Unusual malignant transformation of juvenile recurrent respiratory papillomatosis

Abstract

Recurrent respiratory papillomatosis (RRP) is a chronic upper respiratory condition characterized by the occurrence of diffuse multiple recurring papillomas in the respiratory tract of both adult and children. RRP is the most common benign lesion of the larynx in children. It is usually a self-limiting benign disease but may have a fatal outcome due to pulmonary spread and airway compromise. Rarely, these papillomata may become invasive or undergo spontaneous malignant transformation. The disease therefore presents a perplexing problem to both the patient and the otolaryngologist. We document here a case of human papillomavirus (HPV) type 16 associated RRP in a 12-year-old nonirradiated male, where the lesion underwent a rapid spontaneous malignant transformation within eight months time. The probability of two pathoses coexisting is possible as the cytologic/histologic progression from benign papilloma to invasive tumor could not be proved. The tumoral origin in fresh laryngeal mucosa in close neighborhood to papilloma is less probable albeit not ruled out. Unusual presentation and rarity of this lesion made us report this case. A 12-year-old boy presented with progressive hoarseness for six months and swelling of the neck for one-month duration. Indirect laryngoscopic examination revealed numerous glottic and supraglottic papillomas involving left hemilarynx with mobile cords. There was an associated 1 x 1 cm tender swelling over the left thyroid ala. Computed tomography (CT) revealed a soft tissue mass extending from the supraglottis to the glottis causing asymmetry of the vestibule, displacing the paralaryngeal fat laterally. The subglottic region was free of disease. The child underwent microlaryngoscopic surgery and papillomas were removed. At the end of procedure the larynx looked normal with a minimal bulge in the region of the left false cord, both true cords were.