Aims. The purpose of this study was to review a large cohort of patients and further assess the correlation between the histological response to chemotherapy in patients with Ewing’s sarcoma with the overall (OS) and event-free survival (EFS). Patients and Methods. All patients treated for Ewing’s sarcoma between 1980 and 2012 were reviewed. Of these, 293 patients without metastases at the time of diagnosis and treated with chemotherapy and surgery were included. Patients were grouped according to the percentage of necrosis after chemotherapy: Group I: 0% to 50%, Group II: 51% to 99% and Group III: 100%. Results. The mean age at diagnosis was 16 years (1 to 62) and the mean follow-up was 9.1 years (six months to 32.6 years). The OS and EFS for the series were 75% and 65% at five years. There were significant differences in survival between the groups of necrosis: 0% to 50% (OS: 49% and EFS: 45% at five years, respectively) compared with 51% to 99% (OS: 72% and EFS: 59% at five years, respectively) and 100% (OS: 94% and EFS: 81% at five years, respectively) (p <  0.001). There were no significant differences in survival between patients treated between 1980 and 1989 compared with those treated between 1990 and 1999, and those treated between 2000 and 2012 (p = 0.55). Conclusion. Only patients with 100% necrosis after chemotherapy should be classified as having a good response to chemotherapy because they have significantly better rates of survival compared with those with any viable tumour in the surgical specimen. Cite this article: Bone Joint J 2016;98-B:1138–44

Aims. The aim of this study was to analyse a group of patients with non-metastatic Ewing’s sarcoma at presentation and identify prognostic factors affecting the development of local recurrence, in order to assess the role of radiotherapy. Patients and Methods. A retrospective review of all patients with a Ewing’s sarcoma treated between 1980 and 2012 was carried out. Only those treated with chemotherapy followed by surgery and/or radiotherapy were included. Patients were grouped according to site (central or limb) for further analysis of the prognostic factors. Results. A total of 388 patients were included in the study. Of these, 60 (15%) developed local recurrence at a mean median of 27 months (. sd. 24, range 7 to 150) and the five-year local recurrence-free survival (5yrLRFS) was 83%. For central tumours, the size of the tumour and histological response to chemotherapy were found to be significant factors for local recurrence. For limb tumours, local recurrence was affected by intralesional and marginal resections, but not by the histological response to chemotherapy. Radiotherapy in those with a marginal resection reduced the risk of local recurrence (5yrLRFS: 96% versus 81%, p = 0.044). Conclusion. Local recurrence significantly affects the overall survival in patients with a Ewing’s sarcoma. For those with a tumour in a limb, radiotherapy reduced the risk of local recurrence, especially in those with a marginal margin of excision, but the effect in central tumours was less clear. Radiotherapy for those who have had a wide margin of resection does not reduce the risk of local recurrence, regardless of the histological response to chemotherapy. Cite this article: Bone Joint J 2018;100-B: 247–55

Ewing Sarcoma is the second most common primary bone sarcoma in young patients, however, there remains geographical variation in the treatment of these tumours. All patients receive neoadjuvant chemotherapy and, in most cases, the soft tissue mass diminishes significantly in volume. Controversy surrounds whether to then treat the pre- or post-chemotherapy tumour volume. Many centres advocate either (1) resection of the pre-chemotherapy volume or (2) treatment of the pre-chemotherapy volume with radiation followed by resection of the post-chemotherapy volume. These approaches increase both the short and long-term morbidity for this young patient population. In this study, we retrospectively reviewed our experience resecting only the post-chemotherapy volume without the use of (neo)adjuvant radiotherapy. A retrospective analysis of all patients with Ewing Sarcoma treated at a tertiary orthopaedic oncology centre was conducted. All patients were treated as per the consensus opinion of the multidisciplinary tumour board. Demographic and oncological variables were collected from our institutional database. Presentation and re-staging MRI scans were reviewed to evaluate pre- and post-chemotherapy tumour volumes. Operative and pathology reports were utilized to determine the extent of the surgical resection. Outcome variables included local recurrence free-, metastasis free- and overall survival. Sixty-five patients were identified in our institutional database of which 56 did not receive (neo)adjuvant radiotherapy. Median age at diagnosis was 24 years (range 13–64), 60% of patients were male and 67.6% of tumours were located in the appendicular skeleton. All 56 patients not treated with radiotherapy had resection of the post-chemotherapy tumour volume. There were 3 local recurrences in this group with a mean follow-up of 70.8 months (range 2 to 328). The median overall survival was 47 months and the mean of 70.8months. The rate of local recurrence is comparable to reports in the literature in which patients had their entire pre-chemotherapy tumour volume treated by radiation and/or surgery. Similarly, two-year overall survival for our patient cohort is not significantly different from previous studies in which more aggressive local control measures were employed. Resecting the post-chemotherapy tumour volume in Ewing Sarcoma without the use of (neo)adjuvant radiotherapy does not appear to increase the risk of local recurrence or negatively impact overall survival. This approach should be studied further as it reduces the risk of short and long-term complications for this patient population.”

