Primary Chest Wall Ewing Sarcoma: Treatment and Long-Term Results.

OBJECTIVE: The aim of the study is to evaluate early and long-term results of chest wall primary Ewing's sarcoma patients treated in the time period February 2000-February 2023 by a multidisciplinary approach. METHODS: We retrospectively reviewed the medical records of patients who underwent chest wall resection for a primary tumor. Treatment approach, extent of resection, 30-day mortality, overall survival (OS), local recurrence-free survival (LRFS), and metastasis-free survival (MFS) were analyzed. RESULTS: Overall, n = 15 consecutive patients were treated for chest wall primary Ewing's sarcoma. A median of n = 3 ribs was resected with a median of n = 2 ribs adjacent to the lesion. Resections were extended to the adjacent structures in n = 5 patients (33.3%). In all cases, we performed a prosthetic reconstruction, associated with muscle flap (n = 10, 66.6%) or with rigid titanium bars and muscle flap (n = 6, 40%). A radical resection was accomplished in n = 13 patients (84.6%). The median surgical time was 310 ± 120 min; median hospitalization was 7.8 ± 1.9 days. Post-operative mortality was zero. We recorded n = 4 (30.7%) post-operative complication. The median follow-up (FU) was 26 months. Moreover, 5-year overall and event-free survival were 52% and 48%, respectively. CONCLUSIONS: This case series confirms the benefit of the multidisciplinary approach for Ewing sarcomas in early and long-term results.

Joint-Sparing Resection around the Knee for Osteosarcoma: Long-Term Outcomes of Biologic Reconstruction with Vascularized Fibula Graft Combined with Massive Allograft.

(1) Background: We aim to address the following questions. What was the complication rate of vascularized fibula graft (VFG) combined with massive allograft in patients treated with joint-sparing resection around the knee for a high-grade osteosarcoma? What was the long-term survivorship of VFG free from revision and graft removal? What were the functional results as assessed by the Musculoskeletal Tumor Society (MSTS) score? (2) Methods: 39 patients treated in our unit for osteosarcoma around the knee with intercalary resection and reconstruction with VFG combined with massive allograft were included; 26 patients underwent intercalary tibial resection, while 13 underwent intercalary femoral resection. (3) Results: Mean Follow-Up was 205 months (28 to 424). Complications that required surgery were assessed in requiring surgical revision in 19 patients (49%) after a mean of 31 months (0 to 107), while VFG removal was necessary in three patients (8%). The revision-free survival of the reconstructions was 59% at 5 years and 50% at 10 to 30 years. The overall survival of the reconstructions was 95% at 5 to 15 years and 89% at 20 to 30 years. The mean MSTS score was 29.3 (23 to 30). (4) Conclusions: VFG represents an effective reconstructive option after joint-sparing intercalary resection around the knee for osteosarcoma.

Survival and Results after Resection and Reconstruction with Megaprosthesis at the Hip in Octogenarians.

Few data are available about results after procedures of resection and megaprosthesis at the hip in very elderly patients. The aim of our study was to ascertain survival and complications in patients aged 80 or older undergoing these major orthopedic procedures. A consecutive series of 27 procedures in 26 patients aged 80-93 years was evaluated. In total, 15 procedures were performed due to oncological diseases, 6 were performed following joint arthroplasty failures or periprosthetic fractures, and 6 were performed after trauma or trauma sequelae. Survival of the patients ranged from 0 to 122 months. Overall survival was 56% at 3 years, 24% at 5 years, and 16% at 8 years. An early postoperative death during the first 3 months occurred in five patients (18.5%). The only preoperative parameter negatively affecting survival was preoperative hemoglobin lower than 11 g/dL. Local complications were similar to reported rates in all-age patients' series. In our experience, resection and megaprosthetic reconstruction can also be a valid choice in very elderly patients, with 56% of patients living more than two years from surgery and 24% more than five. Nevertheless, early postoperative deaths are frequent. A multidisciplinary evaluation of frailty of the patient must be accomplished, and patients and relatives must be informed about the risks of the procedure.

