Are IDH1 R132 Mutations Associated With Poor Prognosis in Patients With Chondrosarcoma of the Bone?

BACKGROUND: Because chondrosarcomas vary widely in their behavior, and because anticipating their behavior based on histology alone can be challenging, genetic markers represent an appealing area of inquiry that may help us refine our prognostic approaches. Isocitrate dehydrogenase (IDH) mutations are involved in the pathogenesis of a variety of neoplasms, and recently, IDH1/2 mutations have been found in the tissue of benign cartilage tumors as well as in conventional chondrosarcomas and highly aggressive dedifferentiated chondrosarcomas. However, their association with patient survival is still controversial. QUESTIONS/PURPOSES: (1) What proportion of patients with chondrosarcomas carry IDH mutations, and which IDH mutations can be found? (2) Are any specific IDH mutations associated with poorer overall survival, metastasis-free survival, or local recurrence-free survival? METHODS: Between April 2017 and December 2022, we treated 74 patients for atypical cartilaginous tumors or chondrosarcomas in a musculoskeletal tumor referral center. Patients were considered potentially eligible for the present study if the histologic diagnosis was confirmed by two expert soft tissue and bone pathologists following the current WHO classification, complete preoperative imaging and follow-up data were available, surgical excision was performed by sarcoma orthopaedic surgeons directed by a team leader, and the minimum follow-up was 2 years after surgical treatment unless the patient died. Data including sex, age, diagnosis, grade, type of operation, local recurrence, metastasis, and oncologic follow-up were recorded. Forty-one patients (55%) were eligible for the study. For each patient, DNA was extracted and quantified from paraffin-embedded sections of tumor tissue, and the mutational status of IDH1 (codons 105 and 132) and IDH2 (codons 140 and 172) genes was assessed. Of those, 56% (23 of 41) of patients had adequate DNA for analysis of IDH mutations: 10 male and 13 female patients, with a median age of 59 years (range 15 to 98 years). There were 22 conventional chondrosarcomas (8 atypical cartilaginous tumors, 11 Grade 2, and 3 Grade 3) and 1 dedifferentiated chondrosarcoma. Stage was IA in 3 patients, IB in 5, IIA in 1, IIB in 13, and III in 1, according to the Musculoskeletal Tumor Society classification. At a median follow-up of 3.5 years (range 4 months to 5.6 years), 14 patients were disease-free, 2 were alive with disease, and 7 died (3 within 2 years from surgery). Eight patients had metastases, and 7 developed local recurrence. We determined the proportion of patients who carried IDH mutations, and compared patients with and without those mutations in terms of overall survival, metastasis-free survival, and local recurrence-free survival using Kaplan-Meier curves. RESULTS: Six patients showed wild-type IDH genes, and 17 had IDH mutations (12 had IDH1 R132, 3 had IDH1 G105, and 2 had IDH2 R172). Overall survival at 2 years using the Kaplan-Meier estimator was lower in patients with an IDH mutation than in those with the wild-type gene (75% [95% confidence interval 50% to 99%] versus 100% [95% CI 100% to 100%]; p = 0.002). Two-year metastasis-free survival was also lower in patients with an IDH mutation than in those with the wild-type gene (33% [95% CI 7% to 60%] versus 100% [95% CI 100% to 100%]; p = 0.001), as was 2-year local recurrence-free survival (70% [95% CI 42% to 98%] versus 100% [95% CI 100% to 100%]; p = 0.02). CONCLUSION: We found that IDH1 R132 mutations were negatively associated with the prognosis of patients with bone chondrosarcomas. Nevertheless, more extensive studies (such as multicenter international studies) are needed and advisable to confirm our observations in this preliminary small series. Moreover, evaluating mutational status in fresh samples instead of in paraffin-embedded sections could help to increase the number of patients with adequate DNA for analysis. If our findings will be confirmed, the evaluation of IDH mutational status in biopsy samples or resection specimens could be considered when stratifying patients, highlighting those who may benefit from more aggressive treatment (such as adjuvant chemotherapy) or closer follow-up. LEVEL OF EVIDENCE: Level III, prognostic study.

A rare case of anterior shoulder dislocation in 1-year- and 10-month-old toddler: case report and literature review.

