Angiosarcoma Arising from Right Atrium: Remarkable Response to Concurrent Chemoradiotherapy with Carboplatin and Paclitaxel

Abstract

We read with interest the case report by Fehr et al.1Fehr M Kuhn M Mayer K et al.Metastatic angiosarcoma arising from the right atrium: unusual presentation and excellent response to treatment in a young patient.J Thorac Oncol. 2010; 5: 1301-1302Abstract Full Text Full Text PDF PubMed Scopus (3) Google Scholar and the concise review by Orlandi et al.2Orlandi A Ferlosio A Roselli M et al.Cardiac sarcomas: an update.J Thorac Oncol. 2010; 5: 1483-1489Abstract Full Text Full Text PDF PubMed Scopus (76) Google Scholar regarding cardiac sarcomas. Concurrent chemoradiotherapy (CRT) to cardiac angiosarcomas has been scarcely documented, and only one case was reported with the benefit from concurrent CRT with docetaxel.3Nakamura-Horigome M Koyama J Eizawa T et al.Successful treatment of primary cardiac angiosarcoma with docetaxel and radiotherapy.Angiology. 2008; 59: 368-371Crossref PubMed Scopus (28) Google Scholar Although efficacy of weekly paclitaxel (PTX) for unresectable angiosarcoma has been demonstrated,4Penel N Bui BN Bay JO et al.Phase II trial of weekly paclitaxel for unresectable angiosarcoma: the ANGIOTAX Study.J Clin Oncol. 2008; 26: 5269-5274Crossref PubMed Scopus (387) Google Scholar CRT with weekly PTX was not reported before the report by Fehr et al. We present herein a case of metastatic angiosarcoma arising from the right atrium, which remarkably responded to concurrent CRT with weekly carboplatin and PTX. A 40-year-old man was referred to our institute, presenting a tumor in the right atrium and multiple bilateral lung nodules with the computed tomography (Figures 1A, B). Histology obtained by wedge resection of a lung nodule revealed a high-grade epithelial angiosarcoma, thus the patient was diagnosed with angiosarcoma from right atrium with multiple lung metastases. We judged it to be unresectable and decided to perform concurrent CRT (30 fractions of 2Gy) with weekly carboplatin (area under the curve = 2) and PTX (60 mg/m2) for 6 consecutive weeks. The radiation field was limited to the primary cardiac tumor. After the therapy, almost all bilateral pulmonary nodules disappeared, and a large tumor in the right atrium was markedly reduced (Figure 2A, B). Although grade 2 esophagitis and neutropenia were observed, the treatment was generally well tolerated. No evidence of progression has been confirmed after more than 5 months of follow-up.FIGURE 2A, Almost all bilateral pulmonary nodules disappeared 3 months after the initiation of chemoradiation therapy. B, The cardiac tumor in the right atrium was remarkably reduced 3 months after the initiation of chemoradiation therapy.View Large Image Figure ViewerDownload (PPT) Multimodality treatment combined with surgery and adjuvant chemotherapy and/or radiotherapy is indicated as the standard care for patients with cardiac angiosarcomas. Nevertheless, the majority of patients with cardiac sarcoma are already unresectable and metastatic disease at their initial diagnosis and have a poor prognosis. CRT is desirable in some cases with cardiac sarcoma to control both the primary cardiac tumor and metastatic lesions. In our case, a combined regimen including PTX was chosen to obtain better response of metastatic lesions, and good response was clearly observed. Moreover, the efficacy and tolerability of concurrent CRT with carboplatin + PTX have been recently demonstrated in locally advanced non-small cell lung cancer.5Yamamoto N Nakagawa K Nishimura Y et al.Phase III study comparing second- and third-generation regimens with concurrent thoracic radiotherapy in patients with unresectable stage III non-small-cell lung cancer: West Japan Thoracic Oncology Group WJTOG0105.J Clin Oncol. 2010; 28: 3739-3745Crossref PubMed Scopus (206) Google Scholar To our knowledge, this is the first report demonstrating the efficacy of concurrent CRT with carboplatin + PTX for cardiac angiosarcoma. Nevertheless, there is no other evidence of CRT with doublet regimens. Future studies to explore usefulness of concurrent CRT for cardiac angiosarcomas are warranted.