scholarly journals Neurological outcomes and surgical complications in 221 spinal nerve sheath tumors

2017 ◽  
Vol 26 (1) ◽  
pp. 103-111 ◽  
Author(s):  
Michael M. Safaee ◽  
Russ Lyon ◽  
Nicholas M. Barbaro ◽  
Dean Chou ◽  
Praveen V. Mummaneni ◽  
...  
Keyword(s):  
Surgical Complications ◽  
Spinal Nerve ◽  
Intraoperative Neuromonitoring ◽  
Complication Rates ◽  
Nerve Sheath Tumor ◽  
Tumor Type ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath ◽  
Csf Leakage

OBJECTIVE Among all primary spinal neoplasms, approximately two-thirds are intradural extramedullary lesions; nerve sheath tumors, mainly neurofibromas and schwannomas, comprise approximately half of them. Given the rarity of these lesions, reports of surgical complications are limited. The aim of this study was to identify the rates of new or worsening neurological deficits and surgical complications associated with the resection of spinal nerve sheath tumors and the potential factors related to these outcomes. METHODS Patients were identified through a search of an institutional neuropathology database and a separate review of current procedural terminology (CPT) codes. Age, sex, clinical presentation, presence of neurofibromatosis (NF), tumor type, tumor location, extent of resection characterized as gross total or subtotal, use of intraoperative neuromonitoring, surgical complications, presence of neurological deficit, and clinical follow-up were recorded. RESULTS Two hundred twenty-one tumors in 199 patients with a mean age of 45 years were identified. Fifty-three tumors were neurofibromas; 163, schwannomas; and 5, malignant peripheral nerve sheath tumors (MPNSTs). There were 70 complications in 221 cases, a rate of 32%, which included 34 new or worsening sensory symptoms (15%), 12 new or worsening motor deficits (5%), 10 CSF leaks or pseudomeningoceles (4%), 11 wound infections (5%), 5 cases of spinal deformity (2%), and 6 others (2 spinal epidural hematomas, 1 nonoperative cranial subdural hematoma, 1 deep venous thrombosis, 1 case of urinary retention, and 1 recurrent laryngeal nerve injury). Complications were more common in cervical (36%) and lumbosacral (38%) tumors than in thoracic (18%) lesions (p = 0.021). Intradural and dumbbell lesions were associated with higher rates of CSF leakage, pseudomeningocele, and wound infection. Complications were present in 18 neurofibromas (34%), 50 schwannomas (31%), and 2 MPNSTs (40%); the differences in frequency were not significant (p = 0.834). Higher complication rates were observed in patients with NF than in patients without (38% vs 30%, p = 0.189), although rates were higher in NF Type 2 than in Type 1 (64% vs 31%). There was no difference in the use of intraoperative neuromonitoring when comparing cases with surgical complications and those without (67% vs 69%, p = 0.797). However, the use of neuromonitoring was associated with a significantly higher rate of gross-total resection (79% vs 66%, p = 0.022). CONCLUSIONS Resection is a safe and effective treatment for spinal nerve sheath tumors. Approximately 30% of patients developed a postoperative complication, most commonly new or worsening sensory deficits. This rate probably represents an inevitable complication of nerve sheath tumor surgery given the intimacy of these lesions with functional neural elements.

scholarly journals Association of tumor location, extent of resection, and neurofibromatosis status with clinical outcomes for 221 spinal nerve sheath tumors

2015 ◽  
Vol 39 (2) ◽  
pp. E5 ◽  
Author(s):  
Michael Safaee ◽  
Andrew T. Parsa ◽  
Nicholas M. Barbaro ◽  
Dean Chou ◽  
Praveen V. Mummaneni ◽  
...  
Keyword(s):  
Clinical Outcomes ◽  
Extent Of Resection ◽  
Tumor Location ◽  
Spinal Nerve ◽  
Tumor Type ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath ◽  
The Mean

