17 results on '"schwanomma"'
Search Results
2. Solitary Dorsal Intramedullary Schwanomma – A Rare Lesion
- Author
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Laxmikant Bhople, Hrushikesh Kharosekar, Nimesh Jain, and Vernon Velho
- Subjects
dorsal ,intramedullary ,schwanomma ,Medicine ,Surgery ,RD1-811 - Abstract
Intramedullary schwanommas are rare, and most cases are reported in cervical region. Less than 20 dorsal intramedullary schwanommas have been reported till date in literature. This is due to their cell of origin, the Schwann cell, which is not normally found within the parenchyma of the brain and spinal cord; therefore it is not surprising that these lesions are rare. We report a rare solitary dorsal intramedullary schwanomma in a young adult patient who presented with paraplegia.
- Published
- 2022
- Full Text
- View/download PDF
3. Tongue Base Schwannoma.
- Author
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Tandon, Swati, Meher, Ravi, Chopra, Aditi, Raj, Anoop, Wadhwa, Vikram, Mahajan, Nidhi, and Jain, Avani
- Subjects
- *
SCHWANNOMAS , *ENGLISH literature , *NECK - Abstract
Intraoral schwannomas account for 1% of head and neck schwannoma, with tongue base schwannoma being reported rarely. In an English literature search from 1988 to 2014, not more than 38 cases of tongue base schwannoma have been reported. Large tongue base schwannomas pose a significant risk to airway, thereby calling for an early intervention. We report a rare case of tongue base schwannoma in a 25 year old female managed surgically by lateral pharyngotomy approach. [ABSTRACT FROM AUTHOR]
- Published
- 2019
- Full Text
- View/download PDF
4. Malignant peripheral nerve sheath tumor of the pancreas—A case report.
- Author
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Balineni, Pradeep, Arcot, Rekha, Devygounder, Kannan, Rahaman, Khalilur, Narayansamy, Bharath, Prabhu, Manoj, and Vaitheeswaran, Shantini
- Abstract
Highlights • Pancreatic schwannoma arise from sympathetic and Para sympathetic fibers that cross pancreas. • Macroscopically they are well circumscribed, encapsulated lesions, homogenous lesions. • Microscopically they show Antoni A & B areas. • They demonstrate S100, vimentin, CD56 positivity. • Malignant transformation is extremely rare with only 8 reported cases till now. • Treatment is simple enucleation, but a accurate pre-operative diagnosis is difficult to make hence a oncollogically margin negative resection is done. Abstract Introduction: Nerve sheath tumors are tumors arising from nerve sheaths or which show nerve sheath differentiation. They are divided as benign and malignant. They are associated with Von Recklinghausen syndrome. Characterised by café-au-lait spots, lisch nodules, acoustic neuromas, pheochromocytomas. Presentation of case: A 62 year old gentleman came with complaints of Abdominal discomfort for 1month. He also had Left sided abdominal pain, Anorexia, Vomiting, No history of dyspepsia, fever, bowel disturbance. Patient is a known case of diabetes mellitus II. On abdominal examination a large mass of 10*8 cms was palpated in the left hypochondrium, left lumbar, and umbilical region. It had restricted mobility, not moving with respiration, firm in consistency. On palpation of neck, the thyroid gland was found to be enlarged with palpable right lobe. Contrast enhanced computed tomography showed large lobulated heterogenously enhancing mass with internal necrosis and calcifications in the left hypochondrium, in the region of distal body and tail of pancreas. There were no other foci of metastasis in abdomen or chest. Chest X-ray of patient was normal. Ultrasound of neck revealed a suspicious nodule in right lobe of thyroid measuring 1*1 cms with no nodal enlargement. Fnac of thyroid nodule was done under image guidance which was suggestive of papillary carcinoma of thyroid. As image guided biopsy of abdominal tumour could not be done patient was planned for laparotomy. The tumor was resected en bloc - distal pancreatectomy and splenectomy. Post opereative biopsy showed