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Angiosarcoma complicating generalized lymphangiectasia.

K T Chen, E F Gilbert

    Archives of Pathology & Laboratory Medicine
    |February 1, 1979
    PubMed
    Summary
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    Multifocal angiosarcoma, a rare cancer, can develop in patients with generalized lymphangiectasia. This study details a case in a young adult, adding to the limited reported instances of angiosarcoma associated with lymphedema.

    Area of Science:

    • Oncology
    • Vascular Biology
    • Medical Case Reports

    Background:

    • Angiosarcoma is a rare malignancy arising from endothelial cells.
    • Lymphedema, characterized by lymphatic system swelling, can be congenital or idiopathic.
    • Previous reports document angiosarcoma in patients with underlying lymphedema.

    Observation:

    • A 23-year-old patient presented with multifocal angiosarcoma in the lower extremities.
    • The patient also had a diagnosis of generalized lymphangiectasia.
    • This case represents a rare occurrence of angiosarcoma in a young individual with lymphatic abnormalities.

    Findings:

    • Eighteen cases of angiosarcoma associated with congenital or idiopathic lymphedema have been previously reported.
    • Patient ages in reported cases ranged from 13 to 65 years, with a mean age of 40.

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  • Extremities were the most common site of tumor involvement in these cases.
  • The exact origin of these tumors (blood vessel vs. lymphatic) remains undetermined.

    • Implications:

    • This case highlights a potential association between generalized lymphangiectasia and angiosarcoma development.
    • Further research is needed to elucidate the pathogenetic mechanisms linking lymphatic dysfunction and angiosarcoma.
    • Understanding this association may improve early diagnosis and treatment strategies for patients with lymphedema and suspected malignancy.