Gerhard Martin Hobusch

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: Limb salvage surgery is the preferred treatment for bone and soft tissue tumors. The Toronto Extremity Salvage Score (TESS) is a validated patient-reported outcome measure (PROM). However, its upper extremity section is outdated, lacking assessment of modern technology use. This study adapted TESS (creating TESS 2.0) by adding a question on touchscreen/keyboard use, based on an existing Italian version. Methods: Two independent translations of the new item were synthesized. Linguistic feedback was obtained from two German speakers from Germany and Switzerland. Pretesting with eight individuals refined the wording. Fourteen patients who underwent upper extremity surgery completed the TESS 2.0 twice (once in person, once at home) and the DASH questionnaire once. Reliability was assessed using Cronbach’s alpha and ICC, and validity was assessed using Spearman’s correlation between TESS 2.0 and DASH. Results: TESS 2.0 demonstrated excellent internal consistency (Cronbach’s alpha ≥ 0.98 at both time points). A strong, statistically significant inverse correlation existed between TESS 2.0 and DASH (r = −0.867, p < 0.001). Test–retest reliability was high (ICC = 0.98). Conclusions: German TESS 2.0 is a reliable and valid instrument for assessing upper extremity function in patients treated for bone and soft tissue tumors. Further research is needed to validate its use in postoperative follow-up and a larger, more diverse patient population.

Background: Osteosarcoma (OS) is the most common primary malignant bone tumor, mainly affecting adolescents and young adults. While lung metastases are common, visceral metastases in the hepatopancreatobiliary system are extremely rare and usually associated with a poor prognosis. The limited diagnostic and therapeutic options for such metastases make the treatment of affected patients difficult. The possibility of very late metastatic onset in high-grade OS highlights the potential need for extended follow-up (FU) beyond established intervals. Methods: This study combines a retrospective analysis of prospectively collected data from the Vienna Bone and Soft Tissue Tumor Registry with a review of the literature of patients with OS and metastases to the hepatopancreatobiliary system. A descriptive statistical analysis is presented for the entire cohort. In addition, publications from scientific databases (PubMed, Embase) were analyzed to evaluate the frequency, diagnosis, therapy, and prognosis of visceral metastasis from both conventional OS and primary extraskeletal osteosarcoma (ESOS). Results: A total of six male patients with conventional OS and metastases in the liver (5) and pancreas (1), with a mean lesion size of 38 mm (range, 10–120), were included. The median age at the time of visceral metastasis was 29 years (mean, 32 years; range, 20–62 years), and the mean interval since initial diagnosis was five years and ten months (range, 9 months–10 years and 9 months). Visceral metastases are very rare in general and usually occur in advanced stages of disease. We identified 51 cases of visceral metastases from conventional OS and 34 cases of ESOS in the hepatopancreatobiliary system in the literature. The metastasis interval was three years (range, 15 months before diagnosis–17 years) at a median age of 27 years (mean, 32 years; range, 10–69 years). Conclusions: Visceral metastases from OS are rare but represent a significant therapeutic challenge. Early, targeted imaging in combination with improved methods for diagnosis confirmation and interdisciplinary treatment strategies may potentially improve the results. This study underlines the importance of early diagnosis and highlights the need for individualized long-term surveillance strategies exceeding ten years, especially in high-grade OS, aiming at early detection of late-onset metastasis.

