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.

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.

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.

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.

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.

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.

Expandable distal femur megaprosthesis: A European Musculoskeletal Oncology Society study on 299 cases.

BACKGROUND AND OBJECTIVES: Expandable distal femur prostheses have become more popular over the last decades, but scientific data is limited. METHODS: A retrospective study was performed, including cases treated between 1986 and 2019 in 15 European referral centers for bone sarcomas. RESULTS: A total of 299 cases were included. Average follow-up was 80 months (range, 8-287 months). Mean patient age was 10 years. Most (80%) of the implants were noninvasive growers and a fixed hinge knee was used more often (64%) than a rotating hinge. Most prosthetic designs showed good (>80%) implant survival at 10 years, but repeat surgery was required for 63% of the patients. The most frequent reason for revision procedure was the completion of lengthening potential. Noninvasive expandable implants showed less risk of infection compared to invasive growers (11.8% vs 22.9% at 10 years). No difference in aseptic loosening was found between cemented and uncemented stems. CONCLUSIONS: This study shows the increasing popularity of expandable distal femur prostheses, with overall good results for function and implant survival. However, repeat surgery is frequently required, especially in patients under the age of 10 years old. Infection is less frequent in noninvasive growers compared to implants that require invasive lengthening procedures.

Posterior Tibial Tendon Rupture Associated With Anterolateral Distal Tibial and Medial Malleolar Fracture and a Novel Pattern of Tibiofibular Syndesmotic Injury: A Case Report and Review of the Literature.

A posterior tibial tendon (PTT) rupture associated with ankle fractures is a very rare condition. Ankle pronation and external rotation (PER) movement are the typical traumatic mechanism. This injury is frequently overlooked preoperatively. Early diagnosis and treatment are very important to prevent the serious consequences related to functional PTT insufficiency on biomechanics of the foot. Few cases have been described in the literature that highlight the relationship between PTT rupture and PER type ankle fracture with a medial malleolar fracture. We present a case of a complete PTT rupture in a closed atypical ankle fracture in which a medial malleolar fracture was associated with a very large fragment from the anterolateral distal tibia (Tillaux-Chaput fragment) and a concomitant avulsion fracture from the anteromedial portion of the fibula (Lefort-Wagstaffe fragment), with a novel pattern never described before.

Influence of age and subtype in outcome of operable liposarcoma.

AIM: Liposarcoma (LPS) is rare tumor deriving from adipocytes. LPS is classified into histological subtypes: well-differentiated (WDLPS), dedifferentiated (DDLPS), myxoid (MLPS) and pleomorphic (PLPS). A tailored approach taking into account the specificity of disease subtype and age at presentation could be helpful in delineating therapeutic management of liposarcoma. In this paper, we report a retrospective series of a single-institution cohort of patients with LPS, undergoing surgery and radiotherapy and/or chemotherapy. The aim of this study is to evaluate whether clinical characteristics, tumor- and treatment-related features affect clinical outcome in patients treated with curative intent for non-metastatic liposarcoma. METHODS: Data of patients with locally advanced, non-metastatic liposarcoma treated between 1990 and 2015 were retrospectively reviewed. Data about patient, tumor and treatment features were collected. Two patients subgroups were identified according to age (cutoff: age < 65 years or > 65 years). Statistical analysis was performed to assess correlation between the above-cited variables and local recurrence-free survival (DFS-LR), distant metastasis-free survival, overall survival (OS) and disease-specific survival (DSS); moreover, differences in clinical outcome between the two age groups were identified. RESULTS: Data of 186 patients were collected. At diagnosis, 27.4% of patients were 65 years or older. At a median follow-up of 8.6 years (range 0.1-27.3 years), Kaplan-Meier (KM) survival analysis showed that LR, DM, OS and DSS were 75.5%, 76.6%, 48.1% and 72.1%, respectively. KM analysis showed that age > 65, DDLPS and lower limb localization were related to LR (p = 0.001, p = 0.0001 and p = 0.0001, respectively). Association between LR, age and DDLPS persisted both at univariate (p = 0.003 and p = 0.0001, respectively) and multivariate Cox regression (CR) analysis (p = 0.024 and p = 0.002). Age, tumor depth and grading influenced distant recurrence, both at KM (p = 0.023, p = 0.026 and p = 0.016) and univariate CR (p = 0.026, p = 0.042 and p = 0.012). Age and grading were confirmed at multivariate analysis (p = 0.009 and p = 0.017). Patients with WDLPS and wide excision had significantly better OS (p = 0.001 and p = 0.03, respectively), while histological G3 and age > 65 were related with worse OS (p = 0.008 and p = 0.0001, respectively). Age, DDLPS and grade were related to OS at univariate (p = 0.0001, p = 0.0001 and p = 0.03, respectively) and multivariate CR analysis (p = 0.031, p = 0.0001 and p = 0.001, respectively). However, analyzing the specific causes of death, female died less often for tumor-related causes, with a DSS of 91.0% compared to 57.4% of male counterpart (p = 0.005). At Kaplan-Meier analysis, postoperative radiotherapy resulted in a statistically significant better disease-specific survival than postoperative radiotherapy (82.9% vs. 46.2%, p = 0.045). High grade correlated with poorer disease-specific survival (59.3%) than intermediate and low grade (73.4% and 91.6%, respectively) (p = 0.008). Association between DSS, sex and grade persisted both at univariate (p = 0.008 and p = 0.022, respectively) and multivariate Cox regression (CR) analysis (p = 0.014 and p = 0.038). Histotype-driven schedules of treatment should be developed to take into account biological heterogeneity of this disease. Further studies are needed to stratify patients subgroup and develop tailored treatment strategies (i.e., altered fractionations and different chemotherapy regimens in aggressive subtypes), in particular more prospective trials are needed to develop treatment guidelines in elderly STS, taking into account the frailty and the peculiarity of this subgroup.

