Resection of 2 Intradural Extramedullary Cervical Spine Tumors in a Patient With Neurofibromatosis Type 2: 3-Dimensional Operative Video

2018 ◽  
Vol 16 (2) ◽  
pp. 274-274
Author(s):  
Simone E Dekker ◽  
Chad A Glenn ◽  
Thomas A Ostergard ◽  
Osmond C Wu ◽  
Fernando Alonso ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Cervical Spine ◽  
Treatment Options ◽  
Tumor Resection ◽  
Neurofibromatosis Type ◽  
Cord Compression ◽  
Neurofibromatosis Type 2 ◽  
3 Dimensional ◽  
Intradural Extramedullary

Abstract This 3-dimensional operative video illustrates resection of 2 cervical spine schwannomas in a 19-yr-old female with neurofibromatosis type 2. The patient presented with lower extremity hyperreflexity and hypertonicity. Magnetic resonance imaging (MRI) demonstrated 2 contrast-enhancing intradural extramedullary cervical spine lesions causing spinal cord compression at C4 and C5. The patient underwent a posterior cervical laminoplasty with a midline dural opening for tumor resection. Curvilinear spine cord compression is demonstrated in the operative video. After meticulous dissection, the tumors were resected without complication. The dural closure was performed in watertight fashion followed by laminoplasty using osteoplastic titanium miniplates and screws. Postoperative MRI demonstrated gross total resection with excellent decompression of the spinal cord. The postoperative course was uneventful. The natural history of this disease, treatment options, and potential complications are discussed.

scholarly journals NEUROFIBROMATOSIS TYPE 2: A REVIEW OF PAIN MANAGEMENT OPTIONS

2019 ◽  
pp. 63-68
Author(s):  
Devang Padalia
Keyword(s):  
Chronic Pain ◽  
Pain Management ◽  
Treatment Options ◽  
Disease Process ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
Successful Outcome ◽  
Management Options ◽  
Novel Treatment

Neurofibromatosis is a chronic and painful disease process that can cause significant morbidity. The development of central and peripheral lesions is the primary source of acute and chronic pain. The pain associated with this condition is difficult to treat, but there are a number of options to make the lives of these patients more comfortable. A review of current literature and research data was used to compile a detailed list of treatment options for patients with neurofibromatosis type 2 (NF2). Key points on physiology and pain, as well as current and potential treatment options, are discussed. There are a number of articles, case reports, and studies regarding the treatment options for pain in patients with NF2. However, there is a lack of well-controlled randomized trials that demonstrate the superiority of one treatment modality. There are novel treatment plans being tailored to work at a genetic level, but these still require further research before implementing in daily practice. The management of chronic pain in NF2 is a difficult challenge for the medical community. Novel treatment options are currently being researched to improve the quality of life in these patients. Using a multimodal approach to pain control will improve the chances of a successful outcome. Key words: Neurofibromatosis type 2, schwannomatosis, neurofibromatosis type 1, chronic pain, pain management, neurofibroma, analgesics

Facial Nerve Outcomes After Vestibular Schwannoma Microsurgical Resection in Neurofibromatosis Type 2

2020 ◽  
pp. 019459982095414
Author(s):  
Catherine Sobieski ◽  
Daniel E. Killeen ◽  
Samuel L. Barnett ◽  
Bruce E. Mickey ◽  
Jacob B. Hunter ◽  
...  
Keyword(s):  
Facial Nerve ◽  
Vestibular Schwannoma ◽  
Tumor Volume ◽  
Tumor Resection ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
Tumor Diameter ◽  
Microsurgical Resection

Objective The aim of this study is to investigate facial nerve outcomes after microsurgical resection in neurofibromatosis type 2 (NF2) compared to sporadic tumors. Study Design Single institutional retrospective chart review. Setting Tertiary referral center. Methods All adult patients with NF2 vestibular schwannoma (VS) or sporadic VS who underwent microsurgical resection from 2008 to 2019 with preoperative magnetic resonance imaging (MRI) and 1 year of postsurgical follow-up were included. The primary outcome measure was postoperative House-Brackmann (HB) facial nerve score measured at first postoperative visit and after at least 10 months. Results In total, 161 sporadic VSs and 14 NF2 VSs met inclusion criteria. Both median tumor diameter (NF2, 33.5 mm vs sporadic, 24 mm, P = .0011) and median tumor volume (NF2, 12.4 cm3 vs sporadic, 2.9 cm3, P = .0005) were significantly greater in patients with NF2. The median follow-up was 24.9 months (range, 12-130.1). Median facial nerve function after 1 year for patients with NF2 was HB 3 (range, 1-6) compared to HB 1 (range, 1-6) for sporadic VS ( P = .001). With multivariate logistic regression, NF2 tumors (odds ratio [OR] = 13.9, P = .001) and tumor volume ≥3 cm3 (OR = 3.6, P = .025) were significantly associated with HB ≥3 when controlling for age, sex, extent of tumor resection, translabyrinthine approach, and prior radiation. Conclusion Tumor volume >3 cm3 and NF2 tumors are associated with poorer facial nerve outcomes 1 year following microsurgical resection.

