  • [ISSN] 0031-3998
  • [Journal-full-title] Pediatric research
  • [ISO-abbreviation] Pediatr. Res.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Receptors, Progesterone; 4G7DS2Q64Y / Progesterone
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22. Civit T, Freppel S: [Intraorbital schwannomas and solitary neurofibromas]. Neurochirurgie; 2010 Apr-Jun;56(2-3):137-41
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Intraorbital schwannomas and solitary neurofibromas].
  • Intraorbital schwannomas and solitary neurofibromas account for 2 to 5% of all operated intraorbital tumors.
  • [MeSH-major] Neurilemmoma / surgery. Neurofibroma / surgery. Orbital Neoplasms / surgery
  • [MeSH-minor] Humans. Incidence. Magnetic Resonance Imaging. Optic Nerve Neoplasms / diagnosis. Optic Nerve Neoplasms / pathology. Optic Nerve Neoplasms / radiography. Optic Nerve Neoplasms / surgery

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  • [Copyright] Copyright 2010 Elsevier Masson SAS. All rights reserved.
  • (PMID = 20338600.001).
  • [ISSN] 1773-0619
  • [Journal-full-title] Neuro-Chirurgie
  • [ISO-abbreviation] Neurochirurgie
  • [Language] fre
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] France
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23. Maertens O, Brems H, Vandesompele J, De Raedt T, Heyns I, Rosenbaum T, De Schepper S, De Paepe A, Mortier G, Janssens S, Speleman F, Legius E, Messiaen L: Comprehensive NF1 screening on cultured Schwann cells from neurofibromas. Hum Mutat; 2006 Oct;27(10):1030-40
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Comprehensive NF1 screening on cultured Schwann cells from neurofibromas.
  • Neurofibromatosis type 1 (NF1) is mainly characterized by the occurrence of benign peripheral nerve sheath tumors or neurofibromas.
  • Thorough investigation of the somatic mutation spectrum has thus far been hampered by the large size of the NF1 gene and the considerable proportion of NF1 heterozygous cells within the tumors.
  • We developed an improved somatic mutation detection strategy on cultured Schwann cells derived from neurofibromas and investigated 38 tumors from nine NF1 patients.
  • [MeSH-minor] Adaptor Proteins, Signal Transducing. Carrier Proteins / genetics. DNA Mutational Analysis / methods. DNA Repair / genetics. DNA-Binding Proteins / genetics. Germ-Line Mutation / genetics. Humans. Loss of Heterozygosity. Microsatellite Repeats / genetics. MutS Homolog 2 Protein / genetics. Neurofibroma / genetics. Neurofibroma / pathology. Neurofibromatosis 1 / genetics. Neurofibromatosis 1 / pathology. Nuclear Proteins / genetics. Polymerase Chain Reaction. RNA, Messenger / genetics. RNA, Messenger / metabolism. Sequence Deletion. Tumor Cells, Cultured

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  • (PMID = 16941471.001).
  • [ISSN] 1098-1004
  • [Journal-full-title] Human mutation
  • [ISO-abbreviation] Hum. Mutat.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Adaptor Proteins, Signal Transducing; 0 / Carrier Proteins; 0 / DNA-Binding Proteins; 0 / G-T mismatch-binding protein; 0 / MLH1 protein, human; 0 / Neurofibromin 1; 0 / Nuclear Proteins; 0 / RNA, Messenger; EC 3.6.1.3 / MSH2 protein, human; EC 3.6.1.3 / MutS Homolog 2 Protein
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24. Netsch C, Oberhagemann K, Bach T, Feyerabend B, Gross AJ: [Presacral schwannoma with degenerated areas ("ancient schwannoma")]. Urologe A; 2010 Oct;49(10):1277-82
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  • [Title] [Presacral schwannoma with degenerated areas ("ancient schwannoma")].
  • [Transliterated title] Präsakrales Schwannom mit degenerativ veränderten Anteilen ("ancient schwannoma").
  • A presacral, degenerative schwannoma ("ancient schwannoma") is a rare entity.
  • Tumor heterogeneity with calcifications may be seen in degenerated schwannomas on MRI or CT but not necessarily.
  • We present the case of a 44-year-old male who required surgery for a presacral mass.
  • Histopathological examination revealed the diagnosis of a schwannoma with degenerated areas.
  • [MeSH-major] Neurilemmoma / surgery. Sacrococcygeal Region. Soft Tissue Neoplasms / surgery
  • [MeSH-minor] Adult. Back Pain / etiology. Biomarkers, Tumor / analysis. Calcinosis / diagnosis. Calcinosis / pathology. Calcinosis / surgery. Diagnosis, Differential. Follow-Up Studies. Humans. Magnetic Resonance Imaging. Male. Retroperitoneal Space / surgery. S100 Proteins / analysis. Ultrasonography

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  • (PMID = 20694717.001).
  • [ISSN] 1433-0563
  • [Journal-full-title] Der Urologe. Ausg. A
  • [ISO-abbreviation] Urologe A
  • [Language] ger
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / S100 Proteins
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25. Olszewski J, Konopka W, Radek A, Zieliński K, Pietkiewicz P: [Middle ear schwannoma--case report]. Otolaryngol Pol; 2005;59(6):907-10
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  • [Title] [Middle ear schwannoma--case report].
  • [Transliterated title] Schwannoma ucha środkowego--opis przypadku.
  • Schwannoma is one of the common benign middle ear space tumors.
  • The tumors may present with facial nerve paresis or palsy, otologic symptoms and/or parotid mass middle ear schwannomas may originate from the nerves of the tympanic caviti or by extensions from outside the middle ear space.
  • Schwannomas of the facial nerve can occur along any segment, but they frequently involve the geniculate ganglion and extend proximally or distally from there.
  • We present the clinical and radiologic features of a middle-space schwannoma originating from facial nerve.
  • The tumor was of facial nerve origin and was separated from middle ear.
  • The pathologic diagnosis was schwannoma.
  • [MeSH-major] Ear Neoplasms / pathology. Ear, Middle / pathology. Neurilemmoma / pathology

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  • (PMID = 16521463.001).
  • [ISSN] 0030-6657
  • [Journal-full-title] Otolaryngologia polska = The Polish otolaryngology
  • [ISO-abbreviation] Otolaryngol Pol
  • [Language] pol
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Poland
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26. Wasa J, Nishida Y, Tsukushi S, Shido Y, Sugiura H, Nakashima H, Ishiguro N: MRI features in the differentiation of malignant peripheral nerve sheath tumors and neurofibromas. AJR Am J Roentgenol; 2010 Jun;194(6):1568-74
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  • [Title] MRI features in the differentiation of malignant peripheral nerve sheath tumors and neurofibromas.
  • OBJECTIVE: The objective of this study was to identify the MRI criteria that best differentiate malignant peripheral nerve sheath tumors from benign neurofibromas.
  • MATERIALS AND METHODS: We retrospectively analyzed MR images obtained for 41 histologically diagnosed cases of malignant peripheral nerve sheath tumor and 20 cases of neurofibroma that had been treated at four tertiary institutions.
  • Twenty of the patients with malignant peripheral nerve sheath tumors and 14 patients with neurofibromas developed the disease in association with neurofibromatosis 1.
  • The MR images were evaluated with regard to tumor size, signal intensity, heterogeneity of T1- and T2-weighted MR images, enhancement pattern, definition of margins, presence of perilesional edemalike zone, and presence of intratumoral cystic lesions.
  • RESULTS: Significant differences between malignant peripheral nerve sheath tumors and neurofibromas were noted for the largest dimension of the mass, peripheral enhancement pattern, perilesional edemalike zone, and intratumoral cystic lesion.
  • In cases associated with neurofibromatosis 1, heterogenicity on T1-weighted images was also significant in differentiating neurofibroma from malignant peripheral nerve sheath tumor.
  • The presence of two or more of the four features suggestive of malignancy indicated malignant peripheral nerve sheath tumor with a sensitivity of 61% and a specificity of 90%.
  • CONCLUSION: The MR features described in this study are useful for distinguishing malignant peripheral nerve sheath tumors from neurofibromas.
  • If a tumor has two or more of the four statistically significant features, it can be considered to be highly suspicious of malignancy and should be subjected to a biopsy for early diagnosis.
  • [MeSH-major] Magnetic Resonance Imaging / methods. Nerve Sheath Neoplasms / diagnosis. Neurofibromatosis 1 / pathology

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  • (PMID = 20489098.001).
  • [ISSN] 1546-3141
  • [Journal-full-title] AJR. American journal of roentgenology
  • [ISO-abbreviation] AJR Am J Roentgenol
  • [Language] eng
  • [Publication-type] Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Contrast Media
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27. Salazar F, Machado A, Murthy S, Boulis NM: Thoracoscopically guided transaxillary resection of adjoining intercostal plexiform neurofibromas: review of mosaicism in neurofibromatosis: technical note. Neurosurgery; 2005 Oct;57(4 Suppl):E407; discussion E407
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  • [Title] Thoracoscopically guided transaxillary resection of adjoining intercostal plexiform neurofibromas: review of mosaicism in neurofibromatosis: technical note.
  • OBJECTIVE AND IMPORTANCE: The present article describes a rare presentation of Type I neurofibromatosis (NFI) involving adjoining intercostal plexiform neurofibromas, as well as the novel use of thoracoscopy to guide surgical resection.
  • Biopsy of a subcutaneous nodule in the right chest wall revealed a small neurofibroma.
  • INTERVENTION: The patient underwent right-sided thoracoscopy for identification of the intrathoracic neurofibromas and placement of spinal needles to localize the anterior and posterior extent of the masses.
  • These landmarks were used to guide a transaxillary approach to third rib resection and nerve-sparing neurofibroma resection.
  • Thoracoscopy can be used effectively to guide intercostal nerve sheath tumor resection.
  • [MeSH-major] Abdominal Neoplasms / surgery. Axilla / pathology. Laminectomy / methods. Mosaicism. Neurofibromatosis 1 / surgery. Thoracoscopy
  • [MeSH-minor] Adult. Humans. Magnetic Resonance Imaging / methods. Male. Retroperitoneal Neoplasms. Tomography, X-Ray Computed / methods

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  • (PMID = 16234660.001).
  • [ISSN] 1524-4040
  • [Journal-full-title] Neurosurgery
  • [ISO-abbreviation] Neurosurgery
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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28. Hunter S, Weiss S, Ou CY, Jaye D, Young A, Wilcox J, Arbiser JL, Monson D, Goldblum J, Nolen JD, Varma V: Apolipoprotein D is down-regulated during malignant transformation of neurofibromas. Hum Pathol; 2005 Sep;36(9):987-93
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  • [Title] Apolipoprotein D is down-regulated during malignant transformation of neurofibromas.
  • Apolipoprotein D (apoD) expression was studied in nonneoplastic peripheral nerve, neurofibromas (NFs), and malignant peripheral nerve sheath tumors (MPNSTs) by quantitative polymerase chain reaction, in situ hybridization, and immunohistochemistry.
  • ApoD expression levels were 3.0-fold elevated (DeltaCt = 1.7) in the NFs compared with nonneoplastic peripheral nerve (P < .05).
  • ApoD expression initially increases a small amount with the formation of NFs from nonneoplastic peripheral nerve and subsequently decreases markedly as NFs transform into MPNSTs.
  • This expression pattern may serve as a marker for cell cycle inhibition during peripheral nerve tumorigenesis.
  • [MeSH-major] Apolipoproteins / metabolism. Cell Transformation, Neoplastic. Neurofibroma / metabolism. Neurofibroma / pathology. Peripheral Nervous System Neoplasms / metabolism. Peripheral Nervous System Neoplasms / pathology
  • [MeSH-minor] Apolipoproteins D. Down-Regulation. Humans. Immunohistochemistry. In Situ Hybridization. Nerve Sheath Neoplasms / metabolism. Nerve Sheath Neoplasms / pathology. Polymerase Chain Reaction

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  • (PMID = 16153462.001).
  • [ISSN] 0046-8177
  • [Journal-full-title] Human pathology
  • [ISO-abbreviation] Hum. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Apolipoproteins; 0 / Apolipoproteins D
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29. Dundr P, Povýsil C, Tvrdík D: Actin expression in neural crest cell-derived tumors including schwannomas, malignant peripheral nerve sheath tumors, neurofibromas and melanocytic tumors. Pathol Int; 2009 Feb;59(2):86-90
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  • [Title] Actin expression in neural crest cell-derived tumors including schwannomas, malignant peripheral nerve sheath tumors, neurofibromas and melanocytic tumors.
  • Tumors that originate from neural crest-derived cells represent a heterogeneous group of neoplasms including benign and malignant tumors with melanocytic and schwannian differentiation.
  • The immunophenotype of these tumors is well known but little is known about the expression of smooth muscle/myofibroblastic markers in these tumors.
  • A total of 590 neural crest-derived tumors (50 benign schwannomas, five malignant peripheral nerve sheath tumors, 80 neurofibromas, 240 nevocytic nevi, 115 primary melanomas, and 100 melanoma metastases) were studied with respect to alpha-smooth muscle actin and muscle-specific actin expression. alpha-Smooth muscle actin and muscle-specific actin-positive tumor cells with a co-expression of S-100 protein were found in one benign schwannoma, one primary cutaneous melanoma, and four melanoma metastases.
  • Four of these cases were examined ultrastructurally, but typical actin filaments with focal densities were not found in any of the four.
  • Other immunohistochemical markers examined including desmin, h-caldesmon and smooth muscle myosin heavy chain were negative in the tumor cells.
  • The present results suggest that neural crest-derived tumors could show expression of alpha-smooth muscle actin on rare occasion.
  • [MeSH-major] Actins / metabolism. Melanoma / secondary. Nerve Sheath Neoplasms / pathology. Neurilemmoma / pathology. Neurofibroma / pathology. Nevus, Pigmented / pathology
  • [MeSH-minor] Biomarkers, Tumor / metabolism. Humans. Melanocytes / metabolism. Melanocytes / pathology. Neural Crest / metabolism. Neural Crest / pathology

