ANGIOSARCOMA IN PRIMARY LYMPHEDEMA OF THE LOWER EXTREMITY -- STEWART-TREVES SYNDROME

TH Schmitz-Rixen, S Horsch, G Arnold, PE Peters

Abstract


After a 20-year latent period an angiosarcoma
developed in the edematous leg of a 74-year-old woman
with primary lymphedema. A deep venous thrombosis of
the leg which further aggravated tissue swelling preceded
the appearance of angiosarcoma. Histogenetic
classification of the tumor as hemangiosarcoma rather
than lymphangiosarcoma was favored by positive immunohistochemical
staining for Factor VIII. Despite
high amputation and isolated perfusion with hyperthermal
cytostatic infusion, she developed local recurrence
and distant metastases and died 16 months after operation.
Patients with chronic primary or secondary lymphedema
are susceptible to angiosarcoma although the
overall risk is small.


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