Introduction:Angiosarcomas are very rare, malignant tumors of the endothelial lining of blood vessels and most present cutaneously, while only 10% present as deep soft tissue angiosarcomas. Literature is sparse regarding secondary deep soft tissue angiosarcoma, especially in cases that have formed surrounding a foreign body such as orthopedic implants. Case:A 59-year-old Caucasian male presented with a 1-year history of occasional headaches and non-intentional weight loss. One month prior to consult, he began to experience progressive pelvic pain on weight-bearing, up to 10/10 in severity, with associated swelling of the left leg. Further diagnostics revealed a hypervascular tumor on the anterior left hemipelvis, directly over his initial pelvic injury and orthopedic implants placed 33 years ago. Clinical Discussion:Biopsy revealed a high-grade angiosarcoma surrounding a stainless-steel 316L reconstruction plate adjacent to a titanium alloy (Ti-6Al-4 V) total hip arthroplasty. NextGen Genome sequence analysis done reveals tier II mutations (potential clinical significance) in the HOXB13 p.G84E and RPS6KB1 genes. Conclusion:Few published cases exist regarding secondary deep soft tissue angiosarcoma, and its peculiar presentation in our case arising at the juncture between two orthopedic implants of different compositions suggests a possible effect of galvanic corrosion, and warrants further study.

Background/Objectives: Cancer is the second leading cause of death, and spinal metastases may occur in up to 40% of patients with cancer. The purpose of the current study is to evaluate survival after surgical treatment of spinal metastases, and to identify risk factors that might shorten postoperative survival. Methods: Two-hundred sixty-eight patients who underwent surgery due to spinal metastases at a single center between 1990 and 2019 were evaluated retrospectively. Various variables and prognostic scores were analyzed to assess their impact on postoperative survival. Results: Two-hundred thirty-three patients (86.9%) died with a mean time from surgery to death of 14.84 months. Twelve months postoperatively, the survival rate of the entire cohort was 41.8%. Patients who were 65 years or older and were bedbound or in a wheelchair had a significantly shorter survival rate (p = 0.007). Further risk factors for a worse survival rate were: higher preoperative ASA-score (β = 2.19, 95% CI 1.34–3.57; p = 0.002), higher age at the time of surgery (β = 1.03, 95% CI 1.00–1.05; p = 0.041), presence of preoperative additional extraspinal bone metastases (β = 1.15, 95% CI 1.01–3.76; p = 0.047), ambulatory status (β = 1.21, 95% CI 1.04–1.4; p = 0.013), and elevated CRP-value > 5 mg/dL (β = 3.02, 95% CI 1.59–5.73; p = 0.001). Conclusions: Patients who are 65 years or older and bedbound or in a wheelchair had a significantly shorter survival period. When considering treatment options for patients with spinal metastases, a higher ASA-score, a higher age at the time of surgery, the presence of preoperative additional extraspinal bone metastases, being bedbound or in a wheelchair, and an elevated CRP-value > 5 mg/dL should be considered as significant risk factors for a worse survival rate. Available prognostic scores may only predict a patient’s survival accurately in the short-term follow-up.

Aims:The aim of the present study was to analyze the oncological and neurological outcome of patients undergoing interdisciplinary treatment for primary malignant bone and soft-tissue tumours of the spine within the last seven decades, and changes over time. Methods:We retrospectively analyzed our single-centre experience of prospectively collected data by querying our tumour registry (Medical University of Vienna). Therapeutic, pathological, and demographic variables were examined. Descriptive data are reported for the entire cohort. Kaplan-Meier analysis and multivariate Cox regression analysis were applied to evaluate survival rates and the influence of potential risk factors. Results:A total of 119 consecutive patients (mean age 38 years (SD 37; 1 to 83), mean follow-up 66 months (SD 26; 0 to 505) were investigated. Histological entities included Ewing's sarcoma (EWS; 33), chondrosarcoma (CSA; 20), osteosarcoma (OSA; 22), and soft-tissue sarcoma (STS; 44). Surgery was performed in 88 patients (74%). Neurological parameters improved in 18 patients (20%) after surgery. Overall, 32 patients (36%) suffered from surgical complications requiring revision. The median survival was 42 months (IQR 10 to 204). The one-, five-, and ten-year survival rates were 73%, 47%, and 39%, respectively. Corresponding five-year survival rates for EWS, CSA, OSA, and STS were 63%, 61%, 40%, and 32%, respectively. The decade of diagnosis, histological entity, surgical intervention, resection margin, and the presence of metastases had significant influence on survival. (Neo-)adjuvant therapies alone had no significant influence on overall survival. Conclusion:Our study clearly demonstrates the positive impact of improved surgical techniques, as well as refined imaging methods and evolved adjuvant therapy options, on survival rate in all tumour entities. However, despite a multimodal treatment plan, the long-term mortality of these tumours remains high.