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  • (PMID = 19154261.001).
  • [ISSN] 1440-1827
  • [Journal-full-title] Pathology international
  • [ISO-abbreviation] Pathol. Int.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Australia
  • [Chemical-registry-number] 0 / Actins; 0 / Biomarkers, Tumor
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30. Perrin GQ, Fishbein L, Thomson SA, Thomas SL, Stephens K, Garbern JY, DeVries GH, Yachnis AT, Wallace MR, Muir D: Plexiform-like neurofibromas develop in the mouse by intraneural xenograft of an NF1 tumor-derived Schwann cell line. J Neurosci Res; 2007 May 1;85(6):1347-57
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  • [Title] Plexiform-like neurofibromas develop in the mouse by intraneural xenograft of an NF1 tumor-derived Schwann cell line.
  • Plexiform neurofibromas are peripheral nerve sheath tumors that arise frequently in neurofibromatosis type 1 (NF1) and have a risk of malignant progression.
  • Past efforts to establish xenograft models for neurofibroma involved the implantation of tumor fragments or heterogeneous primary cultures, which rarely achieved significant tumor growth.
  • We report a practical and reproducible animal model of plexiform-like neurofibroma by xenograft of an immortal human NF1 tumor-derived Schwann cell line into the peripheral nerve of scid mice.
  • Localized intraneural injection of the cell line sNF94.3 produced consistent and slow growing tumors that infiltrated and disrupted the host nerve.
  • The xenograft tumors resembled plexiform neurofibromas with a low rate of proliferation, abundant extracellular matrix (hypocellularity), basal laminae, high vascularity, and mast cell infiltration.
  • The histologic features of the developed tumors were particularly consistent with those of human plexiform neurofibroma as well.
  • Intraneural xenograft of sNF94.3 cells enables the precise initiation of intraneural, plexiform-like tumors and provides a highly reproducible model for the study of plexiform neurofibroma tumorigenesis.
  • [MeSH-major] Cell Line, Tumor. Lung Neoplasms / pathology. Neurofibromatosis 1 / pathology. Schwann Cells / cytology
  • [MeSH-minor] Adult. Animals. Blotting, Western. Female. Humans. Mice. Mice, SCID. Neoplasm Transplantation / methods. Nerve Tissue Proteins / metabolism. Neurofibromin 1 / genetics. Transplantation, Heterologous / methods. ras Proteins / metabolism

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  • [Copyright] (c) 2007 Wiley-Liss, Inc.
  • (PMID = 17335073.001).
  • [ISSN] 0360-4012
  • [Journal-full-title] Journal of neuroscience research
  • [ISO-abbreviation] J. Neurosci. Res.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / T32-CA09126-27
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, U.S. Gov't, Non-P.H.S.
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Nerve Tissue Proteins; 0 / Neurofibromin 1; EC 3.6.5.2 / ras Proteins
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31. Fiengo L, Patrizi G, Cancrini G, Cinconze F, Solai F, Arcieri S: [Thyreopathy associated with vagus schwannoma]. Ann Ital Chir; 2007 Jan-Feb;78(1):73-5
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  • [Title] [Thyreopathy associated with vagus schwannoma].
  • [Transliterated title] Tireopatia associata a schwannoma del vago.
  • We report the case of a young woman who underwent total thyroidectomy for a suspect nodular thyreopathy and occasionally happened to be affected simultaneously by vagus nerve schwannoma.
  • [MeSH-major] Cranial Nerve Neoplasms / surgery. Goiter, Nodular / surgery. Neurilemmoma / surgery. Vagus Nerve

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  • (PMID = 17518337.001).
  • [ISSN] 0003-469X
  • [Journal-full-title] Annali italiani di chirurgia
  • [ISO-abbreviation] Ann Ital Chir
  • [Language] ita
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Italy
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32. Chopra R, Morris CG, Friedman WA, Mendenhall WM: Radiotherapy and radiosurgery for benign neurofibromas. Am J Clin Oncol; 2005 Jun;28(3):317-20
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  • [Title] Radiotherapy and radiosurgery for benign neurofibromas.
  • The purpose of this study was to evaluate the efficacy of radiotherapy (RT) and stereotactic radiosurgery (SRS) for neurofibromas.
  • The tumor remained locally controlled in all patients.
  • RT and SRS are likely to locally control neurofibromas in patients who require treatment and are not good candidates for complete resection.
  • [MeSH-major] Head and Neck Neoplasms / therapy. Neurofibroma / therapy. Radiosurgery
  • [MeSH-minor] Adult. Child. Female. Humans. Male. Maxillary Sinus Neoplasms / radiotherapy. Maxillary Sinus Neoplasms / surgery. Orbital Neoplasms / radiotherapy. Orbital Neoplasms / surgery. Peripheral Nervous System Neoplasms / radiotherapy. Peripheral Nervous System Neoplasms / surgery. Pharyngeal Neoplasms / radiotherapy. Pharyngeal Neoplasms / surgery. Radiotherapy, Adjuvant. Retrospective Studies. Spinal Cord Neoplasms / radiotherapy. Spinal Cord Neoplasms / surgery. Spinal Nerve Roots / surgery. Thoracic Nerves. Treatment Outcome

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  • (PMID = 15923807.001).
  • [ISSN] 1537-453X
  • [Journal-full-title] American journal of clinical oncology
  • [ISO-abbreviation] Am. J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Evaluation Studies; Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 32
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33. Ağirdir BV, Turhan M, Derin A: [Endoscopic surgical management of nasal septal schwannoma: a case report]. Kulak Burun Bogaz Ihtis Derg; 2005;14(3-4):83-6
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  • [Title] [Endoscopic surgical management of nasal septal schwannoma: a case report].
  • [Transliterated title] Nazal septal schwannoma ve endoskopik cerrahi tedavisi: olgu sunumu.
  • Although benign tumors arising from the nasal cavity are common, those of the peripheral nerve sheath seen in this localization are unusual.
  • We presented a 45-year-old woman with a nasal septal schwannoma filling the whole nasal cavity.
  • [MeSH-major] Neurilemmoma / diagnosis. Nose Neoplasms / diagnosis

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  • (PMID = 16227730.001).
  • [ISSN] 1300-7475
  • [Journal-full-title] Kulak burun boğaz ihtisas dergisi : KBB = Journal of ear, nose, and throat
  • [ISO-abbreviation] Kulak Burun Bogaz Ihtis Derg
  • [Language] tur
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Turkey
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34. Hornick JL, Bundock EA, Fletcher CD: Hybrid schwannoma/perineurioma: clinicopathologic analysis of 42 distinctive benign nerve sheath tumors. Am J Surg Pathol; 2009 Oct;33(10):1554-61
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  • [Title] Hybrid schwannoma/perineurioma: clinicopathologic analysis of 42 distinctive benign nerve sheath tumors.
  • Benign nerve sheath tumors include neurofibromas, schwannomas, and perineuriomas.
  • In recent years, nerve sheath tumors showing discrete areas of more than one histologic type have been described.
  • We have recently recognized tumors showing hybrid features of schwannoma and soft tissue perineurioma.
  • The tumors arose in a wide distribution: 19 lower limb, 12 upper limb, 6 head and neck, 4 trunk, and 1 colon.
  • Tumor size ranged from 0.7 to 17.5 cm (mean, 3 cm).
  • Most tumors involved superficial subcutis (11 also dermis); only 3 were intramuscular.
  • Histologically, the tumors were usually well circumscribed but unencapsulated, and composed of spindle cells with plump, tapering nuclei, and palely eosinophilic cytoplasm with indistinct cell borders, arranged in a storiform, whorled, and/or lamellar architecture.
  • Only 1 tumor showed infiltrative margins.
  • One tumor showed a plexiform growth pattern.
  • Six tumors showed focally myxoid stroma and 11 contained scattered cells with degenerative nuclear atypia.
  • Mitoses ranged from 0 to 4 per 30 high power fields; 32 tumors had no mitoses.
  • All tumors showed staining for S100 protein and EMA; 98% were positive for CD34, 84% for GFAP, and 80% for claudin-1.
  • Fourteen tumors contained rare neurofilament protein-positive axons.
  • Most tumors were composed of approximately 60% to 70% of Schwann cells and 30% to 40% of perineurial cells.
  • After a mean follow-up of 24 months (range, 6 to 60 mo), 1 tumor recurred locally, after incomplete excision.
  • Benign nerve sheath tumors showing predominantly schwannian cytomorphology and perineurioma-like architecture are composed of an admixture of both cell types.
  • These tumors usually arise in the dermis and subcutis and occur over a wide age range and anatomic distribution.
  • Degenerative nuclear atypia (akin to that seen in ancient schwannoma and atypical neurofibroma) is relatively common.
  • Hybrid schwannoma/perineuriomas have no evident association with neurofibromatosis and rarely recur.
  • [MeSH-major] Nerve Sheath Neoplasms / pathology. Neurilemmoma / pathology. Soft Tissue Neoplasms / pathology
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Biomarkers, Tumor / analysis. Child. Child, Preschool. Female. Humans. Immunohistochemistry. Male. Middle Aged. Mucin-1 / biosynthesis. S100 Proteins / biosynthesis. Young Adult

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  • (PMID = 19623031.001).
  • [ISSN] 1532-0979
  • [Journal-full-title] The American journal of surgical pathology
  • [ISO-abbreviation] Am. J. Surg. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Mucin-1; 0 / S100 Proteins
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35. Grasa Arnal AM, Abad Roger J, Bono Ariño A, Vera Alvarez JJ, Sanz Vélez JI: [Old schwannoma: a rare type of schwannoma]. Arch Esp Urol; 2005 Mar;58(2):164-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Old schwannoma: a rare type of schwannoma].
  • [Transliterated title] Schwannoma antiguo: variante rara de schwannoma.
  • OBJECTIVES: We report one case of retroperitoneal schwannoma with unfrequent histological characteristics evaluating its clinical significance and therapeutic options.
  • METHODS: We report the case of a benign retroperitoneal schwannoma as an incidental MRI finding in a 70 year-old female patient with symptoms of lumbosciatic pain for 6 months.
  • RESULTS: After surgical excision the diagnosis was retroperitoneal old schwannoma.
  • The histological changes apparent in the cases of old schwannoma have been interpreted as degenerative, indicating a long evolution, which has motivated its denomination as old.
  • CONCLUSIONS: The old schwannoma is a rare variety of the benign common schwannoma.
  • [MeSH-major] Neurilemmoma / pathology. Retroperitoneal Neoplasms / pathology

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  • (PMID = 15847275.001).
  • [ISSN] 0004-0614
  • [Journal-full-title] Archivos españoles de urología
  • [ISO-abbreviation] Arch. Esp. Urol.
  • [Language] spa
  • [Publication-type] Case Reports; English Abstract; Journal Article; Review
  • [Publication-country] Spain
  • [Number-of-references] 10
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36. Morais D, Santos J, Alonso M, Ovelar Y: [Schwannoma of the external auditory canal: an exceptional location]. Acta Otorrinolaringol Esp; 2007 Apr;58(4):169-70
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  • [Title] [Schwannoma of the external auditory canal: an exceptional location].
  • [Transliterated title] Schwannoma de conducto auditivo externo: una localización excepcional.
  • Solitary schwannoma is a benign, encapsulated tumour of Schwann cell origin, therefore the olfactory and optic nerves are never affected.
  • About 25%-45% of all schwannomas occur in the head and neck but schwannoma of the external auditory canal is a rare finding, and we have found only 6 previous cases reported in the literature world-wide.
  • Our patient was discovered by chance during a stapedectomy because the tumour was sited in the external auditory canal without distorting it.
  • [MeSH-major] Ear Neoplasms / pathology. Ear, External / pathology. Neurilemmoma / pathology

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  • (PMID = 17428415.001).
  • [ISSN] 0001-6519
  • [Journal-full-title] Acta otorrinolaringológica española
  • [ISO-abbreviation] Acta Otorrinolaringol Esp
  • [Language] spa
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Spain
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37. Maseda E, Ablanedo A, Blanco R, Díaz C, Martín A: [Intra-labyrinthine schwannoma: two-cases report]. Acta Otorrinolaringol Esp; 2007 Feb;58(2):70-2
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  • [Title] [Intra-labyrinthine schwannoma: two-cases report].
  • [Transliterated title] Schwannoma intralaberíntico: a propósito de dos casos.
  • Intralabyrinthine schwannomas are uncommon tumours that arise from neural elements in distal branches of the 8th cranial nerve thus they are confined to or have arisen from the vestibule, the semi-circular canals, or the cochlea.
  • The most common presenting symptom is progressive sensory neural hearing loss with or without tinnitus and vestibular symptoms.
  • We present 2 patients with a diagnosis of intralabyrinthine schwannoma.
  • A discussion of the management of the intralabyrinthine schwannoma follows.

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  • (PMID = 17371686.001).
  • [ISSN] 0001-6519
  • [Journal-full-title] Acta otorrinolaringológica española
  • [ISO-abbreviation] Acta Otorrinolaringol Esp
  • [Language] spa
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Spain
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38. Gokalp G, Hakyemez B, Kizilkaya E, Haholu A: Myxoid neurofibromas of the breast: mammographical, sonographical and MRI appearances. Br J Radiol; 2007 Oct;80(958):e234-7
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  • [Title] Myxoid neurofibromas of the breast: mammographical, sonographical and MRI appearances.
  • Neurofibromas arise from elements in the peripheral nervous system and are rarely detected in the breast.
  • Neurofibromas are more common in neurofibromatosis Type 1.
  • In this paper, we present a patient with neurofibromatosis Type 1 with mammographical, sonographical and MRI findings of myxoid neurofibromas in her left breast.
  • [MeSH-major] Breast Neoplasms / diagnosis. Neurofibroma / diagnosis

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  • (PMID = 17959912.001).
  • [ISSN] 1748-880X
  • [Journal-full-title] The British journal of radiology
  • [ISO-abbreviation] Br J Radiol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
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39. Fornaro R, Frascio M, Stabilini C, Ricci B, Mandolfino F, Picori E, Sticchi C, Boccardo C, Gianetta E: [Excision of a schwannoma of the neck: surgical technique]. G Chir; 2006 Nov-Dec;27(11-12):428-32
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  • [Title] [Excision of a schwannoma of the neck: surgical technique].
  • [Transliterated title] Exeresi di uno schwannoma del collo: note di tecnica chirurgica
  • We present a case of schwannoma of the neck in 49 year old man, symptomatic (paresthesia, cervical mass and dysphagia).
  • The tumour arised from the cervical sympathetic chain.
  • The diagnosis of schwannoma was possible only by histopathologic examen.
  • The identification of the nerve is often difficult until the operation, which is the treatment of choice for the schwannoma.
  • [MeSH-major] Head and Neck Neoplasms / surgery. Neurilemmoma / surgery

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  • (PMID = 17198552.001).
  • [ISSN] 0391-9005
  • [Journal-full-title] Il Giornale di chirurgia
  • [ISO-abbreviation] G Chir
  • [Language] ita
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Italy
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40. Pummi KP, Aho HJ, Laato MK, Peltonen JT, Peltonen SA: Tight junction proteins and perineurial cells in neurofibromas. J Histochem Cytochem; 2006 Jan;54(1):53-61
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  • [Title] Tight junction proteins and perineurial cells in neurofibromas.
  • Cutaneous neurofibromas consist of axonal processes, Schwann cells, fibroblasts, perineurial cells, mast cells, and abundant extracellular matrix.
  • The distribution and role of perineurial cells in neurofibromas has been uncertain, partly because there has not been a specific immunohistochemical marker for perineurial cells.
  • In this study, tight junctions (TJs) of 16 neurofibromas from 12 patients with neurofibromatosis type 1 (NF1) were analyzed using electron microscopy, immunohistochemistry, and Western transfer analysis.
  • Cell-cell contacts with typical ultrastructural morphology of TJs were seen between adjacent perineurial cells surrounding the small nerves and between contacting perineurial cell processes embedded in tumor stroma.
  • Immunohistochemistry showed expression of claudin-1, claudin-3, and ZO-1 in the intercellular junctions of a subpopulation of tumor cells.
  • The results showed that claudin-1 positive cells were also positive for type IV collagen and epithelial membrane antigen but not for S-100 protein.
  • Using claudin-1 as a marker, our results showed that clusters of perineurial cells are distributed around the rudimentary nerves within cutaneous neurofibromas and at the periphery of some neurofibromas.

