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.

What Is the 10-year Survivorship of Cemented Distal Femoral Endoprostheses for Tumor Reconstructions and What Radiographic Features Are Associated with Survival?

BACKGROUND: Aseptic loosening is one of the most common causes of revision of distal femoral endoprostheses and is considered a mid- to long-term complication. There are not many reports of 10-year survivorship free from aseptic loosening and all-cause survivorship in cemented stems. To our knowledge, there are no reports on radiographic features that are associated with aseptic loosening of these implants. QUESTIONS/PURPOSES: (1) What is the 5- and 10-year survivorship free from aseptic loosening in patients undergoing reconstruction with a cemented distal femoral endoprosthesis after a tumor resection? (2) What is the all-cause 5- and 10-year survivorship at in these patients? (3) What radiographic features are associated with aseptic loosening at long-term follow-up? METHODS: We performed a multicenter retrospective study reviewing aseptic loosening in cemented prostheses to determine radiographic features associated with long-term implant survivorship. Patients who underwent a cemented distal femoral reconstruction with a modular endoprosthesis after resection of a musculoskeletal tumor between 1997 and 2017 were reviewed. A total of 246 patients were identified from five institutions and met initial inclusion criteria. Of those, 21% (51) were lost to follow-up before 2 years, leaving 195 patients available for us to evaluate and analyze the survivorship and radiologic features associated with long-term implant survival. The mean (range) follow-up was 78 months (22 to 257). At the time of this analysis, 69% (135 of 195) of the patients were alive. Osteosarcoma was the most common diagnosis in 43% of patients (83 of 195), followed by metastatic carcinoma 13% (25 of 195). Fifty-six percent (110 of 195) of patients received chemotherapy; 15% (30 of 195) had radiation therapy. Aseptic loosening was diagnosed radiographically and was defined as a circumferential radiolucent line on all views, or subsidence around the stem in the absence of infection. We present 5- and 10-year Kaplan-Meier survivorship free from aseptic loosening, 5- and 10-year all-cause survivorship, and a qualitative assessment of radiographic features potentially associated with aseptic loosening (including the junctional radiolucent area, and cortical expansion remodeling). The junctional radiolucent area was defined as a radiolucent area of the bone starting at the bone-endoprosthesis junction to the tip of the femoral stem, and cortical expansion remodeling was defined as an increased cortical thickness at the stem tip. Although we wished to statistically analyze radiographic factors potentially associated with aseptic loosening, we did not have enough clinical material to do so (only nine patients developed loosening). Instead, we will report a few preliminary qualitative observations, which necessarily are preliminary, and which will need to be confirmed or refuted by future studies. We urge caution in interpreting these findings because of the very small numbers involved. RESULTS: Kaplan-Meier survivorship free from aseptic loosening of the femoral component at 5 and 10 years were 95% (95% CI 89 to 98) and 93% (95% CI 86 to 97), respectively. Kaplan-Meier survivorship free from revision for any cause at 5 and 10 years were 74% (95% CI 65 to 79) and 64% (95% CI 49 to 70), respectively. Although the numbers were too small to analyze statistically, all patients with aseptic loosening had a junctional radiolucent area more than 20% of the total length of the stem without cortical expansion remodeling at the stem tip. No aseptic loosening was observed if there was cortical ex remodeling, a junctional radiolucent area less than 20%, or curved stems that were 13 mm or greater in diameter. The numbers of patients with aseptic loosening in this series were too small to analyze statistically. CONCLUSIONS: Cemented distal femoral endoprostheses have a relatively low rate of aseptic loosening and acceptable projected first-decade survivorship. The presence of a radiolucent area more than 20% without cortical expansion remodeling at the stem tip may lead to aseptic loosening in patients with these implants. Close radiographic surveillance and revision surgery may be considered for progressive lucencies and clinical symptoms of pain. If revision is contemplated, we recommend using larger diameter curved cemented stems. These are preliminary and provisional observations based on a low number of patients with aseptic loosening; future studies with greater numbers of patients are needed to validate or refute these findings. LEVEL OF EVIDENCE: Level III, therapeutic study.

Analysis of principles inspiring design of three-dimensional-printed custom-made prostheses in two referral centres.