Primär maligne Knochentumoren sind selten, dürfen aufgrund der damit verbundenen Prognoseverschlechterung im klinischen Alltag aber keinesfalls übersehen werden und müssen daher immer in die Differenzialdiagnose bei der Abklärung muskuloskeletaler Beschwerden einbezogen werden. Eine korrekte Interpretation der diagnostischen Verfahren sowie eine weiterführende Biopsie erlauben die Diagnosesicherung. Das Osteosarkom, das Chondrosarkom und das Ewing-Sarkom sind die 3 häufigsten primär malignen Knochentumoren, andere Läsionen treten nur sporadisch auf. Während beim Osteosarkom und Ewing-Sarkom die Prognose durch eine Chemotherapie erheblich verbessert wurde, spricht das Chondrosarkom auf eine Chemotherapie meist nur schlecht oder nicht an. Die weite Resektion ist Goldstandard in der chirurgischen Therapie aller primär malignen Knochentumoren. Das Ewing-Sarkom spricht überdies auf Bestrahlung an. Die interdisziplinäre Therapie von primär malignen Knochentumoren sollte an darauf spezialisierten Zentren erfolgen.

Summary 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.

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.

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.

Aneurysmal bone cysts (ABC) are benign tumors mostly occurring in children and young adults. Different open and minimal invasive surgical approaches have been proposed for the treatment of ABCs and yet no consensus is defined to date. The aim of this study was to retrospectively review data of a large single center series of ABCs with patients treated by open curettage with or without filling of the cavity or en-bloc resection. We asked: (1) What was the local recurrence rate of ABC after surgical treatment at our institution? (2) What were positive or negative predictors for local recurrence? (3) Was there a benefit from adjuvant burring, phenolization or filling, respectively? (4) Where there changes in recurrence free survival in different time periods of primary surgery? By retrospective data analysis of the Vienna Bone and Soft Tissue Tumor Registry, 123 patients surgically treated for primary aneurysmal bone cysts were identified. After exclusion of 33 patients (27%) due to a postoperative follow up below one year, 90 patients who were treated for primary ABCs between 1986 and 2009 were evaluated. These included 50 males and 40 females with a mean age of 16 years (SD 10 years; range: 2 to 51 years). The mean follow-up was 99 months. (SD 72 months, range: 13 to 329 months) Curettage was performed in 84 patients, while 45 patients received adjuvant phenolization. Local recurrence occurred in 28 patients after a mean time of 16 months, with a corresponding local recurrence free survival (RFS) of 83% after one year, 77% after 2 years and 66% after 5 years. ABCs located in hands and feet (p = 0.044) showed a superior RFS, while younger patients (p = 0.001) displayed an inferior RFS. Regarding adjuvant surgical techniques, mechanical cavity burring (p = 0.004) and filling with autologous cancellous bone graft (p = 0.024) showed protective effects on RFS. Patients treated between 1986 and 1999 (n = 47) had a higher RFS than patients treated between 2000 and 2009 (n = 43, p = 0.011), as surgeons and surgical indications changed over time. Although curettage, burring, phenolization and reconstruction with bone grafts came with a relatively high risk of local recurrence, open surgery is still justified in aggressively growing ABCs of critical localizations. IV; therapeutic study.

