Osteogenic sarcoma in the Rothmund-Thomson syndrome.
Two children who had the Rothmund-Thomson syndrome and developed osteosarcoma are reported. The 10 previously reported cases are reviewed. The 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 chemotherapy agents with prolonged myelosuppression and severe mucositis. It is recommended that doxorubicin in particular should be given with extreme caution in such patients.
Leonard
A
A
Department of Child Health, University of Newcastle upon Tyne, UK.
Craft
A W
AW
Moss
C
C
Malcolm
A J
AJ
D000971:Antineoplastic Combined Chemotherapy Protocols / Q000009:adverse effects; D001853:Bone Marrow / Q000187:drug effects; D001859:Bone Neoplasms / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D004920:Erythropoiesis / Q000187:drug effects; D006801:Humans; D008297:Male; D008534:Megaloblasts / Q000187:drug effects; D012516:Osteosarcoma / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D011884:Radius / Q000473:pathology; D011038:Rothmund-Thomson Syndrome / Q000150:complications* / Q000473:pathology; D013977:Tibia / Q000473:pathology