Two children who had the Rothmund-Thomson syndrome and developed osteo
sarcoma are reported. The 10 previously reported cases are reviewed. T
he osteosarcomas developed at a younger age than normally expected and
66% occurred in the tibia/fibula. Four of the five patients for whom
information was available showed undue sensitivity to cancer chemother
apy agents with prolonged myelosuppression and severe mucositis. It is
recommended that doxorubicin in particular should be given with extre
me caution in such patients. (C) 1996 Wiley-Liss, Inc.