OBJECT Intradural extramedullary spine tumors represent two-thirds of all primary spine neoplasms. Approximately half of these are peripheral nerve sheath tumors, mainly neurofibromas and schwannomas. Given the rarity of this disease and, thus, the limited analyses of clinical outcomes, the authors examined the association of tumor location, extent of resection, and neurofibromatosis (NF) status with clinical outcomes. METHODS Patients were identified through a search of the University of California, San Francisco, neuropathology database and a separate review of current procedural terminology codes. Data recorded included patient age, patient sex, clinical presentation, presence of NF, tumor type, tumor location, extent of resection (gross-total resection [GTR] or subtotal resection [STR]), and clinical follow-up. RESULTS Of 221 tumors in 199 patients (mean age 45 years), 53 were neurofibromas, 163 were schwannomas, and 5 were malignant peripheral nerve sheath tumors. The most common presenting symptom was spinal pain (76%), followed by weakness (36%) and sensory abnormalities (34%). Mean symptom duration was 16 months. In terms of spinal location, neurofibromas were more common in the cervical spine (74% vs 27%, p < 0.001), and schwannomas were more common in the thoracic and lumbosacral spine (73% vs 26%, p < 0.001). Rates of GTR were lower for neurofibromas than schwannomas (51% vs 83%, p < 0.001), regardless of location. Rates of GTR were lower for cervical (54%) than thoracic (90%) and lumbosacral (86%) lesions (p < 0.001). NF was associated with lower rates of GTR among all tumors (43% vs 86%, p < 0.001). The mean follow-up time was 32 months. Recurrence/progression was more common for neurofibromas than schwannomas (17% vs 7%, p = 0.03), although the mean time to recurrence/progression did not differ according to tumor type (45 vs 53 months, p = 0.63). As expected, GTR was associated with lower recurrence rates (4% vs 22%, p < 0.001). According to multivariate analysis, cervical location (OR 0.239, 95% CI 0.110–0.520) and presence of NF (OR 0.166, 95% CI 0.054–0.507) were associated with lower rates of GTR. In a separate model, only GTR (OR 0.141, 95% CI 0.046–0.429) was associated with tumor recurrence. CONCLUSIONS Resection is an effective treatment for spinal nerve sheath tumors. Neurofibromas were found more commonly in the cervical spine than in other regions of the spine and were associated with higher rates of recurrence and lower rates of GTR than other tumor types, particularly in patients with NF Types 1 or 2. According to multivariate analysis, both cervical location and presence of NF were associated with lower rates of GTR. According to a second multivariate model, the only variable associated with tumor recurrence was extent of resection. Maximal safe resection remains ideal for these lesions; however, patients with cervical tumors or NF should be counseled about their increased risk for recurrence.

FORAMINAL NERVE SHEATH TUMORS

Neurosurgery ◽  
2009 ◽  
Vol 64 (suppl_2) ◽  
pp. A33-A43 ◽  
Author(s):  
Judith A. Murovic ◽  
Iris C. Gibbs ◽  
Steven D. Chang ◽  
Bret C. Mobley ◽  
Jon Park ◽  
...  
Keyword(s):  
Peripheral Nerve ◽  
Medical Center ◽  
Nerve Sheath Tumor ◽  
Peripheral Nerve Sheath Tumor ◽  
Central Necrosis ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Neurological Findings ◽  
Nerve Sheath ◽  
Asymptomatic Case

