Radiation-associated angiosarcoma of the breast: An international multicenter analysis
Jana Mergancová, Anna Lierová, Oldřich Coufal, Jan Žatecký, Bohuslav Melichar, Ilona Zedníková, Jindřiška Mergancová, Anna Jesenková, Karel Šťastný, Jiří Gatěk, Otakar Kubala, Jiří Prokop, Daniel Dyttert, Marián Karaba, Ivana Schwarzbacherová, Anna Humeňanská, Jindřich Šuk, M Valenta, Vladimír Moucha, L Sákra, Aleš Hlávka, Mária Hácová, ̈Radovan Vojtíšek, Marek Sochor, Tomáš Jirásek, M Zábojníková, Martina Zemanová, Katarína Macháleková, Dominika Rusnáková, Eva Kúdelová, Marek Smolár
Abstract
INTRODUCTION: Radiation-associated angiosarcoma (RAAS) is a rare and serious complication of breast irradiation. Due to the rarity of the condition, clinical experience is limited and publications on this topic include only retrospective studies or case reports. MATERIALS AND METHODS: All patients diagnosed with RAAS between January 2000 and December 2017 in twelve centers across the Czech Republic and Slovakia were evaluated. RESULTS: Data of 53 patients were analyzed. The median age at diagnosis was 72 (range 44-89) years. The median latency period between irradiation and diagnosis of RAAS was 78 (range 36-172) months. The median radiation dose was 57.6 (range 34-66) Gy. The whole breast radiation therapy with radiation boost to the tumor bed was the most common radiotherapy regimen. Total mastectomy due to RAAS was performed in 43 patients (81%), radical excision in 8 (15%); 2 patients were not surgically treated due to unresectable disease. Adjuvant chemotherapy followed surgical therapy of RAAS in 18 patients, 3 patients underwent adjuvant radiotherapy. The local recurrence rate of RAAS was 43% and the median time from surgery to the onset of recurrence was 7.5 months (range 3-66 months). The 3-year survival rate was 56%, the 5-year survival rate was only 33%. 46% of patients died during the follow-up period. CONCLUSION: The present data demonstrate that RAAS is a rare condition with high local recurrence rate (43%) and mortality (the 5-year survival rate was 33%.). Early diagnosis of RAAS based on biopsy is crucial for treatment with radical intent. Surgery with negative margins constitutes the most important part of the therapy; the role of adjuvant chemotherapy and radiotherapy is still unclear.