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

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

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

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

Predicting medical complications in spine surgery: evaluation of a novel online risk calculator.

PURPOSE: The preoperative prediction of medical complications is essential to optimize perioperative management. SpineSage™ is a free of charge online calculator to predict medical complications in spine surgery. The current study utilizes it in patients undergoing spine surgery to assess whether the predicted risks would correlate with the actual complication rate in clinical practice. METHODS: A total of 273 consecutive patients who underwent spinal surgery were assessed. The risk of medical complications was predicted for each patient, and all medical complications were recorded within 30 days of surgery. Based on their predicted risk of complication, patients were divided into three risk groups (< 15, 15-30, > 30%). RESULTS: The predicted overall risk of medical complications was 14.7% and was comparable to the observed complication rate of 16.1%. The predicted risk for major medical complications (3.8%) was also similar to the observed complication rate (3.3%). Detailed analysis of the segmented risk groups suggests a close correlation between predicted and actual complication rates. Receiver operating characteristic analysis revealed an area under the curve of 0.71 (p < 0.001) for the prediction of overall medical complications and 0.85 (p < 0.001) for major complications. CONCLUSIONS: The online risk calculator predicted both overall and major medical complications. The tool can assist in preoperative planning and counseling of patients. These slides can be retrieved under Electronic Supplementary Material.

Is Total Femur Replacement a Reliable Treatment Option for Patients With Metastatic Carcinoma of the Femur?

BACKGROUND: The majority of metastatic bone lesions to the femoral bone can be treated without surgery or with minimally invasive intramedullary nailing. In rare patients with extensive metastatic disease to the femur, total femur replacement may be the only surgical alternative to amputation; however, little is known about this approach. QUESTIONS/PURPOSES: In a highly selected small group of patients with metastatic carcinoma of the femur, we asked: (1) What was the patient survivorship after this treatment? (2) What was the implant survivorship free from all-cause revision and amputation, and what complications were associated with this treatment? (3) What functional outcomes were achieved by patients after total femur replacement for this indication? METHODS: Eleven patients (three men, eight women) with a mean age of 64 years (range, 41-78 years) received total femur replacements between 1986 and 2016; none were lost to followup. The most common primary disease was breast cancer. In general, during this period, our indications for this procedure were extensive metastatic disease precluding internal fixation or isolated proximal or distal femur replacement, and an anticipated lifespan exceeding 6 months. Our contraindication for this procedure during this time was expected lifespan less than 6 months. Patient survival was assessed by Kaplan-Meier analysis; implant survival free from revision surgery and amputation were assessed by competing risk analysis. Function was determined preoperatively and 6 to 12 weeks postoperatively with the Musculoskeletal Tumor Society (MSTS) score normalized to a 100-point scale, with higher scores representing better function from a longitudinally maintained institutional database. RESULTS: Eleven patients died at a median of 5 months (range, 1-31 months) after surgery. One-year revision-free and limb survival were 82% (95% CI, 51%-98%) and 91% (95% CI, 61%-99%), respectively. Reasons for reoperation were hip dislocation, infection and local recurrence in one patient each. The latter two complications resulted in amputation in two patients. The median MSTS score was 32 (range, 13-57). CONCLUSIONS: Despite attempts to select patients who might have anticipated greater life expectancy, eight of 11 patients died by 6 months after surgery, and an additional two patients had undergone an amputation at 8 and at 17 months postoperatively. Most patients undergoing total femur replacement in this series did not recover from the procedure by the time they died, despite our best attempts to perform the procedure in patients whom we thought would live at least 6 months. Based on this, we believe that most patients with extensive metastatic disease to the femur should be offered palliative care, rather than major reconstruction. LEVEL OF EVIDENCE: Level IV, therapeutic study.

What Sport Activity Levels Are Achieved in Patients After Resection and Endoprosthetic Reconstruction for a Proximal Femur Bone Sarcoma?