Resection of a Lumbar Intradural Extramedullary Schwannoma: 3-Dimensional Operative Video

2018 ◽  
Vol 16 (5) ◽  
pp. 640-640 ◽  
Author(s):  
Simone E Dekker ◽  
Chad A Glenn ◽  
Thomas A Ostergard ◽  
Mickey L Smith ◽  
Brian D Rothstein ◽  
...  
Keyword(s):  
Back Pain ◽  
Treatment Options ◽  
Tumor Resection ◽  
Sensory Nerve ◽  
En Bloc ◽  
3 Dimensional ◽  
Postoperative Mri ◽  
Intradural Extramedullary ◽  
Magnetic Resonance Imaging Mri ◽  
Dural Opening

Abstract This 3-dimensional operative video illustrates resection of a lumbar schwannoma in a 57-yr-old female who presented with right lower extremity numbness, paresthesias, as well as a long history of lower back pain with rest. On magnetic resonance imaging (MRI), there was evidence of an intradural extramedullary enhancing lesion at L5, nearly completely encompassing the spinal canal. This video demonstrates the natural history, treatment options, surgical procedure, risks, and complications of treatment of these types of tumors. The patient underwent a posterior lumbar laminectomy with a midline dural opening for tumor resection. The tumor was encountered intradurally and electromyography recording confirmed that the tumor arose from a lumbar sensory nerve root. The sensory root was then divided and the tumor was then removed. The mass was removed en bloc and histopathologic analysis was consistent with a schwannoma. Postoperative MRI demonstrated gross total resection of the patient's neoplasm with excellent decompression of the spinal cord. The patient had an uneventful postoperative course with full recovery and complete resolution of her back pain and leg paresthesias.

scholarly journals The response of spinal cord ependymomas to bevacizumab in patients with neurofibromatosis Type 2

2017 ◽  
Vol 26 (4) ◽  
pp. 474-482 ◽  
Author(s):  
Katrina A. Morris ◽  
Shazia K. Afridi ◽  
D. Gareth Evans ◽  
Anke E. Hensiek ◽  
Martin G. McCabe ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
Response To Treatment ◽  
Spinal Ependymoma ◽  
Bevacizumab Treatment ◽  
Radiological Changes ◽  
And Behavior ◽  
Serial Images

OBJECTIVE People with neurofibromatosis Type 2 (NF2) have a genetic predisposition to nervous system tumors. NF2-associated schwannomas stabilize or decrease in size in over half of the patients while they are receiving bevacizumab. NF2 patients treated with bevacizumab for rapidly growing schwannoma were retrospectively reviewed with regard to ependymoma prevalence and response to treatment. METHODS The records of 95 NF2 patients receiving bevacizumab were retrospectively reviewed with regard to spinal ependymoma prevalence and behavior. The maximum longitudinal extent (MLE) of the ependymoma and associated intratumoral or juxtatumoral cysts were measured on serial images. Neurological changes and patient function were reviewed and correlated with radiological changes. RESULTS Forty-one of 95 patients were found to have ependymomas (median age 26 years; range 11–53 years). Thirty-two patients with a total of 71 ependymomas had scans appropriate for serial assessment with a mean follow-up of 24 months (range 3–57 months). Ependymomas without cystic components showed minimal change in MLE. Twelve patients had ependymomas with cystic components or syringes. In these patients, reductions in MLE were observed, particularly due to decreases in the cystic components of the ependymoma. Clinical improvement was seen in 7 patients, who all had cystic ependymomas. CONCLUSIONS Bevacizumab treatment in NF2 patients with spinal cord ependymomas results in a decrease in the size of intratumoral and juxtatumoral cysts as well as adjacent-cord syringes and a decrease in cord edema. This may provide clinical benefit in some patients, although the changes do not meet the current criteria for radiological tumor response.

Spinal cord tanycytic ependymoma associated with neurofibromatosis type 2

2010 ◽  
Vol 17 (7) ◽  
pp. 922-924 ◽  
Author(s):  
Bong-Suk Lim ◽  
Sukh Que Park ◽  
Ung Kyu Chang ◽  
Min Suk Kim
Keyword(s):  
Spinal Cord ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
Tanycytic Ependymoma

Spinal cord ganglioglioma in a child with neurofibromatosis Type 2

1999 ◽  
Vol 90 (2) ◽  
pp. 231-233 ◽  
Author(s):  
Paul D. Sawin ◽  
Nicholas Theodore ◽  
Harold L. Rekate
Keyword(s):  
Spinal Cord ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
Histopathological Analysis ◽  
Unique Case ◽  
Content Type ◽  
Complete Surgical Resection ◽  
Disease Entities ◽  
Glial Lineage

