A case of marfan syndrome with osteosarcoma

An eighteen-year-old girl with Marfan syndrome, who was complicated with osteosarcoma of right humerus, was reported. She had the disorders of the skeleton (arachnodactyly, severe scoliosis and pectus excavatum), the eye (ectopia lentis and myopia) and the heart (mitral valve prolapse). According to these findings, she was diagnosed as Marfan syndrome by Pyeritz's criteria.In Nov. 1985, she felt right shoulder pain and swelling and radiograms showed the abnormal shadow in the right proximal humerus. Bone biopsy disclosed typical histological appearance of osteogenic sarcoma and forequater amputation was carried out on May 15, 1986.Relationship between the systemic disorders of the skeleton like Marfan syndrome and the occurrence of malignant bone tumor was discussed.