Aim. Local treatment of Ewing sarcoma of the hip bones and sacrum remains one of the most difficult tasks in the treatment of bone sarcomas. We investigated the difference between size, local treatment and overall survival in Ewing sarcoma of the sacrum and hip bones. Methods. Patients with Ewing sarcoma of the hip bones or sacrum, diagnosed between 1986 and 2009, were identified through the Scandinavian Sarcoma Group registry. Data regarding tumour size, local treatment (radiation or surgery), local recurrence, surgical margin, metastatic disease, and overall survival were analyzed and compared between the two locations (hip bone or sacrum). Results. 99 patients with Ewing tumour in the hip bones (74 patients) or the sacrum (25 patients) were identified. The mean size was 7.8 cm (sacrum) and 10.6 cm (hip bones), p=0.007. For tumours localised to the sacrum, 9% of the patients underwent surgery, 68 % received radiotherapy and 5% received both. For patients with tumours in the hip bones, 28% underwent surgery, while 32% received radiotherapy and 28% received both. All of the 6 patients with local recurrence died. There was a tendency (p=0.059) for better overall 5 year survival for patients with a tumour localised to the sacrum compared with patients with a tumour localised to the hip bones (58 vs. 32%). Conclusion. Ewing tumours are smaller in the sacrum than in the hip bones. Radiotherapy alone appears to give sufficient local control of Ewing sarcoma of the sacrum. Ewing sarcoma of the sacrum most probably has a better prognosis than Ewing sarcoma of the hip bones

Purpose: The purpose of this work was to analyse and compare survival in patients with osteosarcoma (OS) or Ewing sarcoma (EW) of the pelvis as a function of treatment. Material and methods: This retrospective series included 31 patients with OS (n=15) or EW (n=16) of the pelvis who were given a homogeneous therapeutic sequence associating chemotherapy, surgery and/or radiotherapy. Mean follow-up was 37 months (2–144). Mean age was 20 years for EW and 28 years for OS. Localisations in the pelvis were: zone I (n=12), zone I and II (n=4), zone II (n=1), zone II and III (n=7), zone III (n=1), and zone I, II and III (n=6). All patients were given chemotherapy, 15 underwent surgery, and 16 were given radiotherapy alone. Five patients were given complementary radiotherapy after surgery. Actuarial survival curves were compared with the logrank test. Comparison factors were presence of surgical resection, presence of initial or secondary metastasis, tumour response (radiographic measure), and pathology (good or poor responder) after chemotherapy. Results: Five-year survival rate for patients with EW was 53%, 31% for OS. There was no significant difference in survival rates between tumour type. The only factor significantly correlated with lower survival rate was presence of initial metastasis. Discussion and conclusion : The pelvic localisation of osteosarcoma and Ewing sarcoma is a factor of poor prognosis. Unlike data reported in the literature, surgery did not appear to influence outcome, not being found to be a factor of better prognosis. Surgery does however appear to improve short-term survival. In the pelvic localisation, osteosarcoma appears to have a poorer prognosis in terms of survival than Ewing tumour

