Juvenile Onset Recurrent Respiratory Papillomatosis (JORRP) is a rare condition characterized by recurrent growth of squamous papillomas in the upper and lower airways of children younger than twelve years old. It is caused by the cytopathogenic effect of human papilloma virus (HPV) 6, 11, 16/18 and 31/35/51, which is transmitted from active vaginal lesions of the infected mother at the time of birth. JORRP has a prevalence of 1.7–2.6/100,000 children. It typically presents with hoarseness which can progress to stridor. Multiple surgical interventions are often required to achieve local control of the disease and avoid airway obstruction. Involvement of the upper airway is common, especially the larynx. However, papillomas can also spread to the distal trachea and lung parenchyma in 5% of the cases. Several cases of malignant transformation have been reported in patients with JORRP in the setting of concomitant risk factors such as radiation or smoking. However, only few cases have been published describing malignant transformation of papillomas of the lung in the absence of these risks factors. We present a case of a 19 year-old woman with history of JORRP, who developed squamous cell carcinoma of the lung after being in clinical remission since the age of 5.
The patient was a 19 year-old female with history of JORRP who throughout her infancy required several laser interventions for treatment of papillomas of the upper airway. Her last intervention was at age four and her last normal bronchoscopy was at five years of age. She was a non-smoker and was never exposed to radiation. Throughout her adolescence she noticed occasional bronchitis symptoms that were treated symptomatically but no radiologic or invasive studies were performed. Ten month prior to admission she started to complain of frequent thoracic back and pleuritic chest pains. She developed chronic cough, dyspnea on exertion, and was treated with antibiotics for left lower lobe pneumonia. She eventually developed respiratory distress and was emergently intubated. On the day of admission a CT of the chest demonstrated complete collapse of the left lung, a 4.5 cm lower lobe mass and destruction of the fifth and sixth vertebral bodies (Figure 1). Bronchoscopy showed a 1 × 1 cm polyp in the left main stem bronchus, and a biopsy was obtained revealing dysplastic squamous epithelium. Subsequent pleural biopsy from Video-Assisted Thoracic Surgery showed a well differentiated squamous cell carcinoma and she was stratified as T4-N2-MO lung cancer (Figure 2).
We present a case of a 19 year-old female with JORRP, who presented with squamous cell carcinoma of the lung in the absence of smoking or radiation. Prior reported cases of squamous cell carcinoma of the lung in adult patients with JORRP presented after many year of relapsing disease and frequent surgical interventions. In contrast, this patient presented after fourteen years of apparent clinical remission since the age of five. This case highlights that malignant transformation of papillomatous lesions of childhood JORRP is possible even after prolonged clinical remission.
Malignant transformation of benign childhood papillomatosis can occur even in the presence of apparent clinical remission. Development of surveillance guidelines to guide long term follow-up of patients with JORRP is required to facilitate early diagnosis and treatment of malignant transformation.
Natalia Moguillansky, No Financial Disclosure Information; No Product/Research Disclosure Information