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https://www.readbyqxmd.com/read/29781505/cerebral-vasculopathy-in-childhood-neurofibromatosis-type-2-cause-for-concern
#1
Karine Lascelles, Shazia Afridi, Ata Siddiqui, Cheryl Hemingway, Rosalie Ferner, Vijeya Ganesan
Unlike adult neurofibromatosis type 2 (NF2), which presents with symptoms related to bilateral vestibular schwannomas, children with NF2 most frequently present with ocular, dermatological, and neurological symptoms. Arteriopathy, a well-established feature in neurofibromatosis type 1, is not a widely recognized feature of NF2. Here we report three children with NF2 with cerebral arteriopathy and/or arterial ischaemic stroke. Bevacizumab, a vascular endothethial growth factor inhibitor, is an established treatment for rapidly growing vestibular schwannomas; however, it carries a risk of both ischaemic and haemorrhagic stroke...
May 21, 2018: Developmental Medicine and Child Neurology
https://www.readbyqxmd.com/read/29781154/neuroglial-ectopia-of-the-vestibular-nerve-masquerading-as-a-vestibular-schwannoma-a-case-report
#2
John P Sheppard, Carlito Lagman, Thien Nguyen, Négar Khanlou, Quinton Gopen, Isaac Yang
Neuroglial ectopia is a rare entity of undetermined clinical significance. Here, we report a unique case of neuroglial ectopia of the vestibular nerve. A 27-year-old pharmacy student with a previous radiological diagnosis of vestibular schwannoma presented to our clinic for surgical evaluation. Magnetic resonance imaging (MRI) of the brain revealed a 17-mm T1 hypo- to isointense, T2 iso- to hyperintense, poorly enhancing left cerebellopontine angle mass extending into the left internal auditory canal compatible by imaging with a vestibular schwannoma...
May 20, 2018: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/29780700/sporadic-nf2-mosaic-multiple-spinal-schwannomas-presenting-with-severe-intractable-pain-following-pregnancy
#3
Jeffrey H Zimering, Bryan D Choi, Matthew J Koch, John C Dewitt, Anat Stemmer-Rachamimov, John H Shin
The aim of the present paper is to report undiagnosed sporadic neurofibromatosis type 2 presenting with symptomatic compressive spinal tumors following pregnancy. A 36-year-old woman experienced progressive, severe lumbar radicular pain in the second trimester of pregnancy which became intractable soon after delivery. Magnetic resonance imaging revealed a complex heterogeneous hypointense mass lesion around the conus. There were two small punctate lesions in the cauda equina suggestive of myxopapillary ependymoma with 'drop metastases...
December 2017: Interdisciplinary Neurosurgery: Advanced Techniques and Case Management
https://www.readbyqxmd.com/read/29779243/co-occurrence-of-schwannomatosis-and-rhabdoid-tumor-predisposition-syndrome-1
#4
Hildegard Kehrer-Sawatzki, Uwe Kordes, Simone Seiffert, Anna Summerer, Christian Hagel, Ulrich Schüller, Said Farschtschi, Reinhard Schneppenheim, Martin Bendszus, Tim Godel, Victor-Felix Mautner
BACKGROUND: The clinical phenotype associated with germline SMARCB1 mutations has as yet not been fully documented. It is known that germline SMARCB1 mutations may cause rhabdoid tumor predisposition syndrome (RTPS1) or schwannomatosis. However, the co-occurrence of rhabdoid tumor and schwannomas in the same patient has not so far been reported. METHODS: We investigated a family with members harboring a germline SMARCB1 deletion by means of whole-body MRI as well as high-resolution microstructural magnetic resonance neurography (MRN)...
May 20, 2018: Molecular Genetics & Genomic Medicine
https://www.readbyqxmd.com/read/29775147/intraoperative-cochlear-nerve-mapping-with-the-mobile-cochlear-nerve-compound-action-potential-tracer-in-vestibular-schwannoma-surgery
#5
Nobuyuki Watanabe, Takuya Ishii, Kazuhiko Fujitsu, Shogo Kaku, Teruo Ichikawa, Kosuke Miyahara, Tomu Okada, Shin Tanino, Yasuhiro Uriu, Yuichi Murayama
OBJECTIVE The authors describe the usefulness and limitations of the cochlear nerve compound action potential (CNAP) mobile tracer (MCT) that they developed to aid in cochlear nerve mapping during vestibular schwannoma surgery (VSS) for hearing preservation. METHODS This MCT device requires no more than 2 seconds for stable placement on the nerve to obtain the CNAP and thus is able to trace the cochlear nerve instantaneously. Simultaneous bipolar and monopolar recording is possible. The authors present the outcomes of 18 consecutive patients who underwent preoperative useful hearing (defined as class I or II of the Gardner-Robertson classification system) and underwent hearing-preservation VSS with the use of the MCT...
