Standardized multi-planar reformation improves the reliability of the assessment of the anterolateral ligament in ACL-deficient knees.

PURPOSE: The anterolateral ligament (ALL) is an important structure for controlling anterolateral rotatory stability of the knee. Its assessment, however, is difficult using standardized MRI images. The goal of this study was to assess the reliability of judging the integrity of the ALL on multi-planar reformatted (MPR) MRI images and on standard coronal reformatted (SCR) MRI images in knees with an anterior cruciate ligament (ACL) rupture. METHODS: Forty-eight patients (14 females, 34 males, 30 ± 6 years (mean age ± standard deviation)) with acute ACL ruptures (< 2 weeks) and no additional knee injuries (except segond fractures) were included. Images were assessed by two independent raters twice with at least a 2-week interval in between. The assessment was first performed on SCR images and thereafter on MPR images. Images were judged for assessability of the ALL and then the integrity of the ALL was rated. RESULTS: Depending on rater and read, the ALL was judged as "torn" in between 5 (10.4%) and 11 (22.9%) patients out of 48 patients on SCR images. On MRP images, the ALL was judged as "torn" in between 5 (10.4%) and 6 (12.5%) patients out of 48 patients, depending on rater and read. Inter- and intra-rater reliability for the assessment of the ALL using MPR images was "substantial" to "almost perfect". Inter- and intra-rater reliability for the assessment using SCR was "fair" to "substantial". CONCLUSION: MPR images should be used when assessing the integrity of the ALL. Assessment quality is independent of patient positioning during MRI acquisition and the ALL can be displayed in full length on one image. LEVEL OF EVIDENCE: Level III.

Survival, functional outcome and satisfaction of first revision total knee arthroplasty at a mean eleven-year follow-up.

PURPOSE: Providing long-term outcome data after rTKA and compare one- versus two-stage and septic versus aseptic revisions. METHODS: This study represents a single-center retrospective study of first rTKAs performed for any reason with a final follow-up of a minimum of five years. Outcome parameters included stability assessment ROM, radiologic assessment, HSS score, KSS score, OKS score, EQ-5D-3L and VAS. 44 patients were included in the study. Subgroups analysis of one- versus two-stage revision and septic versus aseptic revision was performed. RESULTS: The leading causes of rTKA in this mean 11 year follow-up study were aseptic loosening (36%) and periprosthetic joint infection (27%). At the final follow-up, there was a 89% survivorship of the implants. Patients showed a ROM of 114 ± 13°, HSS score of 78 ± 12, KKS objective score of 77 ± 16, KSS expectation and satisfaction score of 32 ± 11, KSS functional activity score of 50 ± 20, OKS of 30 ± 9, VAS of 53 ± 25 and EQ-5D index of 0.649. Functional outcome scores were not significantly altered in the analyzed subgroups. CONCLUSIONS: In our 11 years follow-up, we obtained 89% implant survivorship. Measurements regarding functional outcome and pain showed results in the medium range of the respective scores, while patient satisfaction lay in the upper third. No significant differences in outcome scores between one- and two-stage revisions and septic versus aseptic revisions were observed. Level of Evidence Level III, retrospective cohort study.

Indications, clinical outcome and survival of rotating hinge total knee arthroplasty in a retrospective study of 63 primary and revision cases.

PURPOSE: The purpose of this study is to report and compare outcome data of both primary and revision cases using a rotating hinge knee (RHK) implant. METHODS: This study retrospectively analyzed 63 cases (19 primary, 44 revisions) at a mean follow-up of 34 ± 8 months after RHK implantation. Outcome parameters were stability, range of motion (ROM), loosening, Hospital of Special Surgery Score (HSS), Knee Society Score (KSS), Oxford Knee Score (OKS), EQ-5D-3L, and Visual Analog Scale (VAS) for overall function. Revision rates and implant survival are reported. RESULTS: Eleven percent showed medio-lateral instability < 5 mm, a mean ROM of 115° ± 17° and radiologic loosening occurred in 8% (2% symptomatic). PROMS showed the following results: HSS 79 ± 18, KSS 78 ± 27, OKS 26 ± 10, EQ-5D index 0.741 ± 0.233 and VAS 70 ± 20. Primary cases revealed better outcomes in HHS (p = .035) and OKS (p = 0.047). KSS, EQ-5D index and VAS did not differ between primary and revision cases (p = 0.070; p = 0.377; p = 0.117). Revision rate was 6.3% with an implant survival of 96.8%. CONCLUSIONS: RHK arthroplasty can be performed with good clinical outcome and low revision rate in revision and complex primary cases. RHK is an option in cases where standard arthroplasty and even implants with a higher degree of constraint have reached their limits. LEVEL OF EVIDENCE: Level III, retrospective cohort study.