✓ Gangliogliomas of the spinal cord are rare disease entities that occur in early childhood. Their occurrence in association with neurofibromatosis Type 2 (NF2) has not been described. The authors describe the unique case of a 2-year-old child with stigmata of NF2 who harbored a spinal cord ganglioglioma that presented as a rapidly growing, exophytic intramedullary mass lesion at the cervicomedullary junction. Treatment consisted of complete surgical resection. Histopathological analysis of the lesion demonstrated a mixed population of neoplastic cells, of both neuronal and glial lineage, that supported the diagnosis of ganglioglioma.

scholarly journals New Developments in Neurofibromatosis Type 2 and Vestibular Schwannoma

2020 ◽  
Author(s):  
Yin Ren ◽  
Divya A Chari ◽  
Sasa Vasilijic ◽  
D Bradley Welling ◽  
Konstantina M Stankovic
Keyword(s):  
Treatment Options ◽  
Neurofibromatosis Type ◽  
Suppressor Gene ◽  
Severe Form ◽  
Neurofibromatosis Type 2 ◽  
Future Research ◽  
Autosomal Dominant Disorder ◽  
Management Options ◽  
Stereotactic Radiation

Abstract Neurofibromatosis type 2 (NF2) is a rare autosomal dominant disorder characterized by the development of multiple nervous system tumors due to mutation in the NF2 tumor suppressor gene. The hallmark feature of the NF2 syndrome is the development of bilateral vestibular schwannomas (VS). Although there is nearly 100% penetrance by 60 years of age, some patients suffer from a severe form of the disease and develop multiple tumors at an early age, while others are asymptomatic until later in life. Management options for VS include surgery, stereotactic radiation, and observation with serial imaging; however, currently there are no FDA-approved pharmacotherapies for NF2 or VS. Recent advancements in the molecular biology underlying NF2 have led to a better understanding of the etiology and pathogenesis of VS. These novel signaling pathways may be used to identify targeted therapies for these tumors. This review discusses the clinical features and treatment options for sporadic- and NF2-associated VS, the diagnostic and screening criteria, completed and ongoing clinical trials, quality of life metrics, and opportunities for future research.

scholarly journals Neurofibromatosis Type 2 Involving the Brain and Spinal Cord

2021 ◽  
Vol 102 (2) ◽  
pp. 98-108
Author(s):  
O. A. Staroseltseva ◽  
N. V. Nudnov ◽  
M. L. Radutnaya ◽  
I. G. Shchelkunova ◽  
A. A. Yakovlev ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
Brain And Spinal Cord ◽  
The Brain

Use of Intraoperative CO2 Laser for the Resection of a Ventral Intradural Extramedullary Cervical Spinal Tumor: 2-Dimensional Operative Video

2019 ◽  
Vol 18 (5) ◽  
pp. E161-E161
Author(s):  
Joseph A Osorio ◽  
Guillermo Victorino T Liabres ◽  
Catherine A Miller ◽  
Michael W McDermott ◽  
Praveen V Mummaneni
Keyword(s):  
Spinal Cord ◽  
Co2 Laser ◽  
Tumor Resection ◽  
Spinal Tumor ◽  
Cord Compression ◽  
Spinal Tumors ◽  
Laser Technique ◽  
Who Grade ◽  
Patient Consent ◽  
Intradural Extramedullary

Abstract Ventral spinal tumors are surgically challenging because the tumor resection should minimize spinal cord and nerve root manipulation to minimize morbidity, while providing access to a complete tumor resection. The CO2 laser has been useful in resection of central nervous system tumors, but little is described about the method used to resect spinal tumors.1 This video demonstrates the removal of a ventral cervical spinal meningioma using the CO2 laser. A 62-yr-old man presented with progressive paresthesias, gait instability, and urinary frequency. A 1-cm intradural extramedullary mass at C5 showed severe spinal cord compression. Patient consent was obtained prior to performing the procedure. A posterior lateral approach is shown, with a bone removal corridor created at C5 for accessing the tumor ventrally. A right-sided facetectomy and pediculectomy at C5 were performed being flush with the posterior vertebral body. A dural opening positioned laterally provided a working corridor between C5 and C6 nerve rootlets. Lateral portions of tumor were excised in wedge-shaped slices starting laterally and working medially. These slices created a successive and enlarging space to safely allow piecemeal tumor dissection and removal, while limiting retraction upon the spinal cord. The CO2 laser was used to cauterize the tumor capsule, create wedge resections of tumor, and coagulate the final dural attachment. The pathology was a meningioma WHO Grade I. The patient did well, with resolution of parasthesias and ataxia. The CO2 laser technique allowed for limited spinal cord retraction throughout the tumor resection and gross total resection of the tumor was achieved.

Sign in / Sign up

Export Citation Format

Share Document