Primary
angiosarcoma of the
spleen is rare and almost always fatal. The pathogenesis is unknown. It has an aggressive
behavior and frequently presents with hematological
abnormalities or metastatic
disease. We
report a 49 year-old
male that presented with
spleen and
lymph node enlargement. He was subjected to a
splenectomy and the
biopsy disclosed an
angiosarcoma of the
spleen.
Metastases were detected in the
lung and
bones and the
patient was considered beyond any
therapeutic option, dying fifteen months later.