[Angiosarcoma of the spleen]

Primitive splenic tumors are extremely rare. We report a case, the first at S. José-Hospitais Civis de Lisboa, of a 70 year old woman who presented splenomegaly. She had pelvic irradiation in her past history. MRI revealed a splenic mass characterized as vascular on angiography. She is doing well 12 months after splenectomy. We have found 61 reported cases of this rare tumor whose etiopathogeny is unknown. Early splenectomy should be the first therapeutical approach, since spontaneous rupture is very common and was the feature presented in 34% of patients in the series by Autry et al. As far as cytostatic chemotherapy is concerned, the rarity of this tumor precludes any conclusion about its efficacy, but in metastasized forms it seems that chemotherapy for soft tissue sarcomas may be effective.