Neoadjuvant Systemic Treatment of Primary Angiosarcoma

Abstract

Angiosarcoma is an extremely rare and aggressive malignancy. Standard of care of localized tumors includes surgery ± radiation. Despite this multimodal treatment, >50% of the angiosarcoma patients develop local or distant recurrent disease. The role of neoadjuvant systemic therapy is still controversial and we therefore performed a systematic review of the literature to define the role of neoadjuvant systemic therapy based on available evidence. We focused on the effects of neoadjuvant systemic therapy on: 1. The success of surgical resection and 2. the long-term survival. All articles published before October 2019 on Ovid Medline, Ovid Embase, Cochrane library and Scopus were evaluated. Eighteen case reports and six retrospective cohort studies were included. There were no randomized controlled trials. This literature showed a beneficial role of neoadjuvant chemotherapy on downsizing of the tumor resulting in an improvement of the resection margins, especially in patients with cardiac or cutaneous angiosarcoma. However, no definitive conclusions on survival can be drawn based on the available literature lacking any prospective randomized studies in this setting. We advise that neoadjuvant chemotherapy should be considered, since this could lead to less mutilating resections and a higher rate of free resection margins. An international angiosarcoma registry could help to develop guidelines for this rare disease.

Clinical Outcomes for Primary and Radiation-Associated Angiosarcoma of the Breast with Multimodal Treatment: Long-Term Survival Is Achievable

“…This paradigm shift from a surgery-only approach to a multidisciplinary approach has resulted in improved local control and decreased risk of distant metastasis at 68% and 38% for PAS and RAAS, respectively [3,[12][13][14]. More recently, case reports and case series have evaluated the role of neoadjuvant chemotherapy with promising results [7,15].…”

New nomograms to predict overall and cancer‐specific survival of angiosarcoma

“…In addition, targeted therapy and immune therapy are also considered to be promising anticancer therapeutic strategies 21–23 . The results of different treatments vary widely and are affected by many factors, such as tumor location, tumor size, resectability, and tumor type 13,24,25 . According to previous studies, 26,27 radical surgery remains the standard therapy for localized AS among different therapeutic approaches.…”

Primary Soft Tissue Sarcoma of the Heart: An Emerging Chapter in Cardio-Oncology

“…Dedicated protocols for chemotherapy in pSTS-h are still a matter of debate due to the paucity of data in the literature and the rarity of these types of cancers. The first-line therapy for pSTS-h is based on Adriamycin at a dose of 75 mg/m 2 administered as a continuous intravenous infusion over 3 days, and ifosfamide at a dose of 10 g/m 2 divided over 4 to 5 days [80][81][82]. Adequate hy-dration therapy should be managed to prevent kidney toxicity, while neurotoxicity related to ifosfamide might be prevented by promoting alkalinization and albumin infusion [82].…”

“…The second-line regimen considers a combination of gemcitabine plus docetaxel [81,82]. Generally, gemcitabine might be given on days 1 and 8 at 900 mg/mq, and docetaxel at 100 mg/mq, although a reduced dose may be considered to reduce side effects [82].…”