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Cardiac Angiosarcoma Management and Outcomes: 20-Year Single-institution Experience

  • Bone and Soft Tissue Sarcomas
  • Published:
Annals of Surgical Oncology Aims and scope Submit manuscript

Abstract

Purpose

To identify the clinicopathologic characteristics, treatments, and outcomes of a series of patients with primary cardiac angiosarcoma (AS).

Methods

This retrospective case series was set in a tertiary referral center with a multidisciplinary clinic. Consecutive patients with institutionally confirmed pathologic diagnosis of cardiac AS from January 1990 to May 2011 were reviewed. Main outcome measures included patient demographics, tumor characteristics, management strategies, disease response, and survival.

Results

Data from 18 patients (78 % male) were reviewed. Sixteen patients (89 %) had AS originating in the right atrium. At diagnosis, eight patients (44 %) had localized/locally advanced disease and ten patients (56 %) had metastatic disease. Initial treatment strategies included resection (44 %), chemotherapy (39 %), and radiotherapy (11 %). Of the eight patients with localized/locally advanced AS, two underwent macroscopically complete resection with negative microscopic margins, one underwent macroscopically complete resection with positive microscopic margins, one underwent macroscopically incomplete resection, two received chemotherapy followed by surgery and intraoperative radiotherapy, one received chemotherapy alone, and one died before planned radiotherapy. Median follow-up was 12 months. Median overall survival (OS) was 13 months for the entire cohort; median OS was 19.5 months for those presenting with localized/locally advanced AS and 6 months for those with metastatic disease at presentation (p = 0.08). Patients who underwent primary tumor resection had improved median OS compared with patients whose tumors remained in situ (17 vs. 5 months, p = 0.01).

Conclusions

Cardiac AS is associated with poor prognosis. Resection of primary tumor should be attempted when feasible, as OS may be improved. Nevertheless, most patients die of disease progression.

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Authors and Affiliations

  1. Division of Surgical Oncology, Brigham and Women’s Hospital, Center for Sarcoma and Bone Oncology, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA, USA

    Nicole J. Look Hong MD, MSc & Chandrajit P. Raut MD, MSc

  2. National Cancer Institute, National Institutes of Heath, Surgery Branch, Bethesda, MD, USA

    Prakash K. Pandalai MD

  3. Department of Pathology, Brigham and Women’s Hospital, Center for Sarcoma and Bone Oncology, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA, USA

    Jason L. Hornick MD, PhD

  4. Division of Cardiac Surgery, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA, USA

    Prem S. Shekar MD

  5. Division of Medical Oncology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA

    David C. Harmon MD

  6. Division of Radiation Oncology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA

    Yen-Lin Chen MD

  7. Division of Medical Oncology, Brigham and Women’s Hospital, Center for Sarcoma and Bone Oncology, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA, USA

    James E. Butrynski MD

  8. Division of Radiation Oncology, Brigham and Women’s Hospital, Center for Sarcoma and Bone Oncology, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA, USA

    Elizabeth H. Baldini MD, MPH

Authors
  1. Nicole J. Look Hong MD, MSc
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  2. Prakash K. Pandalai MD
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  3. Jason L. Hornick MD, PhD
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  4. Prem S. Shekar MD
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  5. David C. Harmon MD
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  8. Elizabeth H. Baldini MD, MPH
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  9. Chandrajit P. Raut MD, MSc
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Corresponding author

Correspondence to Nicole J. Look Hong MD, MSc.

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Look Hong, N.J., Pandalai, P.K., Hornick, J.L. et al. Cardiac Angiosarcoma Management and Outcomes: 20-Year Single-institution Experience. Ann Surg Oncol 19, 2707–2715 (2012). https://doi.org/10.1245/s10434-012-2334-2

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  • DOI: https://doi.org/10.1245/s10434-012-2334-2

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