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

Malignant peripheral nerve sheath tumor with and without neurofibromatosis type 1.
 

Author(s): Roberto André Torres de Vasconcelos, Pedro Guimarães Coscarelli, Regina Papais Alvarenga, Marcus André Acioly

Journal: Arq Neuropsiquiatr. 2017 Jun;75(6):366-371.

 

In this study, we review the institution's experience in treating malignant peripheral nerve sheath tumors (MPNSTs). A secondary aim was to compare outcomes between MPNSTs with and without neurofibromatosis type 1 (NF1).

Last Updated: 28 Jun 2017

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A distal ileum malignant peripheral nerve sheath tumor causing intussusception in a patient in China: a case report.
 

Author(s): Lin-Bo Zhu, Peng-Fei Li, Wei-Hua Xiao, Peng-Bin Zhang, Jun-Qiang Li, Ming-Fei Sun

Journal:

 

Malignant peripheral nerve sheath tumors (MPNSTs) arise from a peripheral nerve or display nerve sheath differentiation. Most MPNSTs typically originate on the trunk, extremities, head, neck, and paravertebral regions. Gastrointestinal MPNSTs are rare entities with only 10 cases reported ...

Last Updated: 20 Jan 2017

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A Case of Occipital Malignant Peripheral Nerve Sheath Tumor with Neurofibromatosis Type 1.
 

Author(s): Ushio Hanai, Tadashi Akamatsu, Megumi Kobayashi, Yotaro Tsunoda, Kenichi Hirabayashi, Tanehumi Baba, Hideki Atsumi, Mitsunori Matsumae

Journal:

 

The prognosis of malignant peripheral nerve sheath tumor (MPNST) with neurofibromatosis type 1 (NF-1) is worse than that of a solitary MPNST, because of the tumor size and location difficult to resect completely. We experienced a case of MPNST in the occipital region with NF-1.

Last Updated: 16 Sep 2016

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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 11 free, full-text review articles on human participants. First 3 results:

Composite pheochromocytoma with a malignant peripheral nerve sheath tumor: Case report and review of the literature.
 

Author(s): Takeshi Namekawa, Takanobu Utsumi, Takashi Imamoto, Koji Kawamura, Takashi Oide, Tomoaki Tanaka, Naoki Nihei, Hiroyoshi Suzuki, Yukio Nakatani, Tomohiko Ichikawa

Journal: Asian J Surg. 2016 Jul;39(3):187-90.

 

Adrenal tumors with more than one cellular component are uncommon. Furthermore, an adrenal tumor composed of a pheochromocytoma and a malignant peripheral nerve sheath tumor is extremely rare. A composite pheochromocytoma with malignant peripheral nerve sheath tumor in a 42-year-old ...

Last Updated: 24 Jun 2016

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Malignant Peripheral Nerve Sheath Tumor of the Liver.
 

Author(s): Satoru Kakizaki, Norio Horiguchi, Toshiyuki Otsuka, Daichi Takizawa, Yuichi Yamazaki, Ken Sato, Yoshihiro Ohno, Motoyasu Kusano, Masanobu Yamada

Journal: Intern. Med.. 2016 ;55(3):245-9.

 

Malignant peripheral nerve sheath tumor (MPNST) of the liver is rare. Most cases of MPNST are accompanied by neurofibromatosis 1 (NF-1, von Recklinghausen's disease). We herein report an autopsy case of MPNST without NF-1 and review the pertinent literature. The tumor occupied the ...

Last Updated: 2 Feb 2016

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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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Clinical Trial Information This information is provided by ClinicalTrials.gov

Multi-Institutional Registry for Malignant Peripheral Nerve Sheath Tumors
 

Status: Recruiting

Condition Summary: Malignant Peripheral Nerve Sheath Tumors

 

Last Updated: 3 May 2017

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A Study of Pembrolizumab in Patients With Malignant Peripheral Nerve Sheath Tumor (MPNST), Not Eligible for Curative Surgery
 

Status: Recruiting

Condition Summary: Malignant Peripheral Nerve Sheath Tumour (MPNST)

 

Last Updated: 7 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: 9 Jan 2017

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