BACKGROUND: Three-dimensional (3D) printing is an emerging technology used in numerous medical fields. Reconstruction of large bone defects after tumor resections or complex revision surgeries is challenging especially in specific sites where modular prostheses are not available. The possibility to realize custom-made 3D-printed prostheses improves their application in surgical field despite the complication rate, gaining a lot of attention for potential benefits. OBJECTIVES: We asked: (1) What are the emerging indications and designs of 3D-printed prostheses for complex bone reconstructions? (2) What complications occur with the use of custom implants considering site? STUDY DESIGN AND METHODS: We performed a retrospective analysis of every patient in whom a custom-made 3D-printed prosthesis was used to reconstruct a bone defect after resection for a bone tumour or challenging revision surgery from 2009 to 2018 in two referral centres. Forty-one patients (11 males [27%], 30 females [73%]) with a mean age of 41 years (range, 10-78 years) were included. Our general indications for using these implants were complex reconstructions of massive bone defects, in the absence of available modular prostheses. Seven were non-oncologic patients, whereas 24 patients were mainly treated for their malignant bone tumours. Custom-made 3D-printed prostheses were used in pelvis (29), forearm (6), scapula (2), distal tibia (2), calcaneus (1), and femoral diaphysis (1). The reconstruction included complete articular replacement in 24 cases (58%) whereas a combined spinopelvic implant has been used in two cases. Flaps were used in 25 cases (61%). Statistical analyses include Kaplan-Meier curves of survival. RESULTS: The mean follow-up was 20 months. In the oncologic group, overall survival was 89% at five year follow-up and only three patients died of disease. Only one patient required implant removal due to deep infection. Overall major and minor complication rate was 22% (14 complications in 9/41 patients), mainly wound-related problems. One patient reported a periprosthetic fracture, one had hip dislocation, and four (12% [4/34 cases]) had local recurrence. Mean MSTS functional outcome score at follow-up was 73% (range, 23-100%), with a full weight bearing at an average time of 73 days from surgery of lower limbs. CONCLUSIONS: Custom-made 3D-printed prostheses represent at today a promising reconstructive technique, maintaining however the correct indications for their use in musculoskeletal oncology and challenging revision surgery. Complication rate is acceptable, with infection and wound healing problems relatively common after complex pelvic reconstructions. We will continue to follow our patients over the longer term to ascertain the role of these implants; however, larger studies will need to confirm indications and control for prognostic factors.

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.

Calcific Myonecrosis of the Leg: A Rare Entity.

Calcific myonecrosis is a rare disease that has been shown to be a late sequela of trauma. This article presents a 68-year-old man with calcific myonecrosis of the leg 40 years after a tibial fracture complicated with peroneal nerve palsy. The soft tissue mass increased in size after another injury to the leg that occurred two years before his presentation. Physical examination at presentation showed a palpable extra-osseous mass at the anterior aspect of the left leg; the mass was not adherent to adjacent soft-tissues and bone, and it was painless but tender to palpation. Radiographs of the left leg showed extensive calcification at the soft-tissue of the anterior and posterior leg. An ultrasonography-guided trocar biopsy was done; histological findings were indicative of calcific myonecrosis. Given the benign entity of the lesion and known high rate of complications, he was recommended for no further treatment except for clinical and imaging observation. Located at the site of the biopsy, he experienced infection with drainage that eventually healed after six months with antibiotics and wound dressing changes. During the last follow-up examination, two years after diagnosis, the patient was asymptomatic without progression of the mass.

The role of 18F-FDG PET/CT in the detection of osteosarcoma recurrence.

AIM: The aim of this study was to investigate the diagnostic accuracy of 18F-FDG-PET/CT in osteosarcoma patients suspicious for disease recurrence after adequate surgical therapy. METHODS: Inclusion criteria were: a) adequate surgical treatment for proven osteosarcoma and documented complete remission after therapy; b) 18F-FDG-PET/CT performed during follow-up for clinical/diagnostic suspicion of relapse; c) new surgical treatment with excision of the suspected lesions; d) histological validation of 18F-FDG-PET/CT findings. Thirty-seven patients matching all inclusion criteria were retrospectively enrolled (20 men and 17 female). Primary surgical treatment consists of resection (31 cases) or amputation (six cases). 18F-FDG-PET/CT performance was assessed with a per-patient and per-site evaluation of sensitivity, specificity, accuracy, positive predicting value (PPV), and negative predicting value (NPV). The sites of relapse were classified as local, lung, lymphnodes (LNs), and distant (other skeletal segments and/or distant soft tissue). The disease-free survival (DFS) and the overall survival (OS) after 18F-FDG PET/CT were evaluated. RESULTS: 18F-FDG-PET/CT was positive in 89.2% (33/37) of patients. Local uptake only was observed in 35.1% patients (13/37); lung uptake only in 18.9% (7/37); distant uptake only in 2.7% (1/37) case; multiple sites of uptake in 32.4% (12/37). Histology resulted positive in 92% (34/37) of patients. A total of 51 pathologic lesions were evaluated (22 local relapse, 11 lung metastasis, 10 metastatic LNs, eight distant metastatic lesions). On a per-patient analysis 18F-FDG-PET/CT showed a sensitivity, specificity, accuracy, PPV, and NPV of 91%, 75%, 89%, 97%, 50%. On a per-site analysis the performance for local relapse was 96%, 100%, 97%, 100%, 93%, while for lung relapse detection was 80%, 100%, 92%, 100%, 88%. The mean follow-up after 18F-FDG-PET/CT was 21.5 months. At the last follow-up, 19% (7/37) of patients were death with disease, 38% (14/37) were alive with disease, and 43% (16/37) had no evidence of disease. Overall survival was 90% and 75% at 24 and 60 months, respectively. CONCLUSION: 18F-FDG-PET/CT showed valuable results for detecting recurrence(s) in osteosarcoma patients with suspicious of relapse after treatment, particularly in the detection of local relapse and lung metastasis.

