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

Tumor expression of survivin, p53, cyclin D1, osteopontin and fibronectin in predicting the response to neo-adjuvant chemotherapy in children with advanced malignant peripheral nerve sheath tumor.
 

Author(s): Gabrielle Karpinsky, Malgorzata A Krawczyk, Ewa Izycka-Swieszewska, Aleksandra Fatyga, Agnieszka Budka, Walentyna Balwierz, Grazyna Sobol, Beata Zalewska-Szewczyk, Magdalena Rychlowska-Pruszynska, Teresa Klepacka, Bozenna Dembowska-Baginska, Bernarda Kazanowska, Anna Gabrych, Ewa Bien

Journal: J. Cancer Res. Clin. Oncol.. 2018 Mar;144(3):519-529.

 

Selected cell-cycle regulators and extracellular matrix proteins were found to play roles in malignant peripheral nerve sheath tumor (MPNST) biology. We aimed to analyze whether initial tumor tissue expressions of survivin, p53, cyclin D1, osteopontin (OPN) and fibronectin (FN) correlate ...

Last Updated: 31 Dec 1969

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Tumor targeted delivery of doxorubicin in malignant peripheral nerve sheath tumors.
 

Author(s): A B Madhankumar, Oliver D Mrowczynski, Becky Slagle-Webb, Vagisha Ravi, Alexandre J Bourcier, Russell Payne, Kimberly S Harbaugh, Elias Rizk, James R Connor

Journal:

 

Peripheral nerve sheath tumors are benign tumors that have the potential to transform into malignant peripheral nerve sheath tumors (MPNSTs). Interleukin-13 receptor alpha 2 (IL13Rα2) is a cancer associated receptor expressed in glioblastoma and other invasive cancers. We analyzed ...

Last Updated: 31 Dec 1969

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[A malignant peripheral nerve sheath tumor developed from the auditory nerve: a case report and a literature review].
 

Author(s): V N Shimanskiy, K V Shevchenko, M V Ryzhova, S V Tanyashin, D A Odamanov, V K Poshataev

Journal: Zh Vopr Neirokhir Im N N Burdenko. ;81(4):95-100.

 

We present a rare clinical case of a patient with a malignant peripheral nerve sheath tumor developed from the auditory nerve as well as a literature review, including 30 reported cases of this disease.

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

Targeted next-generation sequencing of malignant peripheral nerve sheath tumor of the pterygopalatine fossa with intracranial metastatic recurrence.
 

Author(s): Xinjie Bao, Xiangyi Kong, Chengxian Yang, Huanwen Wu, Wenbin Ma, Renzhi Wang

Journal: Medicine (Baltimore). 2018 Jan;97(4):e9636.

 

Malignant peripheral nerve sheath tumor (MPNST) is an uncommon neoplasm that rarely involves the head and neck region. Intracranial MPNSTs unrelated to cranial nerves are highly malignant tumors with poor overall survival, probably because of infiltrating growth into surrounding brain ...

Last Updated: 31 Dec 1969

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Unusual primary breast cancer - malignant peripheral nerve sheath tumor: a case report and review of the literature.
 

Author(s): Md Shuayb, Rabeya Begum

Journal:

 

Sarcomas are a rare type of breast malignancies and malignant peripheral nerve sheath tumors of the breast are even rarer. There are no specific clinical and radiological features for the diagnosis of this tumor and histological features are also reported to be nonspecific. Therefore, ...

Last Updated: 31 Dec 1969

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Histopathologic evaluation of atypical neurofibromatous tumors and their transformation into malignant peripheral nerve sheath tumor in patients with neurofibromatosis 1-a consensus overview.
 

Author(s): Markku M Miettinen, Cristina R Antonescu, Christopher D M Fletcher, Aerang Kim, Alexander J Lazar, Martha M Quezado, Karlyne M Reilly, Anat Stemmer-Rachamimov, Douglas R Stewart, David Viskochil, Brigitte Widemann, Arie Perry

Journal: Hum. Pathol.. 2017 09;67():1-10.

 

Patients with neurofibromatosis 1 (NF1) develop multiple neurofibromas, with 8% to 15% of patients experiencing malignant peripheral nerve sheath tumor (MPNST) during their lifetime. Prediction of transformation, typically from plexiform neurofibroma, is clinically and histologically ...

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: 18 Sep 2018

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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: 30 Jan 2018

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