irregular spindle shaped cells with nuclei exhibiting "a wavy, buckled appearance" malignant peripheral nerve sheath tumor. Patient was reviewed after a period of 2 months. Positron emission tomography was done which revealed para aortic nodes and 2 mesenteric nodes. It also revealed a metabolically active nodule in thyroid with cervical nodal metastasis. Patient was taken up for total thyroidectomy with functional neck dissection. Histopathology confirmed papillary carcinoma of thyroid with positive lymph nodes. Patient was advised to undergo a radioactive iodine scan showed 0.3% uptake. Patient was started on chemotherapy for para aortic nodes with a regimen of paclitaxel, adriamycin, ifosumide and mesma. Discussion: Pancreatic schwannoma arise from sympathetic and Para sympathetic fibers that cross over pancreas. Patients Present with abdominal pain and weight loss commonly. Macroscopically they are well circumscribed, encapsulated lesions, homogenous lesions. Microscopically they show Antoni A & B areas. They demonstrate S100, vimentin, CD56 positivity. Most common differential diagnosis are cystic mucinous / serous neoplasms of pancreas. Malignant transformation is extremely rare with only 8 reported cases till now. Treatment is simple enucleation, but a accurate pre-operative diagnosis is difficult to make hence a oncollogically margin negative resection is done. Adjuvant radiotherapy can be given to reduce the tumor load. Chemotherapy can be given with ifosumide and doxorubicin. Conclusion: Malignant pancreatic nerve sheath tumours are very rare to be found, but have very poor prognosis, hence in cases of pancreatic tumour its better to keep the diagnosis of nerve sheath tumour also a differential diagnosis. In conclusion pancreatic tumours are to be identified at the earliest and surgery should be done for those cases [ABSTRACT FROM AUTHOR]
- Published
- 2019
- Full Text
- View/download PDF
5. A Rare Case of Schwannoma of Hard Palate: A Case Report
- Author
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Vidya Viswanathan, Ashily Koshy, Arpana Dharwadkar, and Shruti Vimal
- Subjects
Hard palate ,S-100 ,schwanomma ,vimentin ,Medicine - Abstract
Schwannoma or neurilemmomas are benign, encapsulated, slow growing nerve sheath tumors of ectodermal origin derived from schwann cells of the nerve sheath. It is commonly seen in the head and neck region. It mainly arises from vestibulocochlear nerve (80%) and rarely in oral cavity (1%), tongue being the most common site in the oral cavity. Palate is a rare site for schwannoma. Herein, we report a rare case of schwannoma of the hard palate in a 21-year-old male. It was excised intraorally. A histopathological diagnosis of schwannoma was made. Immunohistochemistry with S-100 and vimentin were positive which confirmed the diagnosis of schwannoma.
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- 2018
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6. Une cause inhabituelle de dysphagie haute: schwannome de la l oge hyo-thyro-épiglottique.
- Author
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Ayad, Jalila Ben, Nekro, Chaimae, Rokhssi, Safaa, Lassikri, Omar, Benbouzid, Mohamed Anass, and Essakalli, Leila
- Abstract
Schwannomas are well encapsulated mesenchymal tumors of peripheral nerves, with slow growth. The laryngeal schwanomma is exceptional, we present a case not described in the literature of shwannoma in the pre epiglottic space. A 50-year-old woman, who had a history of foreign body sensation for 4 years ago, progressing to high dysphagia and hoarseness. Direct laryngoscopy in suspension demonstrated a regular submucosal mass in the supraglottic space, reducing pharyngo-laryngeal space. CT and MRI concluded with a benign tumor of the preepiglottic space. External surgical excision was performed and a primary tracheotomy was required. The histological examination with an immunochemical study confirmed a benign schwannoma. There was no complication postoperatively. The outcome was excellent after 2 years of follow-up. [ABSTRACT FROM AUTHOR]
