Neurofibromatosis Type 1-Associated MPNST State of the Science: Outlining a Research Agenda for the Future.
J Natl Cancer Inst
; 109(8)2017 08 01.
Article
en En
| MEDLINE
| ID: mdl-29117388
ABSTRACT
Malignant peripheral nerve sheath tumor (MPNST) is an aggressive soft tissue sarcoma for which the only effective therapy is surgery. In 2016, an international meeting entitled "MPNST State of the Science Outlining a Research Agenda for the Future" was convened to establish short- and long-term research priorities. Key recommendations included the 1) development of standardized, cost-efficient fluorodeoxyglucose positron emission tomography and whole-body magnetic resonance imaging guidelines to evaluate masses concerning for MPNST; 2) development of better understanding and histologic criteria for the transformation of a plexiform neurofibroma to MPNST; 3) establishment of a centralized database to collect genetic, genomic, histologic, immunohistochemical, molecular, radiographic, treatment, and related clinical data from MPNST subspecialty centers in a standardized manner; 4) creation of accurate mouse models to study the plexiform neurofibroma-to-MPNST transition, MPNST metastasis, and drug resistance; 5) use of trial designs that minimize regulatory requirements, maximize availability to patients, consider novel secondary end points, and study patients with newly diagnosed disease. Lastly, in order to minimize delays in developing novel therapies and promote the most efficient use of research resources and patient samples, data sharing should be incentivized.
Texto completo:
1
Bases de datos:
MEDLINE
Asunto principal:
Neurofibromatosis 1
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Neoplasias de la Vaina del Nervio
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Terapias en Investigación
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Investigación Biomédica
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Neurilemoma
Tipo de estudio:
Diagnostic_studies
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Guideline
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Prognostic_studies
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Risk_factors_studies
Límite:
Humans
Idioma:
En
Revista:
J Natl Cancer Inst
Año:
2017
Tipo del documento:
Article