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

Multi-centric spinal extradural malignant peripheral nerve sheath tumor: a case report.
 

Author(s): Harsimrat Bir Singh Sodhi, Ankur Kapoor, Pravin Salunke, B D Radotra

Journal: Neurol India. ;62(6):675-7.

 

Last Updated: 16 Jan 2015

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BNIP3 regulates AT101 [(-)-gossypol] induced death in malignant peripheral nerve sheath tumor cells.
 

Author(s): Niroop Kaza, Latika Kohli, Christopher D Graham, Barbara J Klocke, Steven L Carroll, Kevin A Roth

Journal:

 

Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive Schwann cell-derived sarcomas and are the leading cause of mortality in patients with neurofibromatosis type 1 (NF1). Current treatment modalities have been largely ineffective, resulting in a high rate of MPNST recurrence ...

Last Updated: 14 May 2014

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Malignant peripheral nerve sheath tumor of adrenal gland with heterologus osseous differentiation in a case of Von Recklinghausen's disease.
 

Author(s): Manas R Baisakh, Nachiketa Mohapatra, Samiran D Adhikary, Debasis Routray

Journal: Indian J Pathol Microbiol. ;57(1):130-2.

 

Malignant peripheral nerve sheath tumor (MPNST) of the adrenal gland is extremely rare. Most of them occur in association with neurofibromatosis, ganglioneuroma or as part of a composite tumor such as pheochromocytoma. Only seven cases of MPNST of the adrenal gland have been reported ...

Last Updated: 17 Apr 2014

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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 6 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

Study of Everolimus With Bevacizumab to Treat Refractory Malignant Peripheral Nerve Sheath Tumors
 

Status: Recruiting

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

 

Last Updated: 29 Sep 2014

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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: 13 Nov 2014

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Radiation Therapy With or Without Combination Chemotherapy or Pazopanib Hydrochloride Before Surgery in Treating Patients With Newly Diagnosed Non-Rhabdomyosarcoma Soft Tissue Sarcomas That Can Be Removed by Surgery
 

Status: Recruiting

Condition Summary: Adult Fibrosarcoma; Alveolar Soft Part Sarcoma; Angiomatoid Fibrous Histiocytoma; Atypical Fibroxanthoma; Clear Cell Sarcoma of Soft Tissue; Epithelioid Malignant Peripheral Nerve Sheath Tumor; Epithelioid Sarcoma; Extraskeletal Myxoid Chondrosarcoma; Extraskeletal Osteosarcoma; Fibrohistiocytic Neoplasm; Glomus Tumor of the Skin; Inflammatory Myofibroblastic Tumor; Intimal Sarcoma; Leiomyosarcoma; Liposarcoma; Low Grade Fibromyxoid Sarcoma; Low Grade Myofibroblastic Sarcoma; Malignant Cutaneous Granular Cell Tumor; Malignant Peripheral Nerve Sheath Tumor; Malignant Triton Tumor; Mesenchymal Chondrosarcoma; Myxofibrosarcoma; Myxoid Chondrosarcoma; Myxoinflammatory Fibroblastic Sarcoma; Neoplasm With Perivascular Epithelioid Cell Differentiation; Nerve Sheath Neoplasm; Pericytic Neoplasm; Plexiform Fibrohistiocytic Tumor; Sclerosing Epithelioid Fibrosarcoma; Stage IB Soft Tissue Sarcoma; Stage IIB Soft Tissue Sarcoma; Stage III Soft Tissue Sarcoma; Stage IV Soft Tissue Sarcoma; Synovial Sarcoma; Unclassified Pleomorphic Sarcoma of Bone; Undifferentiated (Embryonal) Sarcoma

 

Last Updated: 16 Apr 2015

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