Abstract OBJECTIVE To conduct a retrospective review of outcomes in 15 patients with 18 foraminal tumors, including 17 benign peripheral nerve sheath tumors and 1 malignant peripheral nerve sheath tumor, who underwent CyberKnife (Accuray, Inc., Sunnyvale, CA) radiosurgery at Stanford University Medical Center from 1999 to 2006. METHODS Symptoms and findings, neurofibromatosis (NF) association, previous radiation, imaging, dosimetry, tumor volume, central necrosis, and the relation of these factors to outcomes were evaluated. RESULTS Before treatment, 1 asymptomatic patient had radiculopathic findings, 3 patients experienced local pain with intact neurological examinations, and 7 patients had radiculopathic complaints with intact (1 patient), radiculopathic (4 patients), or radiculomyelopathic examinations (2 patients). Five patients had myelopathic complaints and findings. Three patients had NF1-associated neurofibromas, 1 patient with NF2 had a schwannoma, and 1 patient had a schwannomatosis-related lesion. Two likely radiation-induced lesions, a neurofibroma and a malignant peripheral nerve sheath tumor, were observed. Prescribed doses ranging from 16 to 24 Gy, delivered in 1 to 3 fractions of 6 to 20 Gy, resulted in maximum tumor doses ranging from 20.9 to 30 Gy. Target volumes ranged from 1.36 to 16.9 mL. After radiosurgery, the asymptomatic case remained asymptomatic, and neurological findings improved. Thirteen of 15 symptomatic patients with (12 patients) or without (3 patients) neurological findings improved (3 cases after resection) or remained stable, and 2 patients worsened. Symptoms and examinations remained stable or improved in 8 (80%) of 10 patients with schwannomas and 3 (60%) of 5 patients with neurofibromas. Tumor volumes decreased in 12 (67%) of 18 tumors and increased in 3 tumors. Tumor volumes decreased in 8 of 10 schwannomas and 3 of 7 neurofibromas. Central necrosis developed in 8 (44%) of 18 tumors. CONCLUSION CyberKnife radiosurgery resulted in pain relief and functional preservation in selected foraminal peripheral nerve sheath tumors and a malignant peripheral nerve sheath tumor. Symptomatic and neurological improvements were more noticeable with schwannomas. Myelopathic symptoms may necessitate surgical debulking before radiosurgery.

scholarly journals Retrospective canine skin peripheral nerve sheath tumors data with emphasis on histologic, immunohistochemical and prognostic factors

2015 ◽  
Vol 35 (12) ◽  
pp. 965-974 ◽  
Author(s):  
Gisele S. Boos ◽  
Daniele M. Bassuino ◽  
Fabiana Wurster ◽  
Neusa B. Castro ◽  
Tatiane T.N. Watanabe ◽  
...  
Keyword(s):  
Peripheral Nerve ◽  
Protein S ◽  
Skin Neoplasms ◽  
Tumor Location ◽  
Benign Tumors ◽  
Nerve Sheath Tumor ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath ◽  
S 100

Abstract: In this retrospective study was determined the frequency of canine skin peripheral nerve sheath tumors (PNST) in cases diagnosed by the Setor de Patologia Veterinária of the Universidade Federal do Rio Grande do Sul (SPV-UFRGS), Brazil, between the years 2000 and 2012. The canine profiles, as well as histological, immunohistochemical and prognostic aspects of the tumors were based on 70 samples, comprising 40 females, 29 males and one unspecified sample. Between 2000 and 2012, 2,984 skin tumors of dogs were diagnosed in the SPV-UFRGS, totaling 2.34% of skin neoplasms in dogs. Animals that comprised the largest amount of samples (43%) were those with no breed (SRD), followed by German Shepherds (10%). Females were more affected than males (40/70 - 57% and 29/70 - 41% respectively). Skin PNST of this research showed predominant localization on the limbs (40% in the forelimbs and 29% in the hindlimbs); affecting adult dogs, mostly aged between 8 and 11 years (54%). The samples were routinely processed for hematoxylin and eosin, and were also evaluated by toluidine blue and Masson's trichrome staining, and immunohistochemistry (IHC) anti-vimentin, -S-100, -GFAP, -actin, von Willebrand factor and neurofilament. Anisocytosis and anisokaryosis, mitotic index, intratumoral necrosis, invasion of adjacent tissues, tumor location, local recurrence and metastasis were related to the diagnosis of benign (49/70) or malignant tumor (21/70). The Antoni A histological pattern was observed more frequently in benign tumors. The immunohistochemistry helped to diagnose PNST, and anti-vimentin and anti-protein S-100 showed the highest rates of immunostaining. Throughout statistical analysis of animals with tumor recurrence, it was found that the chance of an animal with a malignant peripheral nerve sheath tumor to develop recurrence is 4.61 times higher than in an animal that had a benign tumor.