BACKGROUND: Limited information is available about sports activities of survivors after resection and reconstruction of primary malignant bone tumors with megaprostheses. Because patients often ask what activities are possible after treatment, objective knowledge about sports activities is needed to help assess the risks of sports participation and to help guide patients' expectations. QUESTIONS/PURPOSES: The aims of this study were to evaluate (1) what proportion of patients with proximal-femoral megaprostheses placed as part of tumor reconstructions can perform sports; (2) what activity levels they achieved; and (3) whether sports activity levels are associated with an increased likelihood of revision. METHODS: This retrospective study considered all 27 living patients in our institutional tumor registry with enduring proximal-femoral reconstructions performed more than 5 years ago who were between the ages of 11 and 49 years at the time of the reconstruction; seven were lost to followup and one was excluded because of paraplegia as a result of a car accident and another because of senile dementia; another two were excluded from statistics because of growing prostheses and skeletal immaturity at the time of followup, leaving 16 (11 male, five female) for analysis. Their mean age was 26 ± 12 years (range, 11-49 years) at surgery, and the mean followup was 18 ± 7 years (range, 5-27 years). Types of sports, frequency per week, duration of each sports session as well as the UCLA and modified Weighted Activity Score were assessed retrospectively by an independent assessor a median of 18 years (range, 5.3-27 years) after surgery. RESULTS: Patients recalled that preoperatively 14 were practicing sports 5 (± 4) hours/week. At followup, 11 of the patients were practicing one or more sports activities 2 (± 3) hours/week on a regular basis. The preoperative UCLA and modified Weighted Activity Score levels of 9 and 6 fell to levels of 6 (p = 0.005) and 3 (p = 0.025), respectively, at followup. With the numbers of patients available for study, we could not determine that prosthetic failures were associated with sport activity levels. CONCLUSIONS: Patients who survive primary malignant bone tumors in the proximal femur reconstructed by megaprostheses are able to perform some sports activities. The estimates of activity levels made in this study probably are best-case estimates, given that some patients were lost to followup; patients unaccounted for might not be doing as well as those represented here. Also, the degree to which sports participation influences implant durability remains, for the most part, unanswered; studies with more patients and longer followup will be needed to determine to what degree prosthesis survivorship relates to sporting activity levels. Most patients perform low-impact sports and at a lower level than they had preoperatively. Because this is a preliminary study of a select group of patients, further information is necessary to weight the benefits of higher sports activity levels against potential risks. If this can be confirmed in a larger number of patients, the information may guide surgeons in their discussion with patients preoperatively and give them some objective assessment of what to expect regarding sports activities. LEVEL OF EVIDENCE: Level IV, therapeutic study.

Computer navigation-assisted surgery for musculoskeletal tumors: a closer look into the learning curve.

BACKGROUND: Computer navigation-assisted surgery for musculoskeletal tumors has shown to reduce the risk of intra-lesional margins in resection. Experiences with this method are still limited to smaller case series. METHOD: We reviewed our first experiences in 24 patients in whom computer navigation-assisted surgery had been performed. In 7 of these patients (6 male and 1 female), this has influenced the surgical treatment plan and navigation was used for both tumor resection and reconstruction. Three of the patients suffered from a chondrosarcoma, 2 from an osteosarcoma, 1 from a fibrosarcoma and 1 from an Ewing's sarcoma. Tumors were localized in the femur (n = 2), the tibia (n = 1), the sacrum (n = 1), the humerus (n = 1), the ilium (n = 1) and in the gluteal region (n = 1). RESULTS: The mean registration error was 0.9 mm. No intra-operative complications occurred. Two postoperative complications were observed which required revision surgery. Except for one marginal resection, all tumors were excised with wide margins. One patient suffered from a local recurrence, and one patient died of disease after distant metastatic dissemination. CONCLUSION: Computer navigation-assisted surgery represents a safe and helpful tool for the resection of musculoskeletal tumors and may influence surgical treatment plans in selected cases to provide more limited resections. Surgeons should be aware of risks considering biomechanical and oncological consequences in doing so. Further investigations and the evaluation of newer techniques (e.g., computed tomography-guided navigation) are needed to assess long-term outcomes of computer navigation assistance in musculoskeletal tumor surgery.

C-reactive protein as a prognostic factor in patients with chordoma of lumbar spine and sacrum--a single center pilot study.