Tumor size and surgical margins are important prognostic factors of synovial sarcoma - A retrospective study.

Background: Synovial Sarcoma (SS) is a rare soft tissue sarcoma. Mean time to get a SS diagnosis from the onset of symptoms is 10 years, furthermore, SS is associated with late metastasis. Surgery is the main treatment option, whose quality deeply affects SS outcomes, and it can be associated to preoperative or post-operative radiotherapy. Chemotherapy is considered very effective in Children, while in adults its efficacy is still under debate. The aim of this study was to investigate the oncologic results in SS treatment and to identify the risk factors for local and systemic control of the disease. Methods: From 1994 to 2018, 211 patients affected by SS were treated in 3 Referral Centres of Orthopaedic Oncology. One hundred seventy-seven patients were included in the study, the median follow-up length was 96 months (5-374). Results: Overall Survival on the Kaplan Meier Analyses was 80%, 70% and 56% at 5, 10 and 20 years. In multivariate analyses, OS correlated with tumour size and negative surgical margins. Chemotherapy use wasn't associated with better survival although patients who underwent CT had bigger and more aggressive tumours. Conclusions: Our findings suggests that surgery with negative margins is the most important factor in Synovial Sarcoma. Adjuvant treatments as chemotherapy and radiation therapy didn't change the disease's course.

Are Vascularized Fibula Autografts a Long-lasting Reconstruction After Intercalary Resection of the Humerus for Primary Bone Tumors?

BACKGROUND: A vascularized fibula graft (VFG) is the vascular autograft most frequently used to restore large segmental long bone defects, particularly in the upper limb. Because the use of a vascularized fibula involves an operation in an uninvolved extremity with potential morbidity, it is important to document that this type of reconstruction is successful in restoring function to the humerus. However, the long-term results of VFG after intercalary resection of the humeral diaphysis for bone tumors are still unknown. QUESTIONS/PURPOSES: (1) What was the complication rate of reconstruction? (2) What was the functional result after surgical treatment, as assessed by the Musculoskeletal Tumor Society (MSTS) score, the American Shoulder and Elbow Society (ASES) score, and Constant score? (3) What was the survivorship of these grafts free from revision and graft removal at 5, 10, and 15 years? METHODS: Between 1987 and 2021, 127 patients were treated at our institution with en bloc resection for a primary malignant or an aggressive benign bone tumor of the humerus; we excluded patients treated with extra-articular resection or amputation. Of those, 14% (18 of 127) were treated with intercalary resection of the humeral diaphysis for primary bone tumors and reconstruction with VFG, with or without a bulk allograft, and were analyzed in this retrospective study. Generally, our indications for reconstruction with VFG are intercalary resection of the humerus for primary malignant or aggressive benign bone tumors in patients with long life expectancy and high functional demands, in whom adequate bone stock of the proximal and distal epiphysis can be preserved. In 13 patients, VFG was used alone, whereas in five patients, a massive allograft was used. Our policy was to use VFG combined with a massive allograft in patients undergoing juxta-articular joint-sparing resections in which proximal osteotomy was performed close to the anatomic neck of the humerus to obtain more stable fixation and better tendinous reattachment of the rotator cuff and deltoid. All 18 patients who were treated with a VFG were available for follow-up at a minimum of 2 years (median follow-up 176 months, range 26 to 275 months), and although three have not been seen in the past 5 years and are not known to have died, they had 172, 163, and 236 months of follow-up, and were included. The median age at surgery was 25 years (range 2 to 63 years), the median humeral resection length was 15 cm (range 8 to 21 cm), and the median fibular length was 16 cm (range 12 to 23 cm). Complications and functional scores were ascertained by chart review that was performed by an individual not involved in patient care. Functional results were assessed with the MSTS score (range 0 to 30), the ASES score (range 0 to 100), and the Constant score (range 0% to 100%). Survivorship was estimated using a Kaplan-Meier survivorship estimator, which was suitable because there were few deaths in this series. RESULTS: Seven patients underwent a revision procedure (one radial nerve transient palsy because of screw impingement, four nonunions in three patients with one humeral head avascular necrosis, treatment for screw-related pain in one patient, and two VFG fractures), and one patient underwent VFG removal. Donor site complications were observed in four patients (one ankle valgus deformity and three claw toes-the first toe in two patients and the other toes in the third). At the final clinical control, at a median follow-up of 176 months (range 26 to 275 months), the median MSTS score was 30 of 30 (range 28 to 30), the median ASES score was 98.3 (range 93 to 100), and the median Constant score was 93.5% (range 79% to 100%). Revision-free survival was 71% (95% CI 53% to 96%) at 5 years and 57% (95% CI 37% to 88%) at 10 and 15 years; VFG removal-free survival was 94% (95% CI 83% to 100%) at 5, 10, and 15 years. CONCLUSION: VFG appears to be an effective reconstructive option after humeral intercalary resection for primary bone tumors. These are complex procedures and should be performed by an experienced team of surgeons who recognize that complications may occur frequently in the first years after the procedure. The frequency of mechanical complications observed in the first 5 years postoperatively may be lessened by using long spanning-plate fixation, and if successful, this reconstruction provides a long-term, durable reconstruction with excellent functional results. LEVEL OF EVIDENCE: Level IV, therapeutic study.

