Avelumab for People With Recurrent Respiratory Papillomatosis

Background * Recurrent respiratory papillomatosis (RRP) is a rare papillomatous disease of the aerodigestive tract that is caused by the Human Papilloma Virus (HPV). * RRP can progress to cause airway compromise, fatal pulmonary lesions, and invasive cancers. * There is no effective systemic therapy for RRP. Patients require repeated interventional procedures for disease control. * Study of a small number of RRP samples has shown programmed death-ligand 1 (PD-L1) expression by inflammatory mononuclear cells and by papilloma epithelial cells. * This clinical trial will evaluate the activity of a PD-L1-targeted drug, avelumab, in the treatment of RRP. This drug was selected for its demonstrated activity in a variety of cancers and for its acceptable safety profile. Objective -Determine the complete response rate for avelumab in the treatment of patients with RRP. Eligibility * Histologically confirmed diagnosis of RRP. * One of the following: * A Derkay anatomic score of 10 or greater and a history of two or more endoscopic interventions in the last 12 months for control of RRP. * Pulmonary RRP with pulmonary disease that is measurable by computed tomography scan. * Tracheal involvement with RRP that has required either two or more endoscopic interventions in the last 12 months or a tracheostomy. * Age 18 years or greater. * Eastern Oncology Cooperative Group Performance Score of 0 or 1. Design * Phase II clinical trial * Simon optimal two-stage design with initial enrollment of 12 patients and expansion to 37 patients if one or more complete response(s) is/are observed in the initial patients.