High fibrinogen levels are associated with poor survival in patients with liposarcoma.

The aim of this study was to evaluate whether (preoperative) plasma levels of fibrinogen, an essential clotting and acute phase protein, are associated with the prognosis of patients with a liposarcoma, a subtype of sarcoma derived from adipose tissue. We performed a retrospective cohort study of 158 patients with liposarcoma treated at the Department of Orthopaedics of the Medical University of Vienna in Austria from May 1994 to October 2021. Kaplan-Meier curves as well as uni- and multivariable Cox proportional hazard models were performed to evaluate the association between fibrinogen levels and overall survival. Elevated fibrinogen was associated with adverse overall survival in cause specific hazards analysis of mortality (hazard ratio [HR] per 10 mg/dL increase: 1.04; 95% CI 1.02-1.06; p < 0.001). This association prevailed in multivariable analysis after adjustment for AJCC tumor stage (HR 1.03; 95% CI 1.01-1.05; p = 0.013). Increasing levels of fibrinogen, a routinely available and inexpensive parameter, predicts the risk of mortality in patients with liposarcoma.

[Growing prostheses after sarcoma resection in children and adolescents]. / Wachstumsprothesen nach Sarkomresektionen im Kindes- und Jugendalter.

BACKGROUND: Growing prostheses are regarded as a valuable alternative to amputation and rotationplasty for the treatment of primary malignant bone and soft-tissue sarcomas in childhood. During the last three decades different devices have been introduced and technically improved from invasively to non-invasively extendable prostheses. THE CURRENT SITUATION OF STUDIES: Despite the long period, only 21 peer-reviewed publications could be detected containing relevant numbers and results. In these papers, 590 patients with mean follow-up times of 81.1 months were reported who had been fitted with growing prostheses at the age of 12.6 years. Besides satisfactory functional results (78.3 out of 100 MSTS points) there was a high complication rate of 27.3% infections and 22.4% mechanical failure. COMPLICATIONS: This increasing risk of infection over a long follow-up period, represents the biggest drawback of this method and, therefore, needs to be discussed extensively with the patients and parents when considering this procedure as an alternative to ablative surgery.

[General diagnostic work-up for benign tumors of the musculoskeletal system]. / Allgemeine diagnostische Grundlagen bei benignen Tumoren des Bewegungsapparats.

INTRODUCTION: Benign bone tumors are heterogeneous and have different biological behaviors. Treatment requires knowledge of the principle diagnosis and clinical behavior to avoid, on the one hand, overtreatment and, on the other hand, incorrect diagnosis of a potentially malignant tumor. METHODS: Bone tumors of stage I (according to Enneking) should be observed clinically and radiologically. For stage II and stage III lesions, a biopsy should be performed, based on the corresponding oncological guidelines. RESULTS AND CONCLUSION: Soft tissue tumors have a different radiological behavior (especially in magnetic resonance images): while a lack of sharp cortical margins in bone tumors indicates an aggressive behavior, sharp margins in soft tissue tumors should not be misinterpreted as benign tumors. Subfascial soft tissue tumors, tumors larger than 2 cm, growing tumors, and persisting tumors after trauma require biopsy.

The outcome of the surgical treatment of pelvic chondrosarcomas: a competing risk analysis of 58 tumours from a single centre.

AIMS: Few studies dealing with chondrosarcoma of the pelvis are currently available. Different data about the overall survival and prognostic factors have been published but without a detailed analysis of surgery-related complications. We aimed to analyse the outcome of a series of pelvic chondrosarcomas treated at a single institution, with particular attention to the prognostic factors. Based on a competing risk model, our objective was to identify risk factors for the development of complications. PATIENTS AND METHODS: In a retrospective single-centre study, 58 chondrosarcomas (26 patients alive, 32 patients dead) of the pelvis were reviewed. The mean follow-up was 13 years (one week to 23.1 years). RESULTS: A total of 26 patients (45%) were alive and 32 patients (55%) had died. Overall survival was 76%, 55% and 45% at one, five and ten years post-operatively, respectively. In a competing risk model the cumulative risk of the development of a surgery-related complication was 64% at six months and 69% at one year, post-operatively, respectively. Endoprosthetic reconstruction was a significant risk factor for the development of complications (p = 0.006). Complications were not significantly related to age or the location or grade of the tumour (p = 0.823, p = 0.976, p = 0.858). The development of complications did not have a negative effect on survival (p = 0.147). CONCLUSION: This is the first study with competing risk analysis of surgery-related complications in patients with a pelvic chondrosarcoma. The surgery in these patients remains prone to complications. Endoprosthetic reconstruction significantly increases the risk of the development of complications (p = 0.006). A competing risk model showed that the development of complications does not have a negative influence on overall survival (p = 0.147). An aggressive, surgical resection with the goal of achieving wide margins whenever possible remains the mainstay of treatment. Cite this article: Bone Joint J 2017;99-B:686-96.