Staged treatment of a comminuted femoral fracture with Masquelet technique and 3D printed reposition guides.

Background: Comminuted femoral fractures pose a challenge to the trauma surgeon due to the absence of bony references during surgery. Therefore, malalignment of length and axis can occur and necessitate revision surgery. During the last decade, 3D-planning has evolved as a surgical aid in difficult cases. Case report: An 18-year-old male patient suffered a polytrauma following a motorcycle accident. This report is about the treatment of a 3rd degree open and comminuted fracture of the left distal femur. The fracture was treated with Masquelet's two-staged technique. With the intent of avoiding malalignment, the second stage surgery was performed with the aid of 3D-planned reduction guides. Despite complex fracture pattern, complete fracture union was achieved with acceptable final alignment (side-to-side comparison of length, axis and femoral torsion). Conclusion: In this case, performing Masquelet's two-staged surgery with the aid of 3D-printed reposition guides yielded favorable results in regards to rotational malalignment. The malrotation of the femur was reduced after the second operation to a clinically acceptable side-to-side difference (10°). This technique remains technically challenging due to soft tissue tension and limited possibility of soft tissue release.

3D printed fracture reduction guides planned and printed at the point of care show high accuracy - a porcine feasibility study.

PURPOSE: After surgical treatment of comminuted diaphyseal femoral and tibial fractures, relevant malalignment, especially rotational errors occur in up to 40-50%. This either results in a poor clinical outcome or requires revision surgery. This study aims to evaluate the accuracy of reduction if surgery is supported by 3D guides planned and printed at the point of care. METHODS: Ten porcine legs underwent computed tomography (CT) and 3D models of femur and tibia were built. Reduction guides were virtually constructed and fitted to the proximal and distal metaphysis. The guides were 3D printed using medically approved resin. Femoral and tibial comminuted diaphyseal fractures were simulated and subsequently reduced using the 3D guides. Postoperative 3D bone models were reconstructed to compare the accuracy to the preoperative planning. RESULTS: Femoral reduction showed a mean deviation ± SD from the plan of 1.0 mm ± 0.9 mm for length, 0.9° ± 0.7° for varus/valgus, 1.2° ± 0.9° for procurvatum/recurvatum and 2.0° ± 1.7° for rotation. Analysis of the tibial reduction revealed a mean deviation ± SD of 2.4 mm ± 1.6 mm for length, 1.0° ± 0.6° for varus/valgus, 1.3° ± 1.4° for procurvatum/recurvatum and 2.9° ± 2.2° for rotation. CONCLUSIONS: This study shows high accuracy of reduction with 3D guides planned and printed at the point of care. Applied to a clinical setting, this technique has the potential to avoid malreduction and consecutive revision surgery in comminuted diaphyseal fractures. LEVEL OF EVIDENCE: Basic Science.

The EOS 3D imaging system reliably measures posterior tibial slope.