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  • (PMID = 16087703.001).
  • [ISSN] 0022-1554
  • [Journal-full-title] The journal of histochemistry and cytochemistry : official journal of the Histochemistry Society
  • [ISO-abbreviation] J. Histochem. Cytochem.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / CLDN1 protein, human; 0 / CLDN3 protein, human; 0 / CLDN5 protein, human; 0 / Claudin-1; 0 / Claudin-3; 0 / Claudin-5; 0 / Membrane Proteins; 0 / OCLN protein, human; 0 / Occludin; 0 / Phosphoproteins; 0 / TJP1 protein, human; 0 / Zonula Occludens-1 Protein
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41. Coscarón Blanco E, Muñoz Herrera A, Serradilla López JM, Maillo Sánchez A, Paniagua Escudero JC: [Clinical picture of 8th pair schwannoma. Is it expressive enough?]. Acta Otorrinolaringol Esp; 2007 Feb;58(2):43-7
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  • [Title] [Clinical picture of 8th pair schwannoma. Is it expressive enough?].
  • [Transliterated title] Caracterización clínica del schwannoma del VIII par. Son suficientemente expresivos sus síntomas?
  • OBJECTIVE: To describe the stage-related clinical features of 8th cranial nerve schwannoma.
  • CONCLUSIONS: There is no typical clinical pattern and no typical first symptom in 8th cranial nerve schwannomas.
  • Any audiovestibular or facial symptom, even the slightest, may be the first expression of 8th cranial nerve schwannoma.

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  • [CommentIn] Acta Otorrinolaringol Esp. 2007 Oct;58(8):381; author reply 382-3 [17949669.001]
  • (PMID = 17371680.001).
  • [ISSN] 0001-6519
  • [Journal-full-title] Acta otorrinolaringológica española
  • [ISO-abbreviation] Acta Otorrinolaringol Esp
  • [Language] spa
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Spain
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42. Song JY, Kim SY, Park EG, Kim CJ, Kim DG, Lee HK, Park IY: Schwannoma in the retroperitoneum. J Obstet Gynaecol Res; 2007 Jun;33(3):371-5
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  • [Title] Schwannoma in the retroperitoneum.
  • Schwannoma (neurilemmoma) is a peripheral nerve sheath tumor and commonly occurs singularly on the head, neck, and trunk.
  • Giant schwannoma is rarely located on the retroperitoneum and pelvic cavity.
  • The majority of symptoms caused by the tumor are due to the effect of its mass.
  • Surgical resection is enough to treat the tumor.
  • Schwannoma is reported usually as benign, and despite incomplete resection of the tumor, the risk of recurrence and metastasis is low.
  • A schwannoma on the retroperitoneum that was preoperatively misdiagnosed as a malignant adnexal mass in a 60-year-old menopausal woman is presented, with a brief review of the literature.
  • [MeSH-major] Neurilemmoma / pathology. Retroperitoneal Neoplasms / pathology. Retroperitoneal Space / pathology

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  • (PMID = 17578370.001).
  • [ISSN] 1341-8076
  • [Journal-full-title] The journal of obstetrics and gynaecology research
  • [ISO-abbreviation] J. Obstet. Gynaecol. Res.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] Japan
  • [Number-of-references] 19
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43. Chen W, Jia JW, Wang JR: Soft tissue diffuse neurofibromas: sonographic findings. J Ultrasound Med; 2007 Apr;26(4):513-8
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  • [Title] Soft tissue diffuse neurofibromas: sonographic findings.
  • OBJECTIVE: The purpose of this study was to describe the sonographic findings of soft tissue diffuse neurofibromas.
  • METHODS: Seven soft tissue diffuse neurofibromas proven pathologically were retrospectively evaluated.
  • CONCLUSIONS: Soft tissue diffuse neurofibromas have a characteristic sonographic appearance; therefore, sonography should be considered a useful tool in the initial screening of this disease.
  • [MeSH-major] Neurofibroma / ultrasonography. Soft Tissue Neoplasms / ultrasonography. Ultrasonography, Doppler, Color

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  • (PMID = 17384049.001).
  • [ISSN] 0278-4297
  • [Journal-full-title] Journal of ultrasound in medicine : official journal of the American Institute of Ultrasound in Medicine
  • [ISO-abbreviation] J Ultrasound Med
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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44. Zehlicke T, Lessle M, Gramer L: [The "ancient schwannoma". A rare tumor of the mouth floor]. HNO; 2006 Apr;54(4):307-11
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  • [Title] [The "ancient schwannoma". A rare tumor of the mouth floor].
  • [Transliterated title] Das "ancient schwannoma". Die seltene Form eines Mundbodentumors.
  • "Ancient schwannomas" of the mouth floor are rare, benign neoplasms derived from the nerve sheath of peripheral nerves.
  • Ancient schwannomas show histopathological features, such as degenerative changes and atypical nuclei, and may easily be confused with malignant neoplasms.
  • Here, we report on a patient with ancient schwannoma of the floor of the mouth.
  • [MeSH-major] Cranial Nerve Neoplasms / diagnosis. Hypoglossal Nerve Diseases / diagnosis. Mouth Floor. Mouth Neoplasms / diagnosis. Neurilemmoma / diagnosis
  • [MeSH-minor] Biomarkers, Tumor / analysis. Diagnosis, Differential. Female. Humans. Ki-67 Antigen / analysis. Lymph Nodes / pathology. Magnetic Resonance Imaging. Male. Middle Aged. Prognosis. Ultrasonography

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  • (PMID = 15951994.001).
  • [ISSN] 0017-6192
  • [Journal-full-title] HNO
  • [ISO-abbreviation] HNO
  • [Language] ger
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Ki-67 Antigen
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45. Kashyap S, Pushker N, Meel R, Sen S, Bajaj MS, Khuriajam N, Mehta M, Chawla B: Orbital schwannoma with cystic degeneration. Clin Exp Ophthalmol; 2009 Apr;37(3):293-8
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  • [Title] Orbital schwannoma with cystic degeneration.
  • BACKGROUND: Schwannoma is a benign, slowly growing, painless, peripheral nerve sheath tumour that is uncommon in orbit.
  • Extensive cystic change in orbital schwannoma is quite rare.
  • We conducted this study to analyse the cases of orbital schwannoma, which were predominantly cystic and describe their clinical, imaging and histopathological features.
  • The clinical, imaging and histopathological features of patients with cystic schwannoma were studied.
  • We found 39 cases of orbital schwannoma.
  • The radiological picture was quite variable resulting in a primary diagnosis of combined venous-lymphatic vascular malformation in 2, lacrimal gland tumour in 2, dermoid cyst in 1, hydatid cyst in 2 and schwannoma in 5 cases.
  • CONCLUSION: Orbital schwannoma is a great masquerader in the orbit.
  • Extensive cystic changes in schwannoma are uncommonly reported.
  • Cystic schwannoma constituted 41% of all orbital schwannomas in our series.
  • [MeSH-major] Cysts / pathology. Neurilemmoma / pathology. Orbital Neoplasms / pathology

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  • (PMID = 19472538.001).
  • [ISSN] 1442-9071
  • [Journal-full-title] Clinical & experimental ophthalmology
  • [ISO-abbreviation] Clin. Experiment. Ophthalmol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Australia
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46. Thomas JA, Bank WO, Myseros JS: Glossopharyngeal schwannoma in childhood. J Neurosurg Pediatr; 2008 Aug;2(2):130-2
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  • [Title] Glossopharyngeal schwannoma in childhood.
  • Glossopharyngeal (that is, cranial nerve IX) schwannomas are extremely rare nerve sheath tumors that frequently mimic the more common vestibular schwannoma in their clinical as well as radiographic presentation.
  • Although rare in adults, this tumor has not been reported in a child.
  • The authors report the case of a 10-year-old boy who presented with several months of unilateral hearing loss.
  • He was found to have a large right cerebellopontine angle tumor.
  • Given the boy's primary complaint of hearing loss and the appearance of the lesion on imaging, the tumor was initially believed to be a schwannoma of the vestibular nerve.
  • It was found intraoperatively, however, to originate from the glossopharyngeal nerve.
  • To the authors' knowledge, this is the first reported case of a glossopharyngeal schwannoma in a child.
  • [MeSH-major] Cranial Nerve Neoplasms / diagnosis. Glossopharyngeal Nerve Diseases / diagnosis. Neurilemmoma / diagnosis

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  • (PMID = 18671618.001).
  • [ISSN] 1933-0707
  • [Journal-full-title] Journal of neurosurgery. Pediatrics
  • [ISO-abbreviation] J Neurosurg Pediatr
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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47. Alfonso C, Lassaletta L, Sarriá J, Gavilán J: [Quality of life following vestibular schwannoma surgery]. Acta Otorrinolaringol Esp; 2007 Feb;58(2):61-5
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  • [Title] [Quality of life following vestibular schwannoma surgery].
  • [Transliterated title] Calidad de vida tras la cirugía del schwannoma vestibular.
  • OBJECTIVE: This study evaluates quality of life (QOL) in patients following surgery for vestibular schwannoma (VS) using the Glasgow Benefit Inventory (GBI), and a pain questionnaire.
  • No differences in postoperative QOL were found when comparing side, gender, age, tumor size, hearing preservation, or post-operative facial function.

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  • (PMID = 17371684.001).
  • [ISSN] 0001-6519
  • [Journal-full-title] Acta otorrinolaringológica española
  • [ISO-abbreviation] Acta Otorrinolaringol Esp
  • [Language] spa
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Spain
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48. Garcia-Escrivà A, Pampliega Pérez A, Martín-Estefania C, Botella C: [Schwannoma of the hypoglossal nerve presenting as a syndrome of Collet-Sicard]. Neurologia; 2005 Jul-Aug;20(6):311-3
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  • [Title] [Schwannoma of the hypoglossal nerve presenting as a syndrome of Collet-Sicard].
  • [Transliterated title] Síndrome de Collet-Sicard como presentación de schwannoma del nervio hipogloso.
  • We describe this entity in relation with a schwannoma of the hypoglossal nerve.
  • The cranial MRI revealed a small intracranial and extracranial tumor adjacent to the hypoglossal foramen.
  • The tumor involved the jugular foramen and was moderately enhanced with gadolinium The patient underwent surgical removal.
  • The pathologic examination of the surgical specimen confirmed the diagnosis of a schwannoma of the hypoglossal nerve.
  • Although schwannoma the hypoglossal nerve is a rare disorder we consider that this entity should be included in the differential diagnosis of the Collet-Sicard syndrome.
  • [MeSH-major] Hypoglossal Nerve Diseases. Neurilemmoma. Paralysis / etiology
  • [MeSH-minor] Accessory Nerve / pathology. Glossopharyngeal Nerve / pathology. Humans. Magnetic Resonance Angiography. Male. Syndrome. Vagus Nerve / pathology

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  • (PMID = 16007514.001).
  • [ISSN] 0213-4853
  • [Journal-full-title] Neurología (Barcelona, Spain)
  • [ISO-abbreviation] Neurologia
  • [Language] spa
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Spain
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49. Gómez García I, Rodríguez Patrón R, Conde Someso S, Sanz Mayayo E, Quicios Dorado C, Palmeiro A: [Benign retroperitoneal schwannoma. Incidental diagnostic in patient with hematuria of the percusionist]. Actas Urol Esp; 2005 May;29(5):511-5
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  • [Title] [Benign retroperitoneal schwannoma. Incidental diagnostic in patient with hematuria of the percusionist].
  • [Transliterated title] Schwannoma retroperitoneal benigno. Diagnostico incidental en paciente con hematuria del percusionista.
  • The incidence of retroperitoneal primitive tumour varies from the 0.3 to 3%.
  • The sarcomas suppose the group but it frequents of retroperitoneal tumour, being the Schwannoma an unusual tumour with an incidence from 1% to 50% of the retroperitoneal primary tumours.
  • The schwannoma also denominated neurinoma or neurolenoma, it is a derived tumour of the cells of Schwann of the outlying nerves.
  • The election treatment is the surgical remove, with wide margins; not being described cases of malignización neither of metastasis at distance, but if the recurrence existence at probably secondary local level to incomplete resection.
  • [MeSH-major] Hematuria / etiology. Neurilemmoma / complications. Retroperitoneal Neoplasms / complications

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  • (PMID = 16013798.001).
  • [ISSN] 0210-4806
  • [Journal-full-title] Actas urologicas españolas
  • [ISO-abbreviation] Actas Urol Esp
  • [Language] spa
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Spain
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50. Martínez-Soto L, Alfaro-Baca R, Torrecilla-Sardón MV, Fernández-Vallejo B, Ferreira-Muñóz R, De Diego T: [A new case of "olfactory schwannoma"; presentation and literature review]. Neurocirugia (Astur); 2009 Jun;20(3):294-7
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  • [Title] [A new case of "olfactory schwannoma"; presentation and literature review].
  • [Transliterated title] Un nuevo caso de "schwannoma del olfatorio"; presentación y revisión de laliteratura.
  • We report the case of a 54-year-old man who presented at the Emergency Department with intense headache of 6-days duration and sporadic nominal dysphasia.
  • He did not present anosmia and the rest of the examination was normal.
  • After total removal by bifrontal craniotomy the histopathological diagnosis was schwannoma of the conventional type.
  • Owing to the unusual frequency of this kind of tumors (26 to the date), we review the literature, the possible radiological differences with olfactory groove meningiomas and the different theories about their origin.
  • [MeSH-major] Brain Neoplasms. Neurilemmoma