Primary malignant peripheral nerve sheath tumor of the spine with acute hydrocephalus: a rare clinical entity

2014 ◽  
Vol 21 (3) ◽  
pp. 367-371 ◽  
Author(s):  
Yaxiong Li ◽  
Fengshi Fan ◽  
Jianguo Xu ◽  
Jie An ◽  
Weining Zhang
Keyword(s):  
Peripheral Nerve ◽  
English Language ◽  
Standard Of Care ◽  
Nerve Sheath Tumor ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath ◽  
History Of ◽  
Brain Ct Scan ◽  
The Right

Primary malignant peripheral nerve sheath tumors (MPNSTs) are extremely rare in patients without a history of neurofibromatosis; only 18 cases have been reported in the English-language literature to this point. The authors report their experience with 1 new case of a primary MPNST. A 33-year-old woman presented with low-back pain radiating to the right calf that progressed over 1 year. Magnetic resonance imaging of the spine revealed an intradural extramedullary lesion at the T12–L1 level. The patient was diagnosed with primary MPNST, underwent two surgical excisions and radiation therapy, and developed leptomeningeal metastases as well as brain metastases. The patient revisited the emergency room with sudden loss of consciousness. A brain CT scan displayed bilateral lateral ventricle enlargement, for which a ventriculoperitoneal shunt was inserted. These symptoms have not been described in any previous report. Primary spinal MPNST is an exceedingly rare entity, and the overall prognosis is very poor. To the authors' knowledge, no standard of care for primary spinal MPNSTs has yet been established. All 19 cases of primary spinal MPNSTs are reviewed, and the authors discuss their clinical, radiological, and therapeutic features and outcomes.

Dumbbell Nerve Sheath Tumors

2018 ◽  
Author(s):  
Hussam Abou-Al-Shaar ◽  
Mark A. Mahan
Keyword(s):  
Imaging Modality ◽  
Spinal Nerve ◽  
Surgical Approaches ◽  
Nerve Sheath Tumor ◽  
Imaging Features ◽  
Neural Foramen ◽  
Dumbbell Tumor ◽  
Nerve Sheath Tumors ◽  
Nerve Sheath ◽  
The Individual

A dumbbell tumor is a nerve sheath tumor that arises from a spinal nerve in the neural foramen and grows as a dumbbell-shaped mass. The differential diagnosis for a dumbbell tumor includes schwannoma, neurofibroma, malignant peripheral nerve sheath tumor, and metastases, among others. MR imaging is considered the gold-standard imaging modality for diagnosis of dumbbell tumors. Surgical approaches that are tailored to the individual patient’s case can be utilized. The chapter reviews dumbbell tumors, including a case example and covers the incidence, clinical presentation, imaging features, decision-making strategy, surgical approaches, outcomes, and potential complications associated with their management.

Nerve Sheath Tumors—Schwannomas and Neurofibromas

2018 ◽  
Author(s):  
Christian Heinen ◽  
Thomas Kretschmer
Keyword(s):  
Peripheral Nerve ◽  
Nerve Sheath Tumor ◽  
Surgical Strategies ◽  
Treatment Timing ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Tumor ◽  
Nerve Sheath ◽  
Contrast Enhanced

A benign peripheral nerve sheath tumor is illustrated in a case presentation of a painful mass in the medial thigh, with paresthesias radiating along the course of the saphenous nerve. The presenting features, appropriate workup, treatment timing, surgical strategies, follow-up, and results for nerve-associated masses are outlined. Specific imaging findings for peripheral nerve sheath tumors on contrast-enhanced MR imaging and the merits of high-resolution ultrasound are detailed. The typical features of a well-defined and noninvasive peripheral nerve tumor, the principles of exploration, and microsurgical enucleation technique are highlighted. Other nerve tumor entities that should be considered in the differential diagnosis, as well as their respective features, are discussed.

scholarly journals Somatic Mutations of lats2 Cause Peripheral Nerve Sheath Tumors in Zebrafish

