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

Purpose. Ewings Sarcoma (ES) and Osteosarcoma (OS) behave and respond differently to chemotherapy and any interpretation of diagnostics tests to predict a patients response to treatment must consider this. We reviewed 18F-FDG PET imaging characteristics of consecutive series of ES and OS patients to determine if any differences in PET imaging existed between them. Method. A retrospective review was performed of 31 patients with ES and OS who received all their treatment by our group and who had pre- and post-chemotherapy 18F-FDG PET scans at the Peter MacCallum Cancer Centre from Jan 1, 1999 to December 1, 2009 (Table 1). Patients who did not have both their pre- and post-chemotherapy PET scans done at Peter MacCallum Cancer Centre were excluded from the study to remove bias from having different PET scanning protocols. Patients received neoadjuvant chemotherapy according to standard protocols, all starting within 2 weeks after the initial pre-chemotherapy PET scans (PET1). The PET scan taken after the last cycle of chemotherapy prior to surgery was considered as the post-chemotherapy scan (PET2). The ratio between pre and post-chemotherapy for each PET parameter was then associated with the histology response for both ES and OS, and positive (PPV) and negative predicting values (NPV) of each parameter were calculated. Results. Standardized Uptake Values (SUV) was not significantly associated with histologic response for both ES and OS. Metabolic tumor volume (MTV) and accumulation percentage of 18F-FDG (%ID) was found to be different for ES and OS. 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. Conclusion. Response to neoadjuvant chemotherapy as reflected by changes in PET characteristics should be interpreted differently for ES and OS

Our purpose was to assess the role of preoperative radio-therapy +/− neoadjuvant chemotherapy in nonmetastatic soft tissue sarcoma of extremities for limb-sparing surgery and identify the role of neoadjuvant therapies on local control and survival rate. Forty-seven patients with soft tissue sarcoma of extremities who were treated at Cerrahpasa Medical Faculty within a limb salvage protocol, including preoperative radiotherapy +/− chemotherapy were retrospectively analized. Median age was 45 years (17–72 years). The tumor size was between 5–33 cm. Seventeen patients were in stage I, 11 in stage II, 19 in stage III. The most common histology was synovial sarcoma. Nine patients were treated for locally recurrent tumour. The tumour and surrounding tissues with probable microscopic tumour involvement observed clinically and radiologically, were irradiated. Thirty-two patients, with a high grade tumour and/or tumours larger than 8 cm, also received neoadjuvant chemotherapy. Neoadjuvant chemotherapy regimen was consisted of doxorubicine and ifosphamide with mesna. Preoperative radiotherapy was applied, usually between the second and third cycles of chemotherapy. Definitive surgery was administered 2–6 weeks after radiotherapy or after the third cycle of chemotherapy. Chemotherapy was completed to 6 courses after the surgery. Postoperative external beam radio-therapy boost of 16 Gy was given who had close or positive surgical margins. Median follow-up time was 67 months (12–217 months). All of the patients had limb-sparing surgery. Patients had; 30 marginal excision, 13 wide local excision, 4 radical resection. Nine patients locally recurred. Limb-sparing surgery was performed for 8 patients. 25 patients had distant metastases. Metastasectomy were applied for 10 patients with lung metastasis. The 5-year local control, disease free survival and overall survival rates were 82.3%, 50.1% and 67.2%, respectively. Preoperative radiotherapy +/− chemotherapy seems to increase the chance of extremity-sparing surgery with good local control and the survival rates which were comparable with the literature

