Malignant peripheral nerve sheath tumor

Common Name(s)

Malignant peripheral nerve sheath tumor

A malignant peripheral nerve sheath tumor (MPNST) is a tumor that develops from nerve tissue.  The first symptom of MPNST is a lump or mass that increases in size, sometimes causing pain or a tingling sensation.   MPNST is considered an aggressive tumor because there is up to a 65% chance of the tumor regrowing after surgery (a recurrence), and approximately 40% chance of spreading to distant parts of the body (a metastasis), most commonly to the lung.   Treatment of MPNST begins with surgery to remove as much of the tumor as possible.  Radiation therapy may be used to decrease the chance of a recurrence.  Chemotherapy might be used if the whole tumor cannot be removed during surgery, or to treat a metastasis.   MPNSTs are quite rare, occurring in 0.001% of the general population.   Approximately 25-50% of MPNSTs are associated with a genetic condition known as neurofibromatosis type 1.
 

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Malignant peripheral nerve sheath tumor" for support, advocacy or research.

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Malignant peripheral nerve sheath tumor" returned 84 free, full-text research articles on human participants. First 3 results:

A neurogenic tumor containing a low-grade malignant peripheral nerve sheath tumor (MPNST) component with loss of p16 expression and homozygous deletion of CDKN2A/p16: a case report showing progression from a neurofibroma to a high-grade MPNST.
 

Author(s): Shogo Tajima, Kenji Koda

Journal:

 

Development of malignant peripheral nerve sheath tumors (MPNSTs) is a stepwise process that involves the alteration of many cell cycle regulators and the double inactivation of the NF1 gene. Inactivation of the TP53 gene and deletion of the CDKN2A/p16 gene are known to play an important ...

Last Updated: 20 Jul 2015

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Classic Ras Proteins Promote Proliferation and Survival via Distinct Phosphoproteome Alterations in Neurofibromin-Null Malignant Peripheral Nerve Sheath Tumor Cells.
 

Author(s): Nicole M Brossier, Amanda M Prechtl, Jody Fromm Longo, Stephen Barnes, Landon S Wilson, Stephanie J Byer, Stephanie N Brosius, Steven L Carroll

Journal: J. Neuropathol. Exp. Neurol.. 2015 Jun;74(6):568-86.

 

Neurofibromin, the tumor suppressor encoded by the neurofibromatosis type 1 (NF1) gene, potentially suppresses the activation of H-Ras, N-Ras, and K-Ras. However, it is not known whether these classic Ras proteins are hyperactivated in NF1-null nerve sheath tumors, how they contribute ...

Last Updated: 18 May 2015

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Antitumor effects of pharmacological EZH2 inhibition on malignant peripheral nerve sheath tumor through the miR-30a and KPNB1 pathway.
 

Author(s): Pingyu Zhang, Xianbin Yang, Xiaoyan Ma, Davis R Ingram, Alexander J Lazar, Keila E Torres, Raphael E Pollock

Journal:

 

Enhancer of zeste homolog 2 (EZH2) is a key epigenetic regulator in cancer cell survival, epithelial-mesenchymal transition, and tumorigenesis. Inhibition of EZH2 has become a promising therapeutic option for various human malignancies. Previously, we demonstrated that the EZH2/miR-30d/karyopherin ...

Last Updated: 19 Apr 2015

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Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Malignant peripheral nerve sheath tumor" returned 7 free, full-text review articles on human participants. First 3 results:

Intraosseous primary malignant peripheral nerve sheath tumor of the calcaneus: an unusual case and review of literature.
 

Author(s): Gaurav Pratap Singh Gahlot, Asit Ranjan Mridha, Devajit Nath, Shah Alam Khan, Shivanand Gamanagatti

Journal: Indian J Pathol Microbiol. ;58(2):220-2.

 

Malignant peripheral nerve sheath tumors (MPNSTs) are uncommon sarcomas that originate from a peripheral nerve or neurofibroma either spontaneously or in association with neurofibromatosis type 1. MPNSTs account for approximately 5% of all soft tissue malignancies. The tumor is commonly ...

Last Updated: 18 Apr 2015

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Microsurgical management of giant malignant peripheral nerve sheath tumor of the scalp: two case reports and a literature review.
 

Author(s): Jun Wang, Shao-wu Ou, Zong-ze Guo, Yun-jie Wang, De-guang Xing

Journal:

 

Malignant peripheral nerve sheath tumors of the scalp are rare lesions of the nervous system. Only 14 cases have been reported to date. The field of neurosurgery has struggled with diagnosing and treating these tumors. In this report, we present two cases of giant malignant peripheral ...

Last Updated: 15 Nov 2013

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Perineurial malignant peripheral nerve sheath tumor in the setting of multiple soft tissue perineuriomas: a rare presentation of an uncommon tumor.
 

Author(s): Bharat Rekhi

Journal: J Cancer Res Ther. ;9(1):131-4.

 

Perineurioma is an uncommon soft tissue tumor with characteristic histological and immunohistochemical features. Herein, this tumor is presented within a rare clinical setting, in a 45-year-old gentleman, with multiple soft tissue swellings and a previous history of surgical excision ...

Last Updated: 11 Apr 2013

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Symptoms, Diagnosis, and Treatment

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Clinical Trial Information This information is provided by ClinicalTrials.gov

Vaccine Therapy in Treating Patients With Malignant Peripheral Nerve Sheath Tumor That is Recurrent or Cannot Be Removed by Surgery
 

Status: Recruiting

Condition Summary: Metastatic Malignant Peripheral Nerve Sheath Tumor; Neurofibromatosis Type 1; Recurrent Malignant Peripheral Nerve Sheath Tumor

 

Last Updated: 17 Jun 2016

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PLX3397 Plus Sirolimus in Unresectable Sarcoma and Malignant Peripheral Nerve Sheath Tumors
 

Status: Recruiting

Condition Summary: Sarcoma; Malignant Peripheral Nerve Sheath Tumors

 

Last Updated: 15 Jan 2016

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Lorvotuzumab Mertansine in Treating Younger Patients With Relapsed or Refractory Wilms Tumor, Rhabdomyosarcoma, Neuroblastoma, Pleuropulmonary Blastoma, Malignant Peripheral Nerve Sheath Tumor, or Synovial Sarcoma
 

Status: Recruiting

Condition Summary: Pleuropulmonary Blastoma; Recurrent Malignant Peripheral Nerve Sheath Tumor; Recurrent Neuroblastoma; Recurrent Rhabdomyosarcoma; Recurrent Synovial Sarcoma; Wilms Tumor

 

Last Updated: 2 Mar 2016

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