Oslam Syndrome

Clinical Features

Mulvihill et al. (1977) described a family in which 3 of 9 children developed typical osteosarcoma. Limb anomalies (clinodactyly, absence of digital ray in foot, bilateral radioulnar synostosis) and macrocytosis without anemia were present in the surviving child with osteosarcoma, several of her sibs and her father. The authors suggested that this is an autosomal dominant syndrome with impaired regulation of bone and bone marrow development.