Radiotherapy for juvenile nasopharyngeal angiofibroma

To explain the concepts that radiation oncologists need to understand to manage patients with juvenile nasopharyngeal angiofibroma (JNA). To accomplish this goal we first describe our institution's experience with radiotherapy for JNA and then use this data set as a framework for explaining the role of radiotherapy in the treatment of this uncommon tumor.

We studied the outcomes of all 24 patients treated with radiotherapy for JNA at our institution. All patients had at least 4 years of follow-up (median follow-up, 18 years). The standard dose in the first half of the series was 30 Gy in 22 treatments (1.43 Gy/treatment). After observing recurrences with this schedule, the prescription was changed to 35 to 36 Gy at 1.8 Gy/treatment. In all cases, the target volume was the primary site without an attempt to cover the regional nodes.

All recurrences were at the primary site and presented within 5 years of completing radiotherapy. There appeared to be a dose response for tumor control: 77% with 30 to 32 Gy versus 91% with 35 to 36 Gy. All recurrences following radiotherapy were successfully salvaged with surgery. The only complications from radiotherapy were cataracts in 2 patients. No patient had a significant growth abnormality or second tumor.

Surgery is the best treatment for JNA when cure is likely with low morbidity, but the threshold for using radiotherapy should be low because moderate-dose radiotherapy cures about 90% of patients with a low risk of serious complications. We recommend 36 Gy at 1.8 Gy per treatment in most cases. Elective nodal irradiation is not necessary. Radiographic response should be almost complete within a year of radiotherapy. Patients should be followed with cross-sectional imaging every 6 months for at least 5 years.