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, SUZ12, and KMT2D [2].

Most Commonly Altered Genes in Malignant Peripheral Nerve Sheath Tumor

CDKN2A Loss, CDKN2B Loss, NF1 Mutation (germline), NF1 Mutation, and NF1 Nonsense 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
Neurogenic Sarcoma, Malignant Peripheral Nerve Sheath Tumour, Malignant Tumor of the Peripheral Nerve Sheath, Malignant Peripheral Nerve Sheath Neoplasm, Malignant Schwannoma, Malignant Neoplasm of the Peripheral Nerve Sheath, Malignant Neurilemmoma, Malig. periph. nerve sheath tum., Malignant Tumor of Peripheral Nerve Sheath, MPNST, Neurofibrosarcoma, Malignant, PERIPHERAL NERVE SHEATH TUMOR, MALIGNANT, Malignant Neurilemoma, Neurofibrosarcoma, Malignant Neoplasm of Peripheral Nerve Sheath
Parent(s)
Sarcoma
Children
Malignant Melanotic Peripheral Nerve Sheath Tumor, Adult Malignant Peripheral Nerve Sheath Tumor, Epithelioid Malignant Peripheral Nerve Sheath Tumor, Malignant Cardiac Peripheral Nerve Sheath Tumor, Malignant Melanotic Peripheral Nerve Sheath Tumor of the Mediastinum, Childhood Malignant Peripheral Nerve Sheath Tumor, Malignant Triton Tumor, and Glandular Malignant Peripheral Nerve Sheath Tumor
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 4. 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.