- Published
- 2017
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- View/download PDF
7. Surgical Management of Multiple-Level Lumbar Spinal Schwannomas: A Case Report.
- Author
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Ekhator C and Rak R
- Abstract
The increase in benign spinal tumors among adults over the last decade has been a major cause of concern. This worrisome trend has been attributed to many factors, including improved detection techniques, enhanced access to medical care, and the aging population. The research primarily focuses on Schwannoma, which is a rare type of tumor that arises from Schwann cells, which are responsible for producing the myelin sheath that surrounds and protects nerves. Although the majority of schwannomas are benign, there have been instances where they have transformed into malignant tumors, potentially leading to significant morbidity and mortality. We report a case of a 68-year-old woman who presented with progressive back pain and weakness in both lower limbs over the past months. The pain was initially localized to the lower back but gradually intensified and radiated down to the legs. The patient reported difficulty walking and a sensation of tingling and numbness in the feet. She denied any recent trauma or significant medical history. On physical examination, there was reduced muscle strength (3/5) in both lower limbs. The patient exhibited hyporeflexia in the knees and ankle. A magnetic resonance imaging (MRI) of the spine was performed, revealing a well-defined mass lesion located in the lumbar region, compressing the spinal cord from L2 to L5. The patient was counseled and prepared for surgical resection of the tumor. Histopathological findings revealed features of peripheral nerve sheath tumors and cellular schwannomas. The patient recovered well postoperatively. The surgeon operating should be mindful of the potential presence of a mobile schwannoma, even though it is rarely mentioned in the literature. Being aware of this possibility can help prevent unnecessary surgical dissection, which can lead to higher rates of complications and morbidity. Although it is plausible that this case could have involved a mobile schwannoma, there was not enough evidence to support it as we performed a laminectomy on multiple levels due to the tumor's size., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2023, Ekhator et al.)
- Published
- 2023
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8. Surgical Management of Large (≥3 cm) Trigeminal Schwannomas: Functional Outcomes and Approach Selection in Multicompartmental Schwannomas.
- Author
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Dwarakanath S, Deora H, Mehta S, Shukla D, A A, Rao K, and Somanna S
- Abstract
Introduction Trigeminal schwannoma surgery has shown a remarkable improvement in functional recovery and tumor resection. In the era of radiosurgery, these outcomes need to be characterized for tumors which are outside the realm of being treated with radiosurgery. We present a series of trigeminal schwannomas larger than 3 cm, surgical approaches used, and outcomes with an emphasis on functional recovery in a high-volume center with radiosurgery facilities. Method All consecutive cases of trigeminal schwannoma from January 2012 to May 2021 which were more than 3 cm in size and underwent microsurgery were included in this series. The surgical approach, neurological outcomes, and extent of resection were defined objectively with pre/postoperative magnetic resonance imaging. Results A total of 83 such cases (>3 cm) were found, with cranial nerve symptoms (5th most common) being the commonest. Twenty three percent cases had blindness due to secondary optic atrophy and eighteen percent had long tract motor symptoms signifying the tumor burden in our series. Radiological gross total excision was achieved in 75.9% cases. Conclusion Large-volume schwannomas present with cranial nerve involvement and may need extensive skull base approaches. Functional outcomes need to be prioritized and can be achieved albeit with lesser gross resection rates. Hearing and facial preservation in addition to relief of trigeminal symptoms should be the goal of resection with minimal additional morbidity., Competing Interests: Conflict of Interest None declared., (Thieme. All rights reserved.)
- Published
- 2023
- Full Text
- View/download PDF
9. Stereotactic radiosurgery does not appear to impact cochlear implant performance in patients with neurofibromatosis type II.
- Author
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Pisa, Justyn, Sulkers, Jacob, Butler, James B., West, Michael, and Hochman, Jordan B.