Multimodal Treatment in Pelvic Ewing Sarcoma: A Prognostic Factor Analysis.

BACKGROUND: Although multidisciplinary therapies have improved local control and overall survival in Ewing sarcoma (ES), the prognosis of pelvic lesions remains markedly worse than that of limb ES. METHODS: We retrospectively evaluated the influence of the type of local treatment, margins, necrosis and sacrum involvement on overall survival (OS) and disease-free survival (DFS) in a series of 21 non-metastatic pelvic ES. RESULTS: The average follow-up was 46.3 months (range 3-156). Only one patient had recurrence, at 11 months after surgery. Eight patients showed pulmonary metastasis and five showed bone metastases. Necrosis was the only significant prognostic factor for overall survival at 5 years (p=0.0132) and disease-free survival (p=0.0086). Overall survival at 5 years was 40.1%. CONCLUSION: Local control in pelvic Ewing sarcoma is comparable for patients treated with surgery (S), surgery plus radiotherapy (S/RT), or definitive radiotherapy (RT). The combination of surgery plus radiotherapy could be indicated in cases of large tumor, a poor necrosis response (< 90%), or an inadequate margin with involvement of the sacrum. A poor response to neoadjuvant therapy is a significant risk factor for both local control and overall survival.

Aneurysmal Bone Cyst: A Review of Management.

Aneurysmal bone cyst is a rare lesion that is most often found in young adults and children. It can have an unpredictable behavior, with a high recurrence rate after treatment. Treatment is based on personal and institutional experience and preferences. Standard treatment consists of curettage (manual + motorized high-speed burr) plus local adjuvants and bone grafting to fill the void. In anatomical locations that are difficult to reach surgically, percutaneous procedures (injection of sclerosant agents, radiofrequency thermal ablation (RFTA)) or selective arterial embolization (SAE) are used. Medical management with bisphosphonates (BPs) or denosumab has also been advocated. Minimally invasive surgical procedures such as "curopsy" and percutaneous demineralized bone matrix (DBM) and/or autologous bone marrow concentrate (BMC) grafting have also been proposed. SAE is used as a pre-operative procedure to reduce intra-operative bleeding in the case of large lesions and as primary treatment for spinal lesions. The purpose of this review is to present currently available options for the management of aneurysmal bone cyst.

Liposarcoma: Clinico-pathological analysis, prognostic factors and survival in a series of 307 patients treated at a single institution.