BACKGROUND: One of the values determined during the assessment of knee issues is the posterior tibial slope (PTS). A new option for measuring the PTS is the EOS 3D imaging system, which provides anteroposterior (AP) and lateral long leg radiographs (LLRs) using less radiation than a conventional LLR. We investigated the reliability of the EOS 3D imaging system with respect to PTS measurements. METHODS: We retrospectively searched our radiological database for patients who underwent an EOS scan and a computed tomography (CT) scan of their lower extremities between January and December 2019. Fifty-six knees were included in the study. Medial and lateral PTSs were determined using both modalities. A radiologist and an orthopaedic surgeon each performed all measurements twice and the intraclass correlation (ICC) was calculated to assess inter- and intrarater reliability. The Student t test and Pearson correlation were used to compare the results of both imaging modalities. RESULTS: The mean medial PTS was 8.5° (95% confidence interval [CI], 8.1-8.9°) for the EOS system and 7.7° (95% CI, 7.3-8.1°) for CT, and the lateral PTS was 7.4° (95% CI, 6.9-7.9°) for the EOS system, and 7.0° (95% CI, 6.5-7.4°) for CT. Interrater reliability (ICC) with respect to medial and lateral PTSs measured on the EOS (0.880, 0.765) and CT (0.884, 0.887) images was excellent. The intrarater reliability of reader 1 (ICC range, 0.889-0.986) and reader 2 (ICC range, 0.868-0.980) with respect to the same measurements was excellent. CONCLUSION: The PTS measurements from the EOS 3D imaging system are as reliable and reproducible as those from CT, the current gold standard method. We recommend using this system if possible, because it acquires more information (sagittal plane) in a scan than a conventional LLR, while exposing the patient to less radiation. LEVEL OF EVIDENCE: Level III, Retrospective cohort study.

Prevalence of combined abnormalities of tibial and femoral torsion in patients with symptomatic hip dysplasia and femoroacetabular impingement.

AIMS: The prevalence of combined abnormalities of femoral torsion (FT) and tibial torsion (TT) is unknown in patients with femoroacetabular impingement (FAI) and hip dysplasia. This study aimed to determine the prevalence of combined abnormalities of FT and TT, and which subgroups are associated with combined abnormalities of FT and TT. METHODS: We retrospectively evaluated symptomatic patients with FAI or hip dysplasia with CT scans performed between September 2011 and September 2016. A total of 261 hips (174 patients) had a measurement of FT and TT. Their mean age was 31 years (SD 9), and 63% were female (165 hips). Patients were compared to an asymptomatic control group (48 hips, 27 patients) who had CT scans including femur and tibia available for analysis, which had been acquired for nonorthopaedic reasons. Comparisons were conducted using analysis of variance with Bonferroni correction. RESULTS: In the overall study group, abnormal FT was present in 62% (163 hips). Abnormal TT was present in 42% (109 hips). Normal FT combined with normal TT was present in 21% (55 hips). The most frequent abnormal combination was increased FT combined with normal TT of 32% (84 hips). In the hip dysplasia group, 21% (11 hips) had increased FT combined with increased TT. The prevalence of abnormal FT varied significantly among the subgroups (p < 0.001). We found a significantly higher mean FT for hip dysplasia (31°; SD 15)° and valgus hips (42° (SD 12°)) compared with the control group (22° (SD 8°)). We found a significantly higher mean TT for hips with cam-type-FAI (34° (SD 6°)) and hip dysplasia (35° (SD 9°)) compared with the control group (28° (SD 8°)) (p < 0.001). CONCLUSION: Patients with FAI had a high prevalence of combined abnormalities of FT and TT. For hip dysplasia, we found a significantly higher mean FT and TT, while 21% of patients (11 hips) had combined increased TT and increased FT (combined torsional malalignment). This is important when planning hip preserving surgery such as periacetabular osteomy and femoral derotation osteotomy. Cite this article: Bone Joint J 2020;102-B(12):1636-1645.

Seventy percent long-term survival of the repaired ACL after dynamic intraligamentary stabilization.

