“…The choice of further treatment is still a matter of debate, owing to the rare incidence of the disease and the absence of prospective, randomized studies. Systemic, doxorubicin-based chemotherapy has been widely used also in patients with localized disease, since early retrospective reports indicated an improved outcome by adding chemotherapy to surgery9/RT (2 Á/4), but in two recently published, retrospective studies no such benefit was noted (17,18). However, in a very large, retrospective, multicentre study (19), Zucca et al reported a survival benefit for patients treated with anthracycline-containing chemotherapy, regardless of disease stage, and a better outcome for patients who had received at least six cycles of chemotherapy.…”