We report our clinical experience of a 1 year and 10 month child with traumatic anterior shoulder dislocation who underwent non-operative reduction and Desault's bandage immobilization for 10 days. No associated fractures were found and after bandage removal, full ROM of the shoulder was immediately assessed. Further research is needed to unified guideline of treatment and the time of immobilization for this type of injury in pediatric population.

The orthopaedic and traumatology scenario during Covid-19 outbreak in Italy: chronicles of a silent war.

BACKGROUND: From February 21, the day of hospitalisation in ICU of the first diagnosed case of Covid-19, the social situation and the hospitals' organisation throughout Italy dramatically changed. METHODS: The CIO (Club Italiano dell'Osteosintesi) is an Italian society devoted to the study of traumatology that counts members spread in public and private hospitals throughout the country. Fifteen members of the CIO, Chairmen of 15 Orthopaedic and Trauma Units of level 1 or 2 trauma centres in Italy, have been involved in the study. They were asked to record data about surgical, outpatients clinics and ER activity from the 23rd of February to the 4th of April 2020. The data collected were compared with the data of the same timeframe of the previous year (2019). RESULTS: Comparing with last year, overall outpatient activity reduced up to 75%, overall Emergency Room (ER) trauma consultations up to 71%, elective surgical activity reduced up to 100% within two weeks and trauma surgery excluding femoral neck fractures up to 50%. The surgical treatment of femoral neck fractures showed a stable reduction from 15 to 20% without a significant variation during the timeframe. CONCLUSIONS: Covid-19 outbreak showed a tremendous impact on all orthopaedic trauma activities throughout the country except for the surgical treatment of femoral neck fractures, which, although reduced, did not change in percentage within the analysed timeframe.

What Is the Survival and Function of Modular Reverse Total Shoulder Prostheses in Patients Undergoing Tumor Resections in Whom an Innervated Deltoid Muscle Can Be Preserved?

BACKGROUND: After proximal humerus resection for bone tumors, restoring anatomy and shoulder function remains demanding because muscles and bone are removed to obtain tumor-free surgical margins. Current modes of reconstruction such as anatomic modular prostheses, osteoarticular allografts, or allograft-prosthetic composites and arthrodeses are associated with relatively poor shoulder function related to loss of the deltoid and rotator cuff muscles. Newer prosthetic designs like the reverse total shoulder arthroplasty (RTSA) are felt to be useful in other reconstructions where rotator cuff function is compromised, so it seemed logical that it might help in tumor reconstructions as well in patients where the deltoid muscle and its innervation can be preserved. QUESTIONS/PURPOSES: In patients with a tumor of the proximal humerus that can be resected with preservation of the deltoid muscle, (1) What complications are associated with tumor resection and reconstruction with a modular RTSA? (2) What are the functional results of modular RTSA in these patients? METHODS: From January 2011 to January 2018, we treated 52 patients for bone tumors of the proximal humerus. Of these, three patients were treated with forequarter amputation, 14 were treated with standard modular proximal humerus implants, seven were treated with allograft-prosthetic composites (RTSA-APC), and 28 were treated with a modular RTSA. Generally, we used anatomic modular prosthetic reconstruction if during the tumor resection none of the abductor mechanism could be spared. Conversely, we preferred reconstruction with RTSA if an innervated deltoid muscle could be spared, but the rotator cuff and capsule could not, using RTSA-APC or modular RTSA if humeral osteotomy was distal or proximal to deltoid insertion, respectively. In this study, we retrospectively analyzed only patients treated with modular RTSA after proximal humerus resection. We excluded three patients treated with modular RTSA as revision procedures after mechanical failure of previous biological reconstructions and three patients treated after December 2016 to obtain an expected minimum follow-up of 2 years. There were nine men and 13 women, with a mean (range) age of 55 years (18 to 71). Reconstruction was performed in all patients using silver-coated modular RTSA protheses. Patients were clinically checked according to oncologic protocol. Complications and function were evaluated at final follow-up by the treating surgeon (PR) and shoulder surgeon (AC). Complications were evaluated according to Henderson classification. Functional results were assessed with the Musculoskeletal Tumor Society score (range 0 points to 30 points), Constant-Murley score (range 0 to 100), and American Shoulder and Elbow Surgeons score (range 0 to 100). The statistical analysis was performed using Kaplan-Meier curves. RESULTS: Complications occurred in five of 22 patients; there was a shoulder dislocation (Type I) in four patients and aseptic loosening (Type II) in one. Function in these patients on the outcomes scales we used was generally satisfactory; the mean Musculoskeletal Tumor Society score was 29, the mean Constant score was 61, and the mean American Shoulder and Elbow Surgeons score was 81. CONCLUSIONS: Although this was a small series of patients with heterogeneous diagnoses and resection types, and we were not able to directly compare the results of this procedure with those of other available reconstructions, we found patients treated with RTSA achieved reasonable shoulder function after resection and reconstruction of a proximal humerus tumor. It may not be valuable in all tumor resections, but in patients in whom the deltoid can be partly spared, this procedure appears to reasonably restore short-term shoulder function. However, future larger studies with longer follow-up are needed to confirm these findings. LEVEL OF EVIDENCE: Level IV, therapeutic study.