- Subjects
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COCHLEAR implants , *NEUROFIBROMATOSIS , *STEREOTACTIC radiosurgery , *SPEECH perception , *AUDITORY perception - Abstract
Introduction: Neurofibromatosis Type 2 (NF2) is a genetic condition associated with the presence of bilateral vestibular schwannoma and progressive sensorineural hearing loss. Treatment options include observation, stereotactic radiosurgery (SRS) or surgical resection. Historically, retrocochlear pathology was a contraindication to cochlear implantation (CI). However it is now recognized as viable, in an attempt to restore hearing and improve communication. Thus, the aim of this study was to contrast auditory function in CI recipients with NF2 whose tumors were either observed versus initially treated with SRS. Methods: A local review of hearing outcomes in 2 CI patients (3 ears total) with NF2 was undertaken and then pooled with the existing medical literature. Comparative post-implant outcome data, including pure-tone average and aided speech perception measurements was analyzed and compared between 8 subjects whose tumors were observed and 11 subjects who received preimplantation SRS. Results: Mean tumor size in the observation cohort was 0.81cm (.13cm to 1.50cm), and 2.34cm in the SRS group (0.10cm to 3.7cm). The mean reported duration of deafness was 22 months as compared to 71 months in the SRS cohort. Both groups demonstrated similar pre-implant candidacy criteria, average age and open-set speech recognition. Although disparate outcome measurements were utilized across studies, an analysis of post-implant open-set speech perception found no significant differences between groups. Conclusion: Despite a small sample size and highly variable post implantation testing methods, patients who undergo SRS for NF2 associated Schwannoma prior to CI have similar hearing performance and benefit by having already completed definitive tumor management. [ABSTRACT FROM AUTHOR]
- Published
- 2017
10. PATTERNS OF MEDIASTINAL TUMORS; A TWO AND HALF YEAR EXPERIENCE.
- Author
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Soomro, Niaz Hussain, Shariff, Ali Shan, Zafar, Aneeqa Ahsan, Ehtisham, Omar, Maxood, Guzel, and Panjwani, Kinza
- Subjects
- *
THORACIC surgery , *COMPUTED tomography , *HISTOPATHOLOGY , *LYMPHOMAS , *TERATOCARCINOMA ,MEDIASTINAL tumors - Abstract
Objective: To review all the mediastinal tumors and cysts operated upon at Department of Thoracic Surgery, OICD, DUHS over a 2.5 year period. Study design: Retrospective descriptive study. Place and duration of study: Ojha Institute of Chest Diseases, Dow University of Health Sciences, Karachi, Pakistan from November 2012-April 2015. Material and methods: 50 patients with mediastinal masses of either sex were included in the study. Tumors were categorized as arising from the anterior, middle and posterior mediastinum on the basis of CT scan chest. Tumors were classified into various type based on the results of histopathology. Data was obtained from hospital medical records and proformas were filled for each patient. Results: A total of 50 patients with mediastinal masses were seen at the Department of Thoracic Surgery, Ojha Institute of Chest Diseases between Nov 2012-April 2015. There were 30 males and 20 females (ratio 1.5:1). Most of the patients were between 11-70 years of age, with mean age of 32 years. The most common location for mediastinal tumors was found to be the anterior mediastinum which comprised of 40% of all tumors, followed by posterior (32%) and middle mediastinum respectively (28.0%). Shwanomma (18%) was the most common type of tumor observed, followed by lymphoma (12%) and germ cell tumors (10%). Conclusion: Mediastinal tumors are relatively uncommon in clinical setting. These tumors represent a group of heterogeneous masses present between two pleural cavities. A definitive early diagnosis is the key in management and prognosis of the patient. We present our 5 year experience of patterns of mediastinal tumors. [ABSTRACT FROM AUTHOR]
- Published
- 2016
- Full Text
- View/download PDF
11. Malignant peripheral nerve sheath tumor of the pancreas—A case report
- Author
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Khalilur Rahaman, Rekha Arcot, Bharath Narayansamy, Shantini Vaitheeswaran, Pradeep Balineni, Manoj Prabhu, and Kannan Devygounder
- Subjects
