Juvenile-onset recurrent respiratory papillomatosis with pulmonary involvement and carcinomatous transformation

Abstract

Papilloma associated with recurrent respiratory papillomatosis (RRP), caused by human papilloma virus (HPV) infection types 6 and 11, is the most common benign neoplasm of the larynx. The clinical features of RRP vary widely from mild to aggressive forms. RRP in children is known as juvenile-onset recurrent respiratory papillomatosis (JORRP). Its outcome may be poor or even fatal due to the high rate of recurrence and eventual spread to the entire respiratory tract. Pulmonary invasion is reported to occur in 3.3% of patients with RRP, and malignant transformation in 0.5% of patients. We report the case of a 39-year-old female patient with a diagnosis of JORRP from the age of 3 years, with extensive bilateral pulmonary involvement and malignant transformation. Analysis of the papilloma and carcinomatous tissues revealed the presence of HPV type 11, which is associated with rapid and aggressive progression. We discussed the case on the basis of a literature review on pulmonary invasion, malignant transformation, and HPV 11 aggressiveness.

Keywords: Carcinoma, Squamous Cell; Human Papillomavirus 11; Lung Neoplasms; Papillomavirus Infections; Recurrent Respiratory Papillomatosis.

Figure 1. Gross view of the larynx with multiple polypoid projections in the larynx.

Figure 2. Photomicrography of the laryngeal papilloma with evident koilocytotic atypia (H&E, 150X).

Figure 3. Gross view of the mediastinal organs showing lungs largely replaced bilaterally by multiple confluent and partially necrotic nodules.

Figure 4. Photomicrography of the lung showing invasive well differentiated squamous cell carcinoma of the lung (H&E, 50X).