Evaluation of the use and efficacy of (neo)adjuvant chemotherapy in angiosarcoma: a multicentre study

Abstract

IntroductionAngiosarcomas constitute approximately 2% to 3% of all soft tissue sarcomas, are characterised by an aggressive clinical behaviour and poor outcome. Optimal management of localised angiosarcomas consists of complete surgical resection with or without radiation. However, due to the infiltrating nature of this disease, complete resection is often not possible. Despite optimal management, the outcome of patients with localised disease remains poor. The role of (neo)adjuvant chemotherapy in angiosarcomas remains undefined. The aim of this study is to document the outcome of patients treated with (neo)adjuvant chemotherapy and assess the feasibility of performing a prospective trial by evaluating the number of patients treated at sarcoma referral centres.MethodsA retrospective search within participating EORTC (European Organisation for Research and Treatment of Cancer) sites for patients treated with (neo)adjuvant chemotherapy was made. Patients treated between January 2007 and January 2016 were included.ResultsA total of 15 institutions participated and 86 patients were evaluable, 43 were treated with neoadjuvant, 27 with adjuvant chemotherapy and 16 with both. At the time of analysis, the median follow-up from diagnosis was 4.6 years. Median overall survival (OS) was 4.9 years (2.9 N) and the percentage alive at 4 years was 57.9 (45.5 to 68.4). The median disease-free survival was 1.4 years (0.9 to 1.7) and the percentage disease-free at 4 years was 26.8% (17.9 to 36.5).ConclusionThe outcome of angiosarcoma patients treated with (neo)adjuvant chemotherapy in this case series compares favourably with previously published data. Due to the aggressive nature of angiosarcoma, a prospective trial of neoadjuvant chemotherapy should be considered.

<p>Is Pathologic Complete Response the Surrogate in Primary Gastric Angiosarcoma Undergoing Doxorubicin-Based Neoadjuvant Chemotherapy? A Case Report</p>

“… 2 , 3 However, these reports mainly describe skin, breast, colon, or mixed tumors due to rare incidence. 1 , 4 In this report, we demonstrate the response of a patient with primary gastric angiosarcoma to doxorubicin-based NAC after three cycles. We present the following case in accordance with the CARE reporting checklist.…”

“…Their results are also diverse, and generally showed poor prognosis. 4,9 Reports of cases involving other sites of angiosarcoma were reviewed. Early reports showed poor experience in management with adjuvant chemotherapy or combinations of chemotherapy and radiation.…”

Right atrial cardiac angiosarcoma treated with concurrent proton beam therapy and paclitaxel: A novel approach to a rare disease

“…The combination of PBT with chemotherapy followed by adjuvant chemotherapy seems to be an effective regimen in patients with cardiac angiosarcoma. Recently, a European study retrospectively examining the treatment approaches in patients with angiosarcoma reported prolonged disease‐free survival (DFS) in patients undergoing multimodality approach combining chemotherapy (neoadjuvant, adjuvant, or both), surgery, and RT 3 . The benefits of PBT in sarcoma stem from the physical characteristics of protons, which lead to improved dose conformality to the tumor volume and help in escalating the doses to the tumor while sparing the surrounding tissue 4 .…”

Optimal Clinical Management and the Molecular Biology of Angiosarcomas

“…Although no prospective randomized trials are available, retrospective studies have suggested that neoadjuvant systemic treatment is feasible with clinical benefit. Data from the European Organisation for Research and Treatment of Cancer (EORTC) institutions with 59 AS patients receiving neoadjuvant systemic treatment demonstrated that various systemic treatments, including chemotherapy and anti-angiogenic agents, have anti-tumour activity and could be maintained until maximum benefit; 10% of patients who received neoadjuvant treatment had disease progression before surgical intervention [ 21 ]. The median disease-free survival (DFS) and overall survival (OS) outcomes of the 86 AS patients, including the 59 patients who received neoadjuvant treatment, was 1.4 years and 4.9 years, respectively [ 21 ].…”