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Atypical Neurofibromatous Neoplasm of Uncertain Biologic Potential

MedGen UID:
1790585
Concept ID:
C5552715
Neoplastic Process
Synonyms: Atypical Neurofibroma; Atypical Neurofibromatous Neoplasm with Uncertain Biologic Potential

Definition

A premalignant neurofibromatous neoplasm characterized by the presence of at least two of the following features: cytologic atypia, hypercellularity, less than 3 mitotic figures per 10 HPFs, and loss of neurofibroma architecture. It is associated with an increased risk of progression to malignant peripheral nerve sheath tumor. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAtypical Neurofibromatous Neoplasm of Uncertain Biologic Potential

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