Between 1986 and 2002, 42 patients with synchronous multifocal osteosarcoma were treated with two different protocols of neoadjuvant chemotherapy. When feasible, the primary and secondary tumours were excised as a combined procedure. After initial chemotherapy 26 patients were excluded from simultaneous excision of all their secondary bone lesions as their disease was too advanced. In 12 patients only isolated excision of the primary lesion was possible. For 16 patients simultaneous operations were conducted to excise the primary and secondary lesions. This involved two supplementary sites in 15 patients and four additional sites in one patient. Of these, 15 attained remission but 12 relapsed and died (11 within two years). Three patients remained disease-free at five, six and 17 years. The histological response to pre-operative chemotherapy of the primary and secondary lesions was concordant in 13 of the 16 patients who underwent simultaneous operations at more than one site. The prognosis for synchronous multifocal osteosarcoma remains poor despite combined chemotherapy and surgery. The homogeneous histological responses in a large proportion of the primary and secondary lesions implies that synchronous multifocal osteosarcoma tumours are not multicentric in origin, but probably represent bone-to-bone metastases from a single tumour

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

Although an osteosarcoma appears to be a solitary lesion clinically, as in this instance, only routine radiographic skeletal survey in such cases will detect multiple osseous involvement. Ross (1964) reported that in ninety-eight cases of osteosarcoma arising in apparently normal bone, fifteen showed metastases to other bones, a much higher incidence than previously recorded. It is also possible that multicentric osteosarcomata, although undoubtedly rare, may be discovered more often if a radiological survey is done. In many large series of osteosarcomata no mention of a skeletal survey has been made, and, while this is routine in some centres, it is not yet general practice

Over a 25-year period we have treated 36 patients with osteosarcoma of the pelvis. Of the tumours, 24 (67%) were primary osteosarcomas and 12 (33%) arose either after irradiation or in association with Paget’s disease. Six patients had a hindquarter amputation and 12 were treated by a limb-salvage procedure with intrapelvic excision. The five-year survival rate of all the patients with pelvic osteosarcoma was 18%, while for 17 treated by chemotherapy and surgery it was 41%. The prognosis for patients presenting with metastases or with secondary osteosarcoma was appalling and none survived after 29 months. No patient over the age of 50 years when seen initially survived for a year. Youth and a good response to chemotherapy along with complete surgical excision offer the best chance of cure

We undertook a prospective study to evaluate the prognostic significance of the serum levels of vascular endothelial growth factor (VEGF) in predicting the survival of patients with osteosarcoma. The levels were measured by an enzyme-linked immunosorbent assay in 15 patients with osteosarcoma before commencing treatment. The patients were divided into two groups, with a high or a low serum VEGF level, and the incidence of metastases and overall survival rate were compared. No significant relationship was observed between the serum VEGF levels and gender, age, the size of the tumour or the response to pre-operative chemotherapy. Patients with a serum VEGF > 1000 pg/ml had significantly worse survival than those with a level < 1000 pg/ml (p = 0.002). The serum VEGF level may be useful in predicting the prognosis for survival in patients with osteosarcoma

Paediatric bone sarcomas are a dual challenge for orthopaedic surgeons in terms of tumour resection and reconstruction, as it is important to minimize functional and growth problems without compromising survival rates. Cañadell’s technique consists of a Type I epiphysiolysis performed using continuous distraction by an external fixator prior to resection. It was designed to achieve a safe margin due to the ability of the physeal cartilage to be a barrier to tumour spread in some situations, avoiding the need for articular reconstruction, and preserving the growth capacity most of the times. Despite initial doubts raised in the scientific community, this technique is now widely used in many countries for the treatment of metaphyseal paediatric bone sarcomas. This annotation highlights the importance of Cañadell’s work and reviews the experience of applying it to bone sarcoma patients over the last 40 years.

Cite this article: Bone Joint J 2023;105-B(1):11–16.

We evaluated the long-term outcome of patients with an osteosarcoma who had undergone prior manipulative therapy, a popular treatment in Asia, and investigated its effects on several prognostic factors. Of the 134 patients in this study, 70 (52%) patients had manipulative therapy and 64 (48%) did not. The age, location, and size of tumour were not significantly different between the groups. The five-year overall survival rate was 58% and 92% in the groups with and without manipulative therapy (p = 0.004). Both the primary and overall rates of lung metastasis were significantly higher in the manipulative group (primary: 32% vs 3%, p = 0.003; overall lung metastasis rate: 51.4% vs 18.8%, p < 0.001). Patients who had manipulative therapy had higher local recurrence rates in comparison to patients who did not (29% vs 6%, p = 0.011). The prognosis for patients with osteosarcoma who had manipulative therapy was significantly poorer than those who had not. Manipulative therapy was an independent factor for survival. This form of therapy may serve as a mechanism to accelerate the spread of tumour cells, and therefore must be avoided in order to improve the outcome for patients with an osteosarcoma

