Dumbbell Nerve Sheath Tumors

Author(s):  
Hussam Abou-Al-Shaar ◽  
Mark A. Mahan

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.

Neurosurgery ◽  
2005 ◽  
Vol 56 (3) ◽  
pp. 510-515 ◽  
Author(s):  
Takahiro Jinnai ◽  
Minoru Hoshimaru ◽  
Tsunemaro Koyama

Abstract OBJECTIVE: Spinal nerve sheath tumors arise from the spinal nerve root and grow along it. There are two sites at which the growth of a tumor is restricted: the dural aperture for the spinal nerve root and the intervertebral foramen. This article describes the growth pattern of a spinal nerve sheath tumor along the spinal nerve root at various spinal levels. METHODS: We retrospectively reviewed the records for 149 patients with spinal nerve sheath tumors who were treated between 1980 and 2001. Of these, 176 resected tumors were classified into five groups according to the relationship to the dura mater and/or the intervertebral foramen. RESULTS: Strictly intradural tumors compose 8% of nerve sheath tumors of the first two cervical nerve roots. The percentage of these tumors increased gradually from the high cervical region to the thoracolumbar region, where it was more than 80%. In contrast, the percentage of strictly extradural tumors gradually decreased from the rostral portion to the caudal portion. Similarly, a percentage of tumors extending outside the spinal canal decreased from the rostral portion to the caudal portion. These changes of the growth pattern may be explained by the anatomic features of the spinal nerve roots, which have a longer intradural component at the more caudal portion of the spinal axis. CONCLUSION: The anatomic relationship of a nerve sheath tumor with the dura mater and the intervertebral foramen varies depending on the level of the tumor. This knowledge may help us to create a strategy for total resection of a nerve sheath tumor.


2019 ◽  
Vol 2019 ◽  
pp. 1-4
Author(s):  
Kofi Effah ◽  
Stefan Seidl ◽  
Edith Gorges ◽  
Patrick Kafui Akakpo

Melanotic schwannoma (MS) is a rare nerve sheath tumor with fewer than 200 cases reported. MS has uncertain malignant potential and comprises 1% of all nerve sheath tumors with a predilection for the spinal nerve roots. An even rarer location for this tumor is the vagina. Up to 55% of MSs that contain psammoma bodies are associated with the Carney complex, an autosomal dominant syndrome. Criteria for malignancy in MS are still not well established and long term follow-up of patients is recommended. A 26-year-old woman presented with a bleeding vaginal tumor which was diagnosed as MS following excision. The clinical, histopathological, and immunohistochemical features of this tumor are discussed.


2005 ◽  
Vol 3 (1) ◽  
pp. 1-11 ◽  
Author(s):  
Paolo Celli ◽  
Giuseppe Trillò ◽  
Luigi Ferrante

Object. The purpose of this study was to analyze the clinical profile of patients harboring extrathecal and intraradicular nerve sheath tumors (NSTs), located inside the sleeve of an extrathecal nerve root and very often within the proximal portion of the spinal nerve, and to evaluate the incidence of long-term dysfunction of the tumor-affected roots if resected. These tumors have not received particular attention in the literature. Methods. A single-institution series of 16 patients who had undergone surgery for intraradicular NSTs during a 50-year period was selected retrospectively. Data pertaining to clinical features, tumor characteristics, and results of surgery were analyzed. Conclusions. Extrathecal and intraradicular neurofibromas or schwannomas more frequently affect the lumbar and S-1 nerve roots, often producing root pain only. Selective en bloc enucleation sparing at least part of the motor rootlets is possible for small schwannomas of the extrathecal—preganglion segment of the radix, whereas total resection of the affected root is generally required for radical removal of neurofibromas and large schwannomas. In the authors' experience, neither deafferentation pain nor severe radicular weakness occurs after division of the nerve root harboring the tumor.


2017 ◽  
Vol 26 (1) ◽  
pp. 103-111 ◽  
Author(s):  
Michael M. Safaee ◽  
Russ Lyon ◽  
Nicholas M. Barbaro ◽  
Dean Chou ◽  
Praveen V. Mummaneni ◽  
...  

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.