Cells ◽  
2019 ◽  
Vol 8 (9) ◽  
pp. 972 ◽  
Author(s):  
Zachary J. Brandt ◽  
Paula N. North ◽  
Brian A. Link
Keyword(s):  
Peripheral Nerve ◽  
Somatic Mutations ◽  
Tumor Formation ◽  
Hippo Signaling ◽  
Nerve Sheath Tumor ◽  
Peripheral Nerve Sheath Tumor ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath ◽  
Early Lethality

The cellular signaling pathways underlying peripheral nerve sheath tumor (PNST) formation are poorly understood. Hippo signaling has been recently implicated in the biology of various cancers, and is thought to function downstream of mutations in the known PNST driver, NF2. Utilizing CRISPR-Cas9 gene editing, we targeted the canonical Hippo signaling kinase Lats2. We show that, while germline deletion leads to early lethality, targeted somatic mutations of zebrafish lats2 leads to peripheral nerve sheath tumor formation. These peripheral nerve sheath tumors exhibit high levels of Hippo effectors Yap and Taz, suggesting that dysregulation of these transcriptional co-factors drives PNST formation in this model. These data indicate that somatic lats2 deletion in zebrafish can serve as a powerful experimental platform to probe the mechanisms of PNST formation and progression.

Primary pediatric intraspinal sarcomas

2009 ◽  
Vol 4 (3) ◽  
pp. 222-229 ◽  
Author(s):  
Paul Klimo ◽  
Patrick J. Codd ◽  
Holcombe Grier ◽  
Liliana C. Goumnerova
Keyword(s):  
Peripheral Nerve ◽  
Ewing Sarcoma ◽  
Pediatric Population ◽  
Spinal Nerve ◽  
Neurological Deficits ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath ◽  
Lumbar Spinal ◽  
Extraosseous Ewing Sarcoma

Sarcomas that arise from within the spinal canal are rare, particularly within the pediatric population. In general, these primary intraspinal sarcomas are highly aggressive, posing unique treatment challenges with respect to surgery and choice of adjuvant therapy. The goal must be to obtain the most complete resection possible to minimize the risk of recurrence and metastasis, while preventing potential neurological deficits that may result from aggressive surgery. Among these primary intraspinal sarcomas are malignant peripheral nerve sheath tumors and members of the Ewing sarcoma family of tumors. The authors present 3 cases of unique spinal sarcomas in children—2 malignant peripheral nerve sheath tumors in patients without neurofibromatosis and an intradural extraosseous Ewing sarcoma arising from the sensory component of a lumbar spinal nerve—and discuss their management and outcome with a review of the current literature.

Nerve Sheath Tumors: Malignant peripheral nerve sheath tumors, Schwannomas, and Neurofibromas

2019 ◽  
Author(s):  
Keila E Torres ◽  
Wei-Lien Wang ◽  
Alexander Lazar ◽  
Ian E McCutcheon
Keyword(s):  
Molecular Biology ◽  
Peripheral Nerve ◽  
Malignant Tumors ◽  
Therapeutic Strategies ◽  
Benign Tumors ◽  
Nerve Sheath Tumor ◽  
High Grade ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath

Peripheral nerve sheath tumors represent a spectrum of entities ranging from benign tumors, such as schwannoma, to high-grade malignant tumors termed malignant peripheral nerve sheath tumors. In this chapter, we discuss current concepts and problematic areas in the diagnosis, treatment, and management of peripheral nerve sheath tumors. We discuss the major categories of nerve sheath tumors including schwannomas, neurofibromas, and malignant peripheral nerve sheath tumors. The different benign variants and their propensity for malignant transformation are highlighted. We suggest useful guidelines for the surgical management of these challenging cases. Finally, we discuss some of the advances in molecular biology that have provided new insights into the nature of the various peripheral nerve sheath tumors and suggest potential novel targeted therapeutic strategies for these tumors. This review contains 11 figures, 5 tables and 66 references. Key Words: malignant peripheral nerve sheath tumors, nerve sheath tumor, neurofibroma, neurofibromatosis, schwannoma, schwannomatosis, NF1, NF2

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