We describe a case of osteosarcoma of the scaphoid bone, which to our knowledge is only the second reported case of osteosarcoma in the carpus. A 38-year-old man complained of intense pain in the right wrist and had curettage and a bone graft for a lesion in the scaphoid. Histological examination showed this to be an osteosarcoma. Below-elbow amputation was performed and adjuvant chemotherapy given. There has been no evidence of recurrence or metastases at 33 months after amputation

This review of 27 cases serves to emphasis that periosteal chondrosarcoma and periosteal osteosarcoma are two distinct entities. Clinically, periosteal chondrosarcoma is less painful than periosteal osteosarcoma and runs a slower course. Radiographically, periosteal chondrosarcoma tends to affect the metaphysis and contains granular or "popcorn" opacities; while periosteal osteosarcoma more often affects the mid-diaphysis and shows lytic lesions with some spicules of reactive bone perpendicular to the underlying cortex. Histologically, periosteal chondrosarcoma shows lobular well-differentiated cartilage with Grade I or II (rarely Grade III) malignancy; periosteal osteosarcoma has a chondroid matrix with some osteoid component and Grade II or III malignancy. The prognosis in periosteal chondrosarcoma is good; conservative surgery is usually effective and metastases are very uncommon. In periosteal osteosarcoma the prognosis is less satisfactory but is better than that of other osteosarcomata; wide surgical excision is, however, needed and the incidence of metastases is about 15 per cent

Aims

Socioeconomic and racial disparities have been recognized as impacting the care of patients with cancer, however there are a lack of data examining the impact of these disparities on patients with bone sarcoma. The purpose of this study was to examine socioeconomic and racial disparities that impact the oncological outcomes of patients with bone sarcoma.

1. Nine patients treated for osteogenic sarcoma by elective radical irradiation are reviewed. Five of the nine patients have survived for from three to fourteen years, but one patient has metastases. 2. These results are compared with those from primary amputation. 3. The importance of histological grading in prognosis is emphasised. 4. It is concluded that radical irradiation should be considered in place of primary amputation for osteogenic sarcoma in the upper limb

We have studied 560 patients with osteosarcoma of a limb, who had been treated by neoadjuvant chemotherapy, in order to analyse the incidence of local and systemic recurrence according to the type of surgery undertaken. Of these, 465 patients had a limb-salvage procedure and 95 amputation or rotationplasty. At a median follow-up of 10.5 years there had been 225 recurrences. The five-year disease-free survival and overall survival rates were 60.7% and 68.5%, respectively, with no significant difference between patients undergoing amputation and those undergoing resection. The incidence of local recurrence was the same for patients treated by either amputation or limb salvage and correlated significantly with the margins of surgical excision and the histological response to chemotherapy. The outcome for patients with a local recurrence was significantly worse than for those who had recurrent disease with metastases only. We conclude that limb-salvage procedures are relatively safe in osteosarcoma treated by neoadjuvant chemotherapy. They should, however, only be performed in institutions where the margins of surgical excision and the histological response to chemotherapy can be accurately assessed. If the margins are inadequate and the histological response to chemotherapy is poor an immediate amputation should be considered

A retrospective study of patients with osteosarcoma was undertaken to determine whether there was a relationship between biopsy and survival. Fifty-seven patients treated at the Karolinska Hospital, Stockholm, between 1938 and 1959 were included in this study, all of whom were less than thirty years old, had a metaphysial osteosarcoma in a long bone but had no pulmonary metastases at the time of diagnosis; all were treated by amputation. No clinical variants of osteosarcoma were included. Twenty-four of the fifty-seven patients had an amputation without a prior biopsy; the others had biopsies before amputation. These two groups were fairly closely matched in age, sex, site and size of tumour, and in the level of amputation; some patients in each group received radiation before operation. Evaluation of these two groups of patients revealed that the performance of a biopsy, with or without a delay of not more than thirty days between the biopsy and the definitive operation, had no adverse effect on survival

1. The case histories of four siblings affected by osteogenic sarcoma are described. 2. The lesions appeared over a period of twelve years. The ages of the patients at the onset of symptoms were fifteen, twenty, eleven and twenty-two years. 3. The diagnosis of osteogenic sarcoma was in each case established by radiological and histological methods. 4. Two patients survived for eight and sixteen years after treatment and both are still alive and well

