ROTHMUND-THOMSON SYNDROME AND OSTEOSARCOMA

The Rothmund-Thomson syndrome (RTS), also called poikiloderma congenit ale is a rare autosomal recessive disease first described in 1868. Thi s syndrome includes most frequently seen skin lesions (atrophy, telang iectases, pigmentation), cataracts and bone defects (dysostosis, dyspl asia). Some authors describe an association with malignancy. We report three cases of Rothmund-Thomson syndrome associated with osteosarcoma . After cutaneous epithelioma, osteosarcoma is the most frequent malig nancy. Thus, patients with RTS need a careful survey. The treatment di d not differ from sporadic osteosarcoma. Chemosensitivity and toxicity are also not different. (C) 1996 Wiley-Liss, Inc.