Examining perceived cancer risk among patients with neurofibromatosis type 1

Neurofibromatosis 1 (NF1) is a genetic disorder in which patients are at significantly increased risk for developing malignant peripheral nerve sheath tumors (MPNST) and malignant gliomas (brain cancer). We sought to develop a measure for assessing perceived risk of developing MPNST and brain cancer among patients with NF1 and to examine patients’ perceived risk of developing these cancers. We assessed 112 NF1 patients’ perceived risk of developing MPNST and brain cancer using an 8-item scale we developed that yielded two subscales in a principal component analysis (PCA). Linear regression models examined factors associated with perceived risk of malignancy. 33.9 % and 47.3 % of patients disagreed that having NF1 placed them at increased risk for MPNST and brain cancer, respectively. The PCA of the perceived risk items yielded a 2-factor solution with an MPNST and a brain subscale (total scale α = 0.90). Level of anxiety was the primary factor associated with perceived risk for both cancers. A significant proportion of NF1 patients underestimate their risk of developing MPNST and brain cancer. Perceived risk was associated with emotional distress, in particular anxiety. Clinicians should actively communicate with NF1 patients about their elevated cancer risk.