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  • (PMID = 19575136.001).
  • [ISSN] 1130-1473
  • [Journal-full-title] Neurocirugía (Asturias, Spain)
  • [ISO-abbreviation] Neurocirugia (Astur)
  • [Language] spa
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Spain
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51. Mingione A, Cirillo C, Martucci N, Mingione S: [Benign retroperitoneal schwannoma: a case report and review of the literature]. Chir Ital; 2009 Jan-Feb;61(1):107-12
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  • [Title] [Benign retroperitoneal schwannoma: a case report and review of the literature].
  • [Transliterated title] Schwannoma benigno del retroperitoneo: caso clinico e revisione della letteratura.
  • Retroperitoneal schwannoma is a very rare benign tumour that accounts for only a small percentage of retroperitoneal tumours.
  • The authors report their experience with a case of a voluminous retroperitoneal mass in a 42-year- old male patient, incidentally discovered during a diagnostic search for a blunt abdominal trauma.
  • Histological examination of the surgical specimen revealed an "ancient schwannoma".
  • The authors, after a short discussion of the subject, go on to examine the diagnostic and therapeutic treatments of this rare benign neoplasm, which always arouses lively clinical and scientific interest.
  • [MeSH-major] Neurilemmoma. Retroperitoneal Neoplasms

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  • (PMID = 19391348.001).
  • [ISSN] 0009-4773
  • [Journal-full-title] Chirurgia italiana
  • [ISO-abbreviation] Chir Ital
  • [Language] ita
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Italy
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52. Son JM, Ahn MI, Cho KD, Yoo J, Park YH: Varying degrees of FDG uptake in multiple benign neurofibromas on PET/CT. Br J Radiol; 2007 Sep;80(957):e222-6
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  • [Title] Varying degrees of FDG uptake in multiple benign neurofibromas on PET/CT.
  • We report fluorodeoxyglucose (FDG) positron emission tomography (PET)/computed tomography (CT) findings of three neurofibromas in the mediastinum and neck in a 26-year-old woman with neurofibromatosis type 1.
  • Surgical resections were carried out and histopathology confirmed three benign neurofibromas with various tissue components and cellularities.
  • [MeSH-major] Fluorodeoxyglucose F18. Head and Neck Neoplasms. Mediastinal Neoplasms. Neurofibromatosis 1. Radiopharmaceuticals
  • [MeSH-minor] Adult. Female. Humans. Incidental Findings. Positron-Emission Tomography / methods. Tracheal Neoplasms / radiography. Tracheal Neoplasms / radionuclide imaging

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  • (PMID = 17928494.001).
  • [ISSN] 1748-880X
  • [Journal-full-title] The British journal of radiology
  • [ISO-abbreviation] Br J Radiol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Radiopharmaceuticals; 0Z5B2CJX4D / Fluorodeoxyglucose F18
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53. Liu K, DeAngelo P, Mahmet K, Phytides P, Osborne L, Pletcher BA: Cytogenetics of neurofibromas: two case reports and literature review. Cancer Genet Cytogenet; 2010 Jan 1;196(1):93-5
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  • [Title] Cytogenetics of neurofibromas: two case reports and literature review.
  • Only a few karyotypes of neurofibromas have been documented in the literature.

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  • (PMID = 19963142.001).
  • [ISSN] 1873-4456
  • [Journal-full-title] Cancer genetics and cytogenetics
  • [ISO-abbreviation] Cancer Genet. Cytogenet.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 16
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54. La Francesca S, Gregoric ID, Cohn WE, Frazier OH: Successful resection of a primary left ventricular schwannoma. Ann Thorac Surg; 2007 May;83(5):1881-2
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  • [Title] Successful resection of a primary left ventricular schwannoma.
  • Nerve sheath neoplasm of the heart is rare.
  • We report the case of a patient with a giant schwannoma of unique ventricular origin.
  • Resection of the schwannoma and subsequent coronary reconstruction were required.
  • [MeSH-major] Graft Occlusion, Vascular / surgery. Heart Neoplasms / surgery. Neurilemmoma / surgery

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  • (PMID = 17462424.001).
  • [ISSN] 1552-6259
  • [Journal-full-title] The Annals of thoracic surgery
  • [ISO-abbreviation] Ann. Thorac. Surg.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Netherlands
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55. Dizdarević K, Link MJ: [Operative treatment of the vestibular schwannoma (acoustic neuroma): correlation between the microsurgical approach and cranial nerve lesion]. Med Arh; 2005;59(3):160-3
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  • [Title] [Operative treatment of the vestibular schwannoma (acoustic neuroma): correlation between the microsurgical approach and cranial nerve lesion].
  • [Transliterated title] Schwannoma vestibulare (neurinoma acustici)--operativni tretman: korelacija izmedu vrste mikroneurohirurskog pristupa i lezije kranijalnih nerava.
  • INTRODUCTION: Vestibular schwannoma (VS) is a benign tumour of cerebellopontine angle which total microsurgical resection is considered as a complex surgery.
  • House-Brackmann grading scale is used for VII nerve deficit appraisal.
  • [MeSH-major] Microsurgery. Neuroma, Acoustic / surgery. Vestibulocochlear Nerve Injuries
  • [MeSH-minor] Facial Nerve Injuries / etiology. Humans. Intraoperative Complications


56. Fornaro R, Canaletti M, Spaggiari P, Davini MD, Masuri M, Sticchi C, Moraglia E, Capellino M, Picori E, Terrizzi A: [Report on a case of schwannoma of the neck: clinical and therapeutic considerations]. Chir Ital; 2005 Jan-Feb;57(1):91-8
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  • [Title] [Report on a case of schwannoma of the neck: clinical and therapeutic considerations].
  • [Transliterated title] A proposito di un caso di schwannoma del collo: considerazioni clinico-terapeutiche.
  • We present a case of schwannoma of the neck in 49-year-old man, which was symptomatic for a long period: paraesthesia for one year, cervical mass for 2-3 months and dysphagia during the last 20 days.
  • The tumour arose from the cervical sympathetic chain.
  • The diagnosis of schwannoma was possible only at histopathological examination.
  • At present, however, the patient presents a moderate lowering of the voice, such as may be due to impairment of the superior laryngeal nerve, though this was already present preoperatively.
  • Identification of the nerve is often difficult up to the time of surgery, which is the treatment of choice for schwannoma.
  • [MeSH-major] Head and Neck Neoplasms / surgery. Neurilemmoma / surgery

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  • (PMID = 15832744.001).
  • [ISSN] 0009-4773
  • [Journal-full-title] Chirurgia italiana
  • [ISO-abbreviation] Chir Ital
  • [Language] ita
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Italy
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57. Lammert M, Mautner VF, Kluwe L: Do hormonal contraceptives stimulate growth of neurofibromas? A survey on 59 NF1 patients. BMC Cancer; 2005 Feb 9;5:16
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  • [Title] Do hormonal contraceptives stimulate growth of neurofibromas? A survey on 59 NF1 patients.
  • BACKGROUND: Neurofibromas are benign tumors of the peripheral nerves and hallmark of neurofibromatosis type 1 (NF1), a tumor suppressor gene syndrome.
  • Neurofibromas mostly start developing at puberty and can increase in size and number during pregnancy.
  • Expression of progesterone receptors has been found in 75% of the tumors.
  • Many female NF1 patients are thus concerned about the possibility that hormonal contraceptives may stimulate the growth of their neurofibromas.
  • METHODS: A survey was carried out on 59 female NF1 patients who are practicing or have practiced hormonal contraception to examine the effect of the various contraceptives on the growth of neurofibromas.
  • RESULTS: Majority (53 out of 58) of patients who received oral estrogen-progestogen or pure progestogen preparations reported no associated tumor growth.
  • In contrast, significant tumor growth was reported by two patients who received depot contraceptive containing high dose of synthetic progesterone.
  • CONCLUSIONS: Oral contraceptives do not seem to stimulate the growth of neurofibromas in NF1 patients.
  • High doses of progesterone might stimulate the growth of neurofibromas and deserve more caution.

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  • [Cites] JAMA. 1997 Jul 2;278(1):51-7 [9207339.001]
  • [Cites] Cancer Res. 2003 Feb 15;63(4):752-5 [12591720.001]
  • (PMID = 15703081.001).
  • [ISSN] 1471-2407
  • [Journal-full-title] BMC cancer
  • [ISO-abbreviation] BMC Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Contraceptives, Oral, Combined; 4G7DS2Q64Y / Progesterone; C2QI4IOI2G / Medroxyprogesterone Acetate
  • [Other-IDs] NLM/ PMC549555
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58. Huesca-Jiménez K, Medina-Franco H: [Gastric Schwannoma: a case report and literature review]. Rev Gastroenterol Mex; 2009;74(3):252-5
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  • [Title] [Gastric Schwannoma: a case report and literature review].
  • [Transliterated title] Schwannoma gástrico: presentación de un caso y revisión de la literatura.
  • We report a 49-year old male with diagnosis of gastric schwannoma who underwent subtotal gastrectomy.
  • We describe clinicopathological and immunohistochemical characteristics of these tumors and performed an extensive literature review.
  • Gastric schwannomas are very rare tumors but they should be taken into account in the differential diagnosis of more common mesenchimal neoplasms like gastrointestinal stromal tumors (GIST).
  • [MeSH-major] Gastrectomy. Neurilemmoma / pathology. Neurilemmoma / surgery. Stomach Neoplasms / pathology. Stomach Neoplasms / surgery

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  • (PMID = 19858018.001).
  • [ISSN] 0375-0906
  • [Journal-full-title] Revista de gastroenterología de México
  • [ISO-abbreviation] Rev Gastroenterol Mex
  • [Language] spa
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Mexico
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59. Spaziani E, Di Filippo A, Vitolo D, Deriu G, Picchio M, Ceci F, Briganti M, Martellucci A, De Angelis F, Nicodemi S, Cipriani B, Stagnitti F: [A possible cause of misdiagnosis in tumors of the axilla: schwannoma of the brachial plexus]. G Chir; 2008 Jan-Feb;29(1-2):38-41
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  • [Title] [A possible cause of misdiagnosis in tumors of the axilla: schwannoma of the brachial plexus].
  • [Transliterated title] Una possibile causa di errore nella diagnosi differenziale delle formazioni ascellari: lo schwannoma asintomatico dei tronchi del plesso brachiale.
  • The Authors report a rare case of a 57 years old man affected by a left radial nerve schwannoma that occurred as an asymptomatic lesion of the axilla.
  • This mistake was due to the low specificity of the instrumental methodology and to the rarity of an asymptomatic schwannoma of the infraclavicular brachial plexus.
  • In the early post-operative follow up, a "falling" attitude of the wrist, the hand and the fingers appeared, peculiar for a lesion of the radial nerve.
  • The sensitive and motor electromyography showed a radial nerve suffering.
  • The "stupor" of the nerve trunk was treated with steroid therapy for 7 days and the patient underwent to some series of neuro-rehabilitative physical therapy for 12 weeks.
  • [MeSH-major] Brachial Plexus. Diagnostic Errors. Neurilemmoma / diagnosis. Peripheral Nervous System Neoplasms / diagnosis. Radial Nerve

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  • (PMID = 18252147.001).
  • [ISSN] 0391-9005
  • [Journal-full-title] Il Giornale di chirurgia
  • [ISO-abbreviation] G Chir
  • [Language] ita
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Italy
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60. Le LQ, Shipman T, Burns DK, Parada LF: Cell of origin and microenvironment contribution for NF1-associated dermal neurofibromas. Cell Stem Cell; 2009 May 8;4(5):453-63
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  • [Title] Cell of origin and microenvironment contribution for NF1-associated dermal neurofibromas.
  • The tumor predisposition disorder neurofibromatosis type I (NF1) is one of the most common genetic disorders of the nervous system.
  • It is caused by mutations in the Nf1 tumor-suppressor gene, which encodes a GTPase-activating protein (GAP) that negatively regulates p21-RAS.
  • Development of malignant nerve tumors and neurofibromas occurs frequently in NF1.
  • However, little is known about the molecular mechanisms mediating the initiation and progression of these complex tumors, or the identity of the specific cell type that gives rise to dermal or cutaneous neurofibromas.
  • In this study, we identify a population of stem/progenitor cells residing in the dermis termed skin-derived precursors (SKPs) that, through loss of Nf1, form neurofibromas.
  • We also provide evidence that additional signals from nonneoplastic cells in the tumor microenvironment play essential roles in neurofibromagenesis.

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  • (PMID = 19427294.001).
  • [ISSN] 1875-9777
  • [Journal-full-title] Cell stem cell
  • [ISO-abbreviation] Cell Stem Cell
  • [Language] ENG
  • [Grant] United States / NINDS NIH HHS / NS / NS052606-040001; United States / NINDS NIH HHS / NS / P50 NS052606; United States / NINDS NIH HHS / NS / P50 NS052606-040001
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, Non-P.H.S.
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Neurofibromin 1
  • [Other-IDs] NLM/ NIHMS116524; NLM/ PMC2737469
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61. Eyenga VC, Eloundou Ngah J, Atangana R, Ngowe Ngowe M, Sosso M: [Results of surgical management of spinal neurinomas and neurofibromas at Yaounde]. Tunis Med; 2008 Jul;86(7):704-6
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  • [Title] [Results of surgical management of spinal neurinomas and neurofibromas at Yaounde].
  • [Transliterated title] Resultats du traitement chirurgical des neurinomes et neurofibromes spinaux a Yaounde.
  • BACKGROUND: Spinal neurinomas and neurofibromas are poorly studied in Sub-Saharan Africa.
  • RESULTS: Of the 62 patients operated for an intraspinal tumor, 12 (19.35%) were selected (nine neurinomas, two neurofibromas, one neurofibrosarcoma).
  • The situation of the tumor was cervical (four cases), dorsal (six cases) and lumbar (two cases).
  • The tumor was extradural, intradural, intra and extradural in six, four and two cases respectively.
  • Tumor excision was macroscopically complete in nine cases and partial in three.
  • CONCLUSION: The diagnosis of neurinomas and neurofibromas are late in our environment, resulting to poor surgical results.
  • [MeSH-major] Neurilemmoma / surgery. Neurofibroma / surgery. Spinal Neoplasms / surgery

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  • (PMID = 19472736.001).
  • [ISSN] 0041-4131
  • [Journal-full-title] La Tunisie médicale
  • [ISO-abbreviation] Tunis Med
  • [Language] fre
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Tunisia
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62. Korets R, Berkenblit R, Ghavamian R: Incidentally discovered adrenal schwannoma. JSLS; 2007 Jan-Mar;11(1):113-5
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  • [Title] Incidentally discovered adrenal schwannoma.
  • Schwannoma is a rare tumor of neural crest cell origin that is rarely seen arising from the adrenal gland.
  • We report a case of an adrenal mass discovered incidentally in a 70-year-old man as part of a hematuria workup.
  • Metabolic evaluation was unremarkable, and imaging studies did not meet strict imaging criteria for a typical adenoma.
  • Following surgical excision and pathologic evaluation with confirmatory immunohistochemical staining, the mass was reported as a benign nerve sheath neoplasm.

