Malignant Transformation and Distal Airway Complications
Recurrent respiratory papillomatosis (RRP) are benign neoplasms of the larynx caused by low-risk human papillomavirus (HPV) viral subtypes, predominantly 6 and 11. Extension beyond the larynx and transformation to dysplasia and invasive carcinoma have been described but the incidence is rare. Overall, juvenile-onset RRP tends to be more aggressive than the adult form, and a higher proportion of children will experience these life-threatening complications. Children are more likely to require a tracheotomy, develop distal spread of RRP, and experience pulmonary complications such as abscess, pneumatocele, and respiratory failure. Patients diagnosed as adults are more likely to develop laryngeal dysplasia but have a far smaller chance of developing pulmonary complications. Treatment strategies for benign papilloma continue to hinge on surgical removal, with considerations for adjuvant therapy when airway obstruction is imminent, frequency of surgery is intolerable, or distal spread is diagnosed. The most common adjuvant therapies have been interferon and cidofovir. The most recent treatments to show promise include HPV vaccination and bevacizumab, a monoclonal antibody that targets angiogenesis by binding to vascular endothelial growth factor. The development of pulmonary papillomatosis and malignant transformation portends a far more dire prognosis.
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