Overview

NCI Definition: An uncommon, highly aggressive malignant tumor, arising from the peripheral nerves and affecting mostly adults in their third to sixth decades of life. It usually occurs in medium-sized and large nerves of the buttock, thigh, upper arm, or the paraspinal region. It may be associated with neurofibromatosis 1 (NF1). [1]

Malignant peripheral nerve sheath tumors most frequently harbor alterations in CDKN2A, NF1, CDKN2B, KMT2D, and TP53 [2].

Most Commonly Altered Genes in Malignant Peripheral Nerve Sheath Tumor

CDKN2A Loss, CDKN2B Loss, NF1 Mutation, NF1 Nonsense, and NF1 Frameshift are the most common alterations in malignant peripheral nerve sheath tumor [2].

Top Alterations in Malignant Peripheral Nerve Sheath Tumor

Significant Genes in Malignant Peripheral Nerve Sheath Tumor

ARAF +

BRAF +

CRKL +

HRAS +

KRAS +

MAP2K1 +

MAP2K2 +

MAP2K4 +

MAP3K1 +

MAPK1 +

NF1 +

NRAS +

PIK3CA +

PTEN +

RAF1 +

SRC +

Disease Details

Synonyms
Malignant Neurilemoma, Malignant Tumor of Peripheral Nerve Sheath, Malignant Schwannoma, Malignant Neoplasm of Peripheral Nerve Sheath, Neurogenic Sarcoma, PERIPHERAL NERVE SHEATH TUMOR, MALIGNANT, Malignant Neurilemmoma, Malignant Neoplasm of the Peripheral Nerve Sheath, Neurofibrosarcoma, Malignant, Malignant Peripheral Nerve Sheath Tumour, Malignant Peripheral Nerve Sheath Neoplasm, Malignant Tumor of the Peripheral Nerve Sheath, Neurofibrosarcoma, Malig. periph. nerve sheath tum., Soft tissue malignant peripheral nerve sheath tumor (MPNST), MPNST
Parent(s)
Sarcoma
Children
Malignant Triton Tumor, Malignant Melanotic Peripheral Nerve Sheath Tumor, Adult Malignant Peripheral Nerve Sheath Tumor, Epithelioid Malignant Peripheral Nerve Sheath Tumor, Malignant Cardiac Peripheral Nerve Sheath Tumor, Glandular Malignant Peripheral Nerve Sheath Tumor, Childhood Malignant Peripheral Nerve Sheath Tumor, and Malignant Melanotic Peripheral Nerve Sheath Tumor of the Mediastinum
OncoTree Name
Malignant Peripheral Nerve Sheath Tumor
OncoTree Code
MPNST

References

1. National Cancer Institute. NCI Thesaurus Version 18.11d. https://ncit.nci.nih.gov/ncitbrowser/ [2018-08-28]. [2018-09-21].

2. The AACR Project GENIE Consortium. AACR Project GENIE: powering precision medicine through an international consortium. Cancer Discovery. 2017;7(8):818-831. Dataset Version 6. This dataset does not represent the totality of the genetic landscape; see paper for more information.

3. All assertions and clinical trial landscape data are curated from primary sources. You can read more about the curation process here.