BACKGROUND: There are few reports on radiotherapy methods for treating extramammary Paget's disease (EMPD). The aim of this study was to explore the outcome of radiotherapy for EMPD. PATIENTS AND METHODS: Between June 1995 and October 2010, 14 patients with EMPD (7 male and 7 female; median age 77 years) underwent radiotherapy at the Tokyo Medical University Hospital. The median total irradiation dose was 50 Gy, delivered in 20-33 fractions. Case reports of patients with EMPD were obtained from the PubMed database for the period of 1991-2012. 66 patients (median age 73 years) were identified. Radiotherapy outcomes were analyzed. RESULTS: All patients achieved complete response within the irradiated volume during a median observation period of 71.4 months. The 5-year locoregional progression-free survival and overall survival were 91.7% (95% confidence interval (CI) 53.9-98.8%) and 84.3% (95% CI 50.3-95.8%), respectively. From the PubMed database, the 5-year local progression-free survival and locoregional progression-free survival were 84.5% (95% CI 65.3-93.6%) and 77.5% (95% CI 57.3-89.0%), respectively. 12 (18%) patients had a recurrence in the inguinal lymph nodes. CONCLUSION: Radiotherapy yielded good local control and survival, which suggests that it was effective for patients with EMPD and in particular medically inoperable EMPD.
BACKGROUND: There are few reports on radiotherapy methods for treating extramammary Paget's disease (EMPD). The aim of this study was to explore the outcome of radiotherapy for EMPD. PATIENTS AND METHODS: Between June 1995 and October 2010, 14 patients with EMPD (7 male and 7 female; median age 77 years) underwent radiotherapy at the Tokyo Medical University Hospital. The median total irradiation dose was 50 Gy, delivered in 20-33 fractions. Case reports of patients with EMPD were obtained from the PubMed database for the period of 1991-2012. 66 patients (median age 73 years) were identified. Radiotherapy outcomes were analyzed. RESULTS: All patients achieved complete response within the irradiated volume during a median observation period of 71.4 months. The 5-year locoregional progression-free survival and overall survival were 91.7% (95% confidence interval (CI) 53.9-98.8%) and 84.3% (95% CI 50.3-95.8%), respectively. From the PubMed database, the 5-year local progression-free survival and locoregional progression-free survival were 84.5% (95% CI 65.3-93.6%) and 77.5% (95% CI 57.3-89.0%), respectively. 12 (18%) patients had a recurrence in the inguinal lymph nodes. CONCLUSION: Radiotherapy yielded good local control and survival, which suggests that it was effective for patients with EMPD and in particular medically inoperable EMPD.
Authors: Gabriel E Molina; Saami Khalifian; Jamie L Mull; Lu Chen; Ilana S Rosman; Beverly E Faulkner-Jones; Kenneth H Ngo; Shadmehr Demehri; Lynn A Cornelius; Peggy A Wu Journal: JAMA Dermatol Date: 2019-05-01 Impact factor: 10.282