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 98 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: 31 Dec 1969

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Hypoxia-inducible factor 1 alpha is a poor prognostic factor and potential therapeutic target in malignant peripheral nerve sheath tumor.
 

Author(s): Suguru Fukushima, Makoto Endo, Yoshihiro Matsumoto, Jun-Ichi Fukushi, Tomoya Matsunobu, Ken-Ichi Kawaguchi, Nokitaka Setsu, Keiichiro IIda, Nobuhiko Yokoyama, Makoto Nakagawa, Kenichiro Yahiro, Yoshinao Oda, Yukihide Iwamoto, Yasuharu Nakashima

Journal:

 

Malignant peripheral nerve sheath tumor (MPNST) is a rare soft tissue sarcoma with poor prognosis. Hypoxia-inducible factor 1 (HIF-1) plays a crucial role in the cellular response to hypoxia and regulates the expression of multiple genes involved in tumor progression in various cancers. ...

Last Updated: 31 Dec 1969

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Doxycycline potentiates antitumor effect of 5-aminolevulinic acid-mediated photodynamic therapy in malignant peripheral nerve sheath tumor cells.
 

Author(s): Ming-Jen Lee, Shih-Hsuan Hung, Mu-Ching Huang, Tsuimin Tsai, Chin-Tin Chen

Journal:

 

Neurofibromatosis type 1 (NF1) is one of the most common neurocutaneous disorders. Some NF1 patients develop benign large plexiform neurofibroma(s) at birth, which can then transform into a malignant peripheral nerve sheath tumor (MPNST). There is no curative treatment for this rapidly ...

Last Updated: 31 Dec 1969

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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: 31 Dec 1969

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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: 31 Dec 1969

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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: 31 Dec 1969

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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: 16 Oct 2017

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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: 19 Sep 2017

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