We report our experience with 5 cases of post-Paget osteosarcoma of th
e skull, a rare lesion of the neurocranium. Four patients were treated
by surgery and radiotherapy and by surgery alone. Two patients receiv
ed chemotherapy. Histologically, the tumor was found to be a osteosarc
oma, fibroblastic in 2 cases, mixed in 2, and osteoblastic in 1. Combi
ned treatment (surgery, radiotherapy and chemotherapy) positively infl
uenced survival (median survival, 6 months). The prognosis for post-Pa
get osteosarcomas of the skull seems to be worse than for primary sarc
omas, probably due to their intense vascularization. This facilitates
the spread of tumor cells to other organs (as observed in our cases) a
nd reduces the reduced effectiveness of chemotherapy. Although the lat
ter lengthens survival and reduces the incidence of metastases, it is
not as efficacious as in primary sarcomas.