Three-dimension-printed custom-made prosthetic reconstructions: from revision surgery to oncologic reconstructions.

BACKGROUND: The use of custom-made 3D-printed prostheses for reconstruction of severe bone defects in selected cases is increasing. The aims of this study were to evaluate (1) the feasibility of surgical reconstruction with these prostheses in oncologic and non-oncologic settings and (2) the functional results, complications, and outcomes at short-term follow-up. METHODS: We analyzed 13 prospectively collected patients treated between June 2016 and January 2018. Diagnoses were primary bone tumour (7 patients), metastasis (3 patients), and revision of total hip arthroplasty (3 patients). Pelvis was the most frequent site of reconstruction (7 cases). Functional results were assessed with MSTS score and complications according to Henderson et al. Statistical analysis was performed using Kaplan-Meier and log-rank test curves. RESULTS: At a mean follow-up of 13.7 months (range, 6-26 months), all patients except one were alive. Oncologic outcomes show seven patients NED (no evidence of disease), one NED after treatment of metastasis, one patient died of disease, and another one was alive with disease. Overall survival was 100% and 80% at one and two years, respectively. Seven complications occurred in five patients (38.5%). Survival to all complications was 62% at two years of follow-up. Functional outcome was good or excellent in all cases with a mean score of 80.3%. CONCLUSION: 3D-printed custom-made prostheses represent a promising reconstructive technique in musculoskeletal oncology and challenging revision surgery. Preliminary results were satisfactory. Further studies are needed to evaluate prosthetic design, fixation methods, and stability of the implants at long-term.

Extra-articular shoulder resections: outcomes of 54 patients.

BACKGROUND: The survival of patients with tumors around the shoulder treated with extra-articular resection, the rates of reconstructions-related complications, and the function of the shoulder cannot be estimated because of limited available data from mainly small published related series and case reports. METHODS: We studied 54 patients with tumors around the shoulder treated with extra-articular shoulder resections and proximal humeral megaprosthetic reconstructions from 1985 to 2012. Mean tumor volume was 549 cm3, and the mean length of the proximal humeral resection was 110 mm. Mean follow-up was 7.8 years (range, 3-21 years). We evaluated the outcomes (survival, metastases, recurrences, and function) and the survival and complications of the reconstruction. RESULTS: Survival of patients with malignant tumors was 47%, 38%, and 35%, at 5, 10, and 20 years, respectively. Rates for metastasis and local recurrence were 60% and 18.5%, respectively. Survival was significantly higher for patients without metastases at diagnosis, tumor volume <549 cm3, and type IV resections. Survival of reconstructions was 56% at 10 years and 48% 20 years. Overall, 19 patients (35.2%) experienced 30 complications (55.5%), the most common being soft tissue failures that required subsequent surgery without, however, implant removal. The mean Musculoskeletal Tumour Society score was 25 points, without any significant difference between the types of extra-articular resections. CONCLUSION: Tumor stage and volume as well as type of resection are important predictors of survival of patients with malignant tumors around the shoulder. Survival of the reconstructions is satisfactory; nevertheless, the complication rate is high. The Musculoskeletal Tumour Society score is similar with respect to the type of resection.

Distal femur reconstruction with modular tumour prostheses: a single Institution analysis of implant survival comparing fixed versus rotating hinge knee prostheses.

