Clinical presentation and long-term outcome of primary spinal intradural malignant peripheral nerve sheath tumors

2019 ◽  
Vol 185 ◽  
pp. 105484
Author(s):  
Jun Chen ◽  
Yifeng Zheng ◽  
Zirong Chen ◽  
Fanfan Fan ◽  
Yu Wang
Keyword(s):  
Peripheral Nerve ◽  
Clinical Presentation ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath

scholarly journals Malignant peripheral nerve sheath tumor in the maxilla. Clinical сase

2019 ◽  
Vol 9 (3) ◽  
pp. 72-76
Author(s):  
V. V. Baryshev ◽  
F. Е. Sevryukov ◽  
V. V. Polkin ◽  
Е. А. Khanenya ◽  
Т. А. Аgababyan ◽  
...  
Keyword(s):  
Peripheral Nerve ◽  
Clinical Case ◽  
Malignant Potential ◽  
Nerve Sheath Tumor ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Peripheral Nerve Sheath Tumor ◽  
Nerve Sheath ◽  
Precise Diagnosis

A clinical case of the malignant peripheral nerve sheath tumor is presented. It is a rare tumor in the head and neck area. The tumor arises from Schwann cells, its malignant potential is unclear. This low-growing tumor is presenting as a solitary nodule. To make a precise diagnosis immunohistochemistry assay is used. Surgical approach provides good long-term outcome.

Role of resection of malignant peripheral nerve sheath tumors in patients with neurofibromatosis Type 1

2013 ◽  
Vol 118 (1) ◽  
pp. 142-148 ◽  
Author(s):  
Gavin P. Dunn ◽  
Konstantinos Spiliopoulos ◽  
Scott R. Plotkin ◽  
Francis J. Hornicek ◽  
David C. Harmon ◽  
...  
Keyword(s):  
Multivariate Analysis ◽  
Radiation Therapy ◽  
Peripheral Nerve ◽  
Neurofibromatosis Type ◽  
Total Resection ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath

Object Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive sarcomas that often arise from major peripheral nerves. Approximately half of MPNSTs arise in patients with neurofibromatosis Type 1 (NF1) who, in comparison with patients without NF1, present at younger ages and with larger tumors that are commonly associated with extensive plexiform neurofibromas. These tumors therefore pose a particularly difficult treatment challenge because of the morbidity often associated with attempted gross-total resection (GTR). Here, the authors aim to examine what role the extent of resection and other covariates play in the long-term survival of patients with NF1 in the setting of MPNST. Methods The authors retrospectively reviewed the records of 23 adult patients with NF1 who underwent surgery for MPNSTs at their institution between 1991 and 2008. The primary end points of the study were mortality, local recurrence, and metastasis. Kaplan-Meier survival curves were evaluated for all patients. Differences for each of the primary end points were evaluated based on cause-specific covariates, which included tiered tumor size, tumor location, grade, resection margin status, postoperative weakness, and use of chemotherapy and radiation therapy. Multivariate analysis was performed using Cox proportional hazards models. Results Gross-total resection (p = 0.01) and surgical margin status (p = 0.034) had a statistically important role in prolonging overall survival in patients with NF1 by univariate analysis. When tumor size, location, grade, postoperative weakness, and radiation therapy were also taken into account using multivariate analysis, GTR continued to be a significant prognostic factor (p = 0.035). Conclusions These findings suggest that GTR offers significant long-term benefit on survival in patients with NF1. Benefit on survival occurred independently of all other covariates, suggesting that complete resection should be the principal goal of treatment in this patient population.

scholarly journals Biology and Management of Undifferentiated Pleomorphic Sarcoma, Myxofibrosarcoma, and Malignant Peripheral Nerve Sheath Tumors: State of the Art and Perspectives

2018 ◽  
Vol 36 (2) ◽  
pp. 160-167 ◽  
Author(s):  
Brigitte C. Widemann ◽  
Antoine Italiano
Keyword(s):  
Peripheral Nerve ◽  
Clinical Presentation ◽  
State Of The Art ◽  
Current Knowledge ◽  
Future Research ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Pleomorphic Sarcoma ◽  
Nerve Sheath ◽  
Aggressive Clinical Behavior

Undifferentiated pleomorphic sarcomas, myxofibrosarcomas, and malignant peripheral nerve sheath tumors are characterized by complex genomic characteristics and aggressive clinical behavior. Recent advances in the understanding of the pathogenesis of these tumors may allow for the development of more-effective innovative therapeutic strategies, including immunotherapies. This review describes the current knowledge of the epidemiology, clinical presentation, treatment, and pathogenesis of these tumors and highlights ongoing and future research.

scholarly journals Management of peripheral nerve sheath tumors: 17 years of experience at Toronto Western Hospital

2018 ◽  
Vol 128 (4) ◽  
pp. 1226-1234 ◽  
Author(s):  
Daipayan Guha ◽  
Benjamin Davidson ◽  
Mustafa Nadi ◽  
Naif M. Alotaibi ◽  
Michael G. Fehlings ◽  
...  
Keyword(s):  
Peripheral Nerve ◽  
Clinical Presentation ◽  
Extent Of Resection ◽  
Tumor Location ◽  
Benign Lesions ◽  
Toronto Western Hospital ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Western Hospital ◽  
Nerve Sheath

OBJECTIVEA surgical series of 201 benign and malignant peripheral nerve sheath tumors (PNSTs) was assessed to characterize the anatomical and clinical presentation of tumors and identify predictors of neurological outcome, recurrence, and extent of resection.METHODSAll surgically treated PNSTs from the Division of Neurosurgery at Toronto Western Hospital from 1993 to 2010 were reviewed retrospectively. Data were collected on patient demographics, clinical presentation, surgical technique, extent of resection, postoperative neurological outcomes, and recurrence.RESULTSOne hundred seventy-five patients with 201 tumors had adequate follow-up for analysis. There were 182 benign and 19 malignant PNSTs. Of the benign lesions, 133 were schwannomas, 21 of which were associated with a diagnosis of schwannomatosis. There were 49 neurofibromas, and 26 were associated with neurofibromatosis Type 1 (NF1). Patients presenting with schwannomas were significantly older than those with neurofibromas. Schwannomas were more readily resected than neurofibromas, with the extent of resection of the former influenced by tumor location. Patients with benign PNSTs typically presented with a painful mass and less frequently with motor deficits. The likelihood of worsened postoperative motor function was decreased in patients with fully resected tumors or preoperative deficits. Recurrence of schwannomas and neurofibromas were seen more frequently in patients diagnosed with NF3 and NF1, respectively. Subtotal resection was associated with the increased recurrence of all benign lesions.CONCLUSIONSOutcomes following resection of benign PNSTs depend on tumor histopathology, tumor location, and genetic predisposition syndrome. Gross-total resection should be attempted for benign lesions where possible. The management of malignant PNSTs remains challenging, requiring a multimodal approach.

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