May 18, 2018: Journal of Neurosurgery
https://www.readbyqxmd.com/read/29774221/magnetic-resonance-imaging-findings-for-differential-diagnosis-of-perianal-plexiform-schwannoma-case-report-and-review-of-the-literature
#6
Xue-Liang Sun, Ke Wen, Zhi-Zhong Xu, Xiao-Peng Wang
Plexiform schwannoma is an extremely rare variant of schwannoma, accounting for approximately 5% of cases. Due to the rarity and lack of typical symptoms, signs and radiological images, a definite diagnosis of plexiform schwannoma may not be made by clinicians prior to biopsy. In the present study, we report the first case (to our knowledge) of perianal plexiform schwannoma arising from the overlapped skin of the ischioanal fossa, and we propose an intratumorally nonenhanced circumferential capsule dividing the tumour into multiple homogeneously enhanced nodules as a magnetic resonance imaging feature to aid in the differential diagnosis of plexiform schwannoma from ancient schwannoma, cavernous haemangioma, liposarcoma and plexiform neurofibroma...
May 16, 2018: World Journal of Clinical Cases
https://www.readbyqxmd.com/read/29771052/-a-case-of-schwannoma-in-cervical-trachea
#7
X Q Shi, J F Luo, Y Yang, X Chen, W S Qu, Y B Wang, F B Cheng
Primary tracheal schwannoma is rare. It is easy to be misdiagnosed as bronchitis, asthma due to the lack of special symptom. Diagnosis of schwannoma depends on a comprehensive examination. Surgery is the only effective mean. Here we reported a 17-year-old male, who suffered from cough for 10 months. A variety of tests confirmed that he had schwannoma. There is no significant change after tumor resection via rigid bronchoscope since two years ago.
May 20, 2017: Journal of Clinical Otorhinolaryngology, Head, and Neck Surgery
https://www.readbyqxmd.com/read/29765830/long-term-facial-nerve-outcomes-after-microsurgical-resection-of-vestibular-schwannomas-in-patients-with-preoperative-facial-nerve-palsy
#8
Michael A Mooney, Benjamin Hendricks, Christina E Sarris, Robert F Spetzler, Kaith K Almefty, Randall W Porter
Objectives  This study aimed at evaluating facial nerve outcomes in vestibular schwannoma patients presenting with preoperative facial nerve palsy. Design  A retrospective review. Setting  Single-institution cohort. Participants  Overall, 368 consecutive patients underwent vestibular schwannoma resection. Patients with prior microsurgery or radiosurgery were excluded. Main Outcome Measures  Incidence, House-Brackmann grade. Results  Of 368 patients, 9 had confirmed preoperative facial nerve dysfunction not caused by prior treatment, for an estimated incidence of 2...
June 2018: Journal of Neurological Surgery. Part B, Skull Base
https://www.readbyqxmd.com/read/29765828/a-cross-sectional-survey-of-the-north-american-skull-base-society-on-vestibular-schwannoma-part-2-perioperative-practice-patterns-of-vestibular-schwannoma-in-north-america
#9
Jamie J Van Gompel, Matthew L Carlson, R Mark Wiet, Nicole M Tombers, Anand K Devaiah M, Devyani Lal, Jacques J Morcos, Michael J Link
Introduction  Perioperative care of vestibular schwannoma (VS) patients is extremely variable across surgeons and institutions making practice patterns difficult to standardize. No data currently exist detailing this practice variability. Methods  The North American Skull Base Society membership was electronically surveyed regarding perioperative care of surgically operated VS patients. Results  There were 87 respondents to the survey. Surgical positioning, surgical approach utilized, and perioperative medical adjuncts are quite variable...