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.

Osteoblastoma of the elbow: analysis of 13 patients and literature review.

PURPOSE: To analyze the clinical and radiographic characteristics, treatment, and outcome of patients with elbow osteoblastoma. PATIENTS AND METHODS: We present 13 patients (7 males and 6 females; mean age, 28 years) diagnosed and treated for an elbow osteoblastoma from 1975 to 2012. Mean follow-up was 29 months (range 12-60 months). Clinical presentation, imaging, surgical treatment, complications, range of elbow motion, and functional outcome were evaluated. The MSTS, DASH, and OXFORD scores were used. RESULTS: Main symptom was pain (all patients) accompanied by stiffness (8 patients) and swelling or tumefaction (7 patients), with a median duration of symptoms of 32 months (range 6-96 months). Distal humerus was affected in 10 patients, proximal ulna in 2 patients, and proximal radius in one patient. All patients underwent surgical therapy that consisted of curettage of the lesion (7 patients), curettage and bone allografting (3 patients), wide resection (2 patients; total distal humerus and resection of the radial head), and radiofrequency thermal ablation (1 patient). One patient experienced a recurrence after surgical treatment. The mean MSTS score after treatment was 87% (range 50-100%), which corresponds to excellent results. CONCLUSIONS: Intralesional surgery is successful in tumor control in most patients with osteoblastoma of the elbow. Thermal ablation may be successful for smaller lesions. Most of the patients had a good-to-excellent functional outcome even if they had tumor-related elbow stiffness at diagnosis. LEVEL OF EVIDENCE: Therapeutic study, Level IV-1.

Chondroblastoma in adult age.

PURPOSE: Chondroblastoma (CB) is a rare benign tumor that occurs most commonly in the second decade of life. No studies on CB in adulthood have been reported. Our purposes were to report a single-institution experience on CB in adults and to discuss the clinical and imaging findings, type of treatment, oncologic and functional outcomes. PATIENTS AND METHODS: All patients diagnosed and treated for CB from 1981 to 2014 were reviewed. The main inclusion criterion was patients above their 30 years of age at diagnosis. Clinical presentation, imaging, surgical treatment, complications and functional outcome were evaluated. Local recurrences were recorded. RESULTS: There were 15 males (65%) and 8 females (35%) with a mean age of 40 years (range 30-59 years). Tumor involved the small bones (talus, calcaneus, acromion, cuneiform, metatarsals) in 15 patients (65%) and the long bones in eight patients (35%). Treatment consisted of curettage only (6 cases), curettage plus phenol (13 cases), curettage plus radiotherapy (one patient with vertebral tumor), resection (two patients with CB of the acromion) and chopart amputation (one patient with a metatarsal tumor). At a mean follow-up of 8.4 years (range 3-30 years), twenty patients (87%) remained continuously disease-free, whereas three had local recurrences. None of the patients received chemotherapy. CONCLUSION: This study confirms that the occurrence and outcome of adult patients with CB are similar with those affecting younger patients. The main difference consists of the involved bones; flat bones and short tubular bones of the foot, especially the talus and calcaneus, are most commonly involved as opposed to long tubular bones. LEVEL OF EVIDENCE: Therapeutic study, level IV-1 (case series).

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.

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.

Infection after surgical resection for pelvic bone tumors: an analysis of 270 patients from one institution.