Surgical treatment for pelvic bone metastatic disease from renal cell carcinoma.

INTRODUCTION: Pelvic bone metastases from renal cell carcinoma (RCC) are challenging due to their destructive pattern, poor response to radiotherapy and hypervascularity. The purpose of our study was to review a series of patients undergoing surgical treatment with the aim to investigate: 1) survival; 2) local disease control; and 3) complications. METHODS: A series of 16 patients was reviewed. A curettage procedure was performed on 12 patients. In eight the lesion involved the acetabulum; a cemented hip arthroplasty with cage was performed in seven; a flail hip in one. Four patients received a resection; in two cases with acetabular involvement, reconstruction was performed with a custom-made prosthesis and with an allograft and prosthesis. RESULTS: Disease-specific survival accounted for 70% at 3 years and 41% at 5 years. Only one local tumor progression after curettage occurred. Revision surgery (flail hip) was necessary for deep infection of the custom-made prosthesis. CONCLUSION: A prolonged survival in patients affected by bone metastatic disease from RCC can justify also major surgical procedures. Due to a low local progression rate after intralesional procedures, curettage, cement and a total hip arthroplasty with cage, when feasible, should be considered as an alternative to more demanding surgeries like resections and reconstructions. LEVEL OF EVIDENCE (OXFORD): Level 4.

A 54-Year-Old Man Who Developed a Femoral Pathologic Fracture from a Giant Popliteal Artery Pseudoaneurysm 7 Years After Ligation and Bypass of a Popliteal Artery Aneurysm: A Case Report and Literature Review.

BACKGROUND This case report describes a giant pseudoaneurysm that grew in size during the years following surgical treatment of a popliteal artery aneurysm, eventually causing a femoral fracture. Bone fractures secondary to vascular injuries are rarely described in the literature. CASE REPORT A 54-year-old man underwent surgical ligation and bypass for left popliteal artery aneurysm. Seven years later, he suffered a left distal femur pathologic fracture surrounded by a giant soft-tissue mass. The patient came to us with a diagnostic hypothesis of angiosarcoma from another hospital at imaging evaluation. After computed tomography angiography (CTA) and angio-magnetic resonance imaging (MRI), we made a diagnosis of femoral pathologic fracture caused by a giant pseudoaneurysm of a treated popliteal artery aneurysm refilled by an aberrant anterior tibial artery (IIA2, Kim classification). We performed excision of the mass and open reduction and internal fixation, with anatomic plate, of the fracture. Fracture healing and good functional outcome were observed at follow-up. CONCLUSIONS A possible complication of surgical treatment of popliteal artery aneurysms is refilling of the excluded aneurysm due to collateral blood flow or, such as in the present case, aberrant vessels. Therefore, the knowledge of anatomical variants of the vessels is important in surgery. Follow-up evaluation after surgery is advisable and a growing mass should be further investigated with an angio-CT scan. In case of a non-pulsating soft-tissue mass causing pathologic bone fracture, a biopsy is mandatory to exclude malignancy.