June 2018: Journal of Neurological Surgery. Part B, Skull Base
https://www.readbyqxmd.com/read/29765827/a-cross-sectional-survey-of-the-north-american-skull-base-society-current-practice-patterns-of-vestibular-schwannoma-evaluation-and-management-in-north-america
#10
Matthew L Carlson, Jamie J Van Gompel, R Mark Wiet, Nicole M Tombers, Anand K Devaiah, Devyani Lal, Jacques J Morcos, Michael J Link
Background  Very few studies have examined vestibular schwannoma (VS) management trends across centers and between providers. The objective of this study is to examine current practice trends, variance in treatment philosophies, and nuanced or controversial aspects of VS care across North America. Methods  This is a cross-sectional survey of North American Skull Base Society (NASBS) members who report regular involvement in VS care. Results  A total of 57 completed surveys were returned. Most respondents claimed to have over 20 years of experience and the majority reported working in an academic practice with an affiliated otolaryngology and/or neurosurgery residency program...
June 2018: Journal of Neurological Surgery. Part B, Skull Base
https://www.readbyqxmd.com/read/29765825/early-career-surgical-practice-for-cerebellopontine-angle-tumors-in-the-era-of-radiosurgery
#11
Giannantonio Spena, Tommaso Sorrentino, Roberto Altieri, Luca Redaelli de Zinis, Roberto Stefini, Pier Paolo Panciani, Marco Fontanella
We analyzed the outcomes of patients with large cerebellopontine angle (CPA) tumors treated by a skull-base team in which two surgeons (one neurosurgeon and one otological surgeon) were in the beginning of their careers (<40 years old). Data of patients operated on between April 2012 and March 2016 were reviewed. All factors related to surgical training were considered. Thirty-one patients had vestibular schwannomas, while 26 had meningiomas. Mean tumor diameter was 30.6 mm (range, 23-49 mm) for schwannomas and 35 mm (range, 22-51 mm) for meningiomas...
June 2018: Journal of Neurological Surgery. Part B, Skull Base
https://www.readbyqxmd.com/read/29764782/eaono-position-statement-on-vestibular-schwannoma-imaging-assessment-what-are-the-indications-for-performing-a-screening-mri-scan-for-a-potential-vestibular-schwannoma
#12
Jérôme Waterval, Romain Kania, Thomas Somers
No abstract text is available yet for this article.
April 2018: Journal of International Advanced Otology
https://www.readbyqxmd.com/read/29764781/eaono-position-statement-on-vestibular-schwannoma-imaging-assessment-question-how-should-growth-of-vestibular-schwannoma-be-defined
#13
Romain Kania, Benjamin Vérillaud, Domitille Camous, Charlotte Hautefort, Thomas Somers, Jérôme Waterval, Sébastien Froelich, Philippe Herman
The relevance of defining the growth of vestibular schwannoma (VS) is that any significant VS growth may impact treatment strategy. A conservative treatment strategy is often proposed as a primary treatment option in the management of VS. Several authors have demonstrated that a significant proportion of VSs do not grow, and those that do, usually grow slowly. Surgical and/or radiosurgical treatment options may be offered to the patient according to the VS growth. Therefore, defining the VS growth is a determinant in managing treatment strategies...
April 2018: Journal of International Advanced Otology
https://www.readbyqxmd.com/read/29764780/what-is-the-required-frequency-of-mri-scanning-in-the-wait-and-scan-management
#14
Thomas Somers, Romain Kania, Jerome Waterval, Tony Van Havenbergh
The wait and scan policy is being increasingly used as the first measure after the diagnosis of a vestibular schwannoma (VS) using magnetic resonance imaging (MRI). As part of the European Academy of Otology and Neuro-Otology (EAONO) position statement on VS, the frequency of imaging has been studied in the literature. Among 163 studies, 29 fulfilled the inclusion criteria and were scored using the Grading of Recommendations, Assessment, Development, and Evaluation system. Because tumor growth rate during the first 5 years of follow-up is predictive of further growth during the upcoming years, a protocol for wait and scan is useful for centers dealing with this condition...
April 2018: Journal of International Advanced Otology
https://www.readbyqxmd.com/read/29764009/a-rare-duodenal-subepithelial-tumor-duodenal-schwannoma
#15
Dong Hwahn Kahng, Gwang Ha Kim, Sang Gyu Park, So Jeong Lee, Do Youn Park
Schwannomas are uncommon neoplasms that arise from Schwann cells of the neural sheath. Gastrointestinal schwannomas are rare among mesenchymal tumors of the gastrointestinal tract, and only a few cases have been reported to date. Duodenal schwannomas are usually discovered incidentally and achieving a preoperative diagnosis is difficult. Schwannomas can be distinguished from other subepithelial tumors on endoscopic ultrasonography; however, any typical endosonographic features of duodenal schwannomas have not been reported due to the rarity of these tumors...