medicine.medical_specialty ,Abdominal pain ,Tail of pancreas ,Pancreatic tumour ,Malignant peripheral nerve sheath tumor ,Schwannoma ,Article ,03 medical and health sciences ,0302 clinical medicine ,medicine ,ath tumour ,business.industry ,Thyroid ,medicine.disease ,Schwanomma ,medicine.anatomical_structure ,030220 oncology & carcinogenesis ,Abdominal examination ,Malignant peripheral nerve she ,Abdomen ,030211 gastroenterology & hepatology ,Surgery ,Radiology ,medicine.symptom ,business ,Umbilical region ,MPNST of pancreas - Abstract
Highlights • Pancreatic schwannoma arise from sympathetic and Para sympathetic fibers that cross pancreas. • Macroscopically they are well circumscribed, encapsulated lesions, homogenous lesions. • Microscopically they show Antoni A & B areas. • They demonstrate S100, vimentin, CD56 positivity. • Malignant transformation is extremely rare with only 8 reported cases till now. • Treatment is simple enucleation, but a accurate pre-operative diagnosis is difficult to make hence a oncollogically margin negative resection is done., Introduction: Nerve sheath tumors are tumors arising from nerve sheaths or which show nerve sheath differentiation. They are divided as benign and malignant. They are associated with Von Recklinghausen syndrome. Characterised by café-au-lait spots, lisch nodules, acoustic neuromas, pheochromocytomas. Presentation of case: A 62 year old gentleman came with complaints of Abdominal discomfort for 1month. He also had Left sided abdominal pain, Anorexia, Vomiting, No history of dyspepsia, fever, bowel disturbance. Patient is a known case of diabetes mellitus II. On abdominal examination a large mass of 10*8 cms was palpated in the left hypochondrium, left lumbar, and umbilical region. It had restricted mobility, not moving with respiration, firm in consistency. On palpation of neck, the thyroid gland was found to be enlarged with palpable right lobe. Contrast enhanced computed tomography showed large lobulated heterogenously enhancing mass with internal necrosis and calcifications in the left hypochondrium, in the region of distal body and tail of pancreas. There were no other foci of metastasis in abdomen or chest. Chest X-ray of patient was normal. Ultrasound of neck revealed a suspicious nodule in right lobe of thyroid measuring 1*1 cms with no nodal enlargement. Fnac of thyroid nodule was done under image guidance which was suggestive of papillary carcinoma of thyroid. As image guided biopsy of abdominal tumour could not be done patient was planned for laparotomy. The tumor was resected en bloc - distal pancreatectomy and splenectomy. Post opereative biopsy showed irregular spindle shaped cells with nuclei exhibiting “a wavy, buckled appearance” malignant peripheral nerve sheath tumor. Patient was reviewed after a period of 2 months. Positron emission tomography was done which revealed para aortic nodes and 2 mesenteric nodes. It also revealed a metabolically active nodule in thyroid with cervical nodal metastasis. Patient was taken up for total thyroidectomy with functional neck dissection. Histopathology confirmed papillary carcinoma of thyroid with positive lymph nodes. Patient was advised to undergo a radioactive iodine scan showed 0.3% uptake. Patient was started on chemotherapy for para aortic nodes with a regimen of paclitaxel, adriamycin, ifosumide and mesma. Discussion: Pancreatic schwannoma arise from sympathetic and Para sympathetic fibers that cross over pancreas. Patients Present with abdominal pain and weight loss commonly. Macroscopically they are well circumscribed, encapsulated lesions, homogenous lesions. Microscopically they show Antoni A & B areas. They demonstrate S100, vimentin, CD56 positivity. Most common differential diagnosis are cystic mucinous / serous neoplasms of pancreas. Malignant transformation is extremely rare with only 8 reported cases till now. Treatment is simple enucleation, but a accurate pre-operative diagnosis is difficult to make hence a oncollogically margin negative resection is done. Adjuvant radiotherapy can be given to reduce the tumor load. Chemotherapy can be given with ifosumide and doxorubicin. Conclusion: Malignant pancreatic nerve sheath tumours are very rare to be found, but have very poor prognosis, hence in cases of pancreatic tumour its better to keep the diagnosis of nerve sheath tumour also a differential diagnosis. In conclusion pancreatic tumours are to be identified at the earliest and surgery should be done for those cases
- Published
- 2019
12. Fatal delayed post-operative cerebral venous thrombosis after excision of hypoglossal nerve schwanomma.
- Author
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Garg, N. and Sampath, S.