Purpose of the study: Nearly all published series of Ewing sarcoma present the present of bone metastasis as a factor of very poor prognosis. Reviewing our experience, we noted that the prognosis is not as bad as expected in these patients if surgical resection of all known foci can be achieved. Case reports: Case n° 1 was a 16-year-old girl who presented a Ewing sarcoma involving the left iliopubic ramus. No other foci could be identified on the plain x-rays, scintigraphy and bone computed tomography. Preopeartive magnetic resonance imaging revealed a metastatic focus in the neck of the homolateral femur. The two foci were resected after preoperative chemotherapy: resection of the left hemi-pelvis and resection of the upper potion of the femur with replacement with a pelvic prosthesis and and massive prosthesis for the proximal femur. Eight years later, the patient has remained in complete primary remission, consulting for orthopedic gait problems related to prosthetic loosening. Case n° 2 was a 13-year-old boy who presented an Ewing sarcoma of the upper tibial metaphysic. Preoperative magnetic resonance imaging revealed three other metastatic localizations in the homolateral femur. Bifocal resection of the tibia and the femur was performed with implantation of an active growth prosthesis. Chemotherapy was continued. Seven years later, the patient remains in primary complete remission. Lengthening the prosthesis has enabled equivalent growth for the two limbs. The patient has a normal life style excepting contact sports which are prohibited. Case n° 3 was a 17-year-old boy who presented a voluminous Ewing sarcoma of the right pelvis. Search for extension revealed a unique metastasis in the fourth lumbar vertebra. The patient was given preoperative chemotherapy before resection of the pelvic tumor then two months later resection of the vertebral metastasis. The patient died 4.5 years later from a traffic accident. He had remained in complete remission. Discussion and conclusion: These three cases of complete long-term primary remission of patients with primary bone metastases show that like other bone sarcomas, eradication of all recognized bone metastases is essential for the prognosis of Ewing sarcoma

Purpose of the study: Prognosis is generally considered poor for patients with an iliac bone localization of Ewing sarcoma because the deep tumor is often large with initial metastases. This study demonstrates that the predictive value of these factors is related to treatment and that early en bloc resection can modify the prognosis. Material and method: We have treated 62 cases of Ewing tumor of the iliac bone since 1976, 36 males and 25 females, mean age 16.5 years (range 4 – 47). Thirteen patients presented primary metastases. Mean tumor size, measured by digital imaging, was 729 cm3. Adapted chemotherapy was given in all cases. Local treatment included exclusive radiotherapy in 20 patients, radiochemotherapy in 15 and exclusive en bloc extratumoral resection in 27. Results: At 15 years mean follow-up, overall relapse-free survival at ten years was 38%. This rate was 43% among patients without metastasis and 18% for those with initial metastasis (the three other patients underwent surgical resection of a primary focus and a bone metastasis). For patients with localized disease, prognosis was essentially determined by type and timing of local treatment. Surgical resection did not appear to have a significant effect on prognosis for patients operated on after three months; for these patients, only those with total histological response survived. Conversely, patient who underwent surgery before three months with en bloc resection and chemotherapy with at least five drugs had a relapse-free survival of 80% at ten years. Conclusion: The prognosis of Ewing sarcoma is seriously dependent on the therapeutic modality, even when the localization is known to have a poor prognosis such as the iliac bone. Early en bloc extratumoral resection (before three months) greatly improved the prognosis of patients without metastasis, even for those with a very large tumor. Conversely, prognosis remained very poor for patients given exclusive radiotherapy or operated on late

Aim: The aim of this study was to look at the presenting features, histological grade, size of primary tumour, method of treatment and patient and doctor delays in upper extremity Ewing sarcoma to observe the effects on local recurrence, metastasis and survival. Methods: 19 patients with upper extremity Ewing sarcoma were identified using the Scottish Bone Tumour Registry which carries clinical, pathological and radiological data on the majority of bone tumours diagnosed in Scotland over the past 50 years. Results: With increasing tumour Enneking grade at presentation there was a significantly higher mortality (X2=8.0606, p=0.0178). Patients with a higher grade also had an increased trend towards local recurrence (X2=5.1154, p=0.0775). Grade did not seem to influence the occurrence of metastasis. Patients with larger tumours tended to have a higher mortality (50% vs 27% dead at 5 years). All patients presented clinically with pain and all but two complained of some sort of swelling. It was found that there was a trend towards a higher grade in patients presenting with a longer duration of symptoms (X2=4.6269,p=0.0989). No difference in survival was noted between patients undergoing surgery and chemotherapy and patients undergoing radiotherapy and chemotherapy. Disease-free survival was 100% at both 5 and 10 years for Enneking Grade IIA, 56% at 5 and 10 years for Grade IIB and 0% at 5 years for Grade III. Conclusions: This study re-emphasises the importance of a delay in diagnosis on outcome. Longer symptom duration results in a higher histological grade at presentation. In turn a higher presenting grade is associated with a higher mortality. In agreement with other studies a larger primary tumour correlates with a poorer outcome. Outcomes in terms of survival are comparable for groups treated with adjuvant radiotherapy or surgery