Survival, failure modes and function of combined distal femur and proximal tibia reconstruction following tumor resection.

BACKGROUND: Tumor spread to the knee joint or skip metastasis to the adjacent bones of the knee require reconstruction with combined distal femur and proximal tibia replacements. The literature on implant survival and failure modes with this type of reconstruction is sparse. The goals of this study were to determine the implant survival, the different failure modes and the functional outcome of this megaendoprosthetic reconstruction. PATIENTS AND METHODS: Thirty-nine patients with combined distal femur and proximal tibia reconstruction were retrospectively reviewed. Median follow-up was 8.8 years (quartiles 4.7-15.5 years). Twenty-one patients received combined distal femur and proximal tibia reconstruction as a primary mode of reconstruction, 18 patients as revision surgery after failed tumor prosthesis. For survival estimations, competing risk analyses were performed. RESULTS: The revision-free survival at five years was 42% (95% CI 22%-56%) and implant survival with exchange of the original implant was 54% (95% CI 35%-68%). Five-year revision-free survival for soft tissue failure was 72% (95% CI 52%-84%), for infection 67% (95% CI 48%-80%), for structural failure 82% (95% CI 63%-91%), for aseptic loosening and tumor progression 97% (95% CI 82%-99%), respectively. Patients with revision surgery had higher risk for infection (p < 0.001), structural failure (p = 0.037) and shorter revision-free- (p = 0.025) and implant-survival (p = 0.006). Limb survival at 20 years was 94%. Mean musculoskeletal Tumor Society score was 76%. CONCLUSION: Despite high failure rates with short revision-free survivals, combined distal femur and proximal tibia reconstruction achieved longtime limb survival in the majority of patients with satisfying function.

Long-term outcome following treatment of Adamantinoma and Osteofibrous dysplasia of long bones.

INTRODUCTION: Adamantinoma (AD) is an ultimately rare, low-grade malignant bone tumor. In most cases it occurs in the tibia of young adults. Osteofibrous dysplasia (OFD) is a rare, benign, lesion that is typically seen in children. Histopathology, ultrastructure, and cytogenetics indicate that these lesions are closely related. Yet, etiology remains a matter of debate. Local recurrence rates are high for both entities as published in literature and long-term outcomes are scarce, due to the rarity of the disease. HYPOTHESIS: AD should be treated by En-Bloc resection while ODF can be treated by curettage or by observation. Consequently, the aim of the present study was to answer following questions: Were local recurrence rates of both entities different based on a retrospective review within a tertiary referral center for orthopedic oncology? MATERIAL AND METHODS: In a retrospective cohort study, 10 patients with AD and 5 patients with OFD (including 1 patient with OFD-like-AD) were reviewed. Primary surgeries for patients with AD were: En-bloc resection in 7, curettage in 2 and amputation in 1. In the OFD group, only 2 patients underwent surgery by curettage. Mean follow-up was 16 years (range: 2-47 years). Nine patients had a minimum follow-up of 10 years (mean: 23 years; range: 10-47 years). RESULTS: Four patients with AD (40%) and 2 patients with OFD (40%) - all of them following surgical removal - suffered from local recurrence. In the "En bloc" resection group of AD, there were 2 LR (29%). All patients of both groups treated with curettage showed LR. One patient with AD had metastasis at time of diagnosis and died of disease. Another patient with AD was diagnosed with metastasis 67 months after surgery and was still alive with disease at latest follow-up (77 month). DISCUSSION: The overall prognosis of AD and OFD is good, yet local recurrence rates are high, irrespective of surgical strategy. While an internationally standardized treatment regime is still missing, a more radical surgical approach should be considered, especially when treating AD. LEVEL OF EVIDENCE: Retrospective study; Level IV.

Complete local tumor control after curettage of chondroblastoma-a retrospective analysis.