- Subjects
- *
VENOUS thrombosis , *CRANIAL nerves , *CARDIOVASCULAR diseases , *NECK blood-vessels , *CEREBROSPINAL fluid pressure , *BLOOD circulation disorders - Abstract
Lower cranial nerve schwanommas are rare tumours. We present a 35 year old female patient who had a lower cranial nerve schwanomma with both intracranial and extracranial components. The internal jugular vein was injured during the dissection of the extracranial portion of the tumour. Ligation of the internal jugular vein is not associated with significant post-operative complications. Our patient however, developed retrograde cortical venous thrombosis on the 14th post-operative day resulting in multiple areas of haemorrhagic venous infarction with raised intracranial pressure. Such a delayed contiguous cortical venous thrombosis has not been reported. We present this report to highlight this event and to outline the probable causes for the same. [ABSTRACT FROM AUTHOR]
- Published
- 2008
- Full Text
- View/download PDF
13. Une cause inhabituelle de dysphagie haute: schwannome de la l oge hyo-thyro-épiglottique
- Author
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Jalila Ben Ayad, Omar Lassikri, Chaimae Nekro, Mohamed Anass Benbouzid, Leila Essakalli, and Safaa Rokhssi
- Subjects
medicine.medical_specialty ,Case Report ,Schwannoma ,Epiglottis ,chirurgie ,surgery ,03 medical and health sciences ,0302 clinical medicine ,loge hyo-thyro-épiglottique ,medicine ,Humans ,030223 otorhinolaryngology ,Laryngeal Neoplasms ,Gynecology ,Schwanomme ,Laryngoscopy ,business.industry ,pre-epiglottic space ,General Medicine ,Middle Aged ,medicine.disease ,Dysphagia ,Schwanomma ,Magnetic Resonance Imaging ,030220 oncology & carcinogenesis ,Schwanomme, loge hyo-thyro-épiglottique, chirurgie ,Female ,medicine.symptom ,Tracheotomy ,business ,Deglutition Disorders ,Tomography, X-Ray Computed ,Neurilemmoma ,Follow-Up Studies - Abstract
Les schwannomes sont des tumeurs mesenchymateuses, bien encapsulees des nerfs peripheriques, de croissance lente. La localisation laryngee est exceptionnelle, nous presentons un cas non decrit dans la litterature de shwannome localise au niveau de la loge hyothyroepiglottique. Une patiente de 50 ans, qui presentait une sensation de corps etranger depuis 4 ans, evoluant vers une dysphagie haute associee a une voix rauque. La laryngoscopie directe en suspension objectivait une masse supraglottique sous muqueuse reguliere, reduisant la lumiere pharyngo-laryngee. La TDM et l'IRM concluaient a une tumeur benigne de la loge hyo-thyro-epiglottique. Une exerese chirurgicale par voie externe a ete realisee et une tracheotomie premiere etait necessaire. L'examen histologique avec une etude immunohistochimique confirmait un schwannome benin. Les suites post operatoires etaient simples. Aucune recidive n'etait objectivee apres 2 ans de recul.
- Published
- 2017
14. Robotic assisted laparoscopic excision of a renal schwannoma from a community hospital: A case report.
- Author
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Madueke I and Shore D
- Abstract
Renal schwannomas are an extremely rare renal tumor with possibility for malignant conversion. Although reports are scant, most reports have been presented from academic institutions. We report on a case of a renal schwannoma that was removed via robotic assisted laparoscopic nephrectomy in a community-based specialty practice under the suspicion of a renal malignancy.
