scholarly journals Outcome and prognostic factors in pediatric malignant peripheral nerve sheath tumors: An analysis of the European Pediatric Soft Tissue Sarcoma Group (EpSSG) NRSTS‐2005 prospective study

2019 ◽  
Vol 66 (10) ◽  
Author(s):  
Max M. Noesel ◽  
Daniel Orbach ◽  
Bernadette Brennan ◽  
Anna Kelsey ◽  
Ilaria Zanetti ◽  
...  
Keyword(s):  
Prognostic Factors ◽  
Soft Tissue ◽  
Soft Tissue Sarcoma ◽  
Peripheral Nerve ◽  
Prospective Study ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath

scholarly journals Trends in systemic therapy for malignant peripheral nerve sheath tumors

2019 ◽  
Vol 2 (2) ◽  
pp. 139-142
Author(s):  
Lucian Alecu ◽  
Cristina Orlov-Slavu ◽  
Adrian Tulin ◽  
Cornelia Niţipir
Keyword(s):  
Targeted Therapy ◽  
Soft Tissue ◽  
Soft Tissue Sarcoma ◽  
Peripheral Nerve ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath ◽  
Type 1 Neurofibromatosis ◽  
Selection Of

AbstractMalignant peripheral nerve sheath tumors are a type of soft tissue sarcoma deriving from Schwann cells that usually appear in type 1 neurofibromatosis patients but also sporadically. Tumors frequently interest the nerves in the limbs. They represent a therapeutical challenge due to difficulty of resection and relative radio-resistance and chemo-resistance. The paper aims to describe targeted therapy used in this setting and news concerning the molecular changes that lead to carcinogenesis initiation and promotion. A short selection of literature data was made using PRISMA criteria on this topic. However, due to the rarity and heterogeneity of these tumors, personalized treatment is necessary.

Malignant peripheral nerve sheath tumors – Outcomes and prognostic factors based on the reference center experience

2020 ◽  
Vol 35 ◽  
pp. 276-284
Author(s):  
Paweł Sobczuk ◽  
Paweł Teterycz ◽  
Anna M. Czarnecka ◽  
Tomasz Świtaj ◽  
Hanna Koseła-Paterczyk ◽  
...  
Keyword(s):  
Prognostic Factors ◽  
Peripheral Nerve ◽  
Reference Center ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath

scholarly journals Brain Metastasis in Bone and Soft Tissue Cancers: A Review of Incidence, Interventions, and Outcomes

Sarcoma ◽  
2014 ◽  
Vol 2014 ◽  
pp. 1-19 ◽  
Author(s):  
Faris Shweikeh ◽  
Laura Bukavina ◽  
Kashif Saeed ◽  
Reem Sarkis ◽  
Aarushi Suneja ◽  
...  
Keyword(s):  
Soft Tissue ◽  
Brain Metastasis ◽  
Brain Metastases ◽  
Peripheral Nerve ◽  
Ewing’S Sarcoma ◽  
Ewing's Sarcoma ◽  
Systemic Disease ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath

Bone and soft tissue malignancies account for a small portion of brain metastases. In this review, we characterize their incidence, treatments, and prognosis. Most of the data in the literature is based on case reports and small case series. Less than 5% of brain metastases are from bone and soft tissue sarcomas, occurring most commonly in Ewing’s sarcoma, malignant fibrous tumors, and osteosarcoma. Mean interval from initial cancer diagnosis to brain metastasis is in the range of 20–30 months, with most being detected before 24 months (osteosarcoma, Ewing sarcoma, chordoma, angiosarcoma, and rhabdomyosarcoma), some at 24–36 months (malignant fibrous tumors, malignant peripheral nerve sheath tumors, and alveolar soft part sarcoma), and a few after 36 months (chondrosarcoma and liposarcoma). Overall mean survival ranges between 7 and 16 months, with the majority surviving < 12 months (Ewing’s sarcoma, liposarcoma, malignant fibrous tumors, malignant peripheral nerve sheath tumors, angiosarcoma and chordomas). Management is heterogeneous involving surgery, radiosurgery, radiotherapy, and chemotherapy. While a survival advantage may exist for those given aggressive treatment involving surgical resection, such patients tended to have a favorable preoperative performance status and minimal systemic disease.

scholarly journals Diagnostic Pathology of Tumors of Peripheral Nerve

Neurosurgery ◽  
2021 ◽  
Vol 88 (3) ◽  
pp. 443-456
Author(s):  
Sarra M Belakhoua ◽  
Fausto J Rodriguez
Keyword(s):  
Soft Tissue ◽  
Peripheral Nerve ◽  
Local Level ◽  
Plexiform Neurofibroma ◽  
Nerve Sheath Tumor ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath ◽  
Biological Potential ◽  
Pathologic Features

