Malignant Nerve Sheath Tumor of the Right Cerebral Peduncle: Case Report

Neurosurgery ◽  
2004 ◽  
Vol 54 (2) ◽  
pp. 500-504 ◽  
Author(s):  
Patrick Beauchesne ◽  
Jean-François Mosnier ◽  
Thierry Schmitt ◽  
Jacques Brunon
Keyword(s):  
Stereotactic Biopsy ◽  
Clinical Presentation ◽  
Positive Response ◽  
Cerebral Peduncle ◽  
Nerve Sheath Tumor ◽  
Chemotherapy Treatment ◽  
Nerve Sheath Tumors ◽  
Nerve Sheath ◽  
The Right ◽  
First Time

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.

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.

scholarly journals Seorang Laki-Laki dengan Tumor Mediastinum Posterior ( Malignant Peripheral Nerve Sheath Tumor )

2019 ◽  
Vol 2 (3) ◽  
pp. 82
Author(s):  
Daniel Maranatha ◽  
Shinta Karina Yuniati
Keyword(s):  
Peripheral Nerve ◽  
Malignant Tumors ◽  
Posterior Mediastinum ◽  
Surrounding Tissue ◽  
Nerve Sheath Tumor ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath ◽  
S 100 ◽  
The Right

Background: Malignant tumors arising from peripheral nerves or displaying differentiation along the lines of the various elements of the nerve sheath are referred to Malignant peripheral nerve sheath tumors (MPNST) and also called malignant schwannomas or neurofibrosarcomas. The common sites of involvement are head, neck, extremities and thorax. This case was discussed due to its rare incidence, namely less than 5% of soft tissue malignant tumor. The overall five year survival rate is approximately 50% in patients with resectable tumor. Case: We report a case of MPNST arising from mediastinum, the patient was a male, 41-years-old, with chief complaint are chest pain and mass in the back. Contrast enhanced chest CT Scan showed a solid mass (10.02 × 6.97 × 10.53 cm) in the right side of the posterior mediastinum. On microscopic examination with hematoxylin eosin imunostaining, of the mass on mediastinum showed typical features of MPNST, which were positive for S-100 imunostaining. Based on convensional histopathologic and imunostaining, this case was concluded as Malignant peripheral nerve sheath tumors (MPNST). Conclusion: The treatment of chest wall MPNSTs is multimodality, including wide local excision, adjuvant radiotherapy, and chemotherapy. In our case, we performed direct excision due to the infiltrating mass to the surrounding tissue, so the prognosis for this case was good. This case indicates the role of chemotherapy in treatment of advanced MPNST. Studies shows the superiority of the doxorubicin–ifosfamide regimen. As in this case the patient showed complete respons after surgery and adjuvant chemotherapy.

scholarly journals Robotic Resection of a Nerve Sheath Tumor Via a Retroperitoneal Approach

2020 ◽  
Author(s):  
Benjamin I Rapoport ◽  
Christina Sze ◽  
Xi Chen ◽  
Ibrahim Hussain ◽  
Mark H Bilsky ◽  
...  
Keyword(s):  
Minimally Invasive ◽  
Intraoperative Ultrasound ◽  
Sensory Nerves ◽  
Retroperitoneal Approach ◽  
Nerve Sheath Tumor ◽  
Robot Assisted ◽  
Neurophysiologic Monitoring ◽  
Nerve Sheath Tumors ◽  
Nerve Sheath ◽  
The Right

Abstract BACKGROUND Resection of large nerve sheath tumors in the lumbar spine using minimally invasive approaches is challenging, as approaches to tumors in this region may require facetectomy or partial resection of adjacent ribs for access to the involved neuroforamen and instrumentation across the involved joint to prevent subsequent kyphotic deformity. OBJECTIVE To describe a robot-assisted retroperitoneal approach for resection of a lumbar nerve sheath tumor, obviating the need for facetectomy and instrumentation. The operation is described, together with intraoperative images and an annotated video, in the context of a schwannoma arising from the right L1 root. METHODS The operation was performed by a urologic surgeon and a neurosurgeon. The patient was placed in lateral position, and the da Vinci Xi robot was used for retroperitoneal access via 5 ports along the right flank. Ultrasound was used to localize the tumor within the psoas. The tumor capsule was defined and released. Encountered nerves were stimulated, allowing small sensory nerves to be identified and safely divided. The tumor was traced into the right L1-L2 neuroforamen and removed. RESULTS Complete en bloc resection of the tumor was achieved, including the paraspinal and foraminal components, without any removal of bone and without violation of the dura. CONCLUSION In selected patients, a robot-assisted retroperitoneal approach represents a minimally invasive alternative to traditional approaches for resection of lumbar nerve sheath tumors. This approach obviates the need for bone removal and instrumented spinal fusion. Interdisciplinary collaboration, as well as use of adjunctive technologies, including intraoperative ultrasound and neurophysiologic monitoring, is advised.

Nerve sheath tumors involving the sacrum

2003 ◽  
Vol 15 (2) ◽  
pp. 1-6 ◽  
Author(s):  
Paul Klimo ◽  
Ganesh Rao ◽  
Richard H. Schmidt ◽  
Meic H. Schmidt
Keyword(s):  
Type I ◽  
Nerve Sheath Tumor ◽  
Delayed Presentation ◽  
Type Ii ◽  
Complete Excision ◽  
Posterior Surgery ◽  
Sacral Nerve ◽  
Type Iii ◽  
Nerve Sheath Tumors ◽  
Nerve Sheath

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.

scholarly journals Hybrid nerve sheath tumor in the orbit: A case report and review of literature

2019 ◽  
Vol 10 ◽  
pp. 250 ◽  
Author(s):  
Sukwoo Hong ◽  
Takayuki Hara
Keyword(s):  
Case Report ◽  
Nerve Sheath Tumor ◽  
Modified Rankin Scale ◽  
Case Description ◽  
Rare Tumor ◽  
Review Of Literature ◽  
Tumor Mass ◽  
First Report ◽  
Nerve Sheath Tumors ◽  
Nerve Sheath

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.

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 Special Issue: “Genomics and Models of Nerve Sheath Tumors”

Genes ◽  
2020 ◽  
Vol 11 (9) ◽  
pp. 1024
Author(s):  
Angela C. Hirbe ◽  
Rebecca D. Dodd ◽  
Christine A. Pratilas
Keyword(s):  
Soft Tissue ◽  
Neurofibromatosis Type ◽  
Benign Tumors ◽  
Nerve Sheath Tumor ◽  
Special Issue ◽  
Peripheral Nerve Sheath Tumor ◽  
Nerve Sheath Tumors ◽  
Nerve Sheath ◽  
Biological Potential

Nerve sheath tumors arising in the context of neurofibromatosis type 1 (NF1) include benign tumors such as cutaneous, diffuse and plexiform neurofibromas; atypical neurofibromas or atypical neurofibromatosis neoplasms of uncertain biological potential (ANNUBP); and the aggressive soft tissue sarcoma, the malignant peripheral nerve sheath tumor (MPNST) [...]

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.

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.

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