Aims. The aim of this study was to determine the rate of indocyanine green (ICG) staining of bone and soft-tissue tumours, as well as the stability and accuracy of ICG fluorescence imaging in detecting tumour residuals during surgery for bone and soft-tissue tumours. Methods. ICG fluorescence imaging was performed during surgery in 34 patients with bone and soft-tissue tumours. ICG was administered intravenously at a dose of 2 mg/kg over a period of 60 minutes on the day prior to surgery. The tumour stain rate and signal-to-background ratio of each tumour were post hoc analyzed. After tumour resection, the tumour bed was scanned to locate sites with fluorescence residuals, which were subsequently inspected and biopsied. Results. The overall tumour stain rate was 88% (30/34 patients), and specific stain rates included 90% for osteosarcomas and 92% for giant cell tumours. For malignant tumours, the overall stain rate was 94%, while it was 82% for benign tumours. The ICG tumour stain was not influenced by different pathologies, such as malignant versus benign pathology, the reception (or lack thereof) of neoadjuvant chemotherapies, the length of time between drug administration and surgery, the number of doses of denosumab for patients with giant cell tumours, or the tumour response to neoadjuvant chemotherapy. The overall accuracy rate of successfully predicting tumour residuals using fluorescence was 49% (23/47 pieces of tissue). The accuracy rate after en bloc resection was significantly lower than that after piecemeal resection (16% vs 71%; p < 0.001). Conclusion. A high percentage of bone and soft-tissue tumours can be stained by ICG and the tumour staining with ICG was stable. This approach can be used in both benign and malignant tumours, regardless of whether neoadjuvant chemotherapy is adopted. The technique is also useful to detect tumour residuals in the wound, especially in patients undergoing piecemeal resection. Cite this article: Bone Joint J 2023;105-B(5):551–558

Neoadjuvant therapy in soft-tissue sarcomas is still a controversial issues regarding indications, patients selection and treatment protocols. In the last fifteen years (1990–2005) at our Institution more than 600 patients affected by soft tissue sarcomas of the limbs and superficial trunk were surgically treated. Among these patients, 49 received preoperative chemotherapy (epirubicin plus ifosfamide, according to Italian Sarcoma Group protocol), associated to preoperative conventional external beam radiationtherapy in 36 cases (73.5%). The histologic types were liposarcoma (30,6%), synovial sarcoma (20,4%), fibrosarcoma (16,3%), pleomorphic sarcoma or malignant fibrous histiocitoma (12,1%), leiomyosarcoma (8,2%), other histotypes (12,1%). Tumor size was 10 cm or larger in 21 cases, 6 to 9 cm in 23 patients and 5 cm or smaller in 5 cases. Neoplasms were high-grade (Broders grade 3 or 4) in all cases but five.

After neoadjuvant treatment we performed a limb-sparing surgical excision of the tumor in 47 patients (96%), while a primary amputation of the limb was necessary in only two cases (4.1%). A vascularized miocutaneous flap was used in 8 cases, and adequate surgical margins were achieved in more than 70% of the cases.

Postoperative chemotherapy was given in 26 cases (53%), postoperative radiotherapy just in 5 (10%). We report the outcome data on these 49 cases, regarding overall survival, local or distant relapse, local and systemic complications, early and long-term limb salvage rate.

According to histologic examination of the resection specimen, average percent of necrosis after neoadjuvant treatment was 70.6% (range 30 – 99%). Wound dehiscence occurred in 6 patients but ultimately healed succesfully in all of them.

At an average follow-up of 23 months (range 3 – 82), 37 patients were continuously disease free (76%), two patients had local recurrence (one amputated), four patients were alive with metastatic disease, five patients had died with disseminated disease (at 4, 19, 28, 37 and 61 months after surgery), one patient had died of unrelated disease.

Due to the inconstant tumor response, neoadjuvant treatment in soft tissue tumors is still a controversial issue. On the basis of data presently available, we think that it can be a useful treatment in high-risk tumors (larger than 5 cm; high grade). In these cases, at a low and acceptable rate of local complications, the conjoined use of preoperative chemotherapy and radiotherapy can help to make a limb-salvage surgery possible and at the same time can maybe reduce the risk of distant metastasis.

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).

