Cancer of the Peripheral Nerve in Neurofibromatosis Type 1

Research output: Contribution to journalArticle

Abstract

The RASopathy neurofibromatosis 1 is an autosomal dominant hereditary cancer syndrome that represents a major risk for the development of malignancies, particularly malignant peripheral nerve sheath tumors (MPNSTs). MPNSTs are unique sarcomas that originate from the peripheral nerve and represent the only primary cancer of the peripheral nervous system. To date, surgery is the only treatment modality proven to have survival benefit for MPNSTs and even when maximal surgery is feasible, these tumors are rarely curable, despite the use of chemotherapy and radiation. In this review, we discuss the current state-of-the-art treatments for MPNSTs, latest therapeutic developments, and critical aspects of the underlying molecular and pathophysiology that appear promising for therapeutic developments in the future. In particular, we discuss the specific elements of cancer in the peripheral nerve and how that may impel development of unique therapies for this form of sarcoma.

Original languageEnglish (US)
Pages (from-to)1-9
Number of pages9
JournalNeurotherapeutics
DOIs
StateAccepted/In press - Mar 27 2017

Fingerprint

Neurofibromatosis 1
Peripheral Nerves
Neurilemmoma
Neoplasms
Sarcoma
Hereditary Neoplastic Syndromes
Therapeutics
Peripheral Nervous System
Radiation
Drug Therapy

Keywords

  • Chemoprevention
  • Malignant peripheral nerve sheath tumor
  • Malignant transformation
  • Neurofibromatosis
  • Sarcoma
  • Treatment

ASJC Scopus subject areas

  • Pharmacology
  • Clinical Neurology
  • Pharmacology (medical)

Cite this

@article{c4a613fc3ae842518a1c171b512968a5,
title = "Cancer of the Peripheral Nerve in Neurofibromatosis Type 1",
abstract = "The RASopathy neurofibromatosis 1 is an autosomal dominant hereditary cancer syndrome that represents a major risk for the development of malignancies, particularly malignant peripheral nerve sheath tumors (MPNSTs). MPNSTs are unique sarcomas that originate from the peripheral nerve and represent the only primary cancer of the peripheral nervous system. To date, surgery is the only treatment modality proven to have survival benefit for MPNSTs and even when maximal surgery is feasible, these tumors are rarely curable, despite the use of chemotherapy and radiation. In this review, we discuss the current state-of-the-art treatments for MPNSTs, latest therapeutic developments, and critical aspects of the underlying molecular and pathophysiology that appear promising for therapeutic developments in the future. In particular, we discuss the specific elements of cancer in the peripheral nerve and how that may impel development of unique therapies for this form of sarcoma.",
keywords = "Chemoprevention, Malignant peripheral nerve sheath tumor, Malignant transformation, Neurofibromatosis, Sarcoma, Treatment",
author = "Verena Staedtke and Renyuan Bai and Jaishri Blakeley",
year = "2017",
month = "3",
day = "27",
doi = "10.1007/s13311-017-0518-y",
language = "English (US)",
pages = "1--9",
journal = "Neurotherapeutics",
issn = "1933-7213",
publisher = "Springer New York",

}

TY - JOUR

T1 - Cancer of the Peripheral Nerve in Neurofibromatosis Type 1

AU - Staedtke, Verena

AU - Bai, Renyuan

AU - Blakeley, Jaishri

PY - 2017/3/27

Y1 - 2017/3/27

N2 - The RASopathy neurofibromatosis 1 is an autosomal dominant hereditary cancer syndrome that represents a major risk for the development of malignancies, particularly malignant peripheral nerve sheath tumors (MPNSTs). MPNSTs are unique sarcomas that originate from the peripheral nerve and represent the only primary cancer of the peripheral nervous system. To date, surgery is the only treatment modality proven to have survival benefit for MPNSTs and even when maximal surgery is feasible, these tumors are rarely curable, despite the use of chemotherapy and radiation. In this review, we discuss the current state-of-the-art treatments for MPNSTs, latest therapeutic developments, and critical aspects of the underlying molecular and pathophysiology that appear promising for therapeutic developments in the future. In particular, we discuss the specific elements of cancer in the peripheral nerve and how that may impel development of unique therapies for this form of sarcoma.

AB - The RASopathy neurofibromatosis 1 is an autosomal dominant hereditary cancer syndrome that represents a major risk for the development of malignancies, particularly malignant peripheral nerve sheath tumors (MPNSTs). MPNSTs are unique sarcomas that originate from the peripheral nerve and represent the only primary cancer of the peripheral nervous system. To date, surgery is the only treatment modality proven to have survival benefit for MPNSTs and even when maximal surgery is feasible, these tumors are rarely curable, despite the use of chemotherapy and radiation. In this review, we discuss the current state-of-the-art treatments for MPNSTs, latest therapeutic developments, and critical aspects of the underlying molecular and pathophysiology that appear promising for therapeutic developments in the future. In particular, we discuss the specific elements of cancer in the peripheral nerve and how that may impel development of unique therapies for this form of sarcoma.

KW - Chemoprevention

KW - Malignant peripheral nerve sheath tumor

KW - Malignant transformation

KW - Neurofibromatosis

KW - Sarcoma

KW - Treatment

UR - http://www.scopus.com/inward/record.url?scp=85016095588&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85016095588&partnerID=8YFLogxK

U2 - 10.1007/s13311-017-0518-y

DO - 10.1007/s13311-017-0518-y

M3 - Article

C2 - 28349408

AN - SCOPUS:85016095588

SP - 1

EP - 9

JO - Neurotherapeutics

JF - Neurotherapeutics

SN - 1933-7213

ER -