Long term outcome of surgical treatment of chondroblastoma: analysis of local control and growth plate/articular cartilage related complications.

BACKGROUND: Chondroblastoma (CBL) is a rare benign chondroid producing bone tumor that typically occurs in epiphysis or apophysis of growing children and young adults. Intralesional curettage is the treatment of choice, while resection is required in selected cases, even though the use of minimally invasive ablation techniques has been advocated. Authors reviewed a series of 75 CBLs with the aim of assess risk factors for local recurrence, the growth plate related complications after epiphyseal curettage and the risk of arthritis of the adjacent joint after epiphyseal curettage. METHODS: We retrospectively review 69 CBLs treated with intralesional curettage and 6 treated with resection from March 1995 to February 2020. The median age was 18.8 years (7 to 42, median 16). The site was proximal humerus in 18 cases, proximal tibia in 17, distal femur in 16, talus in 6, femur's head in 4, calcaneus in 3, acromion in 3, trochanteric region in 2, distal tibia in 2, patella in 2, supracetabular region in 1 and distal humerus in 1 patient. RESULTS: Mean follow-up was 124.2 months (24 to 322, median 116). Among patients treated with curettage, 7.3% of local recurrence was observed and 12 (17.4%) patients developed osteoarthritis of the adjacent joint. Five patients (7.3%) presented limb length discrepancy of the operated limb ranging from 0.5 to 2 cm. Recurrence free survival rate was 94.2% at 5 and 91.6% at 10 years. A mean Musculoskeletal Tumor Society (MSTS) of 29.3 points (20 to 30, median 30) was observed. CONCLUSION: More than 90% of CBLs were successfully treated with aggressive curettage but segmental resection is required in selected cases. In a relatively small proportion of cases long term complications can occur due to growth plate damage or osteoarthritis. TRIAL REGISTRATION: Retrospectively registered.

Salvage of limb salvage in oncological reconstructions of the lower limb with megaprosthesis: how much to push the boundaries?

INTRODUCTION: Megaprosthesis represent the most commonly used limb salvage method after musculoskeletal tumor resections. Nevertheless, they are burdened by high complication rate, requiring several surgical revisions and eventually limb amputation. The aims of this study were to evaluate the effect of rescuing the limb with subsequent revisions on complication rates (a), incidence of amputations (b), and whether complications reduce functional outcome after the first surgical revision (c). MATERIALS AND METHODS: We retrospectively reviewed 444 lower limb megaprosthesis implanted for primary musculoskeletal tumors or metastatic lesions, from February 2000 to November 2017. 59 patients received at least one revision megaprosthesis surgery. MSTS score was used to assess final functional results. Complication-revision-amputation free survival rates were calculated both at 5 and 10 years of follow-up. RESULTS: Complication free survival, revision free survival and amputation free survival at 10 years were 47% and 53%, 61% and 67%, 90% and 86% among all 444 patients and the group of 59 revised patients, respectively. The incidence of further complications after the first complication was 26% in the group treated with no subsequent revision surgeries and 51% in the group with at least one revision surgery. We found a trend of inverse linear relationship between the number of complications needing subsequent revision surgeries and the final MSTS. CONCLUSION: The number of further revision surgeries after limb salvage with megaprosthesis increases the incidence of complications. Repeated surgical revisions, in particular after infection, increase the amputation rate. The most frequent causes of failure were structural failures and infections. MSTS score was superior for patients undergoing limb salvage than amputees. However, MSTS progressively decreased with multiple revisions becoming inferior to the functional score of an amputated patient.