PURPOSE: Primary repair of the anterior cruciate ligament (ACL) is regaining popularity. Long-term results are lacking. The purpose of the current study was to determine the survival of the primarily repaired ACL after dynamic intraligamentary stabilization. METHODS: Between 2011 and 2013, 57 patients with acute proximal ACL ruptures underwent DIS repair within 3 weeks from injury and were available for final follow-up at least 5 years postoperatively. Failure as an end point was defined as conversion to ACL reconstruction, failure to restore stability with persisting laxity side-to-side laxity of > 5 mm or a late-traumatic re-rupture or loss of stability. Kaplan-Meier survival analysis was performed. RESULTS: Kaplan-Meier survival analysis demonstrated an overall survival of 70.0% (standard error SE 6.6%) at 74 months follow-up. Patients performing competitive sports prior to injury demonstrated an inferior long-term ACL survival of 56.4% (SE 11.6%). Patients performing recreational sport activities demonstrated a survival rate of 79.2% (SE 7.7%). The one factor demonstrating a direct influence on failure after adjustment was a high-pre-injury level of physical (odds ratio 4.0 confidence interval 1.0-15.8, p = 0.04). CONCLUSION: The minimum 5 years survival rate after primary ACL repair using this technique was 70%. This value dropped to 56% in highly active patients performing competitive sports. Patients not suffering failure of repair demonstrated adequate restoration of knee laxity and high satisfaction. This study not only underlines the potential of ACL repair, but also highlights the danger of the procedure if strict patient selection is not appreciated. LEVEL OF EVIDENCE: Level IV.

Extra-abdominal desmoid tumours - further evidence for the watchful waiting policy.

PURPOSE: Extra-abdominal desmoid tumours are benign and rare, and lead to a persistent treatment dilemma because of their high recurrence rate and their heterogeneous behaviour. The goal of this retrospective study was to evaluate the results of different treatment modalities for extra-abdominal desmoid tumours at four sarcoma treatment centres. METHODS: The mean follow-up time for the 96 patients included in the study (63.5% female; mean age 38.9 years) was 8.4 years (2.0–40.5 years). The initial treatments were surgery (n = 44), surgery with radiation (n = 16), watchful waiting (n = 15), radiation only (n = 9), or systemic treatment (n = 12). Patient demographics, tumour sites, and the follow-up status of all patients were reviewed and evaluated for each of the treatment modalities. RESULTS: The local recurrence rate was 45.5% in patients with primary surgical treatment and 37.5% following surgery combined with irradiation. Patients who were treated with radiation alone showed regressive (33.3%) or stable disease (66.6%). Systemic treatment alone resulted in disease progression in 41.7% of our patients. In the watchful waiting group, 73.3% showed stable disease, 20.0% showed spontaneous regression, and 6.7% showed progression after a mean follow-up of 4.1 years (2.0–11.5 years). CONCLUSIONS: Our results suggest that a watchful waiting approach should be the first line treatment in asymptomatic desmoid tumours. However, radiation can help improve local control rates in patients who have undergone surgery. Progression and local recurrence rates following systemic treatment were comparable to those observed in surgery combined with radiation.

Obesity and smoking predict the results of two-stage exchange in septic revision hip arthroplasty: A cohort study.

BACKGROUND: Prosthetic joint infection (PJI) is deemed to be the most serious complication following total hip arthroplasty. Obesity and smoking are known risk factors for PJI. However, the influence of these variables on infection free survival, of septic revision hip arthroplasty, is yet to be explored. The aim of this study was to determine the effect of obesity and smoking on the outcome of two-stage prosthetic exchange surgery. PATIENTS AND METHODS: A consecutive series of 97 hips in 94 patients (69 male, 25 female, mean age 66±12 years), undergoing two-stage revision surgery for hip PJI, were investigated retrospectively, after a mean follow-up of 60 (24-170) months. Survival was estimated using Kaplan-Meyer curves. A multivariate cox-regression model was applied to test for the influence of smoking or obesity (BMI≥30) after adjusting 16 potential patient-dependant variables. HYPOTHESIS: The study hypothesis was that smoking and high BMI are predictors for the failure of septic revision hip arthroplasty. Failure of septic revision hip arthroplasty was defined as failure to eradicate the infection or eradication of the infection but failure to preserve the arthroplasty. RESULTS: Kaplan-Meier showed a cumulative survival proportion of 80.4%(standard error S.E 4%), of the definitive implant, at 5 years. Obese patients (BMI≥30) and smokers had a significantly lower 5-year survival of 60.9% (S.E 1%) and 50.6% (S.E 1.4%), respectively (p=0.001). DISCUSSION: Obesity and smoking are both factors determining infection free survival in two-stage revision hip arthroplasty. Clinicians should be aware of potential complications and anticipate a higher likelihood of conversion to a Girdlestone resection or even amputation in this group of patients. LEVEL OF EVIDENCE: III, retrospective cohort study.

