Fast Five Quiz: Neurofibromatosis Type 1 Signs and Symptoms

Heidi Moawad, MD


May 02, 2023

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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