Cemented vs uncemented megaprostheses in proximal femur metastases: a multicentric comparative study.

BACKGROUND: Hip megaprostheses are a long known reconstructive method in the treatment of proximal femur metastases. The use of cemented or uncemented stems is still matter of debate. The aim of this study to compare cemented and uncemented megaprostheses on functional outcomes and complications, in order to establish the role of cementation. METHODS: We retrospectively analysed 51 metastatic patients with proximal femur metastases treated with endoprosthetic reconstruction by megaprostheses, 25 with cementless stems and 26 with cemented ones with different megaprosthetic implants. The primary endpoint was MSTS score, and the secondary endpoint was to state the incidence of surgical and clinical complications in the two groups. An un-paired T test was used to compare anthropometric, anamnestic data, and MSTS. Chi-square test was performed for evaluation of complication in the two group. Multiple linear regression was used to match the functional outcomes and complications' incidence in the population study. Logistic regression was performed to analyse the odds ratio of different parameters and their role in the incidence of complications. RESULTS: The mean follow-up was 50.1 months (+ 12.5). In thirty case right side was involved. No statistical differences were noticed between Group A and B regard the age, gender, active fracture/impending fracture. Comparing the MSTS results within the two groups at last follow-up, the score cemented group was higher than cementless one (17.9 + 7.8 vs 24.2 + 5.3; statistical significance p = 0.001). Regarding surgical complications a logistic regression was performed to analyse the odds ratio of age, cementation and length of resection; cementation confirm and odds ratio of 11 times in the incidence of surgical complications. CONCLUSIONS: Cementation seems to be more liable to complications onset, while improves functional score in metastatic patients compared to uncemented megaprostheses. More studies have to be conducted in order to create a protocol and establish criteria to use cemented or uncemented stems in a frail population like metastatic patients.

Management of Squamous Cell Carcinoma in Chronic Osteomyelitis: Our Experience, Review of the Literature and Role of MRI in Differential Diagnosis.

BACKGROUND: The authors describe a series of patients with chronic osteomyelitis associated with squamous cell carcinoma, whilst analyzing its incidence in chronic osteomyelitis, surgical options for treatment and focusing on the role of MRI in differential diagnosis. METHODS: The authors reviewed 73 cases of chronic osteomyelitis (CO) treated in their department between 1995 and 2019. Six of these patients (8.2%) had a malignant degeneration in squamous cell carcinoma (SCC). All cases with malignancy were evaluated with preoperative gadolinium-enhanced MRI. RESULTS: In this series, the authors observed an incidence rate of 8.2% (6 cases out of 73); all patients were male with a mean age of 63.5 years. The mean time between the occurrence of chronic osteomyelitis and the diagnosis of squamous cell carcinoma was 36 years (range: 21-66). The treatment consisted of amputation in five patients and limb salvage with vascularized fibula autograft in one case. CONCLUSIONS: MRI played a key role in the differential diagnosis between infected and tumoral tissue, and was found to be crucial during follow-up. Amputation is the treatment of choice in extended tumoral involvement of bone and soft tissue.

The Performances of Conventional Titanium and Silver-Coated Megaprostheses in Non-oncological and Post-oncological Patients: An Analysis of Infection Failures in 142 Patients.

Background: Megaprostheses are one of the preferred choices of reconstruction after tumor resection. Periprosthetic joint infections are one of the most serious complications of joint prostheses surgeries. In this study, our aim was to analyze the efficacy of silver-coated megaprostheses in reducing the risk of prosthesis-related infection. Methods: One hundred forty-two patients who had undergone implantation of a mega-endoprosthesis for non-neoplastic or post-neoplastic conditions were included in this retrospective study. The end-point of the survival analysis was the prosthesis failure due to infection. Results: Thirty-eight patients had undergone implantation of a silver-coated megaprosthesis and 104 patients a megaprosthesis without silver coating. The survival analysis showed an overall infection-free survival rate of 82.3% at five years and 61.9% at 10 years. Silver-coated prostheses had an HR of 0.72 (95% CI: 0.26-2.05; P=0.54). Conclusion: Implantation of a silver-coated mega-prosthesis in non-oncological patients did not significantly reduce the risk of prosthesis-related infection.