PURPOSE: To evaluate the incidence of complications in distal femur reconstructions with modular prostheses, comparing fixed vs rotating hinge knee. METHODS: Retrospective analysis of implant survival, complications, and functional results of the Rizzoli series on distal femur megaprosthesis. Between 1983 and 2010, 687 distal femur tumour modular prostheses were implanted: 491 fixed hinge and 196 rotating hinge knee prostheses. Failures of the prostheses were classified in five types: type 1, soft tissue failure; type 2, aseptic loosening; type 3, structural failure; type 4, infection; type 5, tumour progression. RESULTS: Failure rate was 27 % (185/687). Implant survival to all types of failure was 70 % at ten years and 50 % at 20 years with no significant difference between fixed and rotating hinge knee prostheses (p = 0.0928). When excluding type 5 and type 1 failures, the overall survival was 78 % and 58 % at ten and 20 years. There was not a significant difference in implant survival to aseptic loosening (p = 0.5) and infection (p = 0.2) between fixed and rotating hinge knee prostheses. All cases of breakage of prosthetic components occurred in fixed hinge knee prostheses. Functional results, evaluated in 536 pts, were satisfactory in 91.4 % of cases with a mean score of 23.3 with a significantly better function for rotating hinge knee prostheses (p < 0.001). CONCLUSIONS: The most frequent cause of failure was infection followed by aseptic loosening. Even if better results were expected for rotating hinge knee prostheses, there is no significant difference in overall implant survival. No cases of breakage of prosthetic components occurred in rotating hinge knee prosthesis. Functional results were significantly better for the rotating hinge knee prostheses. LEVEL OF EVIDENCE: Therapeutic study, level IV (case series).

Reconstruction of the proximal femur with a modular resection prosthesis.

BACKGROUND: Various megaprostheses are currently available for reconstruction of the proximal femur after tumor resection. This study evaluates the survival and complications of a modular megaprosthesis for reconstruction of the proximal femur. MATERIALS AND METHODS: We studied the medical files of 109 tumor patients (age range 16-86 years) who underwent proximal femoral reconstruction with the MRP(®) megaprosthesis from 2002 to 2011. There were 70 patients with metastases, 34 patients with bone sarcomas, and five patients with hematological malignancies; 82 were primary and 27 were revision reconstructions. Mean follow-up was 2.5 years; 31 patients had a minimum five-year follow-up. We evaluated the survival and function of the patients, and the survival and complications of the megaprostheses. RESULTS: Survival was significantly higher for the patients with bone sarcomas compared to those with metastases and hematological malignancies. Mean MSTS functional score was similar between patients with bone sarcomas and those with hematological malignancies and metastases, and between patients with primary and those with revision reconstructions. Overall survival of the MRP(®) megaprostheses was 74 % at 5 and 9 years. Fourteen (13.6 %) major complications occurred at a mean period of 1.4 years (range 3 months to 4.5 years); these included infection (5.8 %), dislocation (3.9 %), local recurrence (2.9 %), and acetabular fracture (1 %). CONCLUSION: MRP(®) megaprostheses are a valuable reconstruction option after tumor resection of the proximal femur.

Prognostic factors in surgical resection of sacral chordoma.

BACKGROUND AND OBJECTIVES: The best treatment of sacral chordoma is surgical resection, nowadays associated with optimized radiation therapy. We analysed 1) the oncologic outcome in a large series; 2) the effect of previous intralesional surgery, resection level, tumor volume and margins on survivorship to local recurrence (LR) and 3) the complication rate. METHODS: We reviewed 71 patients with sacral chordomas. Forty-eight resections were proximal to S3. Mean tumor volume was 535 cm3. Eleven received previous intralesional surgery elsewhere. Margins were wide in 44 resections, wide-contaminated in 11, marginal in 9 and intralesional in 7. RESULTS: Overall survival was 92%, 65% and 44% at 5, 10 and 15 years. At a mean of 9.5 years 37 were NED (54.4%), 23 died with disease (33.8%) and 8 were alive with disease (11.7%). Relapses included 15 LRs, 6 distant metastases, 17 both. LR rate was significantly higher in patients with previous surgery (p=0.0217), with inadequate margins (p= 0.0339) and large tumors(p<0.01), whereas resection level was not significant. Multivariate analysis confirmed the role of tumor volume. Complication rate was high (80.9%) with an infection rate of 41.2%. CONCLUSIONS: The most prominent adverse factor for LR was previous intralesional surgery. LR rate was related with inadequate margins and tumor volume.

