Despite local treatment with systemic chemotherapy in Ewing’s sarcoma family tumours (ESFT), patients with detectable metastases at presentation have a markedly worse prognosis than those with apparently localised disease. We investigated the clinical, pathological and laboratory differences in 888 patients with ESFT, 702 with localised disease and 186 with overt metastases at presentation, seen at our institution between 1983 and 2006. Multivariate analyses showed that location in the pelvis, a high level of serum lactic dehydrogenase, the presence of fever and a short interval between the onset of symptoms and diagnosis were indicative of metastatic disease. The rate of overt metastases at presentation was 10% without these four risk factors, 22.7% with one, 31.4% with two, and 50% for those with three or four factors. We concluded that in ESFT the site, the serum level of lactic dehydrogenase, fever, and the interval between the onset of symptoms and diagnosis are indicators of tumours having a particularly aggressive metastatic behaviour

The aim of this study was to determine whether the high-sensitivity modified Glasgow prognostic score (Hs-mGPS) could predict the disease-specific survival and oncological outcome in adult patients with non-metastatic soft-tissue sarcoma before treatment. A total of 139 patients treated between 2001 and 2012 were retrospectively reviewed. The Hs-mGPS varied between 0 and 2. Patients with a score of 2 had a poorer disease-specific survival than patients with a score of 0 (p < 0.001). The estimated five-year rate of disease-specific survival for those with a score of 2 was 0%, compared with 85.4% (95% CI 77.3 to 93.5) for those with a score of 0. Those with a score of 2 also had a poorer disease-specific survival than those with a score of 1 (75.3%, 95% CI 55.8 to 94.8; p < 0.001). Patients with a score of 2 also had a poorer event-free rate than those with a score of 0 (p < 0.001). Those with a score of 2 also had a poorer event-free survival than did those with a score of 1 (p = 0.03). A multivariate analysis showed that the Hs-mGPS remained an independent predictor of survival and recurrence. The Hs-mGPS could be a useful prognostic marker in patients with a soft-tissue sarcoma. Cite this article: Bone Joint J 2015; 97-B:847–52

The outcome of tibial allograft reconstruction after resection of a tumour is inconsistent and has a high rate of failure. There are few reports on the use of tibial allografts in children with open growth plates. We performed 21 allograft reconstructions (16 osteoarticular, five intercalary) in 19 consecutive patients between seven and 17 years of age. Two had Ewing’s sarcoma, one an adamantinoma and 16 osteosarcoma, one with multifocal disease. Five patients have died; the other 14 were free from disease at the time of follow-up. Six surviving patients (eight allograft reconstructions) continue to have good or excellent function at a mean of 59 months (14 to 132). One patient has poor function at 31 months. The other seven patients have a good or excellent function after additional procedures including exchange of the allograft and resurfacing or revision to an endoprosthesis at a mean of 101 months (43 to 198). The additional operations were performed at a mean of 47 months (20 to 84) after the first reconstruction. With the use of allograft reconstruction in growing children, joints and growth plates may be preserved, at least partially. Although our results remain inconsistent, tibial allograft reconstruction in selected patients may restore complete and durable function of the limb

We describe a consecutive series of five patients with bone or soft-tissue sarcomas of the elbow and intra-articular extension treated by complex soft tissue, allograft bone and prosthetic joint replacement after wide extra-articular en bloc excision. All had a pedicled myocutaneous latissimus dorsi rotation flap for soft-tissue cover and reconstruction of the triceps. Wide negative surgical margins were obtained in all five patients. No local wound complications or infections were seen. There were no local recurrences at a mean follow-up of 60 months (20 to 105). The functional results were excellent in four patients and good in one. Longer term follow-up is necessary to confirm the durability of the elbow reconstruction