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  • (PMID = 17651570.001).
  • [ISSN] 1086-8089
  • [Journal-full-title] JSLS : Journal of the Society of Laparoendoscopic Surgeons
  • [ISO-abbreviation] JSLS
  • [Language] ENG
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Other-IDs] NLM/ PMC3015789
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63. Santaella Y, Borrego I, López J, Ortiz MJ, Vázquez R: [18-FDG-PET in a case of recurrent malignant schwannoma]. Rev Esp Med Nucl; 2005 Mar-Apr;24(2):127-30
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  • [Title] [18-FDG-PET in a case of recurrent malignant schwannoma].
  • [Transliterated title] Diagnóstico de recurrencia mediante FDG-PET en un caso clínico de schwannoma maligno.
  • The peripheral nerve sarcoma, also called malignant schwannoma, is originally a soft tissue sarcoma.
  • It is mainly located in the peripheral sheath nerve of the limbs and usually infiltrates the nerve fibres.
  • We present the case of a thirty year old woman with a malignant schwannoma in her left leg sciatic nerve who had been treated on several occasions.
  • PET can be a useful technique to detect recurrence for this kind of tumor, mainly in patients who have been previously radiated when the MRI is insufficient to perform a differential diagnosis between postirradiation fibrosis and tumoral recurrence, allowing for suitable therapeutic management of the patient.
  • [MeSH-major] Fluorodeoxyglucose F18. Neurilemmoma / radionuclide imaging. Peripheral Nervous System Neoplasms / radionuclide imaging. Positron-Emission Tomography. Radiopharmaceuticals. Sciatic Neuropathy / radionuclide imaging

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  • (PMID = 15745683.001).
  • [ISSN] 0212-6982
  • [Journal-full-title] Revista española de medicina nuclear
  • [ISO-abbreviation] Rev Esp Med Nucl
  • [Language] spa
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Spain
  • [Chemical-registry-number] 0 / Radiopharmaceuticals; 0Z5B2CJX4D / Fluorodeoxyglucose F18
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64. Vikram M, Pande A, Vasudevan MC, Ravi R: Cervical solitary long segment cystic Schwannoma. Br J Neurosurg; 2010 Apr;24(2):208-10
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  • [Title] Cervical solitary long segment cystic Schwannoma.
  • Cystic neurofibromas are very rare and are of separate entity; occurrence in the cervical spine extending over a long segment intra-durally is very rare.
  • There is a paucity of literature available on cervical cystic Schwannoma and its MRI characteristics.
  • As the preoperative diagnosis of these tumors helps in planning the surgery for this rare tumor the imaging findings reported herein may aid in the preoperative diagnosis and management.
  • [MeSH-major] Cervical Vertebrae / surgery. Cysts / surgery. Neurilemmoma / surgery. Spinal Neoplasms / surgery

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  • (PMID = 19886817.001).
  • [ISSN] 1360-046X
  • [Journal-full-title] British journal of neurosurgery
  • [ISO-abbreviation] Br J Neurosurg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
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65. Sofia L, Currò G, Iapichino G, Melita G, Lorenzini C, Cucinotta E: [Retroperitoneal giant schwannoma: a case report and review of the literature]. Chir Ital; 2008 Jan-Feb;60(1):141-6
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  • [Title] [Retroperitoneal giant schwannoma: a case report and review of the literature].
  • [Transliterated title] Schwannoma gigante retroperitoneale: presentazione di un caso clinico e revisione della letteratura.
  • Schwannomas are rare tumours that originate in the neural sheath and account for only a small percentage of all retroperitoneal tumours.
  • They are usually solitary, circumscribed and encapsulated lesions eccentrically located on proximal nerves or spinal nerve roots.
  • Presentation is typically varied and non-specific, ranging from abdominal pain, an abdominal mass or an incidental finding.
  • We report the case of a 66-year-old female presenting with abdominal pain in her left flank and with an ultrasonographic diagnosis of a left kidney mass.
  • She was diagnosed as suffering from a giant retroperitoneal schwannoma after surgical exploration and complete excision.
  • The role of CT scan and CT-guided needle biopsy is emphasised, in that ultrasonography and fine needle aspiration alone do not provide sufficient information regarding aetiology and malignancy.
  • [MeSH-major] Neurilemmoma / surgery. Retroperitoneal Neoplasms / surgery
  • [MeSH-minor] Aged. Biomarkers, Tumor / analysis. Biopsy, Needle. Female. Humans. Kidney / pathology. Lumbar Vertebrae / pathology. Magnetic Resonance Imaging. Neoplasm Invasiveness. Radiography, Interventional. Tomography, X-Ray Computed

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  • (PMID = 18389759.001).
  • [ISSN] 0009-4773
  • [Journal-full-title] Chirurgia italiana
  • [ISO-abbreviation] Chir Ital
  • [Language] ita
  • [Publication-type] Case Reports; English Abstract; Journal Article; Review
  • [Publication-country] Italy
  • [Chemical-registry-number] 0 / Biomarkers, Tumor
  • [Number-of-references] 31
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66. Gelly-Marty M, Martin E, Assous D, Cuisenier J, Collin F: [Plexiform schwannoma of the delto-pectoral area]. Ann Pathol; 2007 Apr;27(2):133-5
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  • [Title] [Plexiform schwannoma of the delto-pectoral area].
  • [Transliterated title] Schwannome plexiforme profond de la région delto-pectorale gauche.
  • Plexiform schwannoma is a form of schwannoma which usually involves cutaneous tissues.
  • It cannot be easily differentiated from malignant tumors, especially deep or cellular lesions.
  • We report a deep plexiform schwannoma which we place among the various benign or malignant nerve sheath tumors, which may or may not develop within the context of genetic disease.
  • [MeSH-major] Neurilemmoma / pathology. Soft Tissue Neoplasms / pathology. Thoracic Wall

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  • (PMID = 17909473.001).
  • [ISSN] 0242-6498
  • [Journal-full-title] Annales de pathologie
  • [ISO-abbreviation] Ann Pathol
  • [Language] fre
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] France
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67. Ghiluşi M, Pleşea IE, Comănescu M, Enache SD, Bogdan F: Preliminary study regarding the utility of certain immunohistochemical markers in diagnosing neurofibromas and schwannomas. Rom J Morphol Embryol; 2009;50(2):195-202
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  • [Title] Preliminary study regarding the utility of certain immunohistochemical markers in diagnosing neurofibromas and schwannomas.
  • The present study shows the histopathological and immunohistochemical aspects encountered in 49 benign tumors with neural origin diagnosed in the Pathology Department of the Emergency County Hospital of Craiova between 2000 and 2007.
  • Histopathological criteria were used for the histopathological diagnosis, having been diagnosed 22 neurofibromas and 27 schwannomas.
  • Both tumors showed positive immunostaining for S100, CD34, CD57, but of varying intensity and distribution.
  • Schwannomas and neurofibromas showed a low proliferation index (<5%).
  • [MeSH-major] Biomarkers, Tumor / metabolism. Neurilemmoma / pathology. Neurofibroma / pathology. Peripheral Nervous System Neoplasms / pathology

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  • (PMID = 19434310.001).
  • [ISSN] 1220-0522
  • [Journal-full-title] Romanian journal of morphology and embryology = Revue roumaine de morphologie et embryologie
  • [ISO-abbreviation] Rom J Morphol Embryol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Romania
  • [Chemical-registry-number] 0 / Biomarkers, Tumor
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68. Sbidian E, Wolkenstein P, Valeyrie-Allanore L, Rodriguez D, Hadj-Rabia S, Ferkal S, Lacour JP, Leonard JC, Taillandier L, Sportich S, Berbis P, Bastuji-Garin S, Members of NF France Network: NF-1Score: a prediction score for internal neurofibromas in neurofibromatosis-1. J Invest Dermatol; 2010 Sep;130(9):2173-8
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  • [Title] NF-1Score: a prediction score for internal neurofibromas in neurofibromatosis-1.
  • Internal neurofibromas are associated with increased morbidity and mortality through malignant transformation and compression of neighboring organs.
  • Our purpose was to develop and to validate a clinical score for predicting internal neurofibromas in adults.
  • Four variables were independently associated with internal neurofibromas: at least two subcutaneous neurofibromas (odds ratio (OR)=4.7, [2.1-10.5]), age < or =30 years (OR=3.1, [1.4-6.8]), absence of cutaneous neurofibromas (OR=2.6, [0.9-7.5]), and fewer than six café-au-lait spots (OR=2.0 [0.9-4.6]).
  • The probability of internal neurofibromas was computed as exp (-2.93+0.11Score)/exp (1+(-2.93+0.11Score)).
  • The presence of internal neurofibromas can be accurately predicted using a simple clinical score.

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  • [CommentIn] J Invest Dermatol. 2010 Sep;130(9):2167-9 [20711205.001]
  • [CommentIn] J Invest Dermatol. 2010 Sep;130(9):2160 [20711203.001]
  • (PMID = 20428190.001).
  • [ISSN] 1523-1747
  • [Journal-full-title] The Journal of investigative dermatology
  • [ISO-abbreviation] J. Invest. Dermatol.
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article; Validation Studies
  • [Publication-country] United States
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69. De Raedt T, Maertens O, Chmara M, Brems H, Heyns I, Sciot R, Majounie E, Upadhyaya M, De Schepper S, Speleman F, Messiaen L, Vermeesch JR, Legius E: Somatic loss of wild type NF1 allele in neurofibromas: Comparison of NF1 microdeletion and non-microdeletion patients. Genes Chromosomes Cancer; 2006 Oct;45(10):893-904
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  • [Title] Somatic loss of wild type NF1 allele in neurofibromas: Comparison of NF1 microdeletion and non-microdeletion patients.
  • Neurofibromatosis type I (NF1) is an autosomal dominant familial tumor syndrome characterized by the presence of multiple benign neurofibromas.
  • In 95% of NF1 individuals, a mutation is found in the NF1 gene, and in 5% of the patients, the germline mutation consists of a microdeletion that includes the NF1 gene and several flanking genes.
  • We studied the frequency of loss of heterozygosity (LOH) in the NF1 region as a mechanism of somatic NF1 inactivation in neurofibromas from NF1 patients with and without a microdeletion.
  • There was a statistically significant difference between these two patient groups in the proportion of neurofibromas with LOH.
  • None of the 40 neurofibromas from six different NF1 microdeletion patients showed LOH, whereas LOH was observed in 6/28 neurofibromas from five patients with an intragenic NF1 mutation (P = 0.0034, Fisher's exact).
  • LOH of the NF1 microdeletion region in NF1 microdeletion patients would de facto lead to a nullizygous state of the genes located in the deletion region and might be lethal.
  • The mechanisms leading to LOH were further analyzed in six neurofibromas.
  • In two out of six neurofibromas, a chromosomal microdeletion was found; in three, a mitotic recombination was responsible for the observed LOH; and in one, a chromosome loss with reduplication was present.
  • We conclude that NF1 is a familial tumor syndrome in which the type of germline mutation influences the type of second hit in the tumors.
  • [MeSH-minor] DNA Mutational Analysis. DNA, Neoplasm / genetics. DNA, Neoplasm / isolation & purification. Humans. Loss of Heterozygosity. Phenotype. Reverse Transcriptase Polymerase Chain Reaction

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  • [Copyright] (c) 2006 Wiley-Liss, Inc.
  • (PMID = 16830335.001).
  • [ISSN] 1045-2257
  • [Journal-full-title] Genes, chromosomes & cancer
  • [ISO-abbreviation] Genes Chromosomes Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / DNA, Neoplasm; 0 / Neurofibromin 1
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70. Fishbein L, Eady B, Sanek N, Muir D, Wallace MR: Analysis of somatic NF1 promoter methylation in plexiform neurofibromas and Schwann cells. Cancer Genet Cytogenet; 2005 Mar;157(2):181-6
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  • [Title] Analysis of somatic NF1 promoter methylation in plexiform neurofibromas and Schwann cells.
  • Neurofibromatosis 1 (NF1) is an autosomal dominant disorder with the characteristic feature being the neurofibroma.
  • However, often the somatic mutations are not identified, suggesting that epigenetic changes such as methylation could account for the "second hit" in some tumors.
  • The literature reports that the region of the NF1 promoter surrounding the transcription start site is completely unmethylated in several normal tissues and some NF1-related dermal and plexiform neurofibromas.
  • We analyzed the methylation state of the NF1 promoter in normal Schwann cells (the cell type clonally expanded in neurofibromas) and in NF1-related plexiform tumor samples with unidentified somatic mutations.
  • In a region of 451 bp surrounding the transcription start site, a low level of methylation was found at several specific cytosines in 12 of 18 tumor samples.
  • Overall, epigenetic silencing through methylation does not appear to be a major mechanism for the second hit.
  • However, this study, which analyzed the largest number of NF1-related plexiform tumors and is the first to include Schwann cell-enriched tumor cultures, detected greater methylation than in any previous reports.
  • This suggests that methylation, especially at potential transcription factor binding sites, is moderately perturbed in some plexiform neurofibromas and should be investigated further.
  • [MeSH-minor] Cells, Cultured. CpG Islands. Cytosine / metabolism. Gene Expression. Humans. Promoter Regions, Genetic. Tumor Cells, Cultured