Surgical and Pharmacological Management of Periprosthetic Atypical Femoral Fractures: A Narrative Literature Review.

Introduction: An increasing number of patients is annually undergoing total hip arthroplasty (THA), and a significant proportion of these patients are elderly and consequently at a higher risk of complications because of age, osteoporosis, and medical comorbidities. Periprosthetic femoral fractures (PFFs) are one of the worst complications of THA associated with high rates of unfavorable prognosis. Besides, in the last decade, a new independent disease entity called "atypical femoral fracture" (AFF) has been identified and defined by the American Society for Bone and Mineral Research (ASBMR) task force. Some PFFs present clinical history and radiographic aspect consistent with an AFF, meeting the ASBMR criteria for the diagnosis of AFF except that PFFs by themselves are an exclusion criterion for AFF. However, there is an increasing number of published studies suggesting that periprosthetic atypical femoral fractures (PAFFs) exist and should not be excluded by definition. Significance: Nowadays, although there is an increasing interest in PAFFs, there are still very few studies published on the topic and a lack of consensus regarding their treatment. This narrative literature review aims to introduce this new emerging topic to a wider readership describing the characteristics of PAFFs and the state-of-the-art in their management. Conclusions: Many authors agree that PAFFs should be considered as a subgroup of PFFs that have atypical characteristics; they also show a significant correlation with prolonged bisphosphonate use. A correct diagnosis is paramount for proper treatment of the disease that requires both surgical and medical actions to be taken.

The use of a non-biological, bridging, antiprotrusio cage in complex revision hip arthroplasty and periacetabular reconstructive oncologic surgery. Is still today a valid option?: A mid/long-term survival and complications' analysis.

INTRODUCTION: Burch-Schneider-like antiprotrusio cages (B-SlAC) still remain helpful implants to bridge severe periacetabular bone losses. The purpose of this study was to evaluate outcomes and estimate both cages' failures and complication risks in a series of B-SlAC implanted in revision of failed total hip arthroplasties (THA) or after resection of periacetabular primary or secondary bone malignancies. Risk factors enhancing the chance of dislocations and infections were checked. MATERIALS AND METHODS: We evaluated 73 patients who received a B-SlAC from January 2008 to January 2018. Group A, 40 oncological cases (22 primary tumors; 18 metastases); Group B, 33 failed THAs. We compared both Kaplan-Meier estimates of risk of failure and complication with the cumulative incidence function, taking account the competing risk of death. Cox proportional hazards model was utilized to identify possible predictors of instability and infection. Harris hip score HHS was used to record clinical outcomes. RESULTS: Medium follow-up was 80 months (24-137). Average final HHS was 61 (28-92), with no differences within the two groups (p > 0.05). The probabilities of failure and complications were 57% and 26%, respectively, lower in the oncologic group than in the rTHA group (p =0 .176; risk 0.43) (p = 0.52; risk 0.74). Extended ileo-femoral approach and proximal femur replacement (p =0.02, risk ratio = 3.2; p = 0.04, rr = 2.1) were two significant independent predictors for dislocations, while belonging to group B (p = 0.04, rr = 2.6) was predictable for infections. CONCLUSION: Burch-Schneider-like antiprotrusio cages are a classical non-biological acetabular reconstruction method that surgeons should bear in mind when facing gross periacetabular bone losses, independently of their cause. However, dislocation and infection rates are high. Whenever possible, we suggest preserving the proximal femur in revision THA, and to use a less-invasive postero-lateral approach to reduce dislocation rates in non-oncologic cases.

Stereotactic Body Radiotherapy in Oligomestatic/Oligoprogressive Sarcoma: Safety and Effectiveness Beyond Intrinsic Radiosensitivity.