Survival of modern knee tumor megaprostheses: failures, functional results, and a comparative statistical analysis.

BACKGROUND: Modular megaprostheses are now the most common method of reconstruction after segmental resection of the long bones in the lower extremities. Previous studies reported variable outcome and failure rates after knee megaprosthetic reconstructions. QUESTIONS/PURPOSES: The objectives of this study were to analyze the results of a modular tumor prosthesis after resection of bone tumor around the knee with respect to (1) survivorship; (2) failure rate; (3) comparative survivorship against different sites of reconstructions and of primary and revision implants; and (4) functional results on the Musculoskeletal Tumor Society (MSTS) scoring system. METHODS: Between 2003 and 2010, 247 rotating-hinge Global Modular Reconstruction System (GMRS) knee prostheses were implanted in our institute for malignant and aggressive benign tumors. During this time, that group represented 23% of the patients who had oncologic megaprosthesis reconstruction about the knee after resection of primary or metastatic bone tumors (247 of 1086 patients). In the other 77% of cases we used other types of oncologic prostheses. Before 2003 we used the older Howmedica Modular Resection System and Kotz Modular Femur/Tibia Replacement from 2003 we used mostly the GMRS but we continued to use the HMRS in some cases such as patients with poor prognoses, elderly patients, or metastatic patients. Sites included 187 distal femurs and 60 proximal tibias. Causes of megaprosthesis failure were classified according to Henderson et al. in five types: Type 1 (soft tissue failure), Type 2 (aseptic loosening), Type 3 (structural failure), Type 4 (infection), and Type 5 (tumor progression). Followup was at a minimum oncologic followup of 2 years (mean, 4 years; range, 2-8 years). Kaplan-Meier actuarial curves of implant survival to major failures were done. Functional results were analyzed according to the MSTS II system; 223 of the 247 were available for functional scoring (81%). RESULTS: At latest followup, among 175 treated patients for primary reconstruction, 117 are continuously disease-free, 26 have no evidence of disease after treatment of relapse, eight are alive with disease, and 24 died from disease. The overall failure rate of the megaprostheses in our series was 29.1% (72 of 247). Type 1 failure occurred in 8.5% (21 of 247) cases, Type 2 in 5.6% (14 of 247), Type 3 in 0%, Type 4 in 9.3% (23 of 247), and Type 5 in 5.6% (14 of 247). Kaplan-Meier curve showed an overall implant survival rate for all types of failures of 70% at 4 years and 58% at 8 years. Prosthetic survivorship for revisions was 80% at 5 years and for primary reconstructions was 60% at 5 years (p = 0.013). Survivorship to infection was 95% at 5 years for revision patients and 84% at 5 years for primary patients (p = 0.475). The mean MSTS score was 84 (25.2; range, 8-30) with no difference between sites of localization (24.7 in proximal tibia versus 25.4 in distal femur reconstruction; p = 0.306). CONCLUSIONS: Results at a minimum of 2 years with this modular prosthesis are satisfactory in terms of survivorship (both oncologic and reconstructive) and causes and rates of failure. Although these results seem comparable with other like implants, we will continue to follow this cohort, and we believe that comparative trials among the available megaprosthesis designs are called for. LEVEL OF EVIDENCE: Level IV, therapeutic study. See Guidelines for Authors for a complete description of levels of evidence.

Modern treatment of unicameral and aneurysmatic bone cysts.

The best treatment of unicameral bone cyst and aneurismatic bone cyst (ABC) is debated in the literature. For simple bone cysts, multiple treatments were proposed from observation only to open curettage. The historical treatment with intraosseous injection of methylprednisolone acetate into the bone cysts nowadays is reduced due to the morbidity of multiple injections and the risk of multiple pathologic fractures until the healing. Different types of treatments for ABC are reported, including surgery, percutaneous treatments, and medical treatments; however, there is currently no consensus on the best approach. The association of curettage, bone graft, and elastic stable intramedullary nail (ESIN) had a success rate of over 85%. Decompressing the cyst wall is more critical for increasing the healing rate than the type of graft used to fill the cavity. In ABC, sclerotherapy offers the advantages of lower invasiveness and morbidity, associated with better functional scores and faster return to full weight-bearing. Moreover, they can be used in challenging locations. Selective arterial embolization is a complex procedure and often requires association with other treatments. Further studies are needed to confirm the effectiveness of denosumab and its side effects on skeletally immature patients. Curettage with adjuvants and autogenous bone grafting still shows promising results and can be used in larger, aggressive defects or superficial lesions. For simple bone cysts, the combination of curettage, bone graft, and ESIN showed the best results. Sclerotherapy for ABC also shows promising results.

