According to an evaluation of ERN GENTURIS tumour surveillance guidelines for individuals with NF1, 'clinical assessment should include screening for the following clinical features suggestive for a MPNST: rapid tumor growth, substantial pain, new motor deficit/weakness, sensory deficit, sphincter disturbance, swallowing or breathing difficulty, or any changes in tumor consistency'.
MPNTs are sarcomas and display at least partial differentiation towards a connective tissue lineage. Most MPNSTs have nonspecific histologic features, and approximately 5% of NF1-associated MPNSTs are low grade. The lifetime risk of developing MPNST is up to 16%.
Learn more about MPNST.
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Cite this: Heidi Moawad. Fast Five Quiz: Neurofibromatosis Type 1 Signs and Symptoms - Medscape - May 02, 2023.
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