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

Gabriel Lorente Mitsumoto,Barbara Mello,Luisa Lina Villa,Fabiola Del Carlo Bernardi,Geanete Pozzan

doi:10.4322/acr.2018.035

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.

Highlights

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
She denied smoking and drinking alcoholics. She started presenting with shortness of breath and chest pain over the last three years, when the diagnosis of the pulmonary involvement by the papillomatosis was made with the aid of a thoracic computed tomography (CT)
The course of the recurrent respiratory papillomatosis (RRP) is unpredictable, as some patients present a mild form with spontaneous recovery or cure after a single exeresis of the laryngeal papilloma, while others present an aggressive form of the disease with many recurrences requiring multiple interventions and hospitalizations.[4,5]

Summary

Introduction

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.

Results

Conclusion