The Evaluation and Management of Lung Metastases in Patients with Giant Cell Tumors of Bone in the Denosumab Era.

Giant cell tumor of bone (GCTB) is characterized by uncertain biological behavior due to its local aggressiveness and metastasizing potential. In this study, we conducted a meta-analysis of the contemporary literature to evaluate all management strategies for GCTB metastases. A combination of the terms "lung metastases", "giant cell tumor", "bone", "treatment", and "oncologic outcomes" returned 133 patients meeting our inclusion criteria: 64 males and 69 females, with a median age of 28 years (7-63), at the onset of primary GCTB. Lung metastases typically occur at a mean interval of 26 months (range: 0-143 months) after treatment of the primary site, commonly presenting as multiple and bilateral lesions. Various treatment approaches, including surgery, chemotherapy, radiotherapy, and drug administration, were employed, while 35 patients underwent routine monitoring only. Upon a mean follow-up of about 7 years (range: 1-32 years), 90% of patients were found to be alive, while 10% had died. Death occurred in 25% of patients who had chemotherapy, whereas 96% of those not treated or treated with Denosumab alone were alive at a mean follow-up of 6 years (range: 1-19 years). Given the typically favorable prognosis of lung metastases in patients with GCTB, additional interventions beyond a histological diagnosis confirmation may not be needed. Denosumab, by reducing the progression of the disease, can play a pivotal role in averting or delaying lung failure.

When Is a Two-Stage Surgical Procedure Indicated in the Treatment of Pseudotumors of the Hip? A Retrospective Study of 21 Cases and a Review of the Literature.

(1) Background: A pseudotumor of the hip is a sterile, non-neoplastic soft tissue mass associated with total hip arthroplasties. Pseudotumors may mimic soft tissue tumors or infections, and thus a differential diagnosis is crucial, and biopsy is recommended. The purpose of this study was to compare the complications and functional results between one-stage and two-stage procedures. (2) Methods: We retrospectively analyzed 21 patients surgically treated at our institution with "pseudotumors" associated with hip prosthesis (8 male, 13 female with a mean age of 69 years). One-stage revision was performed in 10 cases and two-stage reversion in 10, with excision only in 1 case. Complications were classified as major and minor and functional results assessed using the Harris Hip Score (HHS). (3) Results: Five patients (24%) reported major complications. The survival rate for all complications was 75%. The overall survival rate was 95% at 5 years. The mean HHS ranged from 35 pre-op to 75 post-op, highlighting improved functional results in all cases. We recorded no differences in complications or functional outcomes between the one- and two-stage procedures. (4) Conclusions: In our experience, the two-stage surgical approach is preferable in cases with major bone defects and larger pseudotumor sizes. The use of custom-made 3D-printed prostheses is increasing and is a further reason to prefer two-stage revision.

Primary vascular tumors of bone: A comprehensive literature review on classification, diagnosis and treatment.

Primary vascular tumors of bone are a heterogeneous group of neoplasms, ranging from benign hemangiomas to frankly malignant epithelioid hemangioendotheliomas and angiosarcomas. Over the years, their classification has been a matter of discussion, due to morphologic similarities and uncertainty regarding biologic behavior. Over the past decade, with the development of next-generation sequencing, there has been a significant improvement in the molecular characterization of these lesions. The integration of their morphologic, immunohistochemical and molecular features has led to a better stratification, with important prognostic and therapeutic implications. Nevertheless, primary vascular bone tumors still represent a challenge for medical oncologists. Given their rarity and heterogeneity, in the last few years, there has been no significant progress in medical treatment options, so further research is needed. Here we present a review of the current knowledge regarding primary vascular tumors of the bone, correlating clinicopathologic features with tumor behavior and therapeutic approaches.

Proximal tibial resections and reconstructions: clinical outcome of 225 patients.