STUDY DESIGN: This is a retrospective, diagnostic study, level IV. BACKGROUND: It appears to be necessary to identify prognostic markers for individual risk estimation for progression and survival in patients with chordoma, a rare disease. Are pre-operative serum levels of C-reactive protein (CRP) associated with disease progression and survival? METHODS: Survival rates of 24 patients (18 males, 6 females) (mean age 67 years (SD ± 16; range 20-85 years); minimum follow-up 2 years, mean follow-up 5 years (SD ± 5; range 2-19 years)) with chordoma of the lower spine and sacrum were assessed with a focus on pre-operative CRP levels. RESULTS: The survival rate of patients with pre-operative CRP level of >1.0 mg/dl was lower than that of patients with a CRP level <1.0 mg/dl (p = 0.01). The estimated 10-year survival of patients with pre-operative CRP values <1.0 and >1.0 mg/dl was 76 and 25%, respectively. CRP remained as an independent survival factor (p = 0.025; CI 95% 1.0-2.6) in multivariable analysis. CONCLUSIONS: Pre-operative CRP levels appear to be a biomarker for disease-specific survival in patients with chordoma of the lumbar spine and sacrum. A validation of our finding with larger cohorts and integration of putative risk factor would further elucidate CRP a surrogate for tumor progression.

Use of diagnostic dynamic contrast-enhanced (DCE)-MRI for targeting of soft tissue tumour biopsies at 3T: preliminary results.

OBJECTIVES: To test the feasibility and accuracy of MR-guided soft tissue tumour biopsy at 3T, using the dynamic contrast-enhanced (DCE) information from staging MRI for intralesional targeting. METHODS: After obtaining written informed consent for this institutional review board-approved study, 53 patients with suspected soft tissue tumours prospectively underwent preoperative staging MRI at 3T, including DCE, and subsequent MR-guided core needle biopsy. In 44/53 cases, DCE was heterogeneous and was used for intralesional biopsy targeting. Surgical, whole-specimen histology was used as the gold standard in 43/44 patients and revealed 42 soft tissue tumours (24 men; 18 women; mean age, 52 years; range, 19 - 84). RESULTS: Final surgical histology revealed eight benign lesions, six tumours of intermediate dignity, and 28 malignancies. All malignancies had shown heterogeneous DCE. The diagnostic yield of the biopsies was 100% (42/42). Histological accuracy rates of biopsy were 100% in predicting the dignity (42/42; 95% CI [0.916 - 1.000]), 95.2% for the tissue-specific entity (40/42; 95% CI [0.847 - 0.987]), and 90.5% for the tumour grade (38/42; 95% CI [0.779 - 0.962]). CONCLUSIONS: Our preliminary study indicates that biopsy of soft tissue tumours can be performed accurately and safely with DCE targeted MR-guidance at 3T, using a combined staging/biopsy MRI protocol. KEY POINTS: • MR-guided soft tissue tumour biopsy using DCE for intralesional targeting is feasible. • Targeting by staging-MRI allows reliable planning of the biopsy approach. • The method seems accurate and safe as a combined staging/biopsy procedure in outpatients. • DCE-targeted biopsy seems useful in challenging large and heterogeneous tumours.

Does competing risk analysis give useful information about endoprosthetic survival in extremity osteosarcoma?

BACKGROUND: Conventional survival analysis for endoprosthetic complications does not consider competing events adequately. Patients who die of their disease are no longer at risk for complications; therefore, death as a competing event may alter survivorship estimates in the orthopaedic-oncological setting. QUESTIONS/PURPOSES: This investigation aimed to compare (1) endoprosthetic survivorship after osteosarcoma by Kaplan-Meier analysis; and (2) by a competing risk model. METHODS: Between 1981 and 2009, we performed 247 modular endoprostheses for patients with extremity osteosarcoma; 73 patients had a followup of less than 2 years but all patients were included in statistical analysis. No patients were lost to followup for reasons other than death. Revision-free endoprosthetic survival until soft tissue failure (Type 1), aseptic loosening (Type 2), structural failure (Type 3), infection (Type 4), and local tumor progression (Type 5) was estimated according to a Kaplan-Meier analysis and a competing risk model. Sixty-four patients died throughout followup; the 5- and 10-year overall survival and metastasis-free survival were 72% and 70% and 70% and 69%, respectively. One hundred twenty-two patients (49%) had complications. RESULTS: Competing risk analysis consistently resulted in reduced estimates of the frequency of complications and reconstructive failures compared with Kaplan-Meier analysis. Cumulative risks for complication Types 1 to 5 at 10 years without/with death as a competing event revealed a risk of 19%/16% for Type 1, 26%/20% for Type 2, 51%/38% for Type 3, 23%/20% for Type 4, and 4%/3% for Type 5. CONCLUSIONS: A competing risk model reveals considerably reduced risks for every complication compared with Kaplan-Meier analysis when death is included as a competing event. Because it more realistically represents the risks of complications, competing risk models should be used to arrive at risk estimates for purposes of counseling patients about those risks associated with modular endoprosthetic reconstruction. LEVEL OF EVIDENCE: Level III, therapeutic study. See Guidelines for Authors for a complete description of levels of evidence.