The Ganz acetabular reinforcement ring shows excellent long-term results when used as a primary implant: a retrospective analysis of two hundred and forty primary total hip arthroplasties with a minimum follow-up of twenty years.

PURPOSE: The acetabular reinforcement ring with a hook (ARRH) has been designed for acetabular total hip arthroplasty (THA) revision. Additionally, the ARRH offers several advantages when used as a primary implant especially in cases with altered acetabular morphology. The implant facilitates anatomic positioning by placing the hook around the teardrop and provides a homogenous base for cementing the polyethylene cup. Therefore, the implant has been widely used in primary total hip arthroplasty at our institution. The present study reports the long-term outcome of the ARRH after a minimum follow-up of 20 years. METHODS: Two hundred and ten patients with 240 primary THAs performed between April 1987 and December 1991 using the ARRH were retrospectively reviewed after a minimum follow-up of 20 years. Twenty-three of 240 hips were lost to follow-up, 110 patients with 124 THAs had deceased without having a revision surgery performed. This left 93 hips for final evaluation. Of those, 75 hips were assessed clinically and radiographically after a mean follow-up of 23.1 years (range 21.1-26.1 years). In 18 cases, clinical and radiographic assessment was omitted because implant revision had been performed prior to the follow-up investigation. The primary endpoint was defined as revision for aseptic loosening. RESULTS: Out of the 93 hips available for final evaluation, 14 hips were revised for aseptic loosening; another four were revised for other reasons (deep infection n = 2, recurrent dislocation n = 2). The survival probability of the cup was 0.96 (95% confidence interval 0.93-0.99) after 20 years with aseptic loosening as endpoint. Radiographic analysis of the surviving 75 hips showed at least one sign of radiographic loosening in 24 hips. The mean Merle d'Aubigne score increased from 8 points pre-operatively to 15 points at final follow-up (7.5 ± 1.8 vs 15.0 ± 2.3, p < 0.001). The mean HHS was 85 ± 14 at final follow-up. Radiographic loosening did not correlate with the clinical outcome. CONCLUSIONS: The long-term results of the ARRH in primary THA are comparable to results with standard cemented cups and modern cementless cups. We believe that the ARRH is a versatile implant for primary THA, especially in cases with limited acetabular coverage and altered acetabular bone stock where the ARRH provides sufficient structural support for a cemented cup.

Correction to: Reconstruction of AAOS type III and IV acetabular defects with the Ganz reinforcement ring: high failure in pelvic discontinuity.

The author would like to correct the errors in the publication of the original article. The corrected details are given below for your reading.

Reconstruction of AAOS type III and IV acetabular defects with the Ganz reinforcement ring: high failure in pelvic discontinuity.

BACKGROUND: Large acetabular defects and pelvic discontinuity represent complex problems in revision total hip arthroplasty. This study aimed to investigate whether reconstruction with the Ganz reinforcement ring would provide durable function in large acetabular defects. PATIENTS AND METHODS: 46 hips (45 patients, 19 male, 26 female, mean age 68 years) with AAOS type III and IV defects undergoing acetabular revision with the Ganz reinforcement ring were evaluated at a mean follow-up of 74 months (24-161 months). Fourteen patients died during follow-up. All surviving patients were available for clinical assessment and radiographic studies. Radiographs were evaluated for bone healing and component loosening. A Cox-regression model was performed to identify factors influencing survival of the Ganz-ring. RESULTS: In the group of AAOS III defects, 3 of 26 acetabular reconstructions failed, all due to aseptic loosening. In pelvic discontinuity (AAOS IV), 9 of 20 hips failed due to aseptic loosening (n = 4), deep infection (n = 3), and non-union of the pelvic ring (n = 2). With acetabular revision for any reason as an endpoint, the estimated Kaplan-Meier 5-year survival was 86% in type III defects and 57% in type IV defects, respectively. The presence of pelvic discontinuity was identified as the only independent predictive factor for failure of the Ganz ring acetabular reconstruction (AAOS III vs. IV, Hazard ratio: 0.217, 95%, Confidence interval: 0.054-0.880, p = 0.032). CONCLUSION: The Ganz reinforcement ring remains a favorable implant for combined segmental and cavitary defects. However, defects with pelvic discontinuity demonstrate high failure rates. The indications should therefore be narrowed to acetabular defects not associated with pelvic discontinuity.