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  • (PMID = 15721644.001).
  • [ISSN] 0165-4608
  • [Journal-full-title] Cancer genetics and cytogenetics
  • [ISO-abbreviation] Cancer Genet. Cytogenet.
  • [Language] eng
  • [Grant] United States / NINDS NIH HHS / NS / 1R01 NS34780; United States / NINDS NIH HHS / NS / 1R29 NS31550; United States / NCI NIH HHS / CA / T32-CA01926-22
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, Non-P.H.S.; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] United States
  • [Chemical-registry-number] 8J337D1HZY / Cytosine
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71. Vera-Sempere F, Vera-Sirera B: Intraosseus plexiform schwannoma of the mandible: immunohistochemical differential diagnosis. J Craniofac Surg; 2010 Nov;21(6):1820-4
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  • [Title] Intraosseus plexiform schwannoma of the mandible: immunohistochemical differential diagnosis.
  • Schwannomas and neurofibromas are the most common benign tumors derived from peripheral nerves, and whereas the head and neck region is the most common location for the occurrence of benign neural sheath neoplasms, origin within the oral cavity is uncommon, and occurrence centrally in the jaws is most unusual.
  • Plexiform (multinodular) schwannoma is an anatomically unique variant of schwannoma characterized grossly and/or microscopically by intraneural plexiform and often multinodular growth.
  • In current report, we present the first reported case of intraosseous plexiform schwannoma of the mandible, an extremely rare benign neurogenic tumor, diagnosed by optical and immunohistochemical procedures, showing the importance of differential diagnosis of these unusual intraosseous mandibular tumors.
  • [MeSH-major] Mandibular Neoplasms / diagnosis. Neurilemmoma / diagnosis
  • [MeSH-minor] Ameloblastoma / diagnosis. Cranial Nerve Neoplasms / diagnosis. Diagnosis, Differential. Female. Follow-Up Studies. Humans. Immunohistochemistry. Mandibular Diseases / diagnosis. Mandibular Nerve / pathology. Middle Aged. Odontogenic Cysts / diagnosis

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  • (PMID = 21119430.001).
  • [ISSN] 1536-3732
  • [Journal-full-title] The Journal of craniofacial surgery
  • [ISO-abbreviation] J Craniofac Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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72. Geller M, Mezitis SG, Nunes FP, Ribeiro MG, Araújo AP, Bronstein MD, Siqueira-Batista R, Gomes AP, Oliveira L, Cunha KS: Progesterone and Estrogen Receptors in Neurofibromas of Patients with NF1. Clin Med Pathol; 2008;1:93-7
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  • [Title] Progesterone and Estrogen Receptors in Neurofibromas of Patients with NF1.
  • Neurofibromatosis type 1 (NF1) or von Recklinghausen disease is a genetic disorder affecting the growth of cells in nervous system.
  • One of the most remarkable characteristics of this disease is the development of benign tumors of the nervous system (neurofibromas).The purpose of this study was to test tissue samples taken from neurofibromas and plexiform neurofibromas of NF1 patients for the presence of estrogen and progesterone receptors.
  • We used previously collected samples from patients registered in the database of the Centro Nacional de Neurofibromatose (CNNF-Brazil).
  • Samples from twenty-five patients in the database presenting plexiform neurofibromas (N1 group) and 25 samples from the same database from patients presenting neurofibromas (N2 group) were tested.We observed positive staining for progesterone receptors in 13 of the neurofibroma samples and 19 of the plexiform neurofibroma samples.
  • We suggest further studies to investigate in greater depth possible hormonal influences on the development and growth of neurofibromas and plexiform neurofibromas in NF1.

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  • (PMID = 21876657.001).
  • [ISSN] 1178-1181
  • [Journal-full-title] Clinical medicine. Pathology
  • [ISO-abbreviation] Clin Med Pathol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] New Zealand
  • [Other-IDs] NLM/ PMC3160005
  • [Keywords] NOTNLM ; estrogen receptor / neurofibroma / plexiform neurofibroma / progesterone receptor
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73. Agaram NP, Prakash S, Antonescu CR: Deep-seated plexiform schwannoma: a pathologic study of 16 cases and comparative analysis with the superficial variety. Am J Surg Pathol; 2005 Aug;29(8):1042-8
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  • [Title] Deep-seated plexiform schwannoma: a pathologic study of 16 cases and comparative analysis with the superficial variety.
  • Plexiform schwannoma (PS) is one of the least common histologic variants of schwannoma.
  • However, the frequent cellular morphology associated with hyperchromatic nuclei, increased mitoses, and plexiform growth can suggest a malignant process, mainly a high-grade malignant peripheral nerve sheath tumor (MPNST).
  • Fifteen tumors were located in the deep somatic soft tissue (extremities, 8; retroperitoneum/pelvis, 3; trunk, 2; parotid, 1; vulva, 1) and 1 tumor was located in the thoracic esophagus.
  • Local recurrence was noted in half of the patients with clinical information available, but none had evidence of disease at last follow-up.
  • Worrisome morphologic features included: increased cellularity (68%), mild to moderate pleomorphism (50%), and mitotic activity (93%) ranging from 1 to 10 MF/10 high power fields (HPFs).
  • The 8 superficial PSs showed increased cellularity and mild to moderate pleomorphism in 62% of cases but lacked tumor necrosis.
  • Deep-seated PS is a rare, under-recognized PNST of deep soft tissue, typically not associated with neurofibromatosis.
  • In contrast with the more common superficial (dermal and subcutaneous) tumors, deep PSs have a predilection for females, can occur in congenital settings, and can show necrosis and myxoid change.
  • It is important to differentiate these tumors from plexiform neurofibromas and MPNSTs as they follow a benign clinical course, with complete surgical excision being curative.
  • [MeSH-major] Neurilemmoma / pathology. Soft Tissue Neoplasms / pathology

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  • (PMID = 16006798.001).
  • [ISSN] 0147-5185
  • [Journal-full-title] The American journal of surgical pathology
  • [ISO-abbreviation] Am. J. Surg. Pathol.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / S100 Proteins
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74. Tidmore T, Calhoun KH, Hirschi S, Miick R: Columellar schwannoma. Ear Nose Throat J; 2009 Oct;88(10):E38-40
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  • [Title] Columellar schwannoma.
  • In fact, only 19 cases of schwannoma of the nasal septum have been reported in the otolaryngology literature.
  • We report a case of a columellar schwannoma in a 57-year-old white man.
  • [MeSH-major] Nasal Septum. Neurilemmoma / diagnosis. Nose Neoplasms / diagnosis

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  • (PMID = 19826990.001).
  • [ISSN] 1942-7522
  • [Journal-full-title] Ear, nose, & throat journal
  • [ISO-abbreviation] Ear Nose Throat J
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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75. Joshi D, Gangane N, Kishore S, Vagha S: Unusual histological presentation in neurofibromas: Two case reports. Cases J; 2008;1(1):188
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  • [Title] Unusual histological presentation in neurofibromas: Two case reports.
  • The objective of this paper is to discuss the unusual histological findings seen in two cases of neurofibromas associated with neurofibromatosis type 1 Both cases presented with multiple subcutaneous nodules.
  • Histological examination of case no.1 revealed a benign tumor of the peripheral nerve sheath, of neurofibroma type with presence of mucus producing glands.

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  • (PMID = 18823533.001).
  • [ISSN] 1757-1626
  • [Journal-full-title] Cases journal
  • [ISO-abbreviation] Cases J
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
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76. Overdiek A, Feifel H, Schaper J, Mayatepek E, Rosenbaum T: Diagnostic delay of NF1 in hemifacial hypertrophy due to plexiform neurofibromas. Brain Dev; 2006 Jun;28(5):275-80
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Diagnostic delay of NF1 in hemifacial hypertrophy due to plexiform neurofibromas.
  • Benign tumors of the peripheral nerve sheath, termed neurofibromas, are the hallmark feature of neurofibromatosis type 1 (NF1).
  • These tumors can result in hypertrophy of a limb or another anatomic region.
  • However, despite other typical NF1-associated features (e.g. multiple café-au-lait spots) diagnosis of a plexiform neurofibroma as underlying cause for the hemifacial hypertrophy was significantly delayed in all patients.
  • MRI scans were misinterpreted in all of the cases as lymphangioma because plexiform neurofibromas can resemble mesenchymal tumors or lymphangiomas.
  • A thorough clinical examination of affected patients should focus on typical disease-defining features.

  • The Weizmann Institute of Science GeneCards and MalaCards databases. gene/protein/disease-specific - MalaCards for hemifacial hypertrophy .
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  • (PMID = 16481142.001).
  • [ISSN] 0387-7604
  • [Journal-full-title] Brain & development
  • [ISO-abbreviation] Brain Dev.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] Netherlands
  • [Number-of-references] 10
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77. De Sena G, Molino C, De Riitis MR, Candela S, Cifarelli V, Di Maio V, Chianese F, Rossetti DA, Rossi M, Miranda G: [Surgical management of schwannoma of biliary tract]. Chir Ital; 2009 Jan-Feb;61(1):119-21
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  • [Title] [Surgical management of schwannoma of biliary tract].
  • [Transliterated title] Trattamento chirurgico di schwannoma della via biliare.
  • A borderline Schwann cell tumour with common bile duct compression and jaundice is extremely rare.
  • This paper presents the first report in the Italian literature of a hepatojejunostomy for a symptomatic lesion midway between benign and malignant.
  • [MeSH-major] Bile Duct Neoplasms / surgery. Common Bile Duct. Neurilemmoma / surgery

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  • (PMID = 19391350.001).
  • [ISSN] 0009-4773
  • [Journal-full-title] Chirurgia italiana
  • [ISO-abbreviation] Chir Ital
  • [Language] ita
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Italy
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78. Lee SH, Hong JS, Choi JH, Chung WS: Choroidal schwannoma. Acta Ophthalmol Scand; 2005 Dec;83(6):754-6
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  • [Title] Choroidal schwannoma.
  • PURPOSE: Schwannoma is rarely encountered as an intraocular tumor arising from uveal tract.
  • We describe a case of choroidal schwannoma.
  • To further delineate the tumor, she underwent enucleation of the eyeball.
  • The tumor was examined by light microscopy, electron microscopy and immunohistochemical study.
  • RESULTS: Microscopically, the tumor was composed of a mixture of cellular solid component (Antoni A) and loose myxoid component (Antoni B).
  • Ultrastructurally, the tumor cells showed prominent, continuous basal lamina.
  • CONCLUSIONS: We enucleated the eyeball with a clinical diagnosis of choroidal amelanotic melanoma, but the tumor was finally diagnosed as schwannoma.
  • We think that currently available ancillary studies are still little value in definitely differentiating schwannoma from other choroidal tumors.
  • [MeSH-major] Choroid Neoplasms / pathology. Neurilemmoma / pathology

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  • (PMID = 16396657.001).
  • [ISSN] 1395-3907
  • [Journal-full-title] Acta ophthalmologica Scandinavica
  • [ISO-abbreviation] Acta Ophthalmol Scand
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Denmark
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79. Singh D, Pinjala RK: Schwannoma of the cervical vagus nerve. Pediatr Neurosurg; 2007;43(5):403-5
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  • [Title] Schwannoma of the cervical vagus nerve.
  • Nerve sheath tumors arising from the cervical vagus nerve are extremely rare and difficult to diagnose.
  • Surgical excision of the lesion was carried out and histological examination revealed a schwannoma.
  • Schwannoma is a relatively rare tumor and even rarer in children.
  • The incidence of such tumors and the management of our patients are discussed.
  • [MeSH-major] Cranial Nerve Neoplasms / diagnosis. Neurilemmoma / diagnosis. Vagus Nerve Diseases / diagnosis
  • [MeSH-minor] Adolescent. Humans. Male. Vagus Nerve / pathology. Vagus Nerve / surgery

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  • [Copyright] (c) 2007 S. Karger AG, Basel.
  • (PMID = 17786007.001).
  • [ISSN] 1423-0305
  • [Journal-full-title] Pediatric neurosurgery
  • [ISO-abbreviation] Pediatr Neurosurg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Switzerland
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80. Manolidis S, Higuera S, Boyd V, Hollier LH: Single-stage total and near-total resection of massive pediatric head and neck neurofibromas. J Craniofac Surg; 2006 May;17(3):506-10
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  • [Title] Single-stage total and near-total resection of massive pediatric head and neck neurofibromas.
  • Plexiform neurofibromas traditionally have posed a surgical challenge in pediatric patients.
  • Expert preoperative planning, advanced surgical techniques, and vigilant postoperative care results in minimal morbidity and resolution of tumor symptomatology.
  • A retrospective review of four consecutive pediatric patients with massive head and neck neurofibromas who underwent single-stage total or near-total removal of their tumors was performed.
  • It is possible to safely achieve total or near-total removal of extensive plexiform neurofibromas with minimal morbidity while restoring lost function.
  • [MeSH-major] Head and Neck Neoplasms / surgery. Neurofibroma, Plexiform / surgery
  • [MeSH-minor] Adolescent. Airway Obstruction / surgery. Articulation Disorders / surgery. Bronchitis / etiology. Child. Child, Preschool. Deglutition Disorders / surgery. Follow-Up Studies. Humans. Image Processing, Computer-Assisted. Mediastinal Neoplasms / surgery. Mouth Floor / surgery. Mouth Neoplasms / surgery. Pharyngeal Neoplasms / surgery. Pneumothorax / etiology. Postoperative Complications. Recovery of Function. Retrospective Studies. Skull Neoplasms / surgery. Sleep Apnea, Obstructive / surgery. Tongue Neoplasms / surgery

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  • (PMID = 16770189.001).
  • [ISSN] 1049-2275
  • [Journal-full-title] The Journal of craniofacial surgery
  • [ISO-abbreviation] J Craniofac Surg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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81. Friedrich RE, Schmelzle R, Hartmann M, Mautner VF: Subtotal and total resection of superficial plexiform neurofibromas of face and neck: four case reports. J Craniomaxillofac Surg; 2005 Feb;33(1):55-60
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  • [Title] Subtotal and total resection of superficial plexiform neurofibromas of face and neck: four case reports.
  • INTRODUCTION: Plexiform neurofibromas are benign tumours of the peripheral nerves and connective tissue.
  • Removal of plexiform neurofibromas is usually unsatisfactory because the network-like growth of these tumours often involves multiple nerve fascicles and other adjacent tissues.
  • It has been previously shown that magnetic resonance tomography can distinguish the growth patterns of plexiform neurofibromas into three different categories: superficial, displacing and invasive.
  • PATIENTS AND METHODS: Three cases are described with successful subtotal resections of superficial plexiform neurofibromas, and one case with total resection following the diagnosis of tumour subtype using magnetic resonance imaging (MRI).
  • It enables the distinction to be drawn between this subtype and the other two subtypes of plexiform neurofibromas.
  • [MeSH-major] Head and Neck Neoplasms / surgery. Neurofibroma, Plexiform / surgery