BACKGROUND: Surgical treatment of pelvic tumors with or without acetabular involvement is challenging. Primary goals of surgery include local control and maintenance of good quality of life, but the procedures are marked by significant perioperative morbidity and complications. QUESTIONS/PURPOSES: We wished to (1) evaluate the frequency of infection after limb salvage surgical resection for bone tumors in the pelvis; (2) determine whether infection after these resections is associated with particular risk factors, including pelvic reconstruction, radiotherapy or chemotherapy, type of resection, and age; and (3) analyze treatment of these infections, particularly with respect to the need of additional surgery or hemipelvectomy. METHODS: From 1975 to 2010, 270 patients with pelvic bone tumors (149 with chondrosarcoma, 40 with Ewing's sarcoma, 27 with osteosarcoma, 18 with other primary malignant tumors, 11 with metastatic tumors, and 25 with primary benign tumors) were treated by surgical resection. Minimum followup was 1.1 years (mean, 8 years; range, 1-33 years). The resection involved the periacetabular area in 166 patients. In 137 patients reconstruction was performed; in 133 there was no reconstruction. Chart review ascertained the frequency of deep infections, how they were treated, and the frequency of resection arthroplasty or hemipelvectomies that occurred thereafter. RESULTS: A total of 55 patients (20%) had a deep infection develop at a mean followup of 8 months. There were 20 infections in 133 patients without reconstruction (15%) and 35 infections in 137 patients with reconstruction (26 %). Survivorship rates of the index procedures using infection as the end point were 87%, 83%, and 80% at 1 month, 1 year, and 5 years, respectively. Infection was more common in patients who underwent pelvic reconstruction after resection (univariate analysis, p = 0.0326; multivariate analysis, p = 0.0418; odds ratio, 1.7718; 95% CI, 1.0243-3.0650); no other risk factors we evaluated were associated with an increased likelihood of infection. Despite surgical débridements and antibiotics, 16 patients (46%) had the implant removed and five (9%) underwent external hemipelvectomy (four owing to infection and one as a result of persistent infection and local recurrence). CONCLUSIONS: Infection is a common complication of pelvic resection for bone tumors. Reconstruction after resection is associated with an increased risk of infection compared with resection alone, without significant difference in percentage between allograft and metallic prosthesis. When infection occurs, it requires removal of the implant in nearly half of the patients who have this complication develop, and external hemipelvectomy sometimes is needed to eradicate the infection.

Is still effective massive allograft reconstruction in parosteal osteosarcoma of the distal femur? Review of the literature and advantages of newer technologies.

PURPOSE: Parosteal Osteosarcoma is a well-differentiated, low-grade bone sarcoma. It most commonly occurs in the third decade of life, usually in the distal femur. This study aims to perform a literature review about the types of reconstructions reported and to analyze the results of an updated technique of resection using custom-made 3D-printed cutting guides. METHODS: We perform a systematic literature review about parosteal osteosarcoma, evaluating treatments, margins, local recurrence, complications, and functional results when available. We also report a case treated in our Center with a revisited technique introducing custom-made 3D-printed cutting guides. RESULTS: We analyzed 12 studies with a total of 151 patients. The distal femur was the most frequently reported site (81.5%). After distal femur resection, reconstruction was performed with graft in most cases (48%), followed by prosthetic reconstruction (40%). Margins were wide in 85.5% of cases. The total incidence of local recurrence was 11%. Functional results were excellent in all cases, with a mean MSTS score of 86%. In our case, with the help of the jigs, the surgical technique was relatively easy, graft fusion excellent and fast, margins wide, and functional results excellent. CONCLUSIONS: In the literature, the most commonly used type of reconstruction after resection is biological with graft. Indeed, despite the increasing number of prosthetic reconstructions, the historical diaphysometaphyseal hemiresection and graft is still indicated in parosteal osteosarcoma of the distal femur. New technologies, such as the jigs we used, allow significant advantages during the procedure: reduce the resection and graft preparation time, allow a better match between components, and help to obtain safer margins, sparing as much bone as possible.

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.

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.

Reconstructive surgery after distal fibular resection due to bone tumors: a technical report on surgical strategies and results from the PROSPERO international register of systematic reviews.

Purpose: Primary bone tumors of the fibula are rare. Distal fibular resection has a significant impact on ankle biomechanics and gait, possibly leading to complications such as ankle instability, valgus deformity, and degenerative changes. Question: Is there a need for reconstructive surgery after distal fibular resection, and what reconstructive procedures are available? Materials and methods: The review is registered with the PROSPERO International Register of Systematic Reviews. Inclusion criteria consisted of all levels of evidence, human studies, patients of all ages and genders, publication in English, and resection of the distal portion of the fibula due to tumor pathology. The reviewers defined four different categories of interest by method of treatment. Additional articles of interest during full-text review were also added. Results: The initial search resulted with a total of 2958 records. After screening, a total of 50 articles were included in the study. Articles were divided into 'No reconstruction', 'Soft tissue reconstruction', 'Bone and soft tissue reconstruction', and 'Arthrodesis, arthroplasty or other reconstruction options' groups. Conclusion: Limb salvage surgery should be followed by reconstruction in order to avoid complications. Soft tissue reconstructions should always be considered to stabilize the joint after fibular resection. Bone reconstruction with reversed vascularized fibula is the preferred technique in young patients and in cases of bone defects more than 3 cm, while arthrodesis should be considered in adult patients. Whenever possible for oncologic reason, if a residual peroneal malleolus could be preserved, we prefer augmentation with a sliding ipsilateral fibular graft.

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.