Malignant degeneration of pulmonary juvenile-onset recurrent respiratory papillomatosis

Juvenile-onset recurrent respiratory papillomatosis (JORRP) is a rare disease associated with the human papilloma virus (HPV) in which papillomas form along the aerodigestive tract in children. Pulmonary involvement is uncommon, but associated with worse clinical outcomes, including the rare complication of malignant transformation. We present a patient with JORRP in which lung disease underwent malignant transformation during adolescence. Our goal is to raise awareness of the potential for malignant transformation in children, as well as to familiarize pediatric radiologists with imaging features of malignant lung disease in JORRP. We advocate for the identification of the subgroup of JORPP patients with pulmonary disease who, due to increased risk for malignant transformation, may benefit from closer clinical and imaging surveillance by a multidisciplinary team.