BACKGROUND: Metastatic soft tissue sarcoma (STS) patients may benefit from local ablative treatments due to modest efficacy of systemic chemotherapy. However, use of stereotactic body radiotherapy (SBRT) is controversial because of presumed radioresistance of STS. METHODS: Patients treated with SBRT for oligometastatic and oligoprogressive metastatic STS were retrospectively reviewed to assess results in terms of local control (LC), disease-free survival (DFS), and overall survival (OS). Incidence and grade of adverse events were reported. Statistical analysis was performed to identify variables correlated with outcome and toxicity. RESULTS: Forty patients were treated with SBRT to a median biologic effective dose (BED) of 105 (66-305) Gy5 to 77 metastases. Two-year LC, DFS, and OS were 67%, 23%, and 40%. Improved LC was shown in patients receiving a BED >150 Gy5 (hazard ratio [HR], 3.9; 95% confidence interval [CI], 1.6-9.7; P = 0.028). A delay >24 months between primary tumor diagnosis and onset of metastases was associated with improved DFS (HR, 0.46; 95% CI, 0.22-0.96; P = 0.01) and OS (HR, 0.48; 95% CI, 0.23-0.99; P = 0.03). No toxicity grade ≥3 was observed. CONCLUSIONS: Stereotactic body radiotherapy is effective in metastatic STS with a benign toxicity profile. A BED >150 Gy5 is required to maximize tumor control rates. Metastatic relapse >24 months after diagnosis is correlated to improved survival.

Ewing family tumors of the appendicular skeleton: a retrospective analysis of prognostic factors.

PURPOSE: Authors retrospectively analyzed possible prognostic factors in a series of patients affected by Ewing sarcoma of extremities (eEWS) and treated over a 20-year period at a single institution. METHODS: Between 1997 and 2017, 88 bone eEWS were treated at our institution. Staging, age, gender, tumoral volume, local treatment, surgical margins, post-ChT necrosis were investigated for prognostic correlation with overall survival (OS) and event-free survival (EFS). Median follow-up was 74 months (1-236). RESULTS: Staging of disease correlated with OS (81% vs 59%, p = 0.01) and not with EFS (68% vs 57%, p = 0.28) in localized vs metastatic eEWS at presentation. Age ≥ 14 years (p = 0.002) and volume ≥ 100 cm3 (p = 0.04) were significant negative prognostic factors. No difference was found in local treatment: OS was 76% vs 63% (p = 0.33), while EFS was 68% vs 49% (p = 0.06) after surgery alone or surgery + radiotherapy, respectively. Regarding surgical margins, OS was 76% vs 38% (p = 0.14), and EFS was 65% vs 33% (p = 0.14) in adequate vs not adequate, respectively. OS was 86% and 68% in good and poor responders, respectively (p = 0.13). CONCLUSION: In eEWS, metastatic disease at presentation, age > 14 years and tumoral volume > 100 cm3 are negative prognostic factors. Intensified adjuvant ChT can improve prognosis in poor responders and metastatic patients.

Re-excision after unplanned excision of soft tissue sarcoma: A systematic review and metanalysis. The rationale of systematic re-excision.

In Soft Tissue Sarcomas (STS) referral centre many patients have already had an incomplete tumour resection. In the majority of specimen, tumoral residual is detected and linked to a worsen prognosis. Systematic surgical re-resection of the scar tissue area is often performed. Some authors suggested to postpone re-resections until a clinically evident local recurrence is detected. A searching strategy was applied to Pubmed-Central and Ovid Medline. Odds ratio (OR) for local recurrence (LR), distant metastasis (MTS) or overall survival (OS) were calculated comparing patients who had tumour residual to people who hadn't. OR of local recurrences, distant metastasis and OS were calculated in planned vs unplanned-excisions groups. OR to develop a metastasis and OS after a local recurrences were calculated. Residual tumour led to an OR for LR of 3,56, OR of MTS was 3,42; OR of decreased OS was 3,42. Having a LR lead to a OR of 1,55 for MTS and to a OR of decreased OS of 2,32. Patients who underwent a re-excision compared to planned surgery did not have an increased OR of LR and had an OR to develop a MTS of 0,56. Our data confirm that there is a strong correlation between local recurrences, distant relapses and overall survival. Although there is a selection bias; this analysis highlights the optimal oncological outcome in patients who underwent re-resection. The rationale for systematic re-resection after unplanned excision of soft tissue sarcomas is very strong and this treatment remains the gold standard of care in these patients.