What sports activity levels are achieved in patients with modular tumor endoprostheses of osteosarcoma about the knee?

BACKGROUND: Advances in multimodal treatment have improved survival of patients with nonmetastatic osteosarcoma. At the same time, implant design has improved the outcomes of limb salvage with modular endoprostheses. However, little is known about sports activity in long-term survivors with osteosarcoma. QUESTIONS/PURPOSES: We wanted to evaluate (1) sports activity levels in long-term survivors of osteosarcoma about the knee who received a modular tumor endoprosthesis; (2) to determine if activity level changed over time from initial reconstruction or (3) was predicted from sports activity level before diagnosis; and (4) if complications that occurred affected sports or contributed to prosthetic failures. METHODS: Between 1995 and 2005, we treated 120 patients for osteosarcoma about the knee with resection and modular endoprosthetic reconstruction; of those, 25 (21%) have died, six (5%) had an amputation, 39 (32%) did not speak German and so were ineligible, and 14 (12%) were either lost to followup or refused to participate, leaving 27 patients (14 females, 13 males; median age 19 years [range, 12-60 years); average followup 11 ± 4 years) (54% of the living, German-speaking cohort) for this analysis. Tumors were located in the distal femur (n = 16) and the proximal tibia (n = 11). Sports participation as well as the UCLA Activity Score and the modified Weighted Activity Score were assessed retrospectively. Moreover, postoperative complications were evaluated. RESULTS: Before the diagnosis of osteosarcoma and 1, 3, and 5 years and at the latest followup, respectively, after their reconstructions, 24 (89%), nine (33%), 20 (74%), and 24 patients (89%) were able to perform sports activities. There was a reduction in high-impact activities. Those patients with followup longer than 5 years had no changes in sports activity at their latest followup. Patients who had higher levels of sports activity levels before surgery generally had higher levels of activity at last followup (UCLA Activity Score: r = 0.62, p < 0.0005; modified Weighted Activity Score r = 0.49, p < 0.01). Fourteen patients (51%) underwent revision surgery. With the numbers available, complications had no effect on sports activity. No sports activity-related complications were found. CONCLUSIONS: Some long-term survivors of osteosarcoma can achieve high levels of sports activity. Preoperative activity levels seem to influence the postoperative activity levels. This information is important to give realistic expectations for long-term survivors of osteosarcoma of the knee. LEVEL OF EVIDENCE: Level IV, therapeutic study. See Guidelines for Authors for a complete description of levels of evidence.

Total Femur Replacement After Tumor Resection: Limb Salvage Usually Achieved But Complications and Failures are Common.

BACKGROUND: Primary bone or soft tissue tumors of the femur sometimes present with severe and extensive bone destruction, leaving few limb-salvage options other than total femur replacement. However, there are few data available regarding total femur replacement and, in particular, regarding implant failures. QUESTIONS/PURPOSES: We asked: (1) What are the revision-free and overall implant survival rates of conventional total femur replacements in patients treated for sarcoma of the femur or soft tissues? (2) What are the revision-free and overall implant survival rates of expandable total femur replacements in skeletally immature patients? (3) Using the comprehensive International Society of Limb Salvage failure-mode classification, what types of complications occur with conventional and expandable total femur replacements? PATIENTS AND METHODS: Our retrospective, single-center cohort study was based on data prospectively collected for 50 patients who received a total femur replacement after tumor resection for indications other than carcinoma or metastatic disease. Of the 50 patients, six (12%) were lost to followup before 6 months. Ten of the remaining 44 patients received expandable implants. The mean followup was 57 months (range, 1-280 months) and 172 months (range, 43-289 months) for patients who underwent conventional and expandable total femur replacements, respectively. For implant survival, competing risk analyses were used. RESULTS: At 5 years, revision-free implant survival of conventional total femur replacements was 48% (95% CI, 0.37-0.73), and overall implant survival was 97% (95% CI, 0.004-0.20). Five-year revision-free implant survival of expandable total femur replacements was 30% (95% CI, 0.47-1.00) and overall implant survival was 100%. With conventional total femur replacements soft tissue failures occurred in 13 of 34 patients, structural failures in three, infection in six, and local tumor progression in one. No patient had aseptic loosening with conventional total femur replacements, but hip disarticulation occurred in two patients owing to extensive wound-healing problems and infection. With expandable total femur replacements soft tissue failure, aseptic loosening, and infection occurred in one patient each of 10, and structural failures in three of 10 (two periprosthetic fractures, one loosening of an enhanced tendon anchor). No hip disarticulations were performed. Additionally expandable total femur replacement-related failures included hip instability in eight of 10 patients, contractures attributable to massive scar tissue in six, and defect of the implant's expansion mechanism in four patients. CONCLUSIONS: Although the indications for total femoral resection are rare, we think that total femur replacement is a reasonable treatment option for reconstruction of massive femoral bone defects after tumor resection in adults and skeletally immature patients, and results in limb salvage in most patients. LEVEL OF EVIDENCE: Level IV, therapeutic study.