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  • (PMID = 15694151.001).
  • [ISSN] 1010-5182
  • [Journal-full-title] Journal of cranio-maxillo-facial surgery : official publication of the European Association for Cranio-Maxillo-Facial Surgery
  • [ISO-abbreviation] J Craniomaxillofac Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Scotland
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82. Elwakil TF, Samy NA, Elbasiouny MS: Non-excision treatment of multiple cutaneous neurofibromas by laser photocoagulation. Lasers Med Sci; 2008 Jul;23(3):301-6
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  • [Title] Non-excision treatment of multiple cutaneous neurofibromas by laser photocoagulation.
  • Neurofibromatosis type 1 (NF-1) is an autosomal dominant disorder characterized by skin tumours derived from peripheral nerves.
  • It is a clinically diagnosed disorder of a mainly cosmetic concern.
  • There are different excision modalities for treatment of cutaneous neurofibromas; however, none is considered to be universally accepted treatment.
  • This study was conducted to evaluate a non-excision treatment of multiple cutaneous neurofibromas, using surface and interstitial approaches of neodymium:yttrium aluminum garnet (Nd:YAG) laser (1,064 nm) photocoagulation, depending upon the size and location of the lesions.
  • Twelve patients with multiple cutaneous neurofibromas were included.
  • Within the limitations of the present study, laser photocoagulation has proven to be a promising technique that may be an alternative or additive modality for treatment of multiple cutaneous neurofibromas.
  • [MeSH-major] Laser Coagulation / methods. Neurofibromatosis 1 / surgery. Skin Neoplasms / surgery

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  • (PMID = 17701270.001).
  • [ISSN] 0268-8921
  • [Journal-full-title] Lasers in medical science
  • [ISO-abbreviation] Lasers Med Sci
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 2I87U3734A / Neodymium
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83. Serletis D, Parkin P, Bouffet E, Shroff M, Drake JM, Rutka JT: Massive plexiform neurofibromas in childhood: natural history and management issues. J Neurosurg; 2007 May;106(5 Suppl):363-7
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  • [Title] Massive plexiform neurofibromas in childhood: natural history and management issues.
  • OBJECT: The authors review their experience with massive plexiform neurofibromas (PNs) in patients with pediatric neurofibromatosis Type 1 (NF1) to better characterize the natural history and management of these complex lesions.
  • These patients attended routine follow-up examinations conducted by a number of specialists, and serial neuroimaging studies were obtained to monitor disease progression.
  • The most common presenting feature of PN was that of a painful, expanding lesion.
  • With respect to management, two patients were simply observed, undergoing serial neuroimaging studies; two patients underwent biopsy sampling of their plexiform lesions; two patients underwent attempted medical treatment (farnesyl transferase inhibitor, R11577, and cyclophosphamide chemotherapy); and three patients required surgical debulking of their PNs because the massive growth of these tumors caused functional compromise.
  • Nevertheless, patients with massive PNs will benefit from close surveillance by a team of specialists to monitor for ongoing disease progression.
  • [MeSH-minor] Adolescent. Antineoplastic Agents / therapeutic use. Antineoplastic Agents, Alkylating / therapeutic use. Child. Child, Preschool. Cyclophosphamide / therapeutic use. Humans. Male. Neoplasms, Second Primary / diagnosis. Neoplasms, Second Primary / therapy. Neurosurgical Procedures. Pain / etiology. Quinolones / therapeutic use. Respiration Disorders / etiology. Respiration Disorders / mortality. Retrospective Studies

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  • (PMID = 17566202.001).
  • [ISSN] 0022-3085
  • [Journal-full-title] Journal of neurosurgery
  • [ISO-abbreviation] J. Neurosurg.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Antineoplastic Agents, Alkylating; 0 / Quinolones; 192185-72-1 / tipifarnib; 8N3DW7272P / Cyclophosphamide
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84. Punia RS, Dhingra N, Mohan H: Cutaneous plexiform schwannoma of the finger not associated with neurofibromatosis. Am J Clin Dermatol; 2008;9(2):129-31
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  • [Title] Cutaneous plexiform schwannoma of the finger not associated with neurofibromatosis.
  • Plexiform schwannoma is a rare, benign, peripheral nerve sheath tumor that occurs as an uncommon nodular variant of schwannoma.
  • It is important to recognize this tumor because it can be misdiagnosed as plexiform neurofibroma.
  • In contrast to the latter, however, plexiform schwannoma is not associated with neurofibromatosis (von Recklinghausen disease).
  • We report a case of plexiform schwannoma located on the index finger of a 20-year-old male patient with no signs of neurofibromatosis.
  • [MeSH-major] Neurilemmoma / pathology. Skin / pathology. Skin Neoplasms / pathology

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  • (PMID = 18284268.001).
  • [ISSN] 1175-0561
  • [Journal-full-title] American journal of clinical dermatology
  • [ISO-abbreviation] Am J Clin Dermatol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] New Zealand
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85. Upadhyaya M, Spurlock G, Kluwe L, Chuzhanova N, Bennett E, Thomas N, Guha A, Mautner V: The spectrum of somatic and germline NF1 mutations in NF1 patients with spinal neurofibromas. Neurogenetics; 2009 Jul;10(3):251-63
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  • [Title] The spectrum of somatic and germline NF1 mutations in NF1 patients with spinal neurofibromas.
  • Neurofibromatosis type 1 (NF1) is a common inherited complex multi-system disorder associated with the growth of various benign and malignant tumors.
  • About 40% of NF1 patients develop spinal tumors, of whom some have familial spinal neurofibromatosis (FSNF), a variant form of NF1 in which patients present with multiple bilateral spinal tumors but have few other clinical features of the disease.
  • We have studied 22 spinal neurofibromas derived from 14 unrelated NF1 patients.
  • This is the first study to describe NF1 somatic mutations in spinal neurofibromas.
  • Loss-of-heterozygosity (LOH) was identified in 8/22 of the spinal tumors, 75% of LOH observed was found to result from mitotic recombination, suggesting that this may represent a frequent mutational mechanisms in these benign tumors.
  • No evidence for LOH of the TP53 gene was found in these tumors.
  • [MeSH-major] Germ-Line Mutation. Neurofibroma. Neurofibromatosis 1. Spinal Neoplasms

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  • (PMID = 19221814.001).
  • [ISSN] 1364-6753
  • [Journal-full-title] Neurogenetics
  • [ISO-abbreviation] Neurogenetics
  • [Language] eng
  • [Grant] United Kingdom / Cancer Research UK / /
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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86. Di Lorenzo S, Corradino B, Cordova A, Moschella F: Unexpected ulnar nerve schwannoma. The reasonable risk of misdiagnosis. Acta Chir Plast; 2007;49(3):77-9
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  • [Title] Unexpected ulnar nerve schwannoma. The reasonable risk of misdiagnosis.
  • Peripheral nerve tumors are rare clinical entities that can present with mild symptoms and no neurological deficit.
  • The authors describe one case of asymptomatic schwannoma of the ulnar nerve in a 64-year-old man.
  • The mass was associated with mild and sporadic discomfort due to the mass size; the clinical neurological examination was not conclusive, with Tinel's sign negative; surgery confirmed the nervous origin of the tumor and the histopathologic exam confirmed the diagnosis of schwannoma.
  • These tumors are difficult to diagnose clinically and have often been confused with other benign tumors such as lipomas, hemangiomas, synovial cysts, neurofibromas, etc.
  • Clinical care is important to prevent, during the surgery, the unfortunate resection of the nerve; all too frequently this diagnosis is made intraoperatively or postoperatively with the potential to compromise the outcome of the treatment.
  • An appropriate degree of awareness is necessary for the inclusion of peripheral nerve tumors as a differential diagnosis of an upper extremity mass.
  • [MeSH-major] Neurilemmoma / pathology. Neurilemmoma / surgery. Peripheral Nervous System Neoplasms / pathology. Peripheral Nervous System Neoplasms / surgery. Ulnar Nerve / pathology. Ulnar Nerve / surgery

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  • (PMID = 18051588.001).
  • [ISSN] 0001-5423
  • [Journal-full-title] Acta chirurgiae plasticae
  • [ISO-abbreviation] Acta Chir Plast
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Czech Republic
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87. Ohba T, Shoji F, Kometani T, Yoshino I, Maehara Y: Schwannoma in the peridiaphragm. Gen Thorac Cardiovasc Surg; 2008 Sep;56(9):453-5
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  • [Title] Schwannoma in the peridiaphragm.
  • A rare case of benign diaphragmatic schwannoma in a 50-year-old woman is reported.
  • The 8-cm tumor was located in the right diaphragm.
  • This well-circumscribed tumor highly depressed the liver.
  • Complete extirpation of the tumor was performed, and pathology examination revealed that the benign schwannoma had originated from the diaphragm.
  • [MeSH-major] Diaphragm / pathology. Muscle Neoplasms / pathology. Neurilemmoma / pathology

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  • (PMID = 18791670.001).
  • [ISSN] 1863-6705
  • [Journal-full-title] General thoracic and cardiovascular surgery
  • [ISO-abbreviation] Gen Thorac Cardiovasc Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Japan
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88. Di Giovannantonio L, Bellocci R, Zappacosta R, Zappacosta B, Castrataro A, Liberatore M, Liberati M, Angelucci D: [Primary malignant schwannoma of the uterine cervix: a malignant tumor with unusual behaviour. A case report]. Pathologica; 2005 Feb;97(1):7-9
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  • [Title] [Primary malignant schwannoma of the uterine cervix: a malignant tumor with unusual behaviour. A case report].
  • [Transliterated title] Schwannoma maligno primitivo della cervice uterina: un tumore maligno a comportamento inusuale. Caso clinico.
  • Malignant tumors of peripheral nerves (MPNST) represent approximately 5-10% of all soft tissue sarcomas and usually are in relationship with a major nerve.
  • Primary cervical malignant Schwannomas are very rare neural sheat tumors that, grossly and clinically, are misdiagnosed for other more frequent lesions of the uterine cervix.
  • We report a case of primary cervical malignant Schwannoma in a 27 years old female with atypical bleeding.
  • After 34 months the patient is disease free.
  • [MeSH-major] Neurilemmoma / pathology. Uterine Cervical Neoplasms / pathology


89. Wu J, Williams JP, Rizvi TA, Kordich JJ, Witte D, Meijer D, Stemmer-Rachamimov AO, Cancelas JA, Ratner N: Plexiform and dermal neurofibromas and pigmentation are caused by Nf1 loss in desert hedgehog-expressing cells. Cancer Cell; 2008 Feb;13(2):105-16
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  • [Title] Plexiform and dermal neurofibromas and pigmentation are caused by Nf1 loss in desert hedgehog-expressing cells.
  • Neurofibromatosis type 1 (Nf1) mutation predisposes to benign peripheral nerve (glial) tumors called neurofibromas.
  • We show that inactivation of Nf1 in the glial lineage in vitro at embryonic day 12.5 + 1, but not earlier (neural crest) or later (mature Schwann cell), results in colony-forming cells capable of multilineage differentiation.
  • In vivo, inactivation of Nf1 using a DhhCre driver beginning at E12.5 elicits plexiform neurofibromas, dermal neurofibromas, and pigmentation.
  • Tumor Schwann cells uniquely show biallelic Nf1 inactivation.
  • Peripheral nerve and tumors contain transiently proliferating Schwann cells that lose axonal contact, providing insight into early neurofibroma formation.

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  • (PMID = 18242511.001).
  • [ISSN] 1535-6108
  • [Journal-full-title] Cancer cell
  • [ISO-abbreviation] Cancer Cell
  • [Language] ENG
  • [Grant] United States / NINDS NIH HHS / NS / NS028840-15; United States / NINDS NIH HHS / NS / R01 NS028840; United States / NINDS NIH HHS / NS / 1R01 NS28840; United States / NINDS NIH HHS / NS / R01 NS028840-15
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Hedgehog Proteins; 0 / Neurofibromin 1; 0 / Receptor, Nerve Growth Factor; EC 2.7.7.- / Cre recombinase; EC 2.7.7.- / Integrases
  • [Other-IDs] NLM/ NIHMS40132; NLM/ PMC2846699
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90. Sinha R, Sundaram M, Hegde A, Mahajan C: Pelvic schwannoma masquerading as broad ligament myoma. J Minim Invasive Gynecol; 2008 Mar-Apr;15(2):217-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Pelvic schwannoma masquerading as broad ligament myoma.
  • Two cases of pelvic schwannoma appeared as broad ligament myoma.
  • Both patients underwent complete tumor excision laparoscopically and had uneventful postoperative recovery.
  • Solitary nerve sheath tumors such as benign schwannomas arising in pelvic retroperitoneum are infrequently reported and difficult to diagnose preoperatively.
  • A MEDLINE search did not reveal reports of removing these tumors laparoscopically.
  • [MeSH-major] Broad Ligament. Genital Neoplasms, Female / diagnosis. Myoma / diagnosis. Neurilemmoma / diagnosis. Retroperitoneal Neoplasms / diagnosis

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  • (PMID = 18312995.001).
  • [ISSN] 1553-4650
  • [Journal-full-title] Journal of minimally invasive gynecology
  • [ISO-abbreviation] J Minim Invasive Gynecol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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91. Braunschweig F, Kramer MF, Assmann G, Arbogast S, Leunig A: [Schwannoma of the nasal cavity: a case report]. HNO; 2007 Dec;55(13):1013-6
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  • [Title] [Schwannoma of the nasal cavity: a case report].
  • Most of them are benign and do not recur when totally removed by surgery.
  • It is very important to distinguish between schwannoma and primary benign neurofibroma.
  • Neurofibromas are lesions having the possibility for malignant transformation and recurrence.
  • A case of schwannoma in the nasal cavity is reported, and the diagnostic and therapeutic procedures, as well as recommendations from the literature, are described.
  • The histological and immunohistochemical features are discussed in detail to draw a distinction between schwannoma and neurofibroma.
  • In cases of intranasal and paranasal lesions, the existence of a schwannoma must be considered.
  • Differentiating between schwannoma and neurofibroma is important for estimating the risk of malignant transformation and recurrence.
  • [MeSH-major] Nasal Cavity / pathology. Neurilemmoma / pathology. Neurofibroma / pathology. Nose Neoplasms / pathology

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  • (PMID = 17874054.001).
  • [ISSN] 1433-0458
  • [Journal-full-title] HNO
  • [ISO-abbreviation] HNO
  • [Language] ger
  • [Publication-type] Case Reports; English Abstract; Journal Article; Review
  • [Publication-country] Germany
  • [Number-of-references] 22
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92. Steinmann K, Kluwe L, Friedrich RE, Mautner VF, Cooper DN, Kehrer-Sawatzki H: Mechanisms of loss of heterozygosity in neurofibromatosis type 1-associated plexiform neurofibromas. J Invest Dermatol; 2009 Mar;129(3):615-21
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  • [Title] Mechanisms of loss of heterozygosity in neurofibromatosis type 1-associated plexiform neurofibromas.
  • Plexiform neurofibromas constitute a serious burden for patients with neurofibromatosis type 1 (NF1), a common autosomal dominant disorder characterized by pigmentary changes and tumorous skin lesions (neurofibromas).
  • Despite the prominence of these benign tumors in NF1 patients, the mechanisms underlying the tumor-associated loss of heterozygosity (LOH) in plexiform neurofibromas have not been extensively studied.
  • We performed LOH analysis on 43 plexiform neurofibromas from 31 NF1 patients, the largest study of its kind to date.
  • A total of 13 (30%) plexiform neurofibromas exhibited LOH involving 17q markers.
  • In three tumors, LOH was found to be confined to the NF1 gene region.
  • However, in none of the tumors was a somatic NF1 microdeletion, mediated by non-allelic homologous recombination between either NF1-REPs or SUZ12 genes, detected.
  • Thus, NF1 microdeletions do not appear to be frequent somatic events in plexiform neurofibromas.
  • Determination of NF1 gene copy number by multiplex ligation-dependent probe amplification indicated that although tumors with smaller regions of LOH were characterized by 17q deletions, no NF1 gene copy number changes were detected in six plexiform neurofibromas with more extensive LOH.
  • To our knowledge, mitotic recombination has not previously been reported to be a frequent cause of LOH in plexiform neurofibromas.

