We have reviewed the data from our regional Bone Tumour Registry on patients with osteosarcoma diagnosed between 1933 and 2004 in order to investigate the relationship between survival and changes in treatment. There were 184 patients with non-metastatic appendicular osteosarcoma diagnosed at the age of 18 or under. Survival was calculated using Kaplan-Meier curves, and multivariate analysis was performed using the Cox regression proportional hazards model.

The five-year survival improved from 21% between 1933 and 1959, to 62% between 1990 and 1999. During this time, a multi-disciplinary organisation was gradually developed to manage treatment. The most significant variable affecting outcome was the date of diagnosis, with trends in improved survival mirroring the introduction of increasingly effective chemotherapy. Our experience suggests that the guidelines of the National Institute for Clinical Excellence on the minimum throughput of centres for treatment should be enforced flexibly in those that can demonstrate that their historical and contemporary results are comparable to those published nationally and internationally.

Solitary benign peripheral-nerve tumours are rare and may be difficult to diagnose correctly. Surgical excision may increase the patient's symptoms and may not be necessary. We have reviewed the presentation, clinical findings and histology of 104 solitary tumours presenting at one centre between 1959 and 1990. Male patients predominated for both schwannoma and neurofibroma. There was considerable but variable delay before presentation; 94% of patients complained primarily of a mass and less than half had pain or paraesthesia. The correct diagnosis had been made in only a few cases before operation, and the incidence of neurological symptoms doubled after exploration. We emphasise the need for vigilance, accurate preoperative diagnosis, and careful surgery.

We reviewed the records and radiographs of seven children who presented with knee pain, local tenderness over the medial femoral condyle, and radiological irregularity of the distal medial metaphysis of the femur suggestive of malignancy. In the five patients who had biopsies, histological changes were consistent with musculotendinous avulsion, and the dissection of ten cadavers confirmed the site to be the insertion of part of the adductor magnus. The recognition of this lesion and knowledge of its benign nature may avoid unnecessary anxiety and needless biopsy.

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 report a case of malignant fibrous histiocytoma of the hip which occurred 30 years after the insertion of a single chrome-cobalt alloy screw for a slipped femoral epiphysis. The possible aetiological association between malignant tumours and metallic implants is discussed.