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

In 11 patients juxta-articular osteosarcoma around the knee was treated by intraepiphyseal excision of the tumour and reconstruction of the bone defect by distraction osteogenesis. Preoperative and postoperative chemotherapy was given to eight patients with high-grade tumours. The articular cartilage of the epiphysis and a maximum of healthy soft tissues were preserved. Distraction osteogenesis was then carried out. The mean gain in length was 9.7 cm. Full function of the limb was preserved in all except one patient, with a mean follow-up of 53.8 months. Treatment of juxta-articular osteosarcomas around the knee with joint preservation and biological reconstruction using distraction osteogenesis can give excellent functional results

The method of producing osteogenic sarcoma in rabbits by the injection of beryllium in the form of "zinc beryllium silicate" is presented. In five of ten animals which had such injections, osteogenic sarcomas developed several months later. There was new bone formation in the medullary cavities of the long bones before malignant changes were apparent. It is of particular interest to note that there was atrophy of the spleen in those animals in which bone tumours developed, whereas the spleen seemed to be quite normal in the rabbits which did not develop bone tumours. The tumours usually developed in the metaphysial regions. More than one tumour often developed in the same animal

From the European Osteosarcoma Intergroup study 202 patients were assessed with respect to their surgical treatment. Although treated in three different centres the survival of the three groups was identical (57% at five years). Two of the centres had rates of limb salvage of 85% and 83%, respectively, while the third had a rate of 49%. The corresponding risks of local recurrence were 13.3%, 6.8% and 2.5%, with all local recurrences arising in limbs with attempted limb salvage. Local recurrence was closely related to the adequacy of the margins of excision and to the chemotherapeutic response. Patients who had undergone limb-salvage surgery and who developed local recurrence still survival at five years). Of patients who relapsed, 31% of those with local recurrence alone were cured by further treatment, as compared with only 10% of those with metastases. Limb-salvage surgery with effective chemotherapy remains the optimum treatment for osteosarcoma

A poor response to chemotherapy (≤ 90% necrosis) for osteosarcomas leads to poorer survival and an increased risk of local recurrence, particularly if there is a close margin of excision. We evaluated whether amputation confers any survival benefit over limb salvage surgery (LSS) with narrow margins in patients who respond poorly to chemotherapy. We only analysed patients with an osteosarcoma of the limb, a poor response to chemotherapy and close margins on LSS (marginal/intralesional) or primary amputation: 360 patients (36 LSS (intralesional margins), 197 LSS (marginal margins) and 127 amputations) were included. Local recurrence developed in 13 (36%) following LSS with intralesional margins, and 39 (20%) following LSS with marginal margins. There was no local recurrence in patients who underwent amputation. The five-year survival for all patients was 41% (95% confidence interval (CI) 35 to 46), but for those treated by LSS with marginal margins was 46.2% (95% CI 38 to 53), 36.3% (95% CI 27 to 45) for those treated by amputation, and 28% (95 CI 14 to 44) for those treated by LSS with intralesional margins. Patients who had LSS and then developed local recurrence as a first event had the same survival as those who had primary amputation without local recurrence. Prophylactic adjuvant radiotherapy was used in 40 patients but had no discernible effect in preventing local recurrence. Although amputation offered better local control, it conferred no clear survival benefit over LSS with marginal margins in these patients with a poor overall prognosis. Cite this article: Bone Joint J 2015;97-B:115–20

We undertook a study of the anti-tumour effects of hyperthermia, delivered via magnetite cationic liposomes (MCLs), on local tumours and lung metastases in a mouse model of osteosarcoma. MCLs were injected into subcutaneous osteosarcomas (LM8) and subjected to an alternating magnetic field which induced a heating effect in MCLs. A control group of mice with tumours received MCLs but were not exposed to an AMF. A further group of mice with tumours were exposed to an AMF but had not been treated with MCLs. The distribution of MCLs and local and lung metastases was evaluated histologically. The weight and volume of local tumours and the number of lung metastases were determined. Expression of heat shock protein 70 was evaluated immunohistologically. Hyperthermia using MCLs effectively heated the targeted tumour to 45°C. The mean weight of the local tumour was significantly suppressed in the hyperthermia group (p = 0.013). The mice subjected to hyperthermia had significantly fewer lung metastases than the control mice (p = 0.005). Heat shock protein 70 was expressed in tumours treated with hyperthermia, but was not found in those tumours not exposed to hyperthermia. The results demonstrate a significant effect of hyperthermia on local tumours and reduces their potential to metastasise to the lung