Tumour response of osteosarcoma to neoadjuvant chemotherapy evaluated by magnetic resonance imaging as prognostic factor for outcome.

PURPOSE: This study evaluated the feasibility of computed magnetic resonance imaging (MRI) volumetry in conventional osteosarcomas. Secondly, we investigated whether computed volumetry provides new prognostic indicators for histological response of osteosarcomas after neoadjuvant chemotherapy. METHODS: In a retrospective cohort study, data from the Vienna Bone Tumour Registry was used. MR images from 14 patients (male:female = 1.8, mean age 19 years) were analysed prior to and after neoadjuvant chemotherapy according to current therapy regimens. Histological response to chemotherapy was graded according to the Salzer-Kuntschik classification. Computed volumetry was performed for the intraosseous part, as well as the soft-tissue component and the tumour as a whole. RESULTS: In a setting of appropriate radiological equipment, the method has been considered to be well implementable into clinical routine. The mean tumour volume prior to chemotherapy was 321 (±351) ml. In good responders (n = 6), overall tumour volume decreased by 47% (p = 0.345), whereas poor responders (n = 8) showed a 19% decrease (p = 0.128). Neoadjuvant multidrug therapy remarkably changed the tumour composition. This is seen in a decrease of the mean ratio of soft-tissue to intraosseous tumour volume from 8.67 in poor responders and 1.15 in good responders to 1.26 and 0.45 (p = 0.065), respectively. Interestingly, the bony compartment of good responders showed a volume increase during neoadjuvant chemotherapy (p = 0.073). However, we did not find prognostic markers for histological tumour response to pre-operative chemotherapy. CONCLUSIONS: Separated volumetry of tumour segments revealed interesting insights into therapy-induced growth patterns. If verified in a larger study population, these results should be taken into account when planning ablative surgery.

Initially localized synovial sarcoma in adults: a retrospective single-center analysis of 26 patients registered at the Department of Oncology, University of Vienna between 2004 and 2013.

BACKGROUND: Synovial sarcoma is a rare subgroup of all soft-tissue sarcomas. The aim of this retrospective single-center analysis was to investigate the outcome of patients with initially localized disease. PATIENTS AND METHODS: Twenty-six patients were enrolled in this retrospective single-center analysis. Baseline characteristics, treatment and outcome were evaluated. RESULTS: In 13 patients (50%), the tumor was located in the lower extremity and in 4 patients (15%) in the upper extremity. Surgical resection was done in all but 2 patients (92%). Re-resection was done in 7 patients (27%). Fourteen patients (54%) received adjuvant chemotherapy. After a median follow-up of 23.3 months (range: 2.6-150.3), median disease-free survival was not reached at the time of analysis. Eight patients (31%) relapsed after initial therapy. Surgery was done in 2 patients, amputation in 1 patient, palliative chemotherapy was administered in 3 and radiation therapy in 2 patients. Median overall survival (OS) for all patients was not reached at the time of analysis. The estimated 5-year OS rate was 62%. CONCLUSION: Patients with initially localized synovial sarcoma who were included in this retrospective single-center analysis have an estimated 5-year OS rate of 62%.

Distinctive outcome in patients with non-uterine and uterine leiomyosarcoma.