Pleomorphic rhabdomyosarcoma with an impressive response to chemotherapy: case report and review of the literature.

PURPOSE: Pleomorphic rhabdomyosarcoma (RMS) represents a rare sarcoma subtype of the adult population. Due to its clinical characteristics, treatment is currently based on the guidelines for adult soft tissue sarcoma therapy. Hence, in the metastatic setting, doxorubicin-containing regimens are used in the sarcoma community, with limited treatment benefit. Scanty published data are available on the efficacy of systemic treatment. Whether treatment response and outcome of these patients could be improved by using pediatric protocols used typically in other RMS subtypes, like embryonal and alveolar RMS, is unclear. We report on an impressive effect of multiagent pediatric chemotherapy in an adult patient with metastatic pleomorphic RMS. METHODS: We present the case of a 70-year-old man with metastatic pleomorphic RMS of his left thigh. Systemic chemotherapy according to the VAC regimen (vincristine, actinomycin, cyclophosphamide) was initiated. Follow-up clinical and radiologic assessment demonstrated an impressive treatment response. RESULTS: Sixteen months after primary diagnosis, computed tomography scan shows no signs of tumor progression. CONCLUSIONS: Our case report emphasizes that multiagent systemic therapy according to pediatric protocols should be considered in adult patients with pleomorphic RMS.

Management of pelvic discontinuity in revision total hip arthroplasty: a review of the literature.

Pelvic discontinuity is a complex problem in revision total hip arthroplasty. Although rare, the incidence is likely to increase due to the ageing population and the increasing number of total hip arthroplasties being performed. The various surgical options available to solve this problem include plating, massive allografts, reconstruction rings, custom triflanged components and tantalum implants. However, the optimal solution remains controversial. None of the known methods completely solves the major obstacles associated with this problem, such as restoration of massive bone loss, implant failure in the short- and long-term and high complication rates. This review discusses the diagnosis, decision making, and treatment options of pelvic discontinuity in revision total hip arthroplasty.

Incorporation of RANKL promotes osteoclast formation and osteoclast activity on ß-TCP ceramics.

ß-Tricalcium phosphate (ß-TCP) ceramics are approved for the repair of osseous defects. In large defects, however, the substitution of the material by authentic bone is inadequate to provide sufficient long-term mechanical stability. We aimed to develop composites of ß-TCP ceramics and receptor activator of nuclear factor κ-B ligand (RANKL) to enhance the formation of osteoclasts and promote cell mediated calcium phosphate resorption. RANKL was adsorbed superficially onto ß-TCP ceramics or incorporated into a crystalline layer of calcium phosphate by the use of a co-precipitation technique. Murine osteoclast precursors were seeded onto the ceramics. After 15 days, the formation of osteoclasts was quantified cytologically and colorimetrically with tartrate-resistant acidic phosphatase (TRAP) staining and TRAP activity measurements, respectively. Additionally, the expression of transcripts encoding the osteoclast gene products cathepsin K, calcitonin receptor, and of the sodium/hydrogen exchanger NHA2 were quantified by real-time PCR. The activity of newly formed osteoclasts was evaluated by means of a calcium phosphate resorption assay. Superficially adsorbed RANKL did not induce the formation of osteoclasts on ß-TCP ceramics. When co-precipitated onto ß-TCP ceramics RANKL supported the formation of mature osteoclasts. The development of osteoclast lineage cells was further confirmed by the increased expression of cathepsin K, calcitonin receptor, and NHA2. Incorporated RANKL stimulated the cells to resorb crystalline calcium phosphate. Our in vitro study shows that RANKL incorporated into ß-TCP ceramics induces the formation of active, resorbing osteoclasts on the material surface. Once formed, osteoclasts mediate the release of RANKL thereby perpetuating their differentiation and activation. In vivo, the stimulation of osteoclast-mediated resorption may contribute to a coordinated sequence of material resorption and bone formation. Further in vivo studies are needed to confirm the current in vitro findings.

