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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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 105 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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Successful treatment with doxorubicin and ifosfamide for mediastinal malignant peripheral nerve sheath tumor with loss of H3K27me3 expression.
 

Author(s): Noriko Seno, Toshirou Fukushima, Daisuke Gomi, Takashi Kobayashi, Nodoka Sekiguchi, Hidehiro Matsushita, Takesumi Ozawa, Yoshiko Tsukahara, Keiko Mamiya, Tomonobu Koizumi, Kenji Sano

Journal: Thorac Cancer. 2017 11;8(6):720-723.

 

Malignant peripheral nerve sheath tumor (MPNST) in the thorax is an extremely rare disease, and half of all cases of MPNST are associated with neurofibromatosis type I. Sporadic intrathoracic MPNST is difficult to diagnose and treat. Because of the rarity of intrathoracic MPNST, the ...

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 13 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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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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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: 17 Apr 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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