Multicentric Osteosarcoma, Rothmund-Thomson Syndrome, and Secondary Nasopharyngeal Non-Hodgkinʼs Lymphoma

Abstract

Rothmund-Thomson syndrome (RTS) is an autosomal recessive disease characterized by poikiloderma, photosensitivity, skeletal deformities, 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. A patient with RTS in whom multicentric osteosarcoma developed is described. After surgery and chemotherapy, a secondary malignant neoplasm (SMN) developed consistent with nasopharyngeal non-Hodgkin's lymphoma. The patient was treated with anti-CD20 monoclonal antibody and is in complete remission. This is the first report of a patient with RTS treated for osteosarcoma in whom a SMN developed. Potential factors involved in this SMN include genetic predisposition and previous chemotherapy.