scholarly journals Trends in systemic therapy for malignant peripheral nerve sheath tumors

2019 ◽  
Vol 2 (2) ◽  
pp. 139-142
Author(s):  
Lucian Alecu ◽  
Cristina Orlov-Slavu ◽  
Adrian Tulin ◽  
Cornelia Niţipir
Keyword(s):  
Targeted Therapy ◽  
Soft Tissue ◽  
Soft Tissue Sarcoma ◽  
Peripheral Nerve ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath ◽  
Type 1 Neurofibromatosis ◽  
Selection Of

AbstractMalignant peripheral nerve sheath tumors are a type of soft tissue sarcoma deriving from Schwann cells that usually appear in type 1 neurofibromatosis patients but also sporadically. Tumors frequently interest the nerves in the limbs. They represent a therapeutical challenge due to difficulty of resection and relative radio-resistance and chemo-resistance. The paper aims to describe targeted therapy used in this setting and news concerning the molecular changes that lead to carcinogenesis initiation and promotion. A short selection of literature data was made using PRISMA criteria on this topic. However, due to the rarity and heterogeneity of these tumors, personalized treatment is necessary.

Hybrid Peripheral Nerve Sheath Tumors, Including a Malignant Variant in Type 1 Neurofibromatosis

2013 ◽  
Vol 35 (6) ◽  
pp. 641-649 ◽  
Author(s):  
Denisa Kacerovska ◽  
Michal Michal ◽  
Naoto Kuroda ◽  
Azusa Tanaka ◽  
Radek Sima ◽  
...  
Keyword(s):  
Peripheral Nerve ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath ◽  
Type 1 Neurofibromatosis

scholarly journals New Model Systems and the Development of Targeted Therapies for the Treatment of Neurofibromatosis Type 1-Associated Malignant Peripheral Nerve Sheath Tumors

Genes ◽  
2020 ◽  
Vol 11 (5) ◽  
pp. 477 ◽  
Author(s):  
Kyle B. Williams ◽  
David A. Largaespada
Keyword(s):  
Schwann Cell ◽  
Peripheral Nerve ◽  
Neurofibromatosis Type 1 ◽  
Single Agent ◽  
Neurofibromatosis Type ◽  
Mek Inhibitors ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath

Neurofibromatosis Type 1 (NF1) is a common genetic disorder and cancer predisposition syndrome (1:3000 births) caused by mutations in the tumor suppressor gene NF1. NF1 encodes neurofibromin, a negative regulator of the Ras signaling pathway. Individuals with NF1 often develop benign tumors of the peripheral nervous system (neurofibromas), originating from the Schwann cell linage, some of which progress further to malignant peripheral nerve sheath tumors (MPNSTs). Treatment options for neurofibromas and MPNSTs are extremely limited, relying largely on surgical resection and cytotoxic chemotherapy. Identification of novel therapeutic targets in both benign neurofibromas and MPNSTs is critical for improved patient outcomes and quality of life. Recent clinical trials conducted in patients with NF1 for the treatment of symptomatic plexiform neurofibromas using inhibitors of the mitogen-activated protein kinase (MEK) have shown very promising results. However, MEK inhibitors do not work in all patients and have significant side effects. In addition, preliminary evidence suggests single agent use of MEK inhibitors for MPNST treatment will fail. Here, we describe the preclinical efforts that led to the identification of MEK inhibitors as promising therapeutics for the treatment of NF1-related neoplasia and possible reasons they lack single agent efficacy in the treatment of MPNSTs. In addition, we describe work to find targets other than MEK for treatment of MPNST. These have come from studies of RAS biochemistry, in vitro drug screening, forward genetic screens for Schwann cell tumors, and synthetic lethal screens in cells with oncogenic RAS gene mutations. Lastly, we discuss new approaches to exploit drug screening and synthetic lethality with NF1 loss of function mutations in human Schwann cells using CRISPR/Cas9 technology.

scholarly journals Brain Metastasis in Bone and Soft Tissue Cancers: A Review of Incidence, Interventions, and Outcomes

Sarcoma ◽  
2014 ◽  
Vol 2014 ◽  
pp. 1-19 ◽  
Author(s):  
Faris Shweikeh ◽  
Laura Bukavina ◽  
Kashif Saeed ◽  
Reem Sarkis ◽  
Aarushi Suneja ◽  
...  
Keyword(s):  
Soft Tissue ◽  
Brain Metastasis ◽  
Brain Metastases ◽  
Peripheral Nerve ◽  
Ewing’S Sarcoma ◽  
Ewing's Sarcoma ◽  
Systemic Disease ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath

Bone and soft tissue malignancies account for a small portion of brain metastases. In this review, we characterize their incidence, treatments, and prognosis. Most of the data in the literature is based on case reports and small case series. Less than 5% of brain metastases are from bone and soft tissue sarcomas, occurring most commonly in Ewing’s sarcoma, malignant fibrous tumors, and osteosarcoma. Mean interval from initial cancer diagnosis to brain metastasis is in the range of 20–30 months, with most being detected before 24 months (osteosarcoma, Ewing sarcoma, chordoma, angiosarcoma, and rhabdomyosarcoma), some at 24–36 months (malignant fibrous tumors, malignant peripheral nerve sheath tumors, and alveolar soft part sarcoma), and a few after 36 months (chondrosarcoma and liposarcoma). Overall mean survival ranges between 7 and 16 months, with the majority surviving < 12 months (Ewing’s sarcoma, liposarcoma, malignant fibrous tumors, malignant peripheral nerve sheath tumors, angiosarcoma and chordomas). Management is heterogeneous involving surgery, radiosurgery, radiotherapy, and chemotherapy. While a survival advantage may exist for those given aggressive treatment involving surgical resection, such patients tended to have a favorable preoperative performance status and minimal systemic disease.

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