BACKGROUND: Leiomyosarcomas represent the largest subtype of soft tissue sarcomas. Two subgroups can be distinguished, non-uterine (NULMS) and uterine leiomyosarcomas (ULMS). The aim of this retrospective study was to evaluate differences in clinical features and outcome between these two subgroups. METHODS: Outcome and clinical-pathological parameters between 50 patients with NULMS and 45 patients with ULMS were assessed, and compared between both groups. Univariate and multivariable survival analyses were performed. RESULTS: Patients with ULMS presented with larger tumors when compared to patients with NULMS (p < 0.001). More patients with ULMS initially presented with metastatic disease (67% vs. 36%, p = 0.007). Most common metastatic site was lung for both subtypes (28% and 38%). Five-year overall survival (OS) rates of 82.6% and 41.2% and median OS times of 92.6 (range: 79.7-105.4) and 50.4 (range: 34.8-66.0) months were observed in patients with NULMS and ULMS, respectively (p = 0.006). In multivariate analysis, initial metastatic disease remained an independent prognostic factor in terms of OS (p < 0.0001). CONCLUSION: At time of diagnosis ULMS were larger and more often metastasized. Therefore patients with ULMS showed unfavorable outcome when compared to NULMS. Later diagnosis might be caused by differences in symptoms and clinical presentation or a more aggressive biological tumor behavior.

Primary malignant tumours of the scapula--a review of 29 cases.

PURPOSE: Primary malignant bone tumours of the scapula are very rare. Apart from limited small series and some case reports, international literature on flat bone sarcoma is exiguous and not much is known about the oncological outcome. METHODS: A retrospective analysis of 29 patients diagnosed with a primary malignant tumour of the scapula was performed. The mean age was 40 years. The average time of follow-up was 60 months. Diagnoses included chondrosarcoma in 11 patients, Ewing's sarcoma/PNET in six, osteosarcoma in three, and others in nine. RESULTS: Five patients (19%) had no surgery. Wide resection was performed in 18 patients (74%) and marginal and intralesional resection in three patients (13%) each. Fifteen patients underwent resection without bony reconstruction, seven patients underwent a Tikhoff-Linberg procedure, and two patients received a custom-made scapula prosthesis. Six patients were diagnosed with metastasis after a mean time of nine months after surgery, while three patients suffered local recurrence at an average of six months after surgery. Disease-specific survival was 70% at one year and 47% at five years. The mean MSTS score was 69% and was highest for patients with scapular prosthesis. CONCLUSION: The overall prognosis of primary malignant bone tumours of the scapula remains to be inferior compared to extremity sarcoma. Endoprosthetic reconstruction, however, shows promising functional results even in cases of total scapulectomy.

Incidence and management of hip dislocation in tumour patients with a modular prosthesis of the proximal femur.

PURPOSE: Little data is available about the incidence and especially the management of hip dislocation following the implantation of modular tumor prostheses of the proximal femur. In this retrospective single-centre study we assessed the incidence of hip dislocation following implantation of a proximal femoral modular prosthesis as well as the success of the subsequent surgical or non-surgical treatment in tumor patients. METHODS: Between 1982 and 2008, 166 tumor patients received a modular prosthesis of the proximal femur at our institution. The average age at the time of surgery was 50 ± 20 years (range, six to 84 years). An additional pelvic reconstruction was done in 14 patients. An artificial band for soft tissue reconstruction of the hip was used in 19 patients. The average time of follow-up was 46 ± 64 months (range, one to 277 months). RESULTS: The overall dislocation rate after proximal femoral replacement was 13% after a mean time of seven ± eight months (range, 0.3-33 months) after surgery. Between 1982 and 1986 the dislocation rate was 33% and declined to 9% in subsequent years (1987-2008). Patients who had received an additional pelvic reconstruction had a three fold higher dislocation rate (p <0.05). Patients with closed reduction had a 58% (eight of 12) re-dislocation rate compared to 11% (one of nine) for open reduction (p = 0.0357). CONCLUSIONS: Dislocation of a modular prosthesis of the proximal femur is a common complication, especially in cases with additional pelvic resection with extensive bone and soft-tissue defects. Open surgical management may be more effective in preventing re-dislocation than closed reduction and bracing alone.

Comparison of metal on metal versus polyethylene-ceramic bearing in uncemented total hip arthroplasty in patients with haemophilic arthropathy.

