We studied the pedigrees of 17 index patients with osteosarcoma, recording malignant disease and cause of death for first- and second-degree relatives. There were seven cancers and five cancer deaths per 2151.5 person-years in first-degree relatives of osteosarcoma patients under the age of 50 years, a significantly greater incidence than in an age- and sex-matched population group (p < 0.001). This excess of malignancy was largely due to two families which fulfilled the criteria for the Li-Fraumeni cancer family syndrome. Both of these families were shown to have the genetic alterations in the p53 gene which have been implicated in this syndrome. Our study suggests that orthopaedic surgeons seeing new cases of osteosarcoma should arrange screening for familial malignancy.

We reviewed 28 children with unilateral middle-third fractures of the femoral shaft who had an angular deformity after union of 10 degrees to 26 degrees. At an average follow-up of 45 months (20 to 66), we measured remodelling of the proximal physis, the distal physis and the femoral shaft. The average correction was 85% of the initial deformity. We found that 74% of correction occurred at the physes and only 26% at the fracture site. Neither the direction nor the magnitude of the angulation much influenced the degree of remodelling. Younger children remodelled only a little better than older children. We conclude that in children under 13 years of age, malunion of as much as 25 degrees in any plane will remodel enough to give normal alignment of the joint surfaces.