- Published
- 2019
- Full Text
- View/download PDF
15. Neurosurgical treatment and outcome of Schwanommas arising from lumbosakral plexus or pelvic peripheral nerves. Case series and review of the literature
- Author
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Ahmadi, R, Mehrabi, A, Pham, M, and Unterberg, A
- Subjects
ddc: 610 ,lumbosacralis plexus ,610 Medical sciences ,Medicine ,schwanomma ,retroperitoneal - Abstract
Objective: Schwanommas of lumbosakral plexus or pelvic peripheral nerves are very rare retroperitoneal tumors. Clinical signs are non specific and they are not to be differed from other abdominal and pelvic masses radiologicaly. Complete removing of the tumor by resection of the nerve is a curative [for full text, please go to the a.m. URL], 64. Jahrestagung der Deutschen Gesellschaft für Neurochirurgie (DGNC)
- Published
- 2013
- Full Text
- View/download PDF
16. Hedgehog-Patched pathway aberrations in a malignant triton tumor case study
- Author
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Maja Čretnik, Vesna Musani, Iva Botica, Sonja Levanat, and Miljenko Bura
- Subjects
Patched ,Male ,Patched Receptors ,Cancer Research ,Pathology ,medicine.medical_specialty ,malignant triton tumor ,parotid gland ,Schwanomma ,Hegdkog pathway ,Loss of Heterozygosity ,Receptors, Cell Surface ,Biology ,Zinc Finger Protein GLI1 ,Nerve Sheath Neoplasms ,Receptors, G-Protein-Coupled ,Immunoenzyme Techniques ,03 medical and health sciences ,0302 clinical medicine ,medicine ,Humans ,Hedgehog Proteins ,Allele ,Hedgehog ,Chromatography, High Pressure Liquid ,Cyclin-Dependent Kinase Inhibitor p16 ,030304 developmental biology ,Aged ,0303 health sciences ,Neurofibromin 1 ,Oncogene ,Reverse Transcriptase Polymerase Chain Reaction ,Malignant triton tumor ,Basic Medical Sciences ,General Medicine ,medicine.disease ,Smoothened Receptor ,Hedgehog signaling pathway ,Neoplasm Proteins ,Pedigree ,Oncology ,PTCH1 ,030220 oncology & carcinogenesis ,Mutation ,Cancer research ,Female ,Haploinsufficiency ,Neurilemmoma ,Microsatellite Repeats ,Signal Transduction ,Transcription Factors - Abstract
Transition from malignant schwannoma to malignant triton tumor is analyzed in a case report on a patient with recurring cancers and suspected familial predisposition. It is hypothesized that rhabdomyoblastic differentiation, which distinguishes triton from schwannoma, might be attributable to Hedgehog-Patched pathway malfunctioning. Loss of one Patched gene allele was found in the tissue of advanced triton, but the retained allele had no exon or promoter mutations. Protein levels at early cancer stages indicated possible Patched response to the pathway activation in the first occurrence of triton tumor. Later, in the recurring triton, Patched expression was several times lower than in the control tissue, suggesting that haploinsufficiency was aided by silencing of the remaining allele, although its promoter was not hypermethylated. These findings may justify further investigation of the Hedgehog- Patched pathway role in triton malignancies, especially because of the recent research on the therapeutical potential of the pathway.
- Published
- 2008
17. Preclinical Evaluation of Gene Therapy for NF2 Lesions in Mouse Models Using Amplicon Vectors and Prodrug Activation
- Author
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GENERAL HOSPITAL CORP BOSTON MA, Breakefield, Xandra O., GENERAL HOSPITAL CORP BOSTON MA, and Breakefield, Xandra O.
- Abstract
These studies were designed to characterize tumors in mouse models of NF2 and to evaluate vector mediated therapy. Magnetic resonance, bioluminescence and near-infrared imaging were used in monitoring changes in tumor volume and in tracking gene delivery to there lesions over time in living animals. Several mouse models for NF2 were tested including: transgenic mice which express a dominant mutant form of the NF2 protein and develop spontaneous schwannomas; induction of schwannomas and brain tumors by injection of HSV amplicon vectors expressing Cre recombinase into mice homozygous for a floxed NF2 gene; and implantation of humanschwannoma or meningioma tissue into nude mice. Both schwannomas and meningiomas were shown to be highly infectable with therapeutic vectors derived from herpes simplex virus type-l. Injection of an oncolytic HSV vector G47A into schwannomas of transgenic mice led to a reproducible reduction in tumor volume. An HSV amplicon vector expressing the apostolic protein, caspase-11 was also developed. These vectors can potentially be used to reduce volume of surgically inaccessible tumors in NF2 patients., The original document contains color images. All DTIC reproductions will be in black and white.
- Published
- 2003
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