Abstract Neoplasms of the peripheral nervous system represent a heterogenous group with a wide spectrum of morphological features and biological potential. They range from benign and curable by complete excision (schwannoma and soft tissue perineurioma) to benign but potentially aggressive at the local level (plexiform neurofibroma) to the highly malignant (malignant peripheral nerve sheath tumors [MPNST]). In this review, we discuss the diagnostic and pathologic features of common peripheral nerve sheath tumors, particularly those that may be encountered in the intracranial compartment or in the spine and paraspinal region. The discussion will cover schwannoma, neurofibroma, atypical neurofibromatous neoplasms of uncertain biological potential, intraneural and soft tissue perineurioma, hybrid nerve sheath tumors, MPNST, and the recently renamed enigmatic tumor, malignant melanotic nerve sheath tumor, formerly referred to as melanotic schwannoma. We also discuss the diagnostic relevance of these neoplasms to specific genetic and familial syndromes of nerve, including neurofibromatosis 1, neurofibromatosis 2, and schwannomatosis. In addition, we discuss updates in our understanding of the molecular alterations that represent key drivers of these neoplasms, including neurofibromatosis type 1 and type 2, SMARCB1, LZTR1, and PRKAR1A loss, as well as the acquisition of CDKN2A/B mutations and alterations in the polycomb repressor complex members (SUZ12 and EED) in the malignant progression to MPNST. In summary, this review covers practical aspects of pathologic diagnosis with updates relevant to neurosurgical practice.

Malignant Peripheral Nerve Sheath Tumors

2018 ◽  
Author(s):  
Marie-Noëlle Hébert-Blouin
Keyword(s):  
Decision Making ◽  
Soft Tissue ◽  
Peripheral Nerve ◽  
Soft Tissue Sarcomas ◽  
Nerve Sheath Tumor ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Adjuvant Therapies ◽  
Nerve Sheath ◽  
Schwann Cell Differentiation

Malignant peripheral nerve sheath tumors (PNSTs) are soft tissue sarcomas arising from a peripheral nerve or a pre-existing benign nerve sheath tumor or are sarcomas with features of Schwann-cell differentiation. Differentiating between benign and malignant PNSTs can be challenging. The chapter begins with a case example and then discusses assessment, investigations (including imaging), and diagnosis of malignant PNSTs, as well as the steps involved in decision-making about management of a malignant PNST. The surgical principles and goals for resection of a malignant PNST, the adjuvant therapies used in treatment, and the complications and outcomes of treatment are presented.

Salvage rates and prognostic factors after relapse in children and adolescents with malignant peripheral nerve sheath tumors

2017 ◽  
Vol 65 (2) ◽  
pp. e26816 ◽  
Author(s):  
Luca Bergamaschi ◽  
Gianni Bisogno ◽  
Carla Manzitti ◽  
Paolo D'Angelo ◽  
Giuseppe Maria Milano ◽  
...  
Keyword(s):  
Prognostic Factors ◽  
Peripheral Nerve ◽  
Children And Adolescents ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath

scholarly journals Surgical management of malignant peripheral nerve sheath tumors

2007 ◽  
Vol 22 (6) ◽  
pp. 1-8 ◽  
Author(s):  
John Gachiani ◽  
Daniel Kim ◽  
Adriane Nelson ◽  
David Kline
Keyword(s):  
Soft Tissue ◽  
Peripheral Nerve ◽  
Surgical Techniques ◽  
Soft Tissue Sarcomas ◽  
Neurofibromatosis Type ◽  
Surgical Removal ◽  
Complete Excision ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath

Object The aim of this study was to describe the presentation of patients harboring soft tissue sarcomas involving the nerves, most of which were malignant peripheral nerve sheath tumors (MPNSTs), and provide an algorithm for their treatment. Methods The authors retrospectively analyzed data on 43 surgically treated soft tissue sarcomas involving the nerves, 34 (79%) of which were MPNSTs. Tumor classifications are presented, together with patient numbers, locations of MPNSTs, surgical techniques, and adjunctive treatments. Results The 34 MPNSTs were surgically treated during a period of 40 years. Most of these lesions (19 MPNSTs [56%]) were located in the brachial plexus, whereas the rest were located on other major nerves. Neurofibromatosis Type 1–associated tumors (12 lesions) represented 35% of the total number of MPNSTs. Although the main goal of surgery was complete excision, it was successful in only 16 patients (47%). Of the available records, 18 patients (53%) died due to disease progression. Conclusions Malignant peripheral nerve sheath tumors are rare. Surgical removal with margins when possible coupled by adjuvant radiotherapy offers the best chance of survival. The role of chemotherapy is still being defined.

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