scholarly journals Morphological spectrum of peripheral nerve sheath tumors: An insight into World Health Organization 2013 classification

2016 ◽  
Vol 07 (03) ◽  
pp. 346-354 ◽  
Author(s):  
Panduranga Chikkannaiah ◽  
Mythri M. Boovalli ◽  
Velusamy Nathiyal ◽  
Srinivasamurthy Venkataramappa
Keyword(s):  
Peripheral Nerve ◽  
Granular Cell Tumor ◽  
Malignant Tumors ◽  
Granular Cell ◽  
Cell Tumor ◽  
World Health ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath ◽  
Health Organization

ABSTRACT Introduction: Peripheral nerve sheath tumors (PNSTs) are neuroectodermal in origin. Now these tumors are classified under World Health Organization (WHO) classification of tumors of soft tissue and bone 2013. Objective: To study the morphological spectrum of PNST and to study the secondary degenerative changes associated with it. Materials and Methods: This study was conducted from January 2010 to June 2015. The gross details of tumor and patient's demographic profiles were reviewed. The hematoxylin and eosin stained slides were reassessed and the lesions were categorized and classified as per the WHO 2013 classification. The tumors were also assessed for secondary degenerative changes. Results: Our study comprised 143 cases of PNST. Age of the patients ranged from 5 to 75 years. 21–30 years is the most common age of occurrence with head and neck being the most common site. The PNSTs observed in the present study were neurofibroma (NF) (61.5%), schwannoma (36%), malignant PNST (2%), and granular cell tumor (0.5%). Nearly 10% of NF fulfilled the criteria for neurofibromatosis 1 (NF1). Rare tumors such as plexiform schwannoma and granular cell tumor were also observed. Malignant tumors were larger in dimension than benign. Myxoid, cystic, and hyaline changes were commonly associated with benign tumors while necrosis, hemorrhage, and mitotic activity were seen with malignant tumors. Conclusion: This series highlights the pathological variants of PNST along with their morphological changes and NF1 association. It is essential to be familiar with all these variants of PNST for accurate diagnosis as they have varied biological behavior.

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

scholarly journals Role of high-resolution ultrasound in detection and monitoring of peripheral nerve tumor burden in neurofibromatosis in children

2020 ◽  
Vol 36 (10) ◽  
pp. 2427-2432
Author(s):  
Natalie Winter ◽  
Maike F. Dohrn ◽  
Julia Wittlinger ◽  
Alexander Loizides ◽  
Hannes Gruber ◽  
...  
Keyword(s):  
High Resolution ◽  
Peripheral Nerve ◽  
Malignant Tumors ◽  
Diagnostic Procedures ◽  
Soft Tissue Tumors ◽  
Tumor Burden ◽  
Nerve Sheath Tumor ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath

Abstract Purpose Peripheral nerve sheath tumors are hallmark findings in neurofibromatosis types 1 and 2. With increasing size, they typically lead to neurological symptoms, and NF1 patients have a lifetime risk of 8–13% for developing malignant peripheral nerve sheath tumors. Medical imaging is therefore highly needed for early detection and exact localization of symptomatic or potentially malignant tumors. This review will give an overview of the ultrasound characteristics of peripheral nerve sheath tumors and findings in patients with neurofibromatosis types 1 and 2. Methods A systematic search of electronic databases, reference lists, and unpublished literature was conducted including the keywords “schwannoma,” “neurofibroma,” “neurofibromatosis,” “benign and malignant peripheral nerve sheath tumor.” Results The high-resolution allows a clear analysis of tumor echotexture, definition of margins, and the relation to the parent nerve. The use of color duplex/Doppler and contrast agent adds valuable information for the differentiation of benign and malignant tumors. Conclusion High-resolution ultrasound is a well-established, non-invasive, and easily repeatable first-line tool in diagnostic procedures of soft tissue tumors.

scholarly journals How Effective Are Noninvasive Tests for Diagnosing Malignant Peripheral Nerve Sheath Tumors in Patients with Neurofibromatosis Type 1? Diagnosing MPNST in NF1 Patients

