MULTICENTRIC OSTEOSARCOMA, ROTHMUND-THOMSON SYNDROME, AND SECONDARY NASOPHARYNGEAL NON-HODGKINS-LYMPHOMA - A CASE-REPORT AND REVIEW OF THE LITERATURE

Purpose: Rothmund-Thomson syndrome (RTS) is an autosomal recessive dis ease characterized by poikiloderma, photosensitivity, skeletal deformi ties, and other changes. It is also associated with an increased risk of malignancies, including osteosarcoma. Sixteen previous cases of RTS and osteosarcoma have been reported. Patient: A patient with RTS in w hom multicentric osteosarcoma developed is described. After surgery an d chemotherapy, a secondary malignant neoplasm (SMN) developed consist ent with nasopharyngeal non-Hodgkin's lymphoma. Results: The patient w as treated with anti-CD20 monoclonal antibody and is in complete remis sion. Conclusion: This is the fir st report of a patient with RTS trea ted for osteosarcoma in whom a SMN developed. Potential factors involv ed in this SMN include genetic predisposition and previous chemotherap y.