Extra-abdominal desmoid tumor fibromatosis: a multicenter EMSOS study.

BACKGROUND: Extra-abdominal desmoid tumor fibromatosis (DTF) is a rare, locally aggressive soft tissue tumour. The best treatment modality for this patient cohort is still object of debate. QUESTIONS/PURPOSE: This paper aimed to (1) to compare the outcomes of DTF after different treatment modalities, (2) to assess prognostic factors for recurrence following surgical excision, and (3) to assess prognostic factors for progression during observation. METHODS: This was a retrospective multicenter study under the patronage of the European Musculoskeletal Oncology Society (EMSOS). All seven centres involved were tertiary referral centres for soft tissue tumours. Baseline demographic data was collected for all patients as well as data on the diagnosis, tumour characteristics, clinical features, treatment modalities and whether they had any predisposing factors for DTF. RESULTS: Three hundred eighty-eight patients (240 female, 140 male) with a mean age of 37.6 (±18.8 SD, range: 3-85) were included in the study. Two hundred fifty-seven patients (66%) underwent surgical excision of ADF, 70 patients (18%) were observed without therapy, the residual patients had different conservative treatments. There were no significant differences in terms of tumour recurrence or progression between the different treatment groups. After surgical excision, younger age, recurrent disease and larger tumour size were risk factors for recurrence, while tumours around the shoulder girdle and painful lesions were at risk of progression in the observational group. CONCLUSION: Local recurrence rate after surgery was similar to progression rates under observation. Hence, observation in DTF seems to be justified, considering surgery in case of dimensional progression in 2 consecutive controls (3 and 6 months) and in painful lesions, with particular attention to lesions around the shoulder girdle.

Periprosthetic Atypical Femoral Fractures Exist: A Retrospective Study at a Single Institution. Prevalence on 115 Periprosthetic Femoral Fractures Around a Primary Hip Stem.

BACKGROUND: Some periprosthetic femoral fractures (PFFs) present history and radiographic aspect consistent with an atypical femoral fracture (AFF), fulfilling the criteria for AFF except that PFFs by themselves are excluded from the diagnosis of AFFs. The aim of this study is to evaluate in a single institution series of PFFs if any of them could be considered a periprosthetic atypical femoral fracture (PAFF), and their prevalence. METHODS: Surgical records were searched for PFFs around a primary hip stem from January 2013 to December 2019. Cases were classified according to Vancouver classification. Demographic and medical history was extracted. Fisher's exact test was used for statistical analysis. RESULTS: One hundred fifteen PFFs were identified, 59 of them were type B1 and 16 were type C. Radiographs and medical records were available for all patients. Twenty-four patients (32%) have been treated with bisphosphonates (BPs) for longer than 4 years. Four patients presented a fracture with characteristics of PAFF. When enlarged to all PFFs of the series, no other PAFF was found: prevalence of PAFFs was 5.3% for type B1 and C cases and 3.5% for all surgically treated PFFs. Statistical significative difference between PAFFs and PFFs was found for prolonged BP assumption and for the level of fracture clear of the stem. CONCLUSION: Fracture with characteristics of AFFs can also happen over a prosthetic stem, configuring themselves as PAFFs, and they are related to prolonged BP use. As a correct diagnosis is mandatory for proper treatment, a revision of criteria for AFFs should be considered, accepting that PAFFs exist.