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J.A. De Oliveira



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    P3.24 - Poster Session 3 - Supportive Care (ID 160)

    • Event: WCLC 2013
    • Type: Poster Session
    • Track: Supportive Care
    • Presentations: 1
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      P3.24-051 - Primary Thoracic Angiosarcoma: Treatment and outcomes of 5 patients (ID 3280)

      09:30 - 09:30  |  Author(s): J.A. De Oliveira

      • Abstract

      Background
      Angiosarcomas (AS) are rare aggressive tumors that represent about 1-2% of all soft-tissue sarcomas; 9.5% of them arise in the thorax. We describe five patients diagnosed with thoracic AS who were treated at ICESP.

      Methods
      It is a case series descriptive study with review of the medical files from five consecutively registered patients with AS confirmed by immunohistochemistry at our institution between June 2010 and March 2013.

      Results
      Case 1: A 49-year-old woman was admitted with pulmonary AS presenting progressive dyspnea and a recent hemoptysis. Pneumonectomy was performed in April 2011 and she was treated with adjuvant doxorubicin (every 3 weeks, 4 cycles) and paclitaxel (12 weeks). After 7 months, she developed progressive disease (PD) in liver, bones and lymph nodes. Weekly paclitaxel was restarted, but she had hepatic PD. Since May 2013 she has been treated with liposomal doxorubicin. She is alive after 26 months of diagnosis. Case 2: A 62-year-old woman was diagnosed with metastatic paracardiac AS after cardiac tamponade. She was treated with weekly paclitaxel and developed PD in liver and lungs. She died 3 months after diagnosis. Case 3: A 32-year-old man, was diagnosed with a primary AS in the right ventricle, metastatic to lungs, was admitted with recurrent pericardial effusion for 6 months. The tumor was considered unresectable and he was treated with doxorubicin and ifosfamide (only one cycle), temporarily interrupted due to febrile neutropenia grade 4, but with a partial response. He is alive after 3 months of diagnosis. Case 4: A 31-year-old man was diagnosed with unresectable AS in the right atrium after developing a superior vena cava syndrome. Weekly paclitaxel was started, with initial clinical improvement, but PD was detected after 6 cycles (24 weeks). As a second-line treatment, doxorubicin and ifosfamide were administered, with PD in lungs after 5 cycles. He died 13 months after the beginning of chemotherapy. Case 5: A 58-year-old woman was diagnosed with a right infraclavicular unresectable AS with local pain and edema in the upper right arm for one year. No response was seen after two cycles of doxorubicin and ifosfamide. Palliative radiotherapy followed by weekly paclitaxel was attempted as a second-line therapy with no response. Best supportive care was started and she is alive 6 months after diagnosis.

      Conclusion
      We concluded that thoracic AS presents a very dismal prognosis, due to the primary location and the high incidence of metastatic disease. For those patients with resectable disease and curative intent, surgery must always be considered. Weekly paclitaxel and the combination of doxorubicin and ifosfamide are both active in thoracic AS, but responses usually were not long-lasting. Oral tyrosine kinase inhibitors with antiangiogenic properties may be an option to be better explored.