Prolonged survival have been reached in the last two decades in patients with Ewing’s sarcoma due to combination of chemotherapy and radiotherapy. We report the analysis of 493 patients treated according to 4 different protocols in 23 years (Jan1983- Dec 2006).Aim of this study was to evaluate the occurrence of late toxicities as Second Malignant Neoplasms (SMN), Cardiomyopathies and sterility. Methods: We reviewed our database to find out all those patients aged from 1 to 40 yrs with localized Ewing’s sarcoma who were treated with chemotherapy according to 4 different protocols from 1983 to December 2006. Data were updated at Dec 2008. Results: 493 patients had adequate follow up and meet the eligibility criteria. Median age was 16 yrs (1–40) female/male: 183/310.Median overall survival 69 ms (4–302).220 patients died and 273 are alive. 44 pts received HDCT + PBSCR.Eleven SMN were found : 2 AMLeukemia, 2 parotid adenocarcinoma, 1 melanoma, 1 thyroid cancer and 5 radioinduced osteosarcoma. The interval between Ewing’s sarcoma diagnosis and leukaemia diagnosis was shorter then interval between Ewing’s sarcoma and RT osteosarcoma. Six patients reported a Cardiomyopathy : in 4 cases it was mild and pts are well compensated,2 patients needed heart transplant,. One of these two pts received also a kidney transplant due to chronic renal failure due to previous chemotherapy. Fertility: 17 women became pregnant after chemotherapy, 20 women experienced postTx amenorrea: 7 pts received RT in pelvic area, 9 did HDCT, 3 pts were over 30 yrs old. 9 male became father. 8 male patients did sperm analysis 3 azospermia, 4 oligospermia and 1 normal sperm count. No congenital abnormalities in offsprings were reported. Conclusions: In this casuistic the Cumulative Risk to have a SMN at 5 yrs is 1.8% and 2.9% at 10 yr. The SMN cumulative incidence in Ewing’s sarcoma seems to be lower then in our previous casistic in osteosarcoma patients (ASCO 2006)

Ewing’s sarcoma principally arises in bone but can also present as a soft tissue tumour. Very few studies have assessed the outcomes of extra-skeletal Ewing’s sarcomas. This study compares the oncological outcomes of the two forms of Ewing’s sarcomas to see if there is any difference in prognostic factors. 198 patients with primary, non metastatic Ewing’s sarcoma diagnosed between 1980 and 2005 were identified from our database. There were 118 males and 80 females with a median age of 15 years. The three most common sites of diagnosis were the femur (24%), pelvis (15%) and tibia (13%). There were 169(85%) bony Ewing’s and 29 (15%) extra-skeletal Ewing’s sarcomas. All patients received chemotherapy. 86% of the patients had surgery for local control but 28(14%) patients had radiotherapy. The overall survival at five years was 89% and was related to the age of patient (92% < 16years p=0.005), size (p=0.03) and site of tumour (p=0.004) as well as the response to chemotherapy. There was no difference in the overall survival of patients with bony Ewing’s (90%) and extra-skeletal Ewing’s (85%) (p=0.85). There was a 10% risk of local recurrence at 5 years with site of tumour (p=0.01) and surgical excision (p=0.05) being significant prognostic factors. The risk of local recurrence was also not related to the type of Ewing’s sarcoma. This large series has shown that the oncological outcomes of Ewing’s sarcoma is related to tumour characteristics, patient age and treatment factors and not determined by the tissue component