PURPOSE: We report the results of a consecutive series of 12 cases with haemophilic hip arthropathy treated with uncemented total hip arthroplasty (THA). Our hypothesis was that THA results in the haemophilic group would be inferior to those in the nonhaemophilic group. METHODS: The clinical histories of 12 consecutive THAs in eight patients (all men) with hereditary bleeding disorders (haemophilia A and B and von Willebrand disease) were reviewed retrospectively. The results were compared with an age- and sex-matched control group without haemophilia, with special emphasis on bearing surfaces (Metasul metal-on-metal; polyethylene-ceramic articulation). RESULTS: The mean follow-up of the control group was 9.7 (range five to 24) years and was similar to the haemophilia group, with 10.4. Survival in the Metasul haemophilic group was 22.2 % after 18 years, which significantly differed from the Metasul control group (100 % after 24 years). Survival of the polyethylene-ceramic haemophilic group was similar to the control group (100 % after seven years in both groups). CONCLUSIONS: The metal-on-metal bearing surface in patients with haemophilia gave inferior results compared with nonhaemophilic patients. The use of metal-on-metal bearings in haemophilia is debatable.

[Primary malignant bone tumors]. / Primär maligne Knochentumoren.

Primary malignant bone tumors are rare. Due to an associated worsening of the prognosis by diagnostic delay, these tumors must not be overlooked in the routine clinical practice and should therefore always be included in the differential diagnosis for the clarification of musculoskeletal complaints. A correct interpretation of the diagnostic procedure, radiological investigations and a biopsy of doubtful lesions can confirm the diagnosis. Osteosarcoma, chondrosarcoma and Ewing's sarcoma are the three most frequent primary malignant bone tumors and other entities occur only sporadically. While the prognosis of osteosarcoma and Ewing's sarcoma has been vastly improved with chemotherapy, chondrosarcomas mostly respond only poorly or not at all to systemic chemotherapy. Wide resection represents the gold standard in the surgical management of all primary malignant bone tumors. In addition, Ewing's sarcoma responds well to irradiation. The multidisciplinary management of primary malignant bone tumors should be performed at dedicated and specialized centers.

Patients' perception of changes and consequences after tumor resection : A qualitative study in Austrian patients with musculoskeletal malignancies.

OBJECTIVE: The aim of this study was to investigate the effects and consequences of surgical treatment of patients with musculoskeletal malignancies on everyday life. METHODS: A modified form of grounded theory was used for data collection and analysis. Data collection was systematic and analyzed simultaneously and 16 interviews were conducted: 2 narrative, 11 guided and 3 expert interviews (surgeon, physical therapist, support group). Data collection and analysis alternated until no new codes could be found. Once theoretical saturation was achieved, the main category was formed and described using the literature. RESULTS: The main category results from the combination of all categories and leads to the core category. In the center is the affected person and in the immediate environment are the patient's relatives/partners. In the next instance the primary care physician is necessary to establish a sense of normalcy. This depends on the individuality of the person and the restored possibilities of movement. CONCLUSION: Based on the results, the necessity of implementing psychosocial care involving the social environment is shown. The importance of relatives/partners for recovery is emphasized. Furthermore, the communication between the specialists and family physicians should be simplified.

Surgical treatment of the haemophilic pseudotumour: a single centre experience.

PURPOSE: Haemophilic pseudotumour was defined by Fernandez de Valderrama and Matthews as a progressive cystic swelling involving muscle, produced by recurrent haemorrhage into muscles adjacent to the bone. The pseudotumour mainly occurs in the long bones and the pelvis. The treatment of the haemophilic pseudotumour poses a challenge, and extensive clinical experience is essential to appropriately address this serious complication in patients with haemophilia. Consequently, the aim of this study is to present our own clinical experience and treatment results of the haemophilic pseudotumour. METHODS: We retrospectively reviewed the records of 87 patients with bleeding disorders treated between 1967 and 2011 for musculoskeletal complications of congenital bleeding disorders. We identified six patients with a haemophilic pseudotumour who were treated at our department. RESULTS: The mean age at surgery was 45.9 (range, 40-61) years. The iliac bone was affected in three patients (one right, two left), the right tibia (distal diaphysis) in one, the right thigh in two and the right ulna (proximal part) in one patient. One patient had two pseudotumours. The perioperative course was easily controllable with adequate factor VIII substitution. At the latest follow-up after 8.4 (range, 4-24) years, normal healing with no recurrence was observed. CONCLUSIONS: The haemophilic pseudotumour is a rare but severe complication of hereditary bleeding disorders. In the international literature the resection and postoperative course are described as challenging and difficult, requiring detailed preoperative planning. It is advisable to perform such operations in specialised centres with close co-operation between surgeons and haematologists.