Aims. For paediatric and adolescent patients with growth potential, preservation of the physiological joint by transepiphyseal resection (TER) of the femur confers definite advantages over arthroplasty procedures. We hypothesized that the extent of the tumour and changes in its extent after neoadjuvant chemotherapy are essential factors in the selection of this procedure, and can be assessed with MRI. The oncological and functional outcomes of the procedure were reviewed to confirm its safety and efficacy. Methods. We retrospectively reviewed 16 patients (seven male and nine female, mean age 12.2 years (7 to 16)) with osteosarcoma of the knee who had been treated by TER. We evaluated the MRI scans before and after neoadjuvant chemotherapy for all patients to assess the extent of the disease and the response to treatment. Results. The mean follow-up period was 64.3 months (25 to 148) after surgery and no patients were lost to follow-up. On MRI evaluation, 13 tumours were near but not in contact with the physes and three tumours were partially in contact with the physes before neoadjuvant chemotherapy. Bone oedema in the epiphysis was observed in eight patients. After neoadjuvant chemotherapy, bone oedema in the epiphysis disappeared in all patients. In total, 11 tumours were not in contact and five tumours were in partial contact with the physes. The postoperative pathological margin was negative in all patients. At the last follow-up, 12 patients were continuously disease-free and three had no evidence of disease. One patient died due to the disease. Functionally, the patients with retained allograft or recycled autograft had a mean knee range of flexion of 126° (90° to 150°). The mean Musculoskeletal Tumor Society functional score was 27.6 (23 to 30). Conclusion. TER is an effective limb-salvage technique for treating malignant metaphyseal bone tumours in paediatric and young osteosarcoma patients with open physes when a good response to chemotherapy and no progression of the tumour to the epiphysis have been confirmed by MRI. Cite this article: Bone Joint J 2020;102-B(6):772–778

The December 2022 Oncology Roundup. 360. looks at: Is high-dose radiation therapy associated with early revision with a cemented endoprosthesis?; Neoadjuvant chemotherapy and endoprosthetic reconstruction for lower extremity sarcomas: does timing impact complication rates?; Late amputation after treatment for lower extremity sarcoma; Osteosarcoma prediagnosed as another tumour: a report from the Cooperative Osteosarcoma Study Group; The influence of site on the incidence and diagnosis of solitary central cartilage tumours of the femur: a 21st century perspective

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?

Aims. The aim of this study was to determine the risk of local recurrence and survival in patients with osteosarcoma based on the proximity of the tumour to the major vessels. Patients and Methods. A total of 226 patients with high-grade non-metastatic osteosarcoma in the limbs were investigated. Median age at diagnosis was 15 years (4 to 67) with the ratio of male to female patients being 1.5:1. The most common site of the tumour was the femur (n = 103) followed by tibia (n = 66). The vascular proximity was categorized based on the preoperative MRI after neoadjuvant chemotherapy into four types: type 1 > 5 mm; type 2 ≤ 5 mm, > 0 mm; type 3 attached; type 4 surrounded. Results. Limb salvage rate based on the proximity type was 92%, 88%, 51%, and 0% for types 1 to 4, respectively, and the overall survival at five years was 82%, 77%, 57%, and 67%, respectively (p < 0.001). Local recurrence rate in patients with limb-salvage surgery was 7%, 8%, and 22% for the types 1 to 3, respectively (p = 0.041), and local recurrence at the perivascular area was observed in 1% and 4% for type 2 and 3, respectively. The mean microscopic margin to the major vessels was 6.9 mm, 3.0 mm, and 1.4 mm for types 1 to 3, respectively. In type 3, local recurrence-free survival with limb salvage was significantly poorer compared with amputation (p = 0.025), while the latter offered no overall survival benefit. In this group of patients, factors such as good response to chemotherapy or limited vascular attachment to less than half circumference or longitudinal 10 mm reduced the risk of local recurrence. Conclusion. The proximity of osteosarcoma to major blood vessels is a poor prognostic factor for local control and survival. Amputation offers better local control for tumours attached to the blood vessels but does not improve survival. Limb salvage surgery offers similar local control if the tumour attachment to blood vessels is limited. Cite this article: Bone Joint J 2019;101-B:1024–1031