BACKGROUND AND OBJECTIVES: Liposarcoma (LPS) is a malignant mesenchymal tumor and the most common soft tissue sarcoma. Four different subtypes are described: well differentiated (WD) LPS or atypical lipomatous tumor (ALT), dedifferentiated (DD) LPS, myxoid LPS, and pleomorphic LPS (PLS). The objective of the study was to investigate prognostic factors and clinical outcome of liposarcoma. METHODS: We retrospectively examined the clinico-pathological features of a series of 307 patients affected by Liposarcoma at a mean follow-up of 69 months (range 6-257). ALT/WD LPS were analyzed separately. The influence of site, size, type of presentation, grading, histotype and local recurrence on local and systemic control and survival was assessed. RESULTS: The statistical analysis indicated that only surgical margins represented a significant prognostic factor for local recurrence in ALT/WD LPS (P = 0.0007) and other subtypes of LPS (P = 0.0055). In myxoid, PLS and DD LPS, significant prognostic factors for metastasis free survival (MFS) were surgical margins (P = 0.0009), size of the tumor (P = 0.0358), histology (P = 0.0117) and local recurrence (P = 0.0015). In multivariate analysis, surgical margins (0.0180), size (0.0432) and local recurrence (0.0288) correlated independently with MFS. Margins (P = 0.0315), local recurrence (P = 0.0482) and metastases (P < 0.0001) were prognostic factors for overall survival (OS). CONCLUSION: Marginal surgery can be an accepted treatment for ALT/WD LPS. In other liposarcoma subtypes (Myxoid, DD, PLS) wide or radical surgery is recommended as the margins significantly influence local recurrence-free survival (LRFS), metastasis-free survival (MFS) and overall survival (OS). Local recurrence and metastases were significant prognostic factors for OS.

Is an Intramedullary Nail a Valid Treatment for Limb-Length Discrepancy After Bone Tumor Resection? Case Descriptions.

One of the most frequent outcomes after resection of bone tumors in children is a limb-length discrepancy. An intramedullary nail is a valid method for lengthening the limb. We report our experience with four cases of limb-length discrepancy in the lower limbs several years after the primary treatment of bone tumor resection and subsequent reconstruction. Two femoral PRECICE® nails (NuVasive, Inc., San Diego, CA) were introduced retrograde and two were introduced in an anterograde manner. All four cases healed and showed a reduction of the limb-length discrepancy, early loading, and complete bone osteogenesis. In one case, a reduction of the joint ROM recovered after release of the iliotibial band and a quadriceps release according to Judet's arthrolysis.

Surgical Treatment of Sacral Chordoma: En Bloc Resection with Negative Margins is a Determinant of the Long-Term Outcome.

STUDY DESIGN: Retrospective case series. OBJECTIVE: To report the outcome of a series of patients with sacral chordoma who were surgically treated at a single center. SUMMARY: Chordomas are low-grade malignant tumors that arise from remnants of the notochord. They are most often found in the sacrum, spine and skull-base. These tumors have a slow clinical evolution and may eventually metastasize, even after adequate treatment. Rarely, they can dedifferentiate into high-grade sarcomas. Traditionally, chordomas were considered to be resistant to chemotherapy and standard radiation therapy. However, recently, adrotherapy has been shown to be effective for local and systemic control of the disease. In this study, clinical outcomes and local and systemic recurrence were reviewed to identify prognostic factors for local and systemic control. METHODS: Thirty-three patients with sacral chordoma (19 males, 14 females; median age 61 y, range 43-80) who were surgically treated at our institution between 1994 and 2015 were reviewed. In 24 patients, resection was performed above S2. No patients received pre-operative radiotherapy (RT). Three cases received RT (carbon ion therapy) as treatment for local recurrence. Wide (R0) surgical margins were achieved in 17 patients, marginal (R1) margins in 14 patients and intralesional (R2) margins in 2 patients. RESULTS: At a median follow-up of 53 months (range 0-198), 19 patients were continuously disease-free, 6 were disease-free after local recurrence (5) or metastases (1), 3 were alive with disease (2 local recurrence and 1 metastasis), 4 were dead of disease (1 patient died intraoperatively) and 1 was dead of another cause. Local recurrence was observed in 9 cases (27%); all 9 were treated surgically and 3 received carbon ion therapy after surgical intralesional excision. Overall survival at 10 years was 86.6%. Local recurrence-free survival at 10 years was 51%. A statistical analysis confirmed the importance of negative surgical margins (R0) to achieve local control of the disease (p = 0.0007). High resections (above S2) were associated with lower survival and higher risk of local recurrence. CONCLUSION: Surgical en bloc resection is the primary treatment for sacral chordoma. Carbon ion therapy is used when it is difficult to obtain wide surgical margins. Due to morbidity and the disabling sequelae of surgery, adrotherapy may be considered an alternative to high (above S2-S3) sacral chordoma resections.