The role of radiotherapy and/or surgery in the local treatment of Ewing’s sarcoma has still to be determined. The outcome of Ewing’s sarcoma may differ according to its location and a selection bias towards surgery limits the ability to compare methods of local treatment. We have carried out a retrospective review of 91 consecutive patients treated for non-metastatic Ewing’s sarcoma of the femur. They received chemotherapy according to four different protocols. The primary lesion was treated by surgery alone (54 patients), surgery and radiotherapy (13) and radiotherapy alone (23). One was treated by chemotherapy alone. At a median follow-up of ten years, 48 patients (53%) remain free from disease, 39 (43%) have relapsed, two (2%) have died from chemotherapeutic toxicity and two (2%) have developed a radio-induced second tumour. The probability of survival without local recurrence was significantly (p = 0.01) higher in patients who were treated by surgery with or without radiotherapy (88%) than for patients who received radiotherapy alone (59%). The five- and ten-year overall survival rates were 64% and 57%, respectively. Patients who were treated by surgery, with or without radiotherapy, had a five- and ten-year overall survival of 64%. Patients who received only radiotherapy had a five- and ten-year survival of 57% and 44%, respectively. Our results indicate that in patients with Ewing’s sarcoma of the femur, better local control is achieved by surgical treatment (with or without radiotherapy) compared with the use of radiotherapy alone. Further studies are needed to verify the impact of this strategy on overall survival

In distal fibular resection without reconstruction, the stabilising effect of the lateral malleolus is lost. Thus, the ankle may collapse into valgus and may be unstable in varus. Here, we describe a child who underwent successful staged surgical correction of a severe neglected valgus deformity after excision of the distal fibula for a Ewing’s sarcoma

We studied the CT and MR scans, and the histology of 50 patients with primary Ewing’s sarcoma of bone to determine the association between the change in tumour volume and necrosis after chemotherapy, and to ascertain their influence on prognosis. The mean age of the patients was 17 years. The limbs were involved in 40 and the axial bones in ten. The volume of the tumour at diagnosis varied from 31 to 1790 ml. There was a significant relationship between necrosis and the measured change in volume of the tumour after chemotherapy. Progression of the tumour despite chemotherapy was seen only in patients with necrosis of grades 4 to 6. Necrosis significantly influenced survival (p < 0.05), but the effect of change in volume was less significant. Change in volume of the tumour is a good predictor of necrosis induced by chemotherapy. Necrosis is a strong prognostic factor in Ewing’s sarcoma

In 1999, we developed a technique for biological reconstruction after excision of a bone tumour, which involved using autografts of the bone containing the tumour treated with liquid nitrogen. We have previously reported the use of this technique in 28 patients at a mean follow up of 27 months (10 to 54). In this study, we included 72 patients who underwent reconstruction using this technique. A total of 33 patients died and three were lost to follow-up, at a mean of 23 months (2 to 56) post-operatively, leaving 36 patients available for a assessment at a mean of 101 months 16 to 163) post-operatively. The methods of reconstruction included an osteo-articular graft in 16, an intercalary in 13 and, a composite graft with prosthesis in seven. Post-operative function was excellent in 26 patients (72.2%), good in seven (19.4%), and fair in three (8.3%) according to the functional evaluation system of Enneking. No recurrent tumour occurred within the grafts. The autografts survived in 29 patients (80.6%), and the rates of survival at five and ten years were 86.1% and 80.6 %, respectively. Seven of 16 osteo-articular grafts (44%) failed because of fracture or infection, but all the composite and intercalary grafts survived. The long-term outcomes of frozen autografting, particularly using composite and intercalary grafts, are satisfactory and thus represent a good method of treatment for patients with a sarcoma of bone or soft tissue. Cite this article: Bone Joint J 2014;96-B:555–61

1. Five cases of fibrosarcoma arising after radiotherapy in cases of ankylosing spondylitis are reported. 2. The relationship of the tumours to irradiation is discussed. 3. Objective neurological signs arising in the lower limbs of patients with ankylosing spondylitis after a latent period following radiotherapy may indicate an underlying sarcoma

1. Thirty-nine patients with synovial sarcoma have been reviewed. 2. The average rate of five-year survival was 45 per cent; of ten-year survival, 30 per cent; and of survival for more than ten years, 10 per cent. 3. The only important factor influencing the long-term results was the method of treatment; primary amputation was by far the best

1. Two cases are described in which increased density of the lower femoral epiphysis was due to sarcoma. 2. In one of these, multiple sarcomata were present

We treated five children with non-metastatic Ewing's sarcoma of the distal fibula by distal fibulectomy. At a mean of eight years after surgery they all had nearly normal levels of painfree function. Distal fibulectomy is a good alternative to the more radical amputations recommended in the past