Traditional staging systems for high grade osteosarcoma (Enneking, MSTS) are based largely on gross surgical margins and were developed before the widespread use of neoadjuvant chemotherapy. It is now well known that both microscopic margins and chemotherapy are predictors of local recurrence. However, neither of these variables are used in the traditional surgical staging and the precise safe margin distance is debated. Recently, a novel staging system utilizing a 2mm margin cutoff and incorporating precent necrosis was proposed and demonstrated improved prognostic value for local recurrence free survival (LRFS) when compared to the MSTS staging system. This staging system has not been validated beyond the original patient cohort. We propose to analyze this staging system in a cohort of patients with high-grade osteosarcoma, as well as evaluate the ability of additional variables to predict the risk of local recurrence and overall survival. A retrospective review of a prospectively collected database of all sarcoma patients between 1985 and 2020 at a tertiary sarcoma care center was performed. All patients with high-grade osteosarcoma receiving neo-adjuvant chemotherapy and with no evidence of metastatic disease on presentation were isolated and analyzed. A minimum of two year follow up was used for surviving patients. A total of 225 patients were identified meeting these criteria. Univariate analysis was performed to evaluate variable that were associated with LRFS. Multivariate analysis is used to further analyze factors associated with LRFS on univariate analysis. There were 20 patients (8.9%) who had locally recurrent disease. Five-year LRFS was significantly different for patients with surgical margins 2mm or less (77.6% v. 93.3%; p=0.006) and those with a central tumor location (67.9 v. 94.4; <0.001). A four-tiered staging system using 2mm surgical margins and a percent necrosis of 90% of greater was also a significant predictor of 5-year LRFS (p=0.019) in this cohort. Notably, percent necrosis in isolation was not a predictor of LRFS in this cohort (p=0.875). Tumor size, gender, and type of surgery (amputation v. limb salvage) were also analyzed and not associated with LRFS. The MSTS surgical margin staging system did not significantly stratify groups (0.066). A 2mm surgical margin cutoff was predictive of 5-year LRFS in this cohort of patients with localized high-grade osteosarcoma and a combination of a 2mm margin and percent necrosis outperformed the prognostic value of the traditional MSTS staging system. Utilization of this system may improve the ability of surgeons to stage thier patients. Additional variables may increase the value of this system and further validation is required

There are eight reported cases in the literature of osteosarcomas secreting β-hCG. Our primary aim was to investigate the rate of β-hCG expression in osteosarcoma and attempt to understand the characteristics of osteosarcomas that secrete β-hCG. We reviewed 37 histopathology slides (14 biopsies and 23 surgical specimens) from 32 patients with osteosarcoma. The slides were retrospectively stained for β-hCG expression. Patient and tumour characteristics, including age, gender, tumour location, subtype, proportion of necrosis, presence of metastases and recurrence were recorded. A total of five of the 32 tumours were found to be positive for β-hCG expression (one strongly and four weakly). This incidence of this expression was found in tumours with poor histological response to neoadjuvant chemotherapy. The use of β-hCG expression as a diagnostic, prognostic or follow-up marker is questionable and needs further investigation with a larger sample size

Aims. Clear cell sarcoma (CCS) of soft-tissue is a rare melanocytic subtype of mesenchymal malignancy. The aim of this study was to investigate the clinical and therapeutic factors associated with increased survival, stratified by clinical stage, in order to determine the optimal treatment. Methods. The study was a retrospective analysis involving 117 patients with histologically confirmed CCS, between July 2016 and November 2017, who were enrolled in the Bone and Soft Tissue Tumour Registry in Japan. Results. The five- and ten-year survival rates were 41% (95% confidence interval (CI) 29 to 52) and 37% (95% CI 25 to 49), respectively. On multivariable analysis, the size of the tumour of > 10 cm (p = 0.006), lymph node metastasis at the time of diagnosis (p < 0.001), distant metastases at the time of diagnosis (p < 0.001), and no surgery for the primary tumour (p = 0.019) were independently associated with a poor survival. For N0M0 CCS (n = 68), the development of distant metastases was an independent prognostic factor for survival (early (< 12 months), hazard ratio (HR) 116.78 (95% CI 11.69 to 1,166.50); p < 0.001; late (> 12 months), HR 14.79 (95% CI 1.66 to 131.63); p = 0.016); neoadjuvant/adjuvant chemotherapy (p = 0.895) and/or radiotherapy (p = 0.216) were not significantly associated with survival. The five-year cumulative incidence of local recurrence was 19% (95% CI 8 to 35) and the size of the tumour was significantly associated with an increased rate of local recurrence (p = 0.012). For N1M0 CCS (n = 18), the risk of mortality was significantly lower in patients who underwent surgery for both the primary tumour and lymph node metastases (HR 0.03 (95% CI 0.00 to 0.56); p = 0.020). For M1 CCS (n = 31), excision of the primary tumour was independently associated with better survival (HR 0.26 (95% CI 0.09 to 0.76); p = 0.013). There was no significant difference in survival between the different types of systemic treatment (p = 0.523). Conclusion. Complete excision of the primary tumour and lymph nodes is associated with a better survival in patients with CCS. Systemic treatment appears to provide limited benefits, demonstrating a pressing need for novel systemic agents. Cite this article: Bone Joint J 2023;105-B(11):1216–1225

