A study is presented of the aetiology and results of treatment in a group of 125 proven osteosarcomas present in children under fifteen years of age. These cases have been collected from the records of one English and six European treatment centres. There is a slight male preponderance, but the striking aetiological feature is the very high proportion of tumours of the long bones of the limbs (96 per cent). The two and a half and five year disease-free survival rates were respectively 15 and 12 per cent, with a further 9 per cent still living, but under observation for less than two and a half years. Evidence of metastasis after two and a half years is very unusual, but no child with a tumour of an axial or girdle bone lived this length of time. Although the differences in the results of the different methods of treatment employed are not statistically valid, the largest number of long survivors had been treated by early amputation, which method also provided the lowest rate of local tumour recurrences. Reasons are discussed which indicate that prompt ablation is the treatment of choice, perhaps with certain advantages in the light of recent advances in adjuvant treatment. The past situation in connection with childhood osteosarcoma certainly provides strong support for immediate carefully designed clinical trials of the new adjuvant methods cited.
Forty-seven histologically confirmed cases of Ewing's tumour are reported, with typical age, sex and skeletal distributions. The disease-free survival curves are like those for osteosarcoma of corresponding types of bone, but with higher mortality. The two-year disease-free survival rates were 24 per cent for tumours of long bones and 5 per cent for those of other bones. Osseous metastases were most frequent, although the lung was the commonest single site involved. There were secondary deposits in lymph nodes in nine patients, possibly also in four more with mediastinal enlargement. Although initially radiosensitive, one-third of tumours recurred locally, almost always with the appearance of metastases. Neither whole bone irradiation nor high dosage alone ensures complete control of the primary tumour, and it is suggested that recently reported improved results of treatment should be attributed to adjuvant multi-drug chemotherapy. Inadequate control of the primary tumour diminishes the chance of survival and for some sites radical operation merits reconsideration.
The significance of cobalt as a cause of symptoms after McKee hip arthroplasty is discussed. Seven patients are described in whom such arthroplasties became unsatisfactory after periods varying from nine months to four years. Six of these patients were cobalt-positive but nickel-and chrome-negative on patch testing. Macroscopic and histological necrosis of bone, muscle and joint capsule around the prostheses was found in five patients whose hips were explored. The symptoms were progressive pain, a feeling of instability, and in two cases spontaneous dislocation. Radiological features included acetabular fracture, bone resorption, loosening and dislocation of the prosthesis. Increased cobalt concentrations (determined by atomic absorption spectrophotometry) in the urine of four patients and in a variety of tissues in one patient are presented. Patch testing is recommended in the investigation of patients with troublesome McKee hip arthroplasties.
1. Three cases of infantile pseudarthrosis of the tibia treated successfully by delayed autogenous by-pass graft are reported. 2. The delayed autogenous graft is stouter, stronger and more easily handled and has enhanced osteogenic properties than a graft transferred immediately. 3. The by-pass graft commends itself, firstly, because it does not disturb the pseudarthrosis, which in consequence helps the immobilisation of the graft; secondly, because it is well embedded in healthy bone above and below, well away from the abnormal bone; thirdly, because it lies under compression and, ideally, is vertically disposed between the knee and the ankle; and fourthly, because there is no devitalising stripping of periosteum or introduction of foreign bodies. 4. Support to the grafted leg is needed for at least five years, but only by a polythene splint after four to six months. 5. With early grafting the deformity straightens out and shortening is overcome, as there is early return to normal use of the limb. 6. Prolonged follow-up is called for lest the basic lesion in the tibia should extend. 7. Fibrous dysplasia and similar fibrous lesions of bone account for many cases of infantile pseudarthrosis of the tibia. Many of these lesions are congenital and subsequently lead to fracture. 8. Postponement of surgery should not be countenanced.
