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Table 1 Factors associated with a poor MPNST prognosis

From: Neurofibromatosis 1 French national guidelines based on an extensive literature review since 1966

Factors associated with a poor MPNST prognosis

 Clinical and radiological

Site: axial/trunk [99,100,101]

More than one primary tumor [102]

Larger tumor size [99, 101,102,103]

 Histological

High histological grade [101, 102]

 Genotypic

Telomerase activity and overexpression of TERT[104]

Genomic changes in chromosomes 10, 16, and X [105]

  1. TERT Telomerase reverse transcriptase
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Orphanet Journal of Rare Diseases

ISSN: 1750-1172