Background. - Aneurysmal bone cysts (ABC) are benign tumors mostly occurring in children and young adults. Different open and minimal invasive surgical approaches have been proposed for the treatment of ABCs and yet no consensus is defined to date. The aim of this study was to retrospectively review data of a large single center series of ABCs with patients treated by open curettage with or without filling of the cavity or en-bloc resection. Questions/purposes We asked: (1) What was the local recurrence rate of ABC after surgical treatment at our institution? (2) What were positive or negative predictors for local recurrence? (3) Was there a benefit from adjuvant burring, phenolization or filling, respectively? (4) Where there changes in recurrence free survival in different time periods of primary surgery? Methods. - By retrospective data analysis of the Vienna Bone and Soft Tissue Tumor Registry, 123 patients surgically treated for primary aneurysmal bone cysts were identified. After exclusion of 33 patients (27%) due to a postoperative follow up below one year, 90 patients who were treated for primary ABCs between 1986 and 2009 were evaluated. These included 50 males and 40 females with a mean age of 16 years (SD 10 years; range: 2 to 51 years). The mean follow-up was 99 months. (SD 72 months, range: 13 to 329 months)Results. - Curettage was performed in 84 patients, while 45 patients received adjuvant phenolization. Local recurrence occurred in 28 patients after a mean time of 16 months, with a corresponding local recurrence free survival (RFS) of 83% after one year, 77% after 2 years and 66% after 5 years. ABCs located in hands and feet (p = 0.044) showed a superior RFS, while younger patients (p = 0.001) displayed an inferior RFS. Regarding adjuvant surgical techniques, mechanical cavity burring (p = 0.004) and filling with autologous cancellous bone graft (p = 0.024) showed protective effects on RFS. Patients treated between 1986 and 1999 (n = 47) had a higher RFS than patients treated between 2000 and 2009 (n = 43, p = 0.011), as surgeons and surgical indications changed over time.Conclusion. - Although curettage, burring, phenolization and reconstruction with bone grafts came with a relatively high risk of local recurrence, open surgery is still justified in aggressively growing ABCs of critical localizations. Level of evidence. - IV; therapeutic study.(c) 2021 L'Auteur(s). Publie par Elsevier Masson SAS. Cet article est publie en Open Access sous licence CC BY (http://creativecommons.org/licenses/by/4.0/).

Core needle biopsy (CNB) is gaining in importance due to its advantages in the matter of patient morbidity, time and cost. Nevertheless, controversies still exist regarding the biopsy technique of choice for the accurate diagnosis of soft tissue sarcoma (STS). This retrospective cohort study compared the diagnostic performance between ultrasound-guided CNB and incisional biopsy (IB), both performed by orthopedic surgeons. The aims of the study were to answer the following questions: (1) Is ultrasound-guided CNB a highly reliable modality for diagnosing STSs? (2) Is CNB equally useful to IB for identifying histologic subtype? (3) Had patients who underwent CNB a reduced risk of complications? One-hundred and fifty-three patients who underwent resection of soft tissue sarcoma were classified into two groups according to biopsy technique prior to surgery; CNB group (n = 95) and IB group (n = 58). The final surgical specimens were in 40 patients liposarcoma (myxoid, pleomorphic and dedifferentiated), 39 undifferentiated pleomorphic sarcoma (UPS), 33 myxofibrosarcoma, 10 synovial sarcoma, 10 leiomyosarcoma and in the remaining 21 patients different soft tissue sarcoma entities. Sarcoma location of 71 patients was in the thigh, 19 in the lower leg, 22 in the upper arm and shoulder area; 10 in the knee and gluteal region, 9 in the thoracic region, the residual 12 in other body areas. Malignancy was correctly diagnosed in 87% (83 of 95) for the CNB group and 93% (54/58) for the IB group. Correct identification rate of histologic subtype was 80% (76 of 95) in the CNB group and 83% (48 of 58) in the IB group. There were no significant differences in the correct diagnosis rates of malignancy and subtype between the two techniques. No complications were seen in the CNB group, whereas 2 patients in whom IB was performed developed pulmonary embolism and 1 patient surgical site infection. Ultrasound-guided CNB is highly accurate and not inferior to IB in diagnosing the dignity of lesions and histologic subtype in patients with suspected STSs.

