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 Toward Understanding the Mechanisms of Malignant Peripheral Nerve Sheath Tumor Development

2021 ◽  
Vol 22 (16) ◽  
pp. 8620
Author(s):  
Teddy Mohamad ◽  
Camille Plante ◽  
Jean-Philippe Brosseau
Keyword(s):  
Peripheral Nerve ◽  
Tumor Development ◽  
Neurofibromatosis Type ◽  
Type I ◽  
Nerve Sheath Tumor ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Research Perspectives ◽  
Nerve Sheath ◽  
History Of

Malignant peripheral nerve sheath tumors (MPNSTs) originate from the neural crest lineage and are associated with the neurofibromatosis type I syndrome. MPNST is an unmet clinical need. In this review article, we summarize the knowledge and discuss research perspectives related to (1) the natural history of MPNST development; (2) the mouse models recapitulating the progression from precursor lesions to MPNST; (3) the role of the tumor microenvironment in MPNST development, and (4) the signaling pathways linked to MPNST development.

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.

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.

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

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

2018 ◽  
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

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

2018 ◽  
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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