Ewing sarcoma (ES) and Osteosarcoma (OS) are the 2 most common malignant primary bone tumors. A patient's response to neoadjuvant chemotherapy has important implications in subsequent patient management and prognosis, as a favourable response to chemotherapy allows orthopedic oncologists to be more aggressive in pursuing limb-sparing surgery. An accurate and non-invasive pre-operative marker of response would be ideal for planning surgical margins and as a prognostic tool. ES and OS have differing biological characterisitcs and respond differently to chemotherapy. We reviewed 18F-FDG PET imaging characteristics of ES and OS patients at baseline and following treatment to determine whether this biological variation is reflected in their imaging phenotype. A retrospective review of ES and OS patients treated with neoadjuvant chemotherapy and surgery was done, correlating PET results with histologic response to chemotherapy. Change in the maximum standardized Uptake Value (SUVmax) between baseline and post-treatment scanning was not significantly associated with histologic response for either ES or OS. Metabolic tumor volume (MTV) and the percentage of injected 18F-FDG dose (%ID) in the primary tumor were found to be different for ES and OS response subgroups. A 50% reduction in MTV (MTV2:1 < 0.5) was found to be significantly associated with histologic response in OS. Using the same criteria for ES incorrectly predicted good responders. Increasing the cut-offs for ES to a 90% reduction in MTV (MTV 2:1 < 0.1) resulted in association with histologic response. Response to neoadjuvant chemotherapy as reflected by changes in PET characteristics should be interpreted differently for ES and OS

Background: Intensive chemotherapy in sarcoma treatment may lead to weight loss, and in turn reduce dose intensity. A possible correlation between weight loss under treatment and outcome has never been analysed in sarcoma treatment. Ewing sarcoma (ES) patients undergoing intensive non-corticosteroid-containing chemotherapy commonly experience weight loss. The German Paediatric Oncology/Haematology Society (GPOH) Ewing sarcoma trials (CESS, EICESS) registry was analysed with respect to weight loss and outcome. Patients and Methods: Body weight (BW) both at diagnosis and after a median of 12 courses of chemotherapy was available in 837 of 1549 ES patients, excluding amputees. Changes in BW were calculated as percentage of initial BW; outcome was determined as event-free-survival (EFS) from diagnosis according to Kaplan-Meier. Correlations of BW and outcome and potential confounders like disease stage or tumour volume were estimated uni- and multivariately. Results: Weight loss was not correlated with inferior outcome: A loss of 10%–20% of BW was associated with a slightly more favourable outcome (3-year EFS 0.64 +/−0.106, N=82) than weight gain of 10%–20% of BW (3-year EFS 0.58 +/−0.098, N=97), p=0.101. Multivariate analyses revealed no confounders interfering with these results. Conclusions: In 837 ES-patients analysed, weight loss did not correlate with inferior outcome. This should be observed in discussions about tube feeding and/or parenteral nutrition under cytostatic therapy. Future analyses of the prognostic impact of extreme under- or overweight both at diagnosis or under treatment are warranted in order to develop appropriate guidelines. Validity of this observation should be analysed for other solid tumours. Supported by EC Biomed and Deutsche Krebshilfe

The aim of this study was to identify whether there was any difference in patient, tumour, treatment or outcome characteristics between patients with skeletal or extra-skeletal Ewing’s sarcoma. We identified 300 patients with new primary Ewing’s sarcoma diagnosed between 1980 and 2005 from the centres’ local database. There were 253 (84%) with skeletal and 47 (16%) with extra-skeletal Ewing’s sarcomas. Although patients with skeletal Ewing’s were younger (mean age 16.8 years) than those with extra-skeletal Ewing’s sarcoma (mean age 27.5 years), there was little difference between the groups in terms of tumour stage or treatment. Nearly all the patients were treated with chemotherapy and most had surgery. There was no difference in the overall survival of patients with skeletal (64%) and extra-skeletal Ewing’s sarcoma (61%) (p = 0.85), and this was also the case when both groups were split by whether they had metastases or not. This large series has shown that the oncological outcomes of Ewing’s sarcoma are related to tumour characteristics and patient age, and not determined by whether they arise in bone or soft tissue

