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

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.

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

Clinical Neurology and Neurosurgery ◽

10.1016/j.clineuro.2019.105484 ◽

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

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

Journal of Neurosurgery ◽

10.3171/2017.1.jns162292 ◽

2018 ◽

Vol 128 (4) ◽

pp. 1226-1234 ◽

Cited By ~ 15

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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