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Primary and secondary angiosarcomas: a comparative single-center analysis.

Thorsten Hillenbrand1, Franka Menge1, Peter Hohenberger1

  • 1Sarcoma Unit, Interdisciplinary Tumor Center Mannheim, Mannheim University Medical Center, University of Heidelberg, Theodor-Kutzer-Ufer 1-3, 68167 Mannheim, Germany.

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Summary

This study compared primary (PAS) and secondary angiosarcomas (SAS), finding SAS more common in women and originating in the breast. While surgery is key, both subtypes have poor prognoses with high recurrence or metastasis rates.

Keywords:
ChemotherapyOutcomePrimary angiosarcomaSecondary angiosarcomaTargeted therapy

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Area of Science:

  • Oncology
  • Vascular Malignancies
  • Surgical Pathology

Background:

  • Angiosarcomas (AS) are rare vascular tumors with two main subtypes: primary (PAS) and secondary (SAS).
  • Understanding the distinct characteristics of PAS and SAS is crucial for effective management and treatment strategies.
  • This study aimed to delineate the differences between primary and secondary angiosarcomas.

Purpose of the Study:

  • To compare the epidemiological, clinical, and survival characteristics of primary angiosarcomas (PAS) and secondary angiosarcomas (SAS).
  • To identify key differences in presentation, treatment response, and outcomes between AS subtypes.
  • To inform clinical decision-making for patients diagnosed with angiosarcoma.

Main Methods:

  • A retrospective analysis of 18 primary angiosarcoma (PAS) and 10 secondary angiosarcoma (SAS) patients treated between 2004 and 2012.
  • Data collected included patient demographics, tumor location, treatment modalities (primarily surgery), recurrence rates, metastasis, and overall survival.
  • Statistical comparisons were performed to evaluate differences between the PAS and SAS cohorts.

Main Results:

  • Secondary angiosarcomas (SAS) were predominantly diagnosed in women (80%) and frequently arose from the breast, often being radiation-induced with a median latency of 7.7 years.
  • Primary angiosarcomas (PAS) showed a higher tendency for early metastasis (61.1%) compared to SAS (40.0%).
  • Local recurrence rates were higher in SAS (50.0%) than PAS (27.8%), and overall survival was poor for both subtypes, with median survival of 19 months for PAS and 57 months for SAS.

Conclusions:

  • Radical surgery remains the primary treatment for both primary and secondary angiosarcomas, including recurrence management.
  • Secondary angiosarcomas exhibit a higher local recurrence rate, whereas primary angiosarcomas are more prone to early distant metastasis.
  • Despite treatment, both angiosarcoma subtypes carry a poor overall prognosis, highlighting the need for further research into novel therapeutic approaches.