Ewing sarcoma (ES) is an aggressive sarcoma, and is the second most common bone sarcoma in childhood. Disease specific t(11;22)(~85–90%), t(21;22)(~5–10%), or rarer variant translocations with the involvement of chromosome 22 (~5%) are present. At the gene level, the EWSR1 gene fuses with FLI1, ERG or other ETS transcription factor family members. So far, no ES has been identified with a fusion to transcription factors other than ETS. By using a panel of molecular tools such as multicolor FISH and array-CGH, a ring chromosome containing chromosomes 20 and 22 was identified in four ES cases. Molecular karyotyping showed the translocation and amplification of regions of chromosomes 20q13 and 22q12. Cloning of the breakpoint showed an in-frame fusion between the EWSR1 and NFATc2 genes. The translocation led to the loss of the N-terminal, calcineurin-dependent control region. Consequently, the remaining intact DNA binding domain of NFATc2 is under control of the EWSR1 promoter region permitting oncogenic activation. Intriguingly, in all cases a distinct histological feature was observed. In conclusion: a new translocation involving EWS and NFATc2 was cloned that is associated with a histological variant of ES. The NFATc2 transcription factor is not a member of the ETS family of transcription factors. NFTAC2 has well characterized functions in T-cell differentiation and immune response. For the first time a direct involvement of NFATc2 in oncogenesis has been shown

The April 2023 Oncology Roundup. 360. looks at: Complete tumour necrosis after neoadjuvant chemotherapy defines good responders in patients with Ewing’s sarcoma; Monitoring vascularized fibular autograft: are radiographs enough?; Examining patient perspectives on sarcoma surveillance; The management of sacral tumours; Venous thromboembolism and major bleeding in the clinical course of osteosarcoma and Ewing’s sarcoma; Secondary malignancies after Ewing’s sarcoma: what is the disease burden?; Outcomes of distal radial endoprostheses for tumour reconstruction: a single centre experience over 15 years; Is anaerobic coverage during soft-tissue sarcoma resection needed?; Is anaerobic coverage during soft-tissue sarcoma resection needed?

Cellular mechanisms that account for tumour osteolysis associated with Ewing’s sarcoma are uncertain. Osteoclasts are marrow-derived multinucleated cells that effect tumour osteolysis. Osteoclasts are known to form from macrophages by both receptor activator for nuclear factor κB ligand (RANKL)-dependent and RANKL-independent mechanisms. In this study our aim has been to determine whether tumour-associated macrophages (TAMs) isolated from Ewing’s sarcoma are capable of differentiating into osteoclasts and to characterise the cellular and humoral mechanisms whereby this occurs. TAMs were isolated from two Ewing’s sarcomas and cultured on both coverslips and dentine slices for up to 21 days with soluble RANKL and human macrophage colony stimulating factor (M-CSF). Osteoclast formation from TAMs (CD14+) was evidenced by the formation of tartrate–resistant acid phosphatase and vitronectin receptor-positive multinucleated cells which were capable of carrying out lacunar resorption. This osteoclast formation and resorption was inhibited by the addition of the bisphosphonate, zoledronate. Osteoclast formation was also seen when Ewing’s sarcoma-derived TAMs were cultured with TNF α in the presence of M-CSF. We also found that TC71 Ewing’s sarcoma cells were capable of independently stimulating osteoclast formation through the release of a soluble factor. These results indicate that TAMs in Ewing’s sarcoma are capable of osteoclast differentiation by both RANKL-dependent and RANKL-independent mechanisms and that Ewing’s sarcoma cells produce an osteoclastogenic factor. The role bisphosphonates may play in inhibiting osteoclast formation and osteolysis in Ewing’s sarcoma merits further investigation

Aim: To review the epidemiology, prognostic factors and outcome of treatment for extra-osseous Ewing’s sarcoma. Method: Thirty-four patients with a diagnosis of extra-osseous Ewing’s sarcoma were identified from a prospectively gathered national tumour database between 1961 and 2005. Patient demographics, clinical features, tumour stage, location and size, treatment received, local recurrence, metastasis and survival were all recorded. Survival was analysed using Kaplan-Meier methods. The average follow up was 45 months. Results: The annual incidence of extra-osseous Ewing’s was 0.2 per million of population between 1970 and 2004. The five-year survival rate for extra-osseous Ewing’s was 62%, which is significantly better than previously reported in the literature. Indicators of a poor prognosis were extra-compartment spread of tumour, local recurrence and metastasis. The recorded annual incidence of extra-osseous Ewing’s sarcoma increased during the period of this study. Conclusions: Extra-osseous Ewing’s sarcoma appears to have similar demographics and the outcome following modern treatment is better than previously reported and no worse than that reported for osseous Ewing’s