BACKGROUND: Previous studies reported variable outcome of proximal tibial resections and reconstructions. Therefore, we evaluated the survival, Musculoskeletal Tumor Society (MSTS) function, and complications of patients and reconstructions in this location. MATERIALS AND METHODS: We reviewed the files of 225 patients with proximal tibial tumors treated with proximal tibial resection, fixed or rotating hinge megaprosthetic reconstruction and extensor mechanism reattachment using sutures, mechanical clamping, artificial ligaments, and/or gastrocnemius flap from 1985 to 2010 (mean follow-up, 90 months; median, 56; range, 2-294). Survival of patients and reconstructions, type of hinge, extensor mechanism reconstruction, MSTS function, and complications were analyzed. RESULTS: Survival of patients with sarcomas was 68% and 62% at 5 and 10 years. Survival of megaprosthetic reconstructions was 82% and 78% at 5 and 10 years, without any difference between fixed and rotating hinge megaprostheses. MSTS function was significantly better in univariate and multivariate analysis for rotating compared to fixed hinge megaprostheses, without any difference between the types of extensor mechanism reconstructions. Most common complications were infection (12%), aseptic loosening (6%), and extensor mechanism rupture (3%). CONCLUSIONS: Rotating hinge proximal tibia megaprosthetic reconstructions have better function compared to fixed hinge, regardless of the type of extensor mechanism reconstruction.

Outcome of expandable prostheses in children.

BACKGROUND: Expandable prostheses offer the advantages of limb-salvage and limb-length equality at skeletal maturity. However, what is the cost for achieving that goal, and in how many children this is achieved? MATERIALS AND METHODS: We present 32 children (16 boys and 16 girls; mean age, 9 y) with bone sarcomas of the femur treated with limb salvage using expandable prostheses. The Kotz Growing prosthesis and the noninvasive Repiphysis and Stanmore expandable prostheses were used. The mean follow-up was 49 months. Survival analysis of the children and primary implants and functional evaluation were performed. RESULTS: Survival of the children was 94% and 84% at 48 and 72 months. Survival of the primary prostheses was 78% and 66% at 48 and 72 months; survival was significantly higher only for the Kotz when compared with the Repiphysis prostheses (P=0.026). The rate of implant-related complications was 51.3%; 9 prostheses (23%) were revised because of aseptic loosening, infection, and breakage. A mean total lengthening of 28 mm (4 to 165 mm) was achieved by 84 procedures (2.6 procedures/patient). Three of the 9 children who reached skeletal maturity had limb-length equality and 6 discrepancy of 15 to 30 mm. The mean Musculoskeletal Tumor Society score was excellent (79%) without a significant difference between the type of prostheses (P=0.934). CONCLUSIONS: The Kotz Growing prosthesis, although it requires an open lengthening procedure, has shown higher survival when compared with the noninvasive Repiphysis prosthesis. However, the total lengthening remains small, and the complications rates are high even with the noninvasive prostheses.

New concepts in the surgical treatment of actual and impending pathological fractures in metastatic disease.

INTRODUCTION: Long bone metastases are a disease of high social importance. The goals of surgical treatment are to relieve pain, maintain or restore joint function, and prevent or treat pathological fractures. "Oligometastases" is a disease with a limited number (3-5) of metastatic lesions in the same body district, where an aggressive treatment can be carried out with "curative" intent. This study aimed to evaluate patients with bone metastases surgically treated to determine how surgical treatment can influence prognosis and quality of life, comparing solitary metastasis, oligometastases, and multiple metastases. PATIENTS AND METHODS: This is a retrospective analysis of 130 patients with long bone metastases surgically treated between October 2015 and August 2019: 40 patients had solitary metastasis; 38 had less than three metastases (oligometastases), and 52 had multiple metastases. Surgery was resection and reconstruction with a cemented prosthesis (95) or nailing (35). RESULTS: Overall survival was significantly better in patients with solitary metastasis or oligometastases than in those with multiple metastases (p <0.0001). Patients treated with resection and prosthesis had significantly better survival than those treated with nailing (p <0.0001). Implant complications requiring surgical revision occurred in 20 patients treated with prostheses, while no complications occurred in patients treated with nailing. DISCUSSION: Survival of cancer patients has improved in the last two decades, leading to an increase of diagnosed metastases. Patients with oligometastases have a survival similar to those with a single metastasis. Optimal implants survival curves should stay above the curves of patients survival. CONCLUSIONS: Since there are no differences in survival, patients with oligometastases should be treated as patients with a solitary lesion, with more aggressive surgery (wide resection and reconstruction with prosthesis). Intramedullary nailing is still indicated in metaphyseal or diaphyseal metastases in patients with advanced disease or poor prognosis when the life expectancy does not overcome the expected survival of the nail, avoiding the need for further surgery.

