A CASE OF RECURRENT RESPIRATORY PAPILLOMATOSIS PROGRESSING TO SQUAMOUS CELL CARCINOMA

Abstract

SESSION TITLE: Lung Cancer SESSION TYPE: Fellow Case Report Posters PRESENTED ON: 10/09/2018 01:15 PM - 02:15 PM INTRODUCTION: Recurrent respiratory papillomatosis (RRP) is an uncommon viral disease caused by human papillomavirus (HPV) types 6 and 11. It is characterized by the proliferation of benign squamous papillomas within the aerodigestive tract. It has an unpredictable clinical course with the tendency to recur and spread throughout the gastrointestinal and respiratory tract. In some cases it can undergo malignant transformation. It usually presents with changes in voice and stridor. We present a case of RPR with malignant transformation. CASE PRESENTATION: An 80-year-old Hispanic woman presented to our pulmonary clinic complaining of progressive hoarseness and stridor over the past 3-4 months. At the moment of presentation she denied having any constitutional symptoms, sputum production or hemoptysis. She is a lifetime non-smoker without significant exposures or travel history. She had a past medical history significant for RPR. On examination she was afebrile, well appearing with normal vital signs, pulmonary, cardiac and abdominal examination. Laboratory data was unrevealing. A computed tomography (CT) of the chest showed several cavitating and non-cavitating pulmonary nodules bilaterally, as well as soft tissue nodules in the tracheal walls, which were suggestive of papillomas (figure 1). The patient underwent laryngeal and tracheal ablation and dilation (figure 2). On repeated CT scan of the chest there was an enlarged right lower lobe cavitary lesion. The patient underwent CT guided lung biopsy that showed squamous cell carcinoma, P16 positive, suggesting malignant conversion of RRP. Positron emission tomography (PET) CT showed metastatic disease involving bilateral lungs. She was started on chemotherapy with carboplatin and gemcitabine and was followed by interventional pulmonology for recurrent dilations and ablation procedures for symptom relief. DISCUSSION: Surgical treatment is the mainstay of therapy for RPR with the goal of complete removal of papillomas and preservation of normal structures while avoiding complications. Other emerging surgical techniques and adjuvant treatment modalities are being used such as the carbodioxide (CO2) laser, microdebriders and Argon Plasma Coagulation (APC). Adjuvant therapy may be needed in up to 20% of cases when patients require more than four surgical procedures per year, there is rapid regrowth of papillomata with airway compromise, or there is distal multisite spread of disease. Other therapies include Human Papilloma Virus (HPV) vaccine, interferon, ribavirin, acyclovir, cidofovir, retinoids COX 2 inhibitors, mumps vaccine, among others. CONCLUSIONS: Recurrent Respiratory Papillomatosis (RRP), it is histologically benign, but in some cases it can progress into malignant. It has unpredictable behavior and is difficult to control and has the potential of causing severe morbidity and death. Further investigation is warranted Reference #1: Derkay CS, Wiatrak B. Recurrent respiratory papillomatosis: a review. Laryngoscope 2008;118(7):1236–1247 Reference #2: Larson DA, Derkay CS. Epidemiology of recurrent respiratory papillomatosis. APMIS 2010;118(6–7):450–454. Reference #3: Bonagura VR, Hatam LJ, Rosenthal DW, et al. Recurrent respiratory papillomatosis: a complex defect in immune responsiveness to human papillomavirus-6 and -11. APMIS 2010;118(6–7):455–470 DISCLOSURES: no disclosure on file for Sixto Arias; no disclosure on file for Yoslay Perez; No relevant relationships by Rene Rico, source=Web Response