Sarcoma ◽  
2019 ◽  
Vol 2019 ◽  
pp. 1-8 ◽  
Author(s):  
Maria Schwabe ◽  
Stanislav Spiridonov ◽  
Elizabeth L. Yanik ◽  
Jack W. Jennings ◽  
Travis Hillen ◽  
...  
Keyword(s):  
Peripheral Nerve ◽  
Clinical Symptoms ◽  
Malignant Tumors ◽  
Preoperative Evaluation ◽  
Clinical Findings ◽  
Significant Variable ◽  
Mri Findings ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath

Background. Distinguishing between benign and malignant peripheral nerve sheath tumors (MPNSTs) in neurofibromatosis 1 (NF1) patients prior to excision can be challenging. How can MPNST be most accurately diagnosed using clinical symptoms, magnetic resonance imaging (MRI) findings (tumor size, depth, and necrosis), positron emission tomography (PET) measures (SUVpeak, SUVmax, SUVmax tumor/SUVmean liver, and qualitative scale), and combinations of the above? Methods. All NF1 patients who underwent PET imaging at our institution (January 1, 2007–December 31, 2016) were included. Medical records were reviewed for clinical findings; MR images and PET images were interpreted by two fellowship-trained musculoskeletal and nuclear medicine radiologists, respectively. Receiver operating characteristic (ROC) curves were created for each PET measurement; the area under the curve (AUC) and thresholds for diagnosing malignancy were calculated. Logistic regression determined significant predictors of malignancy. Results. Our population of 41 patients contained 34 benign and 36 malignant tumors. Clinical findings did not reliably predict MPNST. Tumor depth below fascia was highly sensitive; larger tumors were more likely to be malignant but without a useful cutoff for diagnosis. Necrosis on MRI was highly accurate and was the only significant variable in the regression model. PET measures were highly accurate, with AUCs comparable and cutoff points consistent with prior studies. A diagnostic algorithm was created using MRI and PET findings. Conclusions. MRI and PET were more effective at diagnosing MPNST than clinical features. We created an algorithm for preoperative evaluation of peripheral nerve sheath tumors in NF1 patients, for which additional validation will be indicated.

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.

A Rare Perineurioma/Granular Cell Tumor Hybrid Peripheral Nerve Sheath Tumor

2020 ◽  
Vol 42 (10) ◽  
pp. 762-765
Author(s):  
Koorosh Haghayeghi ◽  
Gladys Telang ◽  
Sonja Chen ◽  
Jack Bevivino ◽  
Shamlal Mangray ◽  
...  
Keyword(s):  
Peripheral Nerve ◽  
Granular Cell Tumor ◽  
Granular Cell ◽  
Cell Tumor ◽  
Nerve Sheath Tumor ◽  
Peripheral Nerve Sheath Tumor ◽  
Nerve Sheath

scholarly journals Malignant peripheral nerve sheath tumors

2007 ◽  
Vol 22 (6) ◽  
pp. 1-8 ◽  
Author(s):  
Gaurav Gupta ◽  
Allen Maniker
Keyword(s):  
Peripheral Nerve ◽  
Soft Tissue Sarcomas ◽  
Nerve Fibers ◽  
World Health ◽  
Malignant Schwannoma ◽  
Malignant Neoplasms ◽  
Cell Of Origin ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath

Malignant peripheral nerve sheath tumors (MPNSTs) are rare soft tissue sarcomas of ectomesenchymal origin. The World Health Organization coined the term MPNST to replace previous heterogeneous and often confusing terminology, such as “malignant schwannoma,” “malignant neurilemmoma,” “neurogenic sarcoma,” and “neurofibrosarcoma.” Malignant peripheral nerve sheath tumors arise from major or minor peripheral nerve branches or sheaths of peripheral nerve fibers, and are derived from Schwann cells or pluripotent cells of neural crest origin. The Schwann cell is thought to be the major contributor to the formation of benign as well as malignant neoplasms of the nerve sheath. While this fact remains essentially true, the identity of cell of origin of the MPNST remains elusive, and has not yet been conclusively identified. It has been suggested that these tumors may have multiple cell line origins. In this review, the authors discuss the epidemiology, diagnosis, management, and treatment of MPNSTs.

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