Post-radiation sarcomas. Clinical outcome of 52 Patients.

BACKGROUND: Previous studies reported on post-radiation sarcomas. However, the incidence, latency from radiation therapy, treatment, and survival has been difficult to evaluate. We performed a retrospective, single-institutional study to determine these factors for post-radiation sarcomas. MATERIALS AND METHODS: We retrospectively studied 52 patients with post-radiation sarcomas diagnosed and treated from 1985 to 2011. The mean age was 49 years; 45 patients had bone and 7 soft tissue sarcoma. The mean follow-up was 45 months. Survival was analyzed with respect to age at diagnosis, type (bone vs. soft tissue), histology, location (trunk vs. extremities), size, and surgical treatment (resection vs. amputation). RESULTS: The risk of post-radiation sarcoma was 0.06% at a mean latency of 15 years (3-50 years) after radiation therapy. The most common histology was osteosarcoma followed by malignant fibrous histiocytoma and fibrosarcoma; all sarcomas were high grade. Survival of the patients with post-radiation sarcomas was 85% at 1 year, 51% at 2 years, 48% at 3 years, and 45% at 5 years. Univariate predictor of survival was only the type of the sarcoma. No variable was significant in multivariate analysis. CONCLUSIONS: Prognosis of post-radiation sarcomas is poor; the type of the sarcomas is the only significant variable for survival.

Survival analysis of patients with femoral metastases.

BACKGROUND: Previous studies reported on surgical indications for patients with femoral metastases. However, few studies analyzed the spectrum of femoral metastatic presentation. We performed this study to evaluate the survival of patients with femoral metastases, and clarify the treatment of femoral impending and actual pathological fractures. MATERIALS AND METHODS: We retrospectively studied 110 patients with femoral metastases from various cancers treated with nailing or resection and megaprosthetic reconstruction from 1995 to 2010. The mean follow-up was 18 months. Survival was analyzed with respect to different metastatic presentations regarding gender, type of cancer, number, and location of femoral metastases, type of surgery, and pathological fracture. RESULTS: Univariate predictors of survival were the pathological fracture and type of surgery; multivariate predictor was only the pathological fracture. Survival was significantly higher in patients with resection compared to nailing, impending compared to actual fracture, solitary metastasis and impending fracture, actual fracture treated with resection, proximal femoral actual fracture and distal femoral impending fracture treated with resection. CONCLUSIONS: Patients with femoral metastases have better survival when present with impending compared to actual pathological fracture. Although with a higher rate of complications, patients with pathological fractures of the proximal and distal femur may benefit from resection.

Clinical outcome of central conventional chondrosarcoma.

INTRODUCTION: Aim of this study was to analyze (1) survival, local recurrence (LR), and metastasis rates between the three histological tumor grades; (2) whether type of treatment and tumor site influenced prognosis for each histologic grade. METHODS: We retrospectively studied 296 patients with central conventional chondrosarcomas (CS) (87 grade 1, 162 grade 2, and 47 grade 3). The femur was the most common site (91 cases), followed by the pelvis (82) and other less frequent sites. Type of surgery was related with histologic grade. Margins were wide in 222 cases, marginal in 23, and intralesional in 51 cases. RESULTS: At a mean of 7 years, 201 patients remained continuously NED, 33 were NED after treatment of relapse, 15 were AWD, 35 were died of disease, and 12 of other causes. Survival was 92% at 5 years and 84% at 10 years, significantly influenced by histological grading. In grade 3 CS, two factors influenced survival: type of surgery (resection vs. amputation, P = 0.051) and site (P = 0.039). The two significant factors lost their significance at multivariate analysis. CONCLUSION: Central conventional CS with low/intermediate grade has a good prognosis, while high-grade tumors have poor outcome. Tumor relapses are strictly related with histologic grade.