Radiotherapy for in situ extramammary Paget disease of the vulva

BACKGROUND: Radiotherapy as a first-choice treatment for in situ extramammary Paget disease has been successfully used. OBJECTIVES: To review the most relevant aspects of radiotherapy as first-choice treatment in selected cases of in situ extramammary Paget disease of the vulva. PATIENTS AND METHODS: Two Caucasian females aged 76 and 92 years with in situ extramammary Paget disease localized in the genital region were treated by means of ortovoltage X-rays: 100 kV, 8 mA, 1.7 mm Al filter, field size of 12-cm cone, and source skin distance of 30 cm. Both patients received 40 Gy, 200 cGy per fraction, five fractions per week. RESULTS: Complete regression of in situ extramammary Paget disease was observed in both patients after radical radiation therapy and neither local recurrences nor internal malignancies were detected. CONCLUSIONS: Radiotherapy is a curative treatment in selected cases of in situ extramammary Paget disease affecting the vulva.