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  • (PMID = 18800150.001).
  • [ISSN] 1523-1747
  • [Journal-full-title] The Journal of investigative dermatology
  • [ISO-abbreviation] J. Invest. Dermatol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Oligonucleotide Probes
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93. Muñoz Herrera A, Coscarón Blanco E, Beltrán Mateos LD, Paniagua Escudero J, Morales Ramos F: [Auditory-evoked brainstem response in a schwannoma of the VIIIth cranial nerve: an anatomophysiological and functional correlation. Our experience]. Acta Otorrinolaringol Esp; 2005 Apr;56(4):147-51
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  • [Title] [Auditory-evoked brainstem response in a schwannoma of the VIIIth cranial nerve: an anatomophysiological and functional correlation. Our experience].
  • [Transliterated title] Respuesta evocada auditiva de tronco en el schwannoma del VIII par: correlación anatomofisiológica y funcional. Nuestra experiencia.
  • INTRODUCTION: Schwannomas are tumors charasteristically originated from the nerve sheath.
  • They expande eccentrically from the nerve promoting a disturbance in the neural function either due to vascular compresion or to the effect of the tumor itself on the nerve, disturbance that can be evidenced with the Auditory-evoked Brainstem Response (ABR), fact that constituted the base for their diagnosis in the past.
  • The new radiological techniques developed over the last decades have made possible an earlier diagnosis, and therefore changed their prognosis in terms of nerve function.
  • Diagnosis is more certain today with these techniques so it could seem that ABR may not have any longer a role in the management of this condition.
  • OBJECTIVE: To describe the most frequent ABR patterns in those patients suffering from acoustic schwannoma related to the size of the tumour and the remanent audition thresholds, and to determine the value of this test in our practice.
  • Whose diagnosis was done between 1997 and 2003 in relation to the size of the tumour and the residual auditory function.
  • RESULTS AND CONCLUSIONS: ABR has a good sensibility for detection of acoustic schwannoma.
  • ABR patterns do not relate to the size of the tumour but they do to the auditory function.
  • [MeSH-major] Cranial Nerve Neoplasms / physiopathology. Evoked Potentials, Auditory, Brain Stem. Neurilemmoma / physiopathology

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  • (PMID = 15871289.001).
  • [ISSN] 0001-6519
  • [Journal-full-title] Acta otorrinolaringológica española
  • [ISO-abbreviation] Acta Otorrinolaringol Esp
  • [Language] spa
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Spain
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94. Riccardi VM: The genetic predisposition to and histogenesis of neurofibromas and neurofibrosarcoma in neurofibromatosis type 1. Neurosurg Focus; 2007;22(6):E3
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  • [Title] The genetic predisposition to and histogenesis of neurofibromas and neurofibrosarcoma in neurofibromatosis type 1.
  • He emphasizes the importance of understanding that not all neurofibromas are the same and that the key differences between the types of neurofibromas involve which portions of the nerve sheath contribute to the distinctive behavior of the different types of lesions.
  • Endoneurial neurofibromas derive from cellular elements ordinarily restricted to the endoneurium.
  • Perineurial neurofibromas arise within individual fascicles of a nerve and are largely confined thereby, precluding a breach of the epineurium.
  • Epineurial neurofibromas are contained only by the epineurium, and ultimately that portion of the nerve sheath is breached by these lesions.
  • With respect to surgical treatment, perineurial neurofibromas will have clean planes of dissection about the involved nerve.
  • In contrast, endoneurial and epineurial neurofibromas infiltrate adjacent tissues, leading to surgical challenges.
  • Thus, drugs that might be effective when the perineurium is absent or rent may be less effective (or not effective at all) if the perineurium is intact, as is expected in cases of perineurial neurofibromas.
  • [MeSH-major] Genetic Predisposition to Disease / genetics. Neurofibromatosis 1 / genetics. Neurofibromatosis 1 / pathology. Neurofibrosarcoma / genetics. Neurofibrosarcoma / pathology


95. Valeyrie-Allanore L, Ortonne N, Lantieri L, Ferkal S, Wechsler J, Bagot M, Wolkenstein P: Histopathologically dysplastic neurofibromas in neurofibromatosis 1: diagnostic criteria, prevalence and clinical significance. Br J Dermatol; 2008 May;158(5):1008-12
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  • [Title] Histopathologically dysplastic neurofibromas in neurofibromatosis 1: diagnostic criteria, prevalence and clinical significance.
  • BACKGROUND: Malignant peripheral nerve sheath tumours (MPNSTs) correspond to the most frequent and aggressive neoplasic complications associated with poor prognosis in neurofibromatosis 1.
  • METHODS: According to our database, we retrospectively included, between 1 March 2000 and 31 August 2004, all patients who had subcutaneous and/or plexiform neurofibromas removed surgically.
  • Tumour specimens were systematically reviewed; dysplastic neurofibroma was defined by the association of high cellularity and the presence of atypical cells.
  • Clinically atypical and histopathologically dysplastic neurofibromas were analysed using Fisher's exact test.
  • RESULTS: Among the 89 plexiform and/or subcutaneous neurofibromas surgically removed, high cellularity and cytonuclear atypia were observed in 19% and 17% of cases, respectively.
  • [MeSH-major] Nerve Sheath Neoplasms / pathology. Neurofibroma, Plexiform / pathology. Neurofibromatosis 1 / pathology
  • [MeSH-minor] Adult. Female. Humans. Male. Middle Aged. Peripheral Nervous System Neoplasms / pathology. Prevalence. Retrospective Studies. Subcutaneous Tissue / pathology

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  • (PMID = 18363759.001).
  • [ISSN] 0007-0963
  • [Journal-full-title] The British journal of dermatology
  • [ISO-abbreviation] Br. J. Dermatol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
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96. Brown RM, Klesse LJ, Le LQ: Cutaneous features predict paraspinal neurofibromas in neurofibromatosis type 1. J Invest Dermatol; 2010 Sep;130(9):2167-9
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  • [Title] Cutaneous features predict paraspinal neurofibromas in neurofibromatosis type 1.
  • In neurofibromatosis type 1 (NF-1), malignant transformation of internal plexiform neurofibromas carries a poor prognosis, in part because they are not evident clinically.
  • In this issue, Sbidian et al. describe a novel "NF-1Score" equation that employs four easily observable traits to predict the presence of paraspinal neurofibromas.
  • [MeSH-major] Neurofibromatosis 1 / mortality. Neurofibromatosis 1 / pathology. Skin / pathology. Skin Neoplasms / mortality. Skin Neoplasms / pathology
  • [MeSH-minor] Cell Transformation, Neoplastic. Disease Progression. Humans. Predictive Value of Tests. Risk Factors


97. Komoribayashi N, Arai H, Kojo T, Obonai C, Wakabayashi J, Ogawa A: [Subfrontal schwannoma: case report]. No Shinkei Geka; 2005 Jun;33(6):601-5
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  • [Title] [Subfrontal schwannoma: case report].
  • Schwannoma are benign, slowly growing nerve sheath tumors.
  • They can arise from any peripheral nerve containing Schwann cells including distal portions of cranial nerves.
  • We report a case of subfrontal schwannoma in a 38-year-old woman who presented with seizures in February 2003.
  • The left carotid angiography revealed that the tumor was supplied from the anterior ethomoidal artery, but no remarkable tumor stain was detected.
  • Bifrontal craniotomy with total excision of the tumor revealed a tumor in the left subfrontal lesion.
  • The tumor was attached to the falx, the left frontal lobe, and the skull base, but those structures were not invaded.
  • Unfortunately, we could not detect the left olfactory nerve.
  • Subsequent histological examination proved that this tumor was schwannoma.
  • For this reason they have not been diagnosed preoperatively.
  • Further cases of subfrontal schwannoma are necessary to solve the enigma of the origin of this tumor.
  • [MeSH-major] Brain Neoplasms / surgery. Neurilemmoma / surgery

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  • (PMID = 15952309.001).
  • [ISSN] 0301-2603
  • [Journal-full-title] No shinkei geka. Neurological surgery
  • [ISO-abbreviation] No Shinkei Geka
  • [Language] jpn
  • [Publication-type] Case Reports; English Abstract; Journal Article; Review
  • [Publication-country] Japan
  • [Number-of-references] 28
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98. Gibson JA, Hornick JL: Mucosal Schwann cell "hamartoma": clinicopathologic study of 26 neural colorectal polyps distinct from neurofibromas and mucosal neuromas. Am J Surg Pathol; 2009 May;33(5):781-7
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  • [Title] Mucosal Schwann cell "hamartoma": clinicopathologic study of 26 neural colorectal polyps distinct from neurofibromas and mucosal neuromas.
  • Colorectal polyps containing S-100-positive neural proliferations in the lamina propria that lack ganglion cells have been variously referred to as "neuromas" or "neurofibromas."
  • However, these lesions have not been systematically examined, and whether they are associated with type 1 neurofibromatosis (NF1) or other inherited syndromes is unknown.
  • The aim of this study was to evaluate the clinicopathologic and immunohistochemical features of these lesions, in comparison to colorectal neurofibromas from known NF1 patients.
  • The findings were compared with those in mucosal biopsies of 5 submucosal neurofibromas from NF1 patients.
  • After a mean follow-up of 6.5 years (range, 3 mo to 17.5 y), none of the patients developed other neural polyps, and none had evidence of NF1 or other inherited syndromes.
  • The 5 neurofibromas showed similar histologic features, but were generally less uniformly cellular, showed some intralesional heterogeneity, and showed less extensive staining for S-100 protein; all contained scattered NFP-positive axons.
  • In summary, solitary colorectal polyps containing pure Schwann cell proliferations in the lamina propria are not associated with NF1.
  • Distinguishing these lesions from NF1-associated neurofibromas is difficult based on histologic features; the presence of an underlying submucosal nodule or mass should be excluded endoscopically, and immunohistochemistry should be performed.
  • Although their nature is uncertain, we propose the interim designation "mucosal Schwann cell 'hamartoma'" to avoid confusion with the neural lesions that have significant associations with inherited syndromes.
  • [MeSH-major] Colonic Polyps / pathology. Colorectal Neoplasms / pathology. Hamartoma / pathology. Intestinal Mucosa / pathology. Neurofibromatosis 1 / pathology. Neuroma / pathology. Schwann Cells / pathology

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  • (PMID = 19065103.001).
  • [ISSN] 1532-0979
  • [Journal-full-title] The American journal of surgical pathology
  • [ISO-abbreviation] Am. J. Surg. Pathol.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Actins; 0 / Antigens, CD34; 0 / CLDN1 protein, human; 0 / Claudin-1; 0 / Glial Fibrillary Acidic Protein; 0 / Membrane Proteins; 0 / Mucin-1; 0 / Neurofilament Proteins; 0 / S100 Proteins; EC 2.7.10.1 / Proto-Oncogene Proteins c-kit
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99. Friedrich RE, Hagel C: Appendices of the nipple and areola of the breast in Neurofibromatosis type 1 patients are neurofibromas. Anticancer Res; 2010 May;30(5):1815-7
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  • [Title] Appendices of the nipple and areola of the breast in Neurofibromatosis type 1 patients are neurofibromas.
  • All specimens were found to be composed entirely from neurofibromas with no residues resembling segments of glandular structures.
  • Tumours arising in the areola and nipple area and mimicking an accessory nipple were all neurofibromas.
  • Local excision of neurofibromas was adequate to relief patients from an often unsightly appearance.
  • [MeSH-major] Breast / pathology. Neurofibromatosis 1 / pathology. Nipples / pathology. Skin Neoplasms / pathology


100. Park WC, White WA, Woog JJ, Garrity JA, Kim YD, Lane J, Witte R, Babovic-Vuksanovic D: The role of high-resolution computed tomography and magnetic resonance imaging in the evaluation of isolated orbital neurofibromas. Am J Ophthalmol; 2006 Sep;142(3):456-63
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  • [Title] The role of high-resolution computed tomography and magnetic resonance imaging in the evaluation of isolated orbital neurofibromas.
  • PURPOSE: To analyze the imaging findings in patients diagnosed with isolated orbital neurofibromas.
  • METHODS: Computed tomographic (CT) and magnetic resonance imaging (MRI) findings were reviewed in five patients with orbital neurofibromas in the absence of systemic neurofibromatosis in this multicenter study.
  • In addition, MRI studies in one patient revealed the previously unreported presence of neurofibromas involving extraocular muscle.
  • CONCLUSIONS: Isolated orbital neurofibromas may present with differing radiologic appearances.
  • The combined use of high-resolution CT and MRI may be of benefit in the preoperative diagnosis of these uncommon orbital tumors.
  • [MeSH-major] Magnetic Resonance Imaging / methods. Neurofibroma / diagnosis. Orbital Neoplasms / diagnosis. Tomography, X-Ray Computed / methods

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  • (PMID = 16935591.001).
  • [ISSN] 0002-9394
  • [Journal-full-title] American journal of ophthalmology
  • [ISO-abbreviation] Am. J. Ophthalmol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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