scholarly journals The Lipid Asset Is Unbalanced in Peripheral Nerve Sheath Tumors

2021 ◽  
Vol 23 (1) ◽  
pp. 61
Author(s):  
Ignazio G. Vetrano ◽  
Michele Dei Cas ◽  
Vittoria Nazzi ◽  
Marica Eoli ◽  
Niccolò Innocenti ◽  
...  
Keyword(s):  
Peripheral Nerve ◽  
Biological Behavior ◽  
Malignant Degeneration ◽  
Benign Tumors ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Molecular Features ◽  
Nerve Sheath ◽  
Inflammatory Processes ◽  
Oxidized Triacylglycerols

Peripheral nerve sheath tumors (PNSTs) include schwannomas, neurofibromas (NFs), and plexiform neurofibromas (PNFs), among others. While they are benign tumors, according to their biological behavior, some have the potential for malignant degeneration, mainly PNFs. The specific factors contributing to the more aggressive behavior of some PNSTs compared to others are not precisely known. Considering that lipid homeostasis plays a crucial role in fibrotic/inflammatory processes and in several cancers, we hypothesized that the lipid asset was also unbalanced in this group of nerve tumors. Through untargeted lipidomics, NFs presented a significant increase in ceramide, phosphatidylcholine, and Vitamin A ester. PNFs displayed a marked decrease in 34 out of 50 lipid class analyzed. An increased level of ether- and oxidized-triacylglycerols was observed; phosphatidylcholines were reduced. After sphingolipidomic analysis, we observed six sphingolipid classes. Ceramide and dihydroceramides were statistically increased in NFs. All the glycosylated species appeared reduced in NFs, but increased in PNFs. Our findings suggested that different subtypes of PNSTs presented a specific modulation in the lipidic profile. The untargeted and targeted lipidomic approaches, which were not applied until now, contribute to better clarifying bioactive lipid roles in PNS natural history to highlight disease molecular features and pathogenesis.

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: 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

Malignant peripheral nerve sheath tumors in the setting of segmental neurofibromatosis

2000 ◽  
Vol 92 (2) ◽  
pp. 342-346 ◽  
Author(s):  
Jacob Schwarz ◽  
Allan J. Belzberg
Keyword(s):  
Peripheral Nerve ◽  
Neurological Impairment ◽  
Malignant Degeneration ◽  
Surgical Removal ◽  
Surgical Indications ◽  
Content Type ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath ◽  
Adjacent Structures

✓ Approximately 100 cases of segmental neurofibromatosis (NF5) have been reported in the recent literature. Patients with NF5 present with café-au-lait macules, freckles, and/or neurofibromas limited to one or adjacent dermatomes. Neurofibromas arising in NF5 have been uniformly considered to be benign; patients were thought to have an excellent prognosis without the risk of developing malignant peripheral nerve sheath tumors (PNSTs), which are characteristic in patients with the generalized form of this disease, von Recklinghausen's NF. In this report the authors detail the first observations of malignant PNSTs in two patients with NF5. Indications for surgical removal of a neurofibroma in a patient with NF include pain, neurological impairment, compression of adjacent structures, cosmetic disfigurement, and rapid tumor growth suggestive of malignant degeneration. Surgical indications are similar for patients with NF5. All patients with neurofibromas should be considered at risk for malignant degeneration.

Preserve the Nerve: Microsurgical Resection of Peripheral Nerve Sheath Tumors

2007 ◽  
Vol 61 (suppl_3) ◽  
pp. ONS-113-ONS-118 ◽  
Author(s):  
Stephen M. Russell
Keyword(s):  
Neuropathic Pain ◽  
Peripheral Nerve ◽  
Neurological Deficit ◽  
Benign Tumors ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath ◽  
Microsurgical Techniques ◽  
Microsurgical Resection

Abstract Using a Fascicle sparing approach, schwannomas and nonplexiform neurofibromas can often be removed without causing neurological deficit or neuropathic pain. This article provides a step-by-step description of how to remove these benign tumors using microsurgical techniques.

scholarly journals The Surgical Management of Symptomatic Peripheral Nerve Sheath Tumors

Neurosurgery ◽  
2010 ◽  
Vol 66 (4) ◽  
pp. 833-840 ◽  
Author(s):  
Allan D. Levi ◽  
Andrew L. Ross ◽  
Esteban Cuartas ◽  
Rabah Qadir ◽  
H. Thomas Temple
Keyword(s):  
Peripheral Nerve ◽  
Surgical Management ◽  
Tumor Size ◽  
Motor Deficit ◽  
Benign Tumors ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath ◽  
Neurologic Deficits ◽  
Increased Risk

Abstract OBJECTIVE To determine the clinical presentation and morbidity of the surgical management of peripheral nerve sheath tumors (PNSTs). METHODS We performed a retrospective chart review of surgically treated PNSTs at the University of Miami between 1991 and 2008. RESULTS There were a total of 140 cases, including 87 schwannomas, 34 neurofibromas, and 19 malignant peripheral nerve sheath tumors (MPNSTs). The average age of the total study group was 49.0 years; it was significantly lower for patients with neurofibroma. There was a high correlation between neurofibroma tumors and neurofibromatosis-1. Most patients with benign tumors presented with a painful mass, paresthesias, or numbness without significant weakness. Patients who had previously undergone attempted resections and preoperative biopsy had a significantly increased risk (41%) for developing postoperative neurologic deficits when compared with patients who presented with de novo tumors (15%). Intraoperative monitoring appeared to reduce the risk of postoperative motor deficit, particularly in neurofibromas. Most MPNSTs (>80%) were diagnosed at stage IIB or higher and had a combined mortality rate of 31.6% at 78 months. Tumor size was the best predictor of adverse outcome, as all MPNST mortalities occurred in patients with a tumor size of more than 7 cm. CONCLUSION PNSTs are a heterogeneous group of lesions. Benign tumors respond well to marginal excision, whereas MPNSTs are aggressive sarcomas that require multimodal management. There was a significantly increased risk of postoperative neurologic deficits in patients who had undergone a previous biopsy, and thus tertiary referral without biopsy is recommended when a PNST is suspected.

Malignant Peripheral Nerve Sheath Tumors of the 8th Cranial Nerve Arising without Prior Irradiation

2016 ◽  
Vol 77 (S 01) ◽  
Author(s):  
Matthew Carlson ◽  
Jeffrey Jacob ◽  
Elizabeth Habermann ◽  
Amy Wagie ◽  
Aditya Raghunathan ◽  
...  
Keyword(s):  
Peripheral Nerve ◽  
Cranial Nerve ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath

MR imaging of benign peripheral nerve sheath tumors

1994 ◽  
Vol 35 (3) ◽  
pp. 282-286 ◽  
Author(s):  
Veli Soderlund ◽  
H. Goranson ◽  
H. C. F. Bauer
Keyword(s):  
Mr Imaging ◽  
Peripheral Nerve ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath
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