1. An analytical study of eighty histologically proven cases of Paget's sarcoma confirms and elaborates existing knowledge of the etiology of this tumour. The frequency with which this tumour occurs in certain parts of England may be seen by comparison with the numbers of osteosarcoma and fibrosarcoma cases registered at Bristol and Leeds. An overall incidence figure for Paget's sarcoma of 0·l6 and 0·18 per 100,000 population per annum has been calculated for the Bristol and Leeds areas respectively. The similarity of the age incidence curves of Paget's disease and Paget's sarcoma are shown and discussed in the light of demographic information. 2. Histologically, most Paget's sarcomata resemble recognised tumour types as seen in otherwise normal bones, the most frequent forms being osteosarcoma and fibrosarcoma. The type of tumour arising in an osteitic bone is uninfluenced by age, sex or site of origin. Thirty-eight per cent of the cases were complicated by fracture, which occurred with slightly greater frequency in fibrosarcoma and with advancing age. A causal connection between sarcoma and fracture is supported but not vice versa. 3. Survival statisticsfor this series are compared with Bristol records for osteosarcoma and fibrosarcoma (without Paget's disease), the mean survival of seventy-four patients being 11·1 months from the date of the initial symptoms of malignancy. Four long-term survivors are reported, the five-year and ten-year survival rates being respectively 5 per cent and 3·1 per cent. The effects of age, site, sex, fracture and treatment upon survival are tabulated and discussed.
1. Fifty cases of fibrosarcoma from the records of the Bristol Bone Tumour Registry are reviewed. They present further clinical and radiological evidence supporting the concept of fibrosarcoma as a distinctive type of bone tumour. 2. The radiographic appearances have often been those ofan osteolytic lesion totally enclosed by reactive subperiosteal new bone. 3. Included in the group are eleven cases with Paget's osteitis deformans, three cases in which the sarcoma was associated with calcified cartilage and one case that is regarded as a post-irradiation sarcoma. 4. Several clinically differing modes of presentation are described. 5. Fibrosarcoma in bone has often been said to be "central" or "medullary" but because the present series includes tumours with eccentric origin and unilateral cortical destruction this description is inappropriate. 6. The histological differentiation from other fibroblastic lesions is briefly discussed. 7. The five-year and ten-year survival rates were respectively 28 per cent and 1 2 per cent, with an average survival oftwenty-four months in the thirty-six patients who died. Among the long survivors were two patients with Paget's disease.
1. The clinical, radiological, operative and histological features of twelve aneurysmal bone cysts are recorded. 2. Attention is drawn to atypical features, and the problem of diagnosis is discussed. 3. It is recommended that treatment should be complete excision, except where this would interfere with function. 4. Various theories on pathogenesis are considered and favour given to the view that these cysts result from a local change in haemodynamics occurring possibly in a pre-existing lesion.
1. Alkaline and acid phosphatase, non-specific esterase and beta-glucuronidase have been estimated and demonstrated histochemically in a series of bone tumours and allied lesions, of which ten were osteogenic sarcomata, ten were giant-cell lesions, eleven were fibroblastic lesions and seven were tumours of cartilage. 2. Osteogenic sarcoma was found to be characterised by high levels of alkaline phosphatase, with rich staining for this enzyme in the tumour cells. Similar high levels of alkaline phosphatase were found in other bone-forming lesions, such as fibrous dysplasia, a giant-cell sarcoma with osteogenic matrix, and fracture callus. 3. Giant-cell lesions were characterised by high levels of acid phosphatase, and intense staining for this enzyme in the osteoclasts. These cells were also found to be rich in non-specific esterase (as shown by the alpha-naphthyl acetate method) and in beta-glucuronidase, but almost or entirely lacking in alkaline phosphatase. High levels of alkaline phosphatase were not found in giant-cell lesions except in relation to osteogenic matrix. 4. Fibroblastic tumours were characterised by moderate levels of all four enzymes, with little or no staining for phosphatases in the tumour cells; non-specific esterase was generally present in a proportion of the cells. 5. In certain lesions intermediate stages in the differentiation of fibroblasts to osteoblasts were found, notably in fibrous dysplasia, in which the biochemical change preceded the histological. In such lesions high total levels of alkaline phosphatase were found. 6. Cartilaginous tumours were characterised by low levels of all four enzymes, and little histochemical staining except in hypertrophied cells in areas of ossification. 7. It was found in general that the enzyme distributions in these neoplasms and other lesions reflected the findings in comparable reactive and growing normal tissues.