We investigated the effect of adjuvant and neoadjuvant chemotherapy regimens on the tibial regenerate after removal of the external fixator in a rabbit model of distraction osteogenesis using New Zealand white rabbits. Forty rabbits were randomly distributed into two groups. In the neoadjuvant group, half of the rabbits received 1mg/kg cisplatinum & 2mg/kg adriamycin at eight weeks of age followed by 1mg/kg cisplatinum & 4mg/kg adriamycin at ten weeks of age. The remaining ten received an identical volume of normal saline using the same regimen. The adjuvant group differed only in the timing of the chemotherapy infusion. Half received the initial infusion ten days prior to the osteotomy, with the second infusion four days following the osteotomy. Again, the remaining ten rabbits received an identical volume of normal saline using the same regimen. This produced an identical interval between infusions and identical age at osteotomy in both groups. All rabbits underwent a tibial osteotomy at 12 weeks of age. Distraction started 24hours after osteotomy at a rate of 0.75mm a day for 10 days, followed by 18 days without correction to allow for consolidation of the regenerate. At week 16 there was no difference in Bone Mineral Density (BMD), Bone Mineral Content (BMC) or volumetric Bone Mineral Density (vBMD) in the adjuvant group. Neoadjuvant chemotherapy appears to have a significant detrimental effect on BMD, vBMD and BMC. Despite this there were no significant alterations in the mechanical properties of the regenerate. Histologically there was a trend for increased cortical thickness in the control groups compared to intervention however this did not prove statistically significant. In conclusion, adjuvant chemotherapy may be more beneficial for cases where distraction osteogenesis is being considered to replace segmental bone loss after tumour excision

Introduction. Telangiectatic osteosarcoma (TOS) is a rare subtype of osteosarcoma. We review our experience to characterize its prevalence, treatment, relapse and survivorship at long term follow-up. Methods. Eighty-seven patients aged from 4 to 60 years (mean 20 years), were treated from 1985 to 2008. Lesions affected the femur (38), humerus (20), tibia (19), fibula (4), pelvis (3), foot (2) and radius (1). Eight patients had metastatic disease at diagnosis. Seventy-eight patients were treated with neoadjuvant chemotherapy with three or more drugs according to different protocols, nine had surgery as first treatment. Limb salvage surgery was performed in 71 cases, amputation in 14 and rotationplasty in one. One patient died before surgery. Prognostic factors were evaluated with Kaplan-Meier analysis. Results. At a mean follow-up of 8 years, overall survival was 81%, 65% and 65% at 2, 5 and 10 years respectively. Fifty-two patients were disease-free, three were alive with disease, twenty-nine died with disease and three died of other causes. Thirteen local recurrences were observed. Twenty-three patients developed lung (20) or bone (3) metastases. Pathologic fracture did not significantly influence survivorship. Prognostic influence of age of the patients was evaluated at three different cut-off (15, 20 and 25 years-old): younger patients had better survivorship, without statistical significance. Induced necrosis according to Huvos’ classification was significant at both univariate and multivariate regression Cox analysis (p=0.0001). Conclusion. TOS does not have a poor prognosis as previously reported in the literature. A high percentage of patients can be cured with neoadjuvant chemotherapy and surgery. In most patients, limb sparing surgery is possible and safe

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

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

Introduction: Seeding of bone or soft tissue tumour along the biopsy tract is a known complication of percutaneous biopsies. Correct surgical management requires preoperative Identification and excision of the biopsy tract at time of surgery. We aim to audit how well biopsy tract sites can be identified preoperatively and investigate factors influencing their Identification. Method: Prospective audit of patients who had tissue biopsies for bone and soft tissue tumours at the RNOH Stanmore and presented for surgery between February and April 2008. Case note analysis, patient history and examination at the time of surgery used to collect data. Results: 13/23 patients had their biopsy tract site accurately identified preoperatively, with a mean time gap of 43 days (6–118) between biopsy and excision. In 10/23 patients the biopsy site could not be accurately identified preoperatively. In these patients the mean time between biopsy and excision was 106 days (55–158) (p=< 0.05). 7 patients had neoadjuvant chemotherapy with a mean time gap of 110 days; in 5/7 the tract site was unidentifiable. One patient had preoperative radiotherapy and the biopsy site was unidentifiable. Discussion: This audit has shown that Identification of the biopsy site is more difficult after 40 days. In order to ensure accurate Identification of the biopsy site an Indian ink tattoo should be considered at time of biopsy. It may be particularly advisable for patients who are likely to require neoadjuvant chemotherapy or preoperative radiotherapy. On this basis we would recommend that all patients have the biopsy site marked at the time of biopsy and a further audit will be carried out to evaluate this change in practice