Biological Intercalary Reconstruction with Bone Grafts After Joint-Sparing Resection of the Lower Limb: Is this an Effective and Durable Solution for Joint Preservation?

Due to advances in neoadjuvant therapies and preoperative imaging modalities, joint-sparing resections have become appealing in bone tumor surgery. However, the intercalary reconstruction of metadiaphyseal bone defects of the femur and the tibia after juxta-articular tumor resection remains challenging. Both biological and prosthetic reconstructions have been used for joint-sparing resections, but little is known about the long-term outcome of these procedures. The authors reviewed a consecutive series of 64 patients treated with joint-sparing intercalary resection and reconstruction with bone grafts. Inclusion criteria were an osteotomy line within 5 cm from the knee and ankle joint surface and an osteotomy line proximal to 1 cm below the lesser trochanter at the hip level. Intra-epiphyseal resection was performed in 25 patients (39%)and intercalary resection was performed in 39 (61%). Reconstruction included 49 allograft + vascularized fibular graft (VFG), 10 allografts, and 5 VFG + structural allogenic grafts. At a mean follow-up of 117 months (range 12-305), 51 patients (80%) were continuously disease-free, and 6 showed no evidence of disease after treatment of local recurrence or metastatic lesion. One patient was alive with lung metastases at 26 months of follow-up and six patients died of disease. In the entire series of 64 patients, 26 had a non-oncological complication that required surgical revision (40.6%). Overall survival (OS) of reconstruction was 92% at 5 years and 90% at 10 and 15 years. Limb salvage survival (LSS) was 94% at 5, 10 and 15 years. Twenty-two fractures occurred in 17 patients (26.5%). There were a total of nine non-unions (14%). Six patients (9.3%) presented early wound dehiscence (average 1.8 months, range 0-6). A deep infection occurred in 3 cases (4.7 %). In 12 patients treated with VGF reconstruction (12/54:22%), a donor-site complication was observed. The overall Musculoskeletal Tumor Society (MSTS) functional score in 54 evaluable patients, who were alive with reconstruction in situ, was 27 points (range 18-30). Biologic intercalary reconstructions with bone grafts resulted in effective joint-sparing resections of the lower limb, allowing joint preservation in all but one case who required a total knee replacement for varus osteoarthritis. Despite the high rate of complications requiring surgical revision, at 15 years, overall survival of the reconstruction was 90% and limb salvage survival was 94%. In our experience, revision-free survival was better with VFG reconstruction than with allograft alone and the combination of VFG and allogenic graft seems to favor spontaneous fracture-healing and to decrease the non-union rate.

Hydatid cyst in the vastus lateralis muscle: a case report.

Hydatidosis is a zoonotic disease; human infection occurs through the consumption of food and water contaminated with the eggs of parasites of the Echinococcus type. While the liver is the most common site of infection, involvement of the musculoskeletal system is extremely rare. In the context of musculoskeletal involvement, the spine is the most commonly infected site, while the muscles are rarely infected and account for approximately <1% of cases. It has been suggested that muscles provide an unsuitable environment for the parasite, because of the presence of lactic acid. The cysts appear as slow-growing masses of soft tissue, and signs of inflammation and fistulization often coexist. We report a rare case of an hydatid cyst located in the vastus lateralis muscle of a 41-year-old man. Muscular echinococcosis is an extremely rare disease. A MRI evaluation should be taken into account as gold standard in the diagnosis. Surgical cystectomy is often indicated, and an excision with wide margins is mandatory to avoid the rupture of the cyst and anaphylaxis. Adjuvant pharmacological therapy is recommended to minimize the risk of recurrence.