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.

There are currently no organizations listed in Disease InfoSearch that support this condition. Create a listing.

 

 

General Support Organizations

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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 59 free, full-text research articles on human participants. First 3 results:

Genomic amplification and high expression of EGFR are key targetable oncogenic events in malignant peripheral nerve sheath tumor.
 

Author(s): Xiaoling Du, Jilong Yang, Antti Ylipää, Ze Zhu

Journal:

 

The dismal outcome of malignant peripheral nerve sheath tumor (MPNST) highlights the necessity of finding new therapeutic methods to benefit patients with this aggressive sarcoma. Our purpose was to investigate epidermal growth factor receptor (EGFR) as a potential therapeutic target in MPNSTs.

Last Updated: 23 Dec 2013

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Forward genetic screen for malignant peripheral nerve sheath tumor formation identifies new genes and pathways driving tumorigenesis.
 

Author(s): Eric P Rahrmann, Adrienne L Watson, Vincent W Keng, Kwangmin Choi, Branden S Moriarity, Dominic A Beckmann, Natalie K Wolf, Aaron Sarver, Margaret H Collins, Christopher L Moertel, Margaret R Wallace, Bernat Gel, Eduard Serra, Nancy Ratner, David A Largaespada

Journal: Nat. Genet.. 2013 Jul;45(7):756-66.

 

Malignant peripheral nerve sheath tumors (MPNSTs) are sarcomas of Schwann cell lineage origin that occur sporadically or in association with the inherited syndrome neurofibromatosis type 1. To identify genetic drivers of MPNST development, we used the Sleeping Beauty (SB) transposon-based ...

Last Updated: 26 Jun 2013

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Sensitivity of malignant peripheral nerve sheath tumor cells to TRAIL is augmented by loss of NF1 through modulation of MYC/MAD and is potentiated by curcumin through induction of ROS.
 

Author(s): David E Reuss, Jana Mucha, Christian Hagenlocher, Volker Ehemann, Lan Kluwe, Victor Mautner, Andreas von Deimling

Journal: PLoS ONE. 2013 ;8(2):e57152.

 

Malignant peripheral nerve sheath tumor (MPNST) is a rare aggressive form of sarcoma often associated with the tumor syndrome neurofibromatosis type 1 (NF1). We investigated the effects of tumor necrosis factor-related apoptosis inducing ligand (TRAIL) on NF1 associated MPNST and ...

Last Updated: 25 Feb 2013

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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:

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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Survival meta-analyses for >1800 malignant peripheral nerve sheath tumor patients with and without neurofibromatosis type 1.
 

Author(s): Matthias Kolberg, Maren Høland, Trude H Agesen, Helge R Brekke, Knut Liestøl, Kirsten S Hall, Fredrik Mertens, Piero Picci, Sigbjørn Smeland, Ragnhild A Lothe

Journal: Neuro-oncology. 2013 Feb;15(2):135-47.

 

There are conflicting reports as to whether malignant peripheral nerve sheath tumor (MPNST) patients with neurofibromatosis type 1 (NF1) have worse prognosis than non-NF1 MPNST patients. Large clinical studies to address this problem are lacking due to the rareness of MPNST. We have ...

Last Updated: 21 Jan 2013

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Symptoms, Diagnosis, and Treatment

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Clinical Trial Information This information is provided by ClinicalTrials.gov

A Trial of Ganetespib Plus Sirolimus: Phase 1 Includes Multiple Sarcoma Subtypes and Phase 2 MPNST
 

Status: Recruiting

Condition Summary: Malignant Peripheral Nerve Sheath Tumors (MPNST); Sarcoma

 

Last Updated: 20 Jun 2014

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Natural History Study of Patients With Neurofibromatosis Type I
 

Status: Recruiting

Condition Summary: Neurofibromatosis Type 1; Malignant Peripheral Nerve Sheath Tumor; Plexiform Neurofibroma; Optic Glioma; Neurofibroma

 

Last Updated: 21 May 2014

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Gemcitabine and Docetaxel in Combination With Pazopanib (Gem/Doce/Pzb) for the Neoadjuvant Treatment of Soft Tissue Sarcoma (STS)
 

Status: Recruiting

Condition Summary: Sarcoma; Leiomyosarcoma; Malignant Peripheral Nerve Sheath Tumor; Malignant Fibrous; Histiocytoma/Undifferentiated Pleomorphic Sarcoma

 

Last Updated: 5 Aug 2014

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