Sclerosing epitheloid fibrosarcoma (SEF) is an extremely rare soft tissue sarcoma arising from connective tissue cells of mesenchymal origin. SEF mostly occurs in extraosseous sites in the soft tissue; however two cases of primary localization in the bone have been described. Despite benign cytological features the clinical course is complicated by a high local recurrence rate and late metastases. SEF represents a clinically challenging entity especially because no standardized treatment regimens are available. We report a 16-year old female patient who showed persistent load-dependent pain focused on the right proximal tibia. Radiological evaluation revealed an osteolytic lesion and the diagnosis of a benign bone cyst was consented. The tumor was surgically removed. Only after recurrence of the tumor and repeated histopathological analysis diagnosis of SEF could be established. Because of the bone localization of the tumor the patient underwent standardized neoadjuvant chemotherapy analogous to the European-American EURAMOS-1 protocol for the treatment of osteosarcoma followed by tumor resection and endoprothesis. Histopathological analysis of the resected tumor showed > 90% vital tumor cells suggesting no response to the neoadjuvant chemotherapy. Therefore, therapy was reassigned to the CWS protocol of the German Society for Pediatric Oncology and Hematology (GPOH) for treatment of soft tissue sarcoma. To date, the patient is alive and no metastases of the primary tumor can be detected. SEF represents a taunting clinical entity due to deceptive histopathological features and rare occurrence. Localization in the bone represents an additional challenge with regards to the therapeutical approach. Standardized treatment regimens are currently not available for SEF. This case report, to our knowledge, is the first outlining a therapeutic approach in detail. Our data suggest that SEF may be resistant to a chemotherapy regimen containing Cisplatin, Doxorubicin and Metho-trexate despite close association to the bone, possibly indicative of the soft tissue histogenesis of this tumor. The response to the soft tissue sarcoma targeting CWS chemotherapy remains to be determined

Patients with high-grade osteosarcoma who have been previously misdiagnosed as benign lesions or infection and accordingly been treated by curettage, internal fixation or drainage present a challenge in deciding the most appropriate treatment plan. Since one of the contraindications of limb salvage is the inability to achieve a wide surgical margin, there has been a tendency to treat these patients by amputation. Due to contamination by previous surgeries, limb salvage surgery was thought to be associated with a higher risk of local recurrence. The aim of this study was to evaluate the oncologic outcome following limb salvage surgery done for high-grade osteosarcoma patients who were initially treated inadequately by curettage, internal fixation or drainage. The study included 24 patients (14 males and 10 females) with an average age of 19 years (range 7 to 39 years). All the patients had high-grade osteosarcoma of the extremities. Seven were located in proximal tibia, six distal femur, four proximal humerus, three proximal femur, two distal tibia, one distal radius and one fibula. 14 patients were previously diagnosed as benign lesions and treated by curettage. 5 patients were diagnosed as regular fracture and internally fixed. 5 patients were diagnosed as osteomyelitis and treated by drainage. The patients were staged then treated by neoadjuvant chemotherapy and limb salvage surgery. The average time between the initial procedure and the limb salvage procedure was 7 months (range 3 to 36 months). A wide resection margin was achieved in all patients. The average follow up period was 40 months (range 18 to 110 months). Local recurrence occurred in three patients (12.5%). Three patients developed chest metastases and one patient developed bone metastases. We conclude that patients who had an inadequate surgical procedure prior to the diagnosis of a high-grade osteosarcoma could still be treated by neoadjuvant chemotherapy and limb salvage surgery without a significant increased risk of local recurrence and chest metastases