Combined Modality Therapy Results In Improved Overall Survival For Angiosarcoma

Angiosarcoma is an aggressive soft tissue sarcoma for which there is limited consensus on treatment recommendations given the rare nature of the disease. We examined disease course and evaluated predictors for improved clinical outcomes in a cohort of angiosarcoma patients. We conducted a retrospective review of 63 patients treated at our institution for angiosarcoma between 2000 and 2015. Outcome metrics included overall survival, progression-free survival, time to recurrence, and prognostic factors for survival. Twenty-eight men and 35 women with a median age of 65 (range, 21-87) years were treated for angiosarcoma, of which 75% were primary sarcomas and 25% were secondary (from prior radiation). The most common angiosarcoma sites were breast/chest (n=21, 33%), head/neck (n=18, 29%), and extremity (n=11, 17%), and a majority of patients had grade 3 disease (54%). Initial treatment included surgery in 79% of patients, chemotherapy in 38% (docetaxel+gemcitabine most common), and radiation in 33% (median dose 54 Gy, range 30-72 Gy). Forty-one percent of patients had surgery alone, 25% surgery followed by adjuvant therapy (chemotherapy, radiation, or both), 13% neoadjuvant therapy followed by surgery, 16% chemotherapy and/or radiation without surgery, and 5% no treatment. Almost half (46%) of patients experienced locoregional or distant recurrences, and half of these patients experienced multiple recurrences. The majority of initial recurrences (62%) were locoregional, while 38% were distant. The median time to recurrence from initial treatment was 9.4 months. R0 resection (P=0.028) and chemotherapy given as part of initial treatment (P=0.045) were associated with fewer recurrences. Median progression-free survival from initial treatment was 14.6 months (range, 0.5-122.0 months), and median overall survival (OS) from initial diagnosis was 43.8 months (range, 0.5-171.8 months). Improved OS was seen in patients with breast/chest angiosarcoma (median 84.6 months vs 39.6 months for head/neck and 12.7 months for extremity, P=0.008), those with secondary angiosarcoma (median 81.7 months vs 32.2 months for primary sarcoma, P=0.024), and patients treated with surgery followed by adjuvant therapy (median 74.3 months vs 39.6 months for surgery alone, 14 months for treatments without surgery, and 2.4 months for no treatment, P=0.002). Frequent recurrences after initial therapy are observed in patients with angiosarcoma. However, surgery followed by adjuvant therapy improves overall survival. Combined modality therapy including surgical resection and chemotherapy should be utilized to treat patients with this aggressive malignancy.