Pathomorphology and treatment of femoroacetabular impingement.

Femoroacetabular impingement is recognized as a major cause of hip pain and early hip joint osteoarthritis in young adults. The dynamic conflict between the femoral neck and the acetabular rim has been shown to result in labral tears, cartilage lesions, and early osteoarthritis. To be successful, the treatment strategy should address the underlying pathomorphology at the femoral neck, the acetabulum, or both. An overview of the various pathomorphologies leading to femoroacetabular impingement and a treatment algorithm intended to preserve the native hip joint should be helpful to the orthopaedic surgeon treating patients with this condition.

Incidence, predictive factors, and prognosis of chondrosarcoma in patients with Ollier disease and Maffucci syndrome: an international multicenter study of 161 patients.

BACKGROUND: Enchondromatosis is characterized by the presence of multiple benign cartilage lesions in bone. While Ollier disease is typified by multiple enchondromas, in Maffucci syndrome these are associated with hemangiomas. Studies evaluating the predictive value of clinical symptoms for development of secondary chondrosarcoma and prognosis are lacking. This multi-institute study evaluates the clinical characteristics of patients, to get better insight on behavior and prognosis of these diseases. METHOD: A retrospective study was conducted using clinical data of 144 Ollier and 17 Maffucci patients from 13 European centers and one national databank supplied by members of the European Musculoskeletal Oncology Society. RESULTS: Patients had multiple enchondromas in the hands and feet only (group I, 18%), in long bones including scapula and pelvis only (group II, 39%), and in both small and long/flat bones (group III, 43%), respectively. The overall incidence of chondrosarcoma thus far is 40%. In group I, only 4 patients (15%) developed chondrosarcoma, in contrast to 27 patients (43%) in group II and 26 patients (46%) in group III, respectively. The risk of developing chondrosarcoma is increased when enchondromas are located in the pelvis (odds ratio, 3.8; p = 0.00l). CONCLUSIONS: Overall incidence of development of chondrosarcoma is 40%, but may, due to age-dependency, increase when considered as a lifelong risk. Patients with enchondromas located in long bones or axial skeleton, especially the pelvis, have a seriously increased risk of developing chondrosarcoma, and are identified as the population that needs regular screening on early detection of malignant transformation.

Recurrent giant cell tumor of long bones: analysis of surgical management.

BACKGROUND: Treatment of giant cell tumor of bone (GCT) often is complicated by local recurrence. Intralesional curettage is the standard of care for primary GCTs. However, there is controversy whether intralesional curettage should be preferred over wide resection in recurrent GCTs. QUESTIONS/PURPOSES: We investigated the rerecurrence-free survival after surgical treatment of recurrent GCTs to determine the influence of the surgical approach, adjuvant treatment, local tumor presentation, and demographic factors on the risk of further recurrence. PATIENTS AND METHODS: We retrospectively reviewed the medical records of 46 patients with recurrent GCTs of long bones treated with wide resection or intralesional curettage and compared these cohorts. Recurrence rates, risk factors for recurrence, and the development of pulmonary metastases were determined. The minimum followup was 37 months (mean, 134 months; range, 37-337 months). RESULTS: The rate of rerecurrence after wide resection was 6%. Intralesional curettage showed an overall rerecurrence rate of 32%. Implantation of polymethylmethacrylate (PMMA) instead of bone grafting was associated with a lower risk of subsequent recurrence in intralesional procedures (14% versus 50%). Extracompartmental disease did not increase the risk of rerecurrence. Pulmonary metastases occurred in seven patients and appeared independent of the surgical treatment modality chosen. CONCLUSIONS: Intralesional curettage with methylmethacrylate for recurrent GCT provided equivalent tumor control compared with resection in this retrospective study. If joint salvage is possible, we advocate this treatment over resection in recurrent GCTs to preserve the native joint articulation. LEVEL OF EVIDENCE: Level III, therapeutic study. See Guidelines for Authors for a complete description of levels of evidence.