BACKGROUND: Chondroblastoma is an uncommon benign bone tumor with an incidence of 1 to 2% among all primary bone tumors. In the past, treatment for chondroblastoma has been highly variable leading to different rates of recurrences. Therefore we aimed to determine: (1) the rate of recurrence, (2) the complication rate, (3) and functional outcome after intralesional curettage of chondroblastoma. HYPOTHESES: Intralesional curettage with high speed burring and packing can avoid local recurrences. PATIENTS AND METHODS: Experiences of 22 patients with chondroblastoma of the bone were retrospectively reviewed. The patient group consisted of 16 men; 6 women; mean age 24years (range; 12-58years) affecting in 15 the lower- (68%) and in seven the upper extremity (32%). RESULTS: There was no local recurrence or malignant transformation. All patients underwent intralesional curettage, followed by defect filling presenting in 19 patients (87%) excellent clinical and oncological results (mean MSTS 98.9). Complications were seen in two patients. Pain was the main revealing symptom of the chondroblastoma (n=16, 73%). Mean follow-up of all patients was 114months (range, 25 to 480months). DISCUSSION: Aggressive curettage and packing provided excellent local tumor control and functional results in our patients with chondroblastoma. Malignant transformation is extremely rare, however, present in literature but was not seen in any of our patients. LEVEL OF EVIDENCE: Level IV, retrospective study.

Vascularised or non-vascularised autologous fibular grafting for the reconstruction of a diaphyseal bone defect after resection of a musculoskeletal tumour.

Resection of a primary sarcoma of the diaphysis of a long bone creates a large defect. The biological options for reconstruction include the use of a vascularised and non-vascularised fibular autograft. The purpose of the present study was to compare these methods of reconstruction. Between 1985 and 2007, 53 patients (26 male and 27 female) underwent biological reconstruction of a diaphyseal defect after resection of a primary sarcoma. Their mean age was 20.7 years (3.6 to 62.4). Of these, 26 (49 %) had a vascularised and 27 (51 %) a non-vascularised fibular autograft. Either method could have been used for any patient in the study. The mean follow-up was 52 months (12 to 259). Oncological, surgical and functional outcome were evaluated. Kaplan-Meier analysis was performed for graft survival with major complication as the end point. At final follow-up, eight patients had died of disease. Primary union was achieved in 40 patients (75%); 22 (42%) with a vascularised fibular autograft and 18 (34%) a non-vascularised (p = 0.167). A total of 32 patients (60%) required revision surgery. Kaplan-Meier analysis revealed a mean survival without complication of 36 months (0.06 to 107.3, sd 9) for the vascularised group and 88 months (0.33 to 163.9, sd 16) for the non-vascularised group (p = 0.035). Both groups seem to be reliable biological methods of reconstructing a diaphyseal bone defect. Vascularised autografts require more revisions mainly due to problems with wound healing in distal sites of tumour, such as the foot.

[Soft tissue sarcoma]. / Weichteilsarkome.

Soft tissue sarcomas are rare tumours of the connective tissue. The knowledge of this rare entity is necessary for an accurate diagnosis. The incidence in Europe is about 3,6/100 000 per year. The first line treatment consists of a wide resection of the tumour, possibly combined with an adjuvant radiation therapy. A chemotherapy can be indicated and has to be evaluated for each case individually. A biopsy should be done before any treatment and in accordance with the oncological resection approach. The management of the soft tissue sarcoma has to be carried out by a specialist sarcoma multidisciplinary team.

Clinical signs of neurofibromatosis impact on the outcome of malignant peripheral nerve sheath tumors.

OBJECTIVE: Malignant peripheral nerve sheath tumors (MPNST) are a rare subtype of sarcoma, with a poor outcome. MPNST are regarded as being sporadic or associated with neurofibromatosis type 1 (NF1). Few comparative overall-survival (OS) data in these 2 subsets of MPNST patients exist. The aim of this retrospective study was to assess OS in sporadic and NF1-associated MPNST patients. METHODS: Fourteen consecutive patients with initial localized as well as initial metastatic MPNST were diagnosed and treated in our department. Patients with sporadic MPNST were assigned to group A and those with NF1-associated MPNST to group B. RESULTS: Eight versus 6 patients were allocated to groups A and B. Primary tumors were located on the extremities in all but 1 patient. Two patients in group A and 4 patients in group B experienced a relapse. Four patients died in each of the 2 groups. Median follow-up was 66.2 and 57.2 months in group A and group B, respectively. Median OS in group A was 46.9 months versus 12.7 months in group B. CONCLUSIONS: In this small, single-center study, sporadic-MPNST patients had a longer median OS than those with NF1-associated MPNST.

Radio-lunate arthrodesis in rheumatoid arthritis: outcome and techniques.