May 15, 2018: Clinical Endoscopy
https://www.readbyqxmd.com/read/29761250/sarcoma-in-neurofibromatosis-2-case-report-and-review-of-the-literature
#16
C Linder, M J Smith, M Bulman, A Wallace, A J Freemont, D C Mangham, D G R Evans
Neurofibromatosis type 2 (NF2) is associated with the development of several types of benign nervous system tumours, while malignancies are rare. We report a 22-year-old man who presented with retroperitoneal and spinal high-grade sarcomas with epithelial features. Samples showed a mixed epithelioid and spindled cell content with little associated matrix and inconclusive immunochemistry. Genetic analysis of a schwannoma and matched blood samples demonstrated a constitutional de novo substitution at the splice donor site of intron 8 of the NF2 gene and aa acquired large deletion of the entire NF2 gene as a second hit, with some loss of SMARCB1...
May 14, 2018: Familial Cancer
https://www.readbyqxmd.com/read/29756618/intraparotid-facial-nerve-schwannoma-two-case-reports-and-a-review-of-the-literature
#17
M Simone, E Vesperini, C Viti, A Camaioni, L Lepanto, F Raso
Schwannomas are rare benign tumours that arise from Schwann cells. The most known and studied is the intracranial vestibular schwannoma, even if it is not the most frequent. More often schwannomas arise from peripheral sensitive nerves, and the vagous is most involved among the cranial nerves. Intraparotid schwannomas account for just 10% of all facial involvement, so they are an extremely rare localisation. At present, there are less than 100 cases described in the literature. We performed a retrospective analysis of parotidectomy in two Italian hospitals and present two cases of intraparotid schwannoma and a review of the literature...
February 2018: Acta Otorhinolaryngologica Italica
https://www.readbyqxmd.com/read/29756611/cochlear-obliteration-following-a-translabyrinthine-approach-and-its-implications-in-cochlear-implantation
#18
B Delgado-Vargas, M Medina, R Polo, A Lloris, M Vaca, C Pérez, A Cordero, I Cobeta
The most frequent sequelae following a translabyrinthine approach for vestibular schwannoma resection is complete hearing loss on the affected side. Such patients could benefit from a cochlear implant, provided that two essential requisites are met before surgery: a preserved cochlear nerve and a patent cochlea to accommodate the electrode array. The goal of our study is to determine the prevalence and extent of cochlear ossification following a translabyrinthine approach. Postoperative MRI of 41 patients were retrospectively reviewed...
February 2018: Acta Otorhinolaryngologica Italica
https://www.readbyqxmd.com/read/29756228/comparison-of-ultrasonographic-findings-of-schwannomas-and-angioleiomyomas
#19
Dai Ogata, Masaaki Takeji, Takuo Murakami, Hiroto Yanagisawa, Akira Kuramochi, Tetsuya Tsuchida
A subcutaneous tumor accompanied by tenderness has a myriad of differential diagnoses. Indeed, using physical findings alone to achieve a diagnosis is often challenging. In this study, we focused on schwannomas and angioleiomyomas, which usually develop as hard subcutaneous tumors and are often associated with tenderness. We aimed to determine significant differentiating features between the tumor types, using ultrasonography. We compared clinical findings and ultrasonographic imaging and calculated the statistical significance for each item...
May 14, 2018: Journal of Dermatology
https://www.readbyqxmd.com/read/29753078/isolated-pediatric-intramedullary-schwannoma-case-report-and-review-of-the-literature
#20
Alessandro Landi, Giovanni Grasso, Fabrizio Gregori, Giorgia Iacopino, Andrea Ruggeri, Roberto Delfini
BACKGROUND: Intramedullary (IM) schwannomas are rare entities representing the 0.3-1% of intramedullary tumors and 1.1% of spinal schwannomas. Beside many theories proposed, their rare occurrence might be related to the absence of Schwann cells into the spinal cord. Pediatric IM schwannomas are uncommon and in absence of neurofibromatosis (NF) they are extremely rare. To date, very few cases have been reported in the literature. CASE DESCRIPTION: We describe the case of a 8 year-old female affected by a progressive paraparesis...
May 9, 2018: World Neurosurgery
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