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.

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.

Fragestellung Die endoprothetische Rekonstruktion des gesamten Femurs stellt eine hochspezielle Operationsmethode dar, die prinzipiell für die Tumorendoprothetik entwickelt wurde. In Fällen mit hochgradigen, multiplen Komplikationen nach Hüft- und Knietotalendoprothesen stellt die totale Femurrekonstruktion häufig die einzige extremitätenerhaltende Versorgungsmethode dar. Neben der Möglichkeit der raschen Mobilisierung ist die Methode jedoch mit einem hohen Komplikationsrisiko behaftet.

BACKGROUND:There is scant evidence to guide decision-making for patients considering total femoral replacement (TFR). We aimed to identify the indication, patient, disease, and surgical technique-related factors associated with failure. We hypothesized that failure occurs more frequently in the setting of revision surgical procedures, with infection as the predominant failure mode. METHODS:We performed a retrospective cohort study of patients receiving total femoral endoprostheses for oncological and revision arthroplasty indications; 166 patients met these criteria. Our primary independent variable of interest was TFR for a revision indication (arthroplasty or limb salvage); the primary outcome was failure. Analyses were performed for patient variables (age, sex, diagnosis group, indication), implant variables (model, decade, length, materials), and treatment variables. We analyzed TFR failures with respect to patient factors, operative technique, and time to failure. We conducted bivariate logistic regressions predicting failure and used a multivariate model containing variables showing bivariate associations with failure. RESULTS:Forty-four patients (27%) had treatment failure. Failure occurred in 24 (23%) of 105 primary TFRs and in 20 (33%) of 61 revision TFRs; the difference was not significant (p = 0.134) in bivariate analysis but was significant (p = 0.044) in multivariate analysis. The mean age at the time of TFR was 37 years in the primary group and 51 years in the revision group (p = 0.0006). Of the patients who had mechanical failure, none had reoccurrence of their original failure mode, whereas all 8 patients from the nonmechanical cohort had reoccurrence of the original failure mode; this difference was significant (p = 0.0001). CONCLUSIONS:TFR has a high failure rate and a propensity for deep infection, especially in the setting of revision indications and prior infection. All failed TFRs performed for revision indications for infection or local recurrence failed by reoccurrence of the original failure mode and resulted in amputation. LEVEL OF EVIDENCE:Therapeutic Level IV. See Instructions for Authors for a complete description of levels of evidence.

Summary Background The aim of the study was to assess (1) sports activity, (2) sports involving the upper extremities, (3) functional outcome and (4) sports-related complications of long-term survivors of primary malignant bone tumors of the proximal humerus. Methods A total of 18 patients with an endoprosthetic reconstruction for primary malignant bone sarcoma of the proximal humerus (8 male, 10 female, mean age 19.9 ± 8.4 years, range 7.8–37.4 years) with an average follow-up of 18.1 ± 7.4 years (range 6.7–29.8 years) were included. The type of sport, frequency, duration of each sport session and the University of California, Los Angeles (UCLA) activity score were assessed before surgery, at 1 year, 3 years and at the latest follow-up. Functional outcome was assessed by the Toronto extremity salvage score (TESS). Results The mean UCLA activity score decreased from 8.0 (±1.3, range 5–9) preoperative to 4.2 (±1.7, range 3–8) at 1‑year follow-up (p < 0.05). After 3 years it increased to 5.1 (±1.75, range 3–8) and further to 7 (±1.8, range 4–9) at the last follow-up. The mean postoperative TESS was 80.8 (±6.4, range 75.7–91.4) at the latest follow-up. Patients who were initially more active without reconstruction including a synthetic mesh were more likely to develop soft tissue complications accompanied by proximal endoprothesis migration. Conclusion Patients with a modular endoprosthetic reconstruction of the humerus following primary bone sarcoma resume participation in sports. Regarding the low incidence of periprosthetic infections, utilization of a synthetic mesh for reconstruction to prevent soft tissue complications in active patients should be considered.

Aim Periprosthetic joint infections are a devastating complication after modular endoprosthetic reconstruction following resection of a musculoskeletal tumour. Due to long operating times, soft tis...