Introduction: A retrospective analysis was performed to determine the oncological outcome of patients with Ewing’s sarcoma of the spine treated with combined chemotherapy and radiotherapy for definitive local control. Materials and Methods: Fifteen patients were identified from the Scottish Bone Tumour Registry with a histologically confirmed Ewing’s sarcoma affecting the axial skeleton. All case notes and imaging were retrospectively reviewed. Results: Primary vertebral Ewing’s sarcoma accounted for 8.3% of all malignant spinal lesions in our registry. The mean age was 17.8 years (between 4 and 39 years). There was a male predilection with 9 male and 6 female patients. Site was evenly distributed between cervical (4), dorsal (5) and lumbosacral (6) regions. Progressively worsening back pain was the first symptom in all the patients. Satisfactory imaging studies were available in all with plain radiographs (15), bone scan (11), CT-scan (12) and MR Scan (9) patients. Biopsy was performed in 11 patients and surgical treatment was carried out in 3 patients including curettage (2) and excision with bone grafting (1). All patients were treated with adjuvant radiotherapy while 87% also received adjuvant chemotherapy. Seven patients were alive with no evidence of disease at a mean 6 year follow-up. Six patients died of metastatic disease, one due to local recurrence and one with persistent primary disease. The mean follow-up time was 65 months (median 28 months; ranging from 12 to 218 months). Conclusions: Primary vertebral Ewing’s sarcoma comprised 8.3% of our National Registry’s primary malignant spinal lesions. Progressive vertebral pain in the late second decade and male gender should raise the suspicion of Ewing’s sarcoma. Ewing’s sarcoma of the spine treated with combined chemotherapy and radiotherapy for definitive local control achieved a 45% five year survival

We review the treatment of pelvic Ewing’s sarcoma by the implantation of extracorporeally-irradiated (ECI) autografts and compare the outcome with that of other reported methods. We treated 13 patients with ECI autografts between 1994 and 2004. There were seven males and six females with a median age of 15.7 years (interquartile range (IQR) 12.2 to 21.7). At a median follow-up of five years (IQR 1.8 to 7.4), the disease-free survival was 69% overall, and 75% if one patient with local recurrence after initial treatment elsewhere was excluded. Four patients died from distant metastases at a mean of 17 months (13 to 23). There were three complications which required operative intervention; one was a deep infection which required removal of the graft. The functional results gave a mean Musculoskeletal Tumor Society score of 85% (60% to 97%), a mean Toronto extremity salvage score of 86% (69% to 100%) and a mean Harris hip score of 92 (67 to 100). We conclude that ECI grafting is a suitable form of treatment for localised and resectable pelvic Ewing’s sarcoma

Aims. Ilium is the most common site of pelvic Ewing’s sarcoma (ES). Resection of the ilium and iliosacral joint causes pelvic disruption. However, the outcomes of resection and reconstruction are not well described. In this study, we report patients’ outcomes after resection of the ilium and iliosacral ES and reconstruction with a tibial strut allograft. Methods. Medical files of 43 patients with ilium and iliosacral ES who underwent surgical resection and reconstruction with a tibial strut allograft between January 2010 and October 2021 were reviewed. The lesions were classified into four resection zones: I. 1. , I. 2. , I. 3. , and I. 4. , based on the extent of resection. Functional outcomes, oncological outcomes, and surgical complications for each resection zone were of interest. Functional outcomes were assessed using a Musculoskeletal Tumor Society (MSTS) score and Toronto Extremity Salvage Score (TESS). Results. The mean age of the patients was 17 years (SD 9.1). At a mean follow-up of 70.8 months (SD 50), the mean functional outcomes were 24.2 points (SD 6.3) for MSTS and 81 points (SD 11) for TESS. The mean MSTS and TESS scores were associated with the iliac resection zone (< 0.001). Nine patients (20.9%) had local recurrence. The recurrence was not associated with the zone of iliac resection (p = 0.324). The two-year disease-free survival of the patients was 69.4%. The mean time to graft union was longer in patients with the I. 4. resection zone (p < 0.001). The complication rate was 34.9%, and nerve palsy (11.6%) was the most common. The rate of surgical complications was not associated with the resection zone. Conclusion. Reconstruction using tibial strut allograft is an efficient procedure after the resection of the ilium and iliosacral ES. Functional outcomes and complications of iliac ES depend on the resection zone, and inferior outcomes could be generally expected when more segments of the pelvic ring are resected, even if it is reconstructed. Cite this article: Bone Jt Open 2024;5(5):385–393