We analysed 256 patients with primary soft-tissue sarcoma of the extremities diagnosed between 1970 and 1990 to see if tumour-related and host-related prognostic factors influenced both the selection of operation and the margin of clearance obtained at surgery. Amputation was more often performed in young patients, in those with distal tumours, and in those with deep-seated tumours. Inadequate surgical margins (those with a high risk of local recurrence) were more common in patients with deep-seated and large tumours than in patients with superficial and small tumours. These features of the tumour and the host, which have been shown to be prognostic for survival, also influenced the choice and performance of surgical procedures

Out of 21 900 cases filed at the Latin-American Registry of Bone Pathology between April 1940 and July 1981, there were 987 with Paget's disease (4.51 per cent); 62 of these (6.28 per cent) were complicated by sarcoma and two were associated with giant-cell tumours of bone (osteoclastoma) without signs of malignancy. There was a slight predominance of men and the ages ranged from 45 to 87 years, with an average of 66 years. The most frequent sites were the femur (23 cases), the humerus (nine), the pelvis (10), and the tibia (nine). The low incidence of vertebral involvement (five cases) is noteworthy and is in sharp contrast to uncomplicated Paget's disease. The most common tumour type was osteosarcoma (39 cases), followed by fibrosarcoma (15 cases); other varieties (chondrosarcoma, malignant fibrous histiocytoma and reticulum-cell sarcoma) were much rarer. Most of the sarcomata occurred when the Paget's disease was polyostotic. Tumours often developed simultaneously, or at short time intervals, in the same or different bones; these bones had, in all cases, been affected by Paget's disease. The histological features of the osteosarcomata were characteristic, with large numbers of osteoclast giant cells, alternating with atypical osteoblasts, thus exaggerating the anarchic remodelling process of Paget's disease. The neighbouring areas of the pagetic bone showed an increased number of osteoclasts. These facts suggest a possible pathogenetic relationship between sarcoma and Paget's disease; the possibility of both processes having a viral aetiology is discussed

A case is reported in which an epithelioid sarcoma developed in an apparently benign enchondroma or bone infarct at the site of a chrome-cobalt total knee replacement

A patient with Paget's sarcoma of the femur, alive and well seven years after amputation, is reported. Long survival in two previously reported cases is also mentioned

We analysed metastasis-free survival after local recurrence in a population-based series of 432 patients treated for soft-tissue sarcoma of the extremities or trunk wall. Local recurrence was found in 124 patients; of these, 73 had no detectable metastases when the last local recurrence was diagnosed. No patient received chemotherapy for local recurrence. The 73 patients had a 5-year metastasis-free survival rate of 0.73 after the last local recurrence, compared with 0.47 in all 124 patients with local recurrence. The 5-year metastasis-free survival rate was 0.76 in the 308 patients who had never developed local recurrence, and 0.68 in the whole series. The selection of patients with local recurrence but no concurrent metastasis creates a subset of the population with a good prognosis; this makes it difficult to evaluate the effect of treatment for local recurrence on survival rate. In studies of prognosis this group of patients should be analysed separately from patients with primary tumours

Seven patients with Ewing's sarcoma of the pelvis were treated by chemotherapy followed by wide resection of the primary tumour. Although good function after operation is possible, survival in this series reflects the poor prognosis associated with the disease; two patients died, two are alive with local recurrence and metastases and three patients are alive with no evidence of disease

The results are presented of thirty-seven patients with Ewing's sarcoma; ten were treated by a combination of operation, radiotherapy and cyclic chemotherapy, the remainder by radiotherapy and chemotherapy but without operation. The drugs, vincristine, cyclophosphamide and adriamycin were used in combination and were continued for two years. The follow-up ranged from twelve to sixty-two months. The mortality rate and the incidence of metastases were both markedly lower than in a comparable previous series treated by radiotherapy alone, or by operation plus radiotherapy, but all without chemotherapy. The percentage of local recurrences and of metastases was much higher in the twenty-seven patients who had radiotherapy and adjuvant chemotherapy, than in the ten in whom operation was also performed. It is suggested that on the basis of these results (and on theoretical grounds) treatment should consist of radiotherapy combined with chemotherapy plus, whenever feasible, operative excision of the primary tumour