Neurosurgery ◽  
2004 ◽  
Vol 54 (2) ◽  
pp. 500-504 ◽  
Author(s):  
Patrick Beauchesne ◽  
Jean-François Mosnier ◽  
Thierry Schmitt ◽  
Jacques Brunon

Abstract OBJECTIVE AND IMPORTANCE Schwannomas occurring in the neuraxis are very rare. Usually, these tumors are benign. Primary malignant intracerebral nerve sheath tumors are extremely rare, with only five documented cases in the international literature. We report one case of a primary malignant intracerebral nerve sheath tumor occurring in the right cerebral peduncle of a 35-year-old man. CLINICAL PRESENTATION Magnetic resonance imaging revealed a heterogeneous peripherally enhancing mass of the right cerebral peduncle, surrounded by a small edema. INTERVENTION Unlike the five cases previously reported, this is the first time a stereotactic biopsy has been performed, and this is the only patient who responded to cranial radiation therapy for approximately 2 years. When the tumor recurred, a systemic chemotherapy treatment was prescribed. No positive response was seen, and the patient died 29 months after the initial diagnosis. CONCLUSION An accurate diagnosis and planned aggressive treatment seem to be the key elements in the management of the disease.


2014 ◽  
Vol 72 (4) ◽  
pp. 312-317 ◽  
Author(s):  
Rudi Lenck Fernandes ◽  
José Carlos Lynch ◽  
Leonardo Welling ◽  
Mariangela Gonçalves ◽  
Rodrigo Tragante ◽  
...  

Objective: Observe whether a microsurgical gross total removal (GTR) of a spinal nerve sheath tumors (SNSTs) is safe and decreases the tumor recurrence. Method: We identify 30 patients with 44 SNSTs. Results: We operated upon 15 males and 15 females patients; mean age 40 years. GTR was achieved in 29 (96.6%) instances. Surgical mortality was 3.3% and the recurrence rate was 3.3%. The median follow-up time was 6.2 years. Conclusion: The surgical approach used in this group of patients afford that the great majority of tumors could be totally removed with low mortality and low recurrence rates, proving to be safe and effective.


2003 ◽  
Vol 15 (2) ◽  
pp. 1-6 ◽  
Author(s):  
Paul Klimo ◽  
Ganesh Rao ◽  
Richard H. Schmidt ◽  
Meic H. Schmidt

Nerve sheath tumors that involve the sacrum are rare. Delayed presentation is common because of their slow-growing nature, the permissive surrounding anatomical environment, and nonspecific symptoms. Consequently, these tumors are usually of considerable size at the time of diagnosis. The authors discuss a case of a sacral nerve sheath tumor. They also propose a classification scheme for these tumors based on their location with respect to the sacrum into three types (Types I–III). Type I tumors are confined to the sacrum; Type II originate within the sacrum but then locally metastasize through the anterior and posterior sacral walls into the presacral and subcutaneous spaces, respectively; and Type III are located primarily in the presacral/retroperitoneal area. The overwhelming majority of sacral nerve sheath tumors are schwannomas. Neurofibromas and malignant nerve sheath tumors are exceedingly rare. Regardless of their histological features, the goal of treatment is complete excision. Adjuvant radiotherapy may be used in patients in whom resection was subtotal. Approaches to the sacrum can generally be classified as anterior or posterior. Type I tumors may be resected via a posterior approach alone, Type III may require an anterior approach, and Type II tumors usually require combined anterior–posterior surgery.


2019 ◽  
Vol 10 ◽  
pp. 250 ◽  
Author(s):  
Sukwoo Hong ◽  
Takayuki Hara

Background: In neurosurgical practice, we rarely encounter hybrid nerve sheath tumors (HNST) in the orbit. We recently had a patient of this rare tumor. We believe that this is the first report where we resected the tumor transcranially. Case Description: A 54-year-old male presented with the left proptosis and intraconal tumor of 43 mm. We performed fronto-orbital craniotomy to resect the tumor mass. His proptosis completely improved and discharged home with a modified Rankin Scale of 1. Conclusion: Transcranial resection of orbital HNST was a safe and effective way to treat. Since we do not have much data regarding this rare tumor, we need to accumulate more cases.


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 ◽  
...  

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.


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