1. The average number of cases of osteogenic sarcoma found in the years 1946-58 inclusive in the northern division of the South-west Hospital region of England was 6·7 for each year, varying from three to ten. 2. The annual incidence of this sarcoma in this area was one tumour per 230,000 population. 3. These figures were compared with similar figures from Norway which indicate a rather similar tumour incidence among juveniles, but a very much smaller number of tumours in elderly persons. 4. Among the eighty-seven sarcomata collected in the specified area in thirteen years twenty-Six were associated with Paget's disease. 5. It is estimated that among the population of the specified area there were probably 26,000 persons at any time with Paget's disease, of whom one in 650 (0·15 per cent) would eventually develop sarcoma. 6. This study suggests that Paget's disease increases the risk of sarcoma about thirty-fold in persons over forty years of age. 7. It is tentatively suggested that the incidence of Paget's disease is influenced more by heredity than environment.
1. The relationship between histological grading and survival has been studied in a consecutive series of eighty-eight patients with osteogenic sarcomata. The grading has been based entirely upon the mitotic activity of these tumours. 2. There is a positive correlation ("r"=+0·59) between the two variates mitotic ratio and survival in months. 3. The frequency distributions of the mitotic ratios and survivals are similar and, from the observed range, mean and mode of mitotic ratios a more precise definition is proposed for the terms "low," "medium" and "high" malignancy. 4. Comment is made on those patients (20 per cent) in whom actual survival was very different from that expected on the basis of histological grading. 5. The five-year survival rates were: Grade I–67 per cent, Grade II–15 per cent, Grade III–nil, all–17 per cent. For the whole series the average survival period from the time of the presenting symptom was thirty-six months. 6. The five-year survival rate for forty-five tumours of the femur was 20 per cent; no further analysis by sites is attempted.
1. Analyses are presented of comparable groups of the two commonest bone-forming tumoursâosteochondroma and osteogenic sarcomaâderived from the following sources: Bristol Bone Tumour Register; British Empire Cancer Campaign Annual Report (1949); Stocks and Barrington (1925); Meyerding (1927); and Geschickter and Copeland (1949). 2. From this study, the following features emerge as characteristic and common to both tumours. 1) Both tumours are commoner in the male during the age periods 0-34 years, and over fifty years, with the possible exceptions of tumours of the humerus during early life and tumours of the pelvis. 2) The male preponderance is most marked for the appendicular long bones. 3) The male preponderance is greatest during the age period 15-34 years. 4) In the separate bones, these tumours tend to arise at a somewhat earlier age in the female. 5) In both sexes both tumours tend to arise at an earlier age in the bones of the upper arm and shoulder girdle than in those of the lower limb and pelvis. 6) Both tumours are related to an aberration of endochondral growth in length of bone. 3. The greater growth of the male, and differences in skeletal development in the two sexes, offer a simple and reasonably consistent explanation of these peculiarities and make it possible to discern the interplay of the three factors of age, sex and site of origin of the tumour. 4. From this concept it is possible to make a crude estimate of the factors of time and bulk of tissue which when combined may well account for the observed male preponderance of these tumours, and their anatomical distribution.
1. The detailed findings are presented of a woman aged fifty who had widely distributed ossifying skeletal tumours, the structure and form of which have been shown to be low-grade osteoblastic osteogenic sarcoma. The patient was treated with heavy doses of P32, to which is attributed some clinical improvement, but which induced a fatal aplastic anaemia. 2. The study of the several tumours, together with the history, the radiographs and the post-mortem findings, suggests that the lesions are multifocal skeletal primary tumours rather than numerous osseous metastases secondary to a solitary new growth in any one bone. 3. The focal neoplastic lesion is discussed in the light of personal experience of other osteogenic sarcomata of low grade but of solitary origin, and of the multifocal form of bone sarcoma which may complicate Paget's osteitis deformans.