AOS27 Extensive juvenile nasopharyngeal angiofibroma: A long-term study of 40 patients successfully treated with radiotherapy

Abstract

Background Juvenile nasopharyngeal angiofibroma (JNA) is a rare, highly vascular tumour, which is primarily seen in male adolescents and is characterised by aggressive local growth. Methods During 1990–2009, 40 male patients (aged 9–22 years) with extensive JNA were treated with definitive radiotherapy. The tumours were staged according to JNA classification (1987) based on clinicoradiological extensions. Radiologically, on computed tomography (CT) scan, all patients had extensive disease with tumours in paranasal sinus or orbits(s) or the intracranial compartment. Because of extensive disease, all patients were treated with a median radiotherapy dose of 50 Gy in 25 fractions over 5 weeks on a 6 MV linear accelerator or tele-cobalt unit. Findings Excellent symptomatic relief was seen during treatment in all patients, but radiological regression on follow-up CT scan was slow (range 2–8 years). None of the patients developed recurrence of the symptoms or progression of the disease during the 2–19 years of follow-up. One patient developed radiation-induced osteosarcoma of the mandible 17 years after the radiotherapy. Interpretation This series is one the largest number of angiofibroma cases that were successfully treated with radiation. Most of our patients had locally advanced lesions and many of them had only biopsy or incomplete resections before definitive radiation therapy. A course of definitive radiation not only made these patients asymptomatic but also halted the disease progression. Because of the extensive nature of the disease a higher than conventionally used dose of radiation was used in our patients. These patients must be followed up for the rest of their lives to detect radiation-induced squeal-like malignant lesions at the earliest manifestation in the irradiated region. The author declared no conflicts of interest.