Atypical neurofibromas in neurofibromatosis type 1 are premalignant tumors.

  title={Atypical neurofibromas in neurofibromatosis type 1 are premalignant tumors.},
  author={Eline Beert and Hilde Brems and Bruno Dani{\"e}ls and Ivo de Wever and Frank van Calenbergh and Joseph Schoenaers and Maria Debiec-Rychter and Olivier Gevaert and Thomas de Raedt and Annick Van den Bruel and Thomy de Ravel and Karen Cichowski and Lan Kluwe and Victor -F. Mautner and Rafa{\"e}l Sciot and Eric Legius},
  journal={Genes, chromosomes & cancer},
  volume={50 12},
Benign peripheral nerve sheath tumors (PNSTs) are a characteristic feature of neurofibromatosis type I (NF1) patients. NF1 individuals have an 8-13% lifetime risk of developing a malignant PNST (MPNST). Atypical neurofibromas are symptomatic, hypercellular PNSTs, composed of cells with hyperchromatic nuclei in the absence of mitoses. Little is known about the origin and nature of atypical neurofibromas in NF1 patients. In this study, we classified the atypical neurofibromas in the spectrum of… CONTINUE READING
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