BACKGROUND AND PURPOSE: The wrist is one of the most affected joints in rheumatoid arthritis. The purpose of this retrospective study was to assess clinical, functional and radiographic results of radio-lunate arthrodesis. Two different operation and fixation techniques are compared and detailed outcome after this intervention is presented. METHODS: Twenty-seven patients with long-standing rheumatoid arthritis were operated on, either by stabilisation of the arthrodesis with Shapiro staples (n = 14) or by Herbert screw (n = 13) and followed for a mean of 5.4 years. RESULTS: Radio-lunate arthrodesis resulted in high overall and subjective satisfaction concerning function, grip and return to work. Grip strength was 35 kPa for the dominant and 26 kPa for the non-dominant hand. No revision, pseudoarthrosis or hardware failure was observed; only two conservatively treated wound healing problems were reported. The procedure resulted in a mean flexion of 26° and a mean extension of 24°; a clear improvement was also seen in activities of daily life. No difference between both groups was observed for pain, complication rate or functional outcome. INTERPRETATION: Due to high patient satisfaction and functional outcome, radio-lunate wrist arthrodesis can be recommended independent of fixation method.

Age predicts outcome of high-tibial osteotomy.

This study compares the predictive value of age at surgery in high tibial osteotomy. Twenty-seven high-tibial osteotomies in patients who are 65 years or older (mean age at surgery 68+/-4 years, follow up 12+/-2 years) were compared to 67 osteotomies in patients younger than 65 years (mean age at surgery 56+/-6 years, follow up 13+/-3) with respect to the outcome by Cox regression analysis. Failure, i.e. endpoint, was defined as implantation of a knee endoprosthesis and assessed by Kaplan-Meier analysis. There is a significantly higher risk for failure of high tibial osteotomies in patients of 65 years or more compared to younger patients (failure rate 38.4+/-11.3% vs. 23.1+/-5.8%) resulting in a relative risk of 1.5 (P=0.0461). The hazard of failure increased 7.6% per year of age. We conclude that in regard to the increasing risk of failure per year of age and the higher failure rate in older patients, high-tibial osteotomy should not be performed on patients older than 65 years.

Outcome of surgical concepts in haemophilic arthropathy of the hindfoot.

The ankle is one of the most frequent joint affected by haemophilia, in the second decade of life it can be considered as the most common site for haemophilic arthropathy. The aim of this study is to evaluate the results of 15 surgical interventions of the hindfoot performed on 11 patients. Twenty-seven of 70 male patients suffering from haemophilia were treated for haemophilic arthropathy of the hindfoot. We performed 15 operations on 11 patients with a mean age of 33.5 (range: 10-53) years. The surgical treatment consisted of 15 operations, 12 of which concerned the talocrural joint (six synovectomies, three radiosynoviorthesis, four arthrodeses, one achilles tendon lengthening) and one the talocalcaneonavicular joint (one triplearthrodesis). The arthrodeses of the talocrural joint were undertaken by screw, the triplearthrodesis by clamp fixation. The synovectomies were all late synovectomies and performed by open means. The mean follow-up period was 32.0 (range: 12-84) months. The mean age at surgery was 29.6 (range: 8-51) years. No perioperative complications were registered when adequate replacement therapy was carried out. The consolidation of the arthrodeses was accomplished within 8-12 weeks. All synovectomies stopped or reduced significantly recurrent joint bleeding. With both procedures we achieved pain relief and walking ability improvement. The aim of synovectomy is to reduce bleeding episodes. If synovectomy can halt the progression of the haemophilic arthropathy is controversially discussed, especially when surgery was carried out in the presence of an established arthropathy. If synovectomy fails, the arthrodesis proves to be a good treatment option.

Outcome of osteotomies for the treatment of haemophilic arthropathy of the knee.

BACKGROUND AND AIMS: The knee is one of the most commonly affected joints in haemophilic arthropathy leading to stiffness and disability. It is the aim of this study to investigate the outcome of corrective osteotomies around the knee. PATIENTS AND METHODS: We report on the long-term results of ten osteotomies around the knee for severe haemophilic arthropathy and axial deviation at an average of 7.25+/-1.8 years postoperatively. Seven high tibial (preoperatively 7.2+/-2 degrees varus) and three supracondylar osteotomies (preoperatively 7+/-3 degrees valgus) were performed on seven patients (three of them bilateral). RESULTS: The clinical score of the Advisory Board of the World Federation of Haemophilia (average 7.4 points preoperatively) remained unchanged in two patients, improved in three patients and deteriorated in five patients. The radiological Pettersson score (average 8.2 points preoperatively) showed a worsening of 2.5 points over the time. Patients reported a subjective improvement for seven osteotomies, with sports activity in three patients, although the range of motion did not change significantly. Total knee arthroplasty was considered to be a failure, i.e. endpoint of follow-up. Six knees were replaced in four patients by total arthroplasty after a mean of 6.6 years. CONCLUSION: Although survival of osteotomies around the knee in haemophilic arthropathy is lower than in non-haemophilic patients, we think that it is a choice of treatment, which, at least, postpones the indication for total knee arthroplasty in this young patient group.