Background: Rotationplasty was first introduced as an alternative to above-the-knee amputation after resection of bone sarcomas of the distal femur by Salzer in 1974. Although the procedure involves a substantial body image issue, it has many advantages such as durability of the reconstruction (compared with limb salvage procedures) and avoidance of phantom pain (compared with amputation). Although several reports have shown intermediate outcomes of rotationplasty, very long-term results in terms of function, activity levels, and quality of life (QoL) in comparison with above-the-knee amputation have not been reported. This work aims to fill this gap left by prior reports. Questions/purposes: (1) Is there a difference in revision-free survival in very long-term follow-up after rotationplasty and transfemoral amputation or knee disarticulation? (2) Are patient activity levels after rotationplasty comparable with those after transfemoral amputation or knee disarticulation in the very long term? (3) Do activity levels differ in terms of QoL? (4) Within the group of patients who have undergone rotationplasty, is the ROM in the neo-knee associated with QoL 20 to 40 years later? Methods: Between 1961 and 1995, a total of 360 patients were treated for bone and soft tissue sarcoma of the lower extremity. Fifty-four patients were treated with A1 rotationplasty, 124 were treated with an amputation, and 182 were treated with a limb salvage procedure. Of those who underwent amputation or rotationplasty, 9% (11 of 124) and 15% (8 of 54), respectively, were lost to follow-up before a period of 20 years without meeting a study endpoint, and another 71% (88 of 124) and 44% (24 of 54), respectively, died prior to 20 years with intact residual limbs, leaving 20% (25 of 124) and 41% (22 of 54), respectively, of the original group who had a follow-up time of at least 20 years. Four patients with amputations declined to participate in the study, while three patients with transtibial amputations and one patient with a complete language barrier after rotationplasty were excluded. These 39 patients with a minimum follow-up time of 20 years (mean [range] 36 years [23 to 55]) were available and gave their consent to this retrospective comparative study at the local orthopaedic department. The decision between rotationplasty and endoprosthetic replacement was made after thorough consultation and according to the patient’s choice. As general guidance, Salzer’s idea was to provide rotationplasty to patients who had a strong desire for higher levels of activity. Endoprosthetic reconstructions were more likely indicated given a patient’s preference for a cosmetically uncompromised limb. Amputation was primarily performed one decade before the availability of rotationplasty and was mostly an alternative to rotationplasty because of patient refusal or surgical limitations. Therefore, the two groups differed in age at follow-up; however, they did not differ in age at surgery, BMI, gender ratio, tumor entities, or tumor localization. All-cause revisions presented in Kaplan-Meier curves, pain sensation, and functional and QoL outcomes, such as ROM, University of California Los Angeles (UCLA) activity scores, 5-level EuroQol 5-domain (EQ-5D-5L) scores, and 36-Item Short Form survey scores, were assessed. Results: There was no difference between patients after rotationplasty and amputation in terms of survivorship free from revision of unpredictable events at 20 years (86% [95% confidence interval (CI) 85% to 95%] versus 67% [95% CI 64% to 94%]; p = 0.27). There was no difference in median UCLA activity scores between the groups (rotationplasty 6 versus amputation 5; p = 0.18). Patients treated with a rotationplasty had less pain than those treated with amputation (EQ-5D-5L pain/discomfort, p < 0.01). The EuroQol (EQ) index was higher in patients who had rotationplasty in comparison with patients who underwent amputation (0.92 versus 0.81; p = 0.01). A linear regression model that controlled for length of follow-up, gender, age, and type of surgery found that having rotationplasty was associated with a better EQ index than undergoing amputation (R = 0.538, R² corrected = 0.212; p = 0.011). There were positive correlations between the EQ index and both flexion (ρ = 0.53 [95% CI 0.03 to 0.82]; p = 0.03) and ROM (ρ = 0.54 [95% CI 0.05 to 0.82]; p = 0.03) in the neo-knee. Conclusion: After rotationplasty and amputation, patients show similarities at long-term follow-up in the use of external prostheses and in cosmetic issues after limb loss. Both groups might have benefitted from the advancements in prosthetics that have occurred and will continue to do so; however, in this study, patients seem to have better QoL after rotationplasty compared with those with amputation. This study intentionally did not compare outcomes after an extendible or modular endoprosthesis with outcomes after rotationplasty. However, failures after endoprosthetic reconstructions occur frequently in the long term, whereas they rarely exist after rotationplasty. The use of rotationplasty, therefore, might be a benefit not only to individual patients but also to stakeholders in healthcare systems. Furthermore, this study emphasizes the benefit of rotationplasty as a durable surgical method that enables patients for high physical performance. Therefore, tumor centers around the world should be aware of these benefits, and patients must be given the opportunity to receive information about it. Currently, rotationplasty might be beneficial in certain situations, at least when patients and/or parents accept this kind of reconstruction. Children younger than 5 to 7 years and small in height, patients considered for megaprostheses with borderline or insufficient soft tissue coverage, and even patients who are highly active and feel that sporting activities are most important for their lifestyle are potential candidates for rotationplasty. Furthermore, rotationplasty might be an option after failed limb salvage surgery. Level of Evidence: Level III, therapeutic study.

Objective: This study focuses on how patients experience the time following amputation after primary limb salvage surgery due to musculoskeletal malignancies. Limb salvage is state of the art in the treatment of musculoskeletal tumours. Nonetheless, in some cases, limb salvage can become problematic over time, resulting in poorer limb function and septic outcomes. This raises the question of whether amputation is indicated sooner rather than later. Patients who have undergone secondary amputation might retrospectively prefer a different approach. Design: Interview study. Subjects/patients: Patients who underwent primary limb salvage surgery followed by later ablative sarcoma treatment. Methods: Semi-structured interviews and the standardized “Prosthetic Limb Users Survey of Mobility” questionnaire were conducted. Interviews were analysed according to Mayring content analysis method. Results: Amputation is perceived as an improvement after a long course of illness with little quality of life. By enhancing the amputation environment and providing detailed information regarding quality of life afterwards, emotional pressure could be reduced and patient satisfaction improved. Conclusion: Surgical options for ablation should be openly communicated earlier when consulting patients experiencing recurrent complications that might eventually lead to amputation. Supporting factors that subsequently may help to improve quality of life after amputation were further identified.

Objective: To describe and evaluate the combination of osseointegration and nerve transfers in 3 transhumeral amputees. Design: Case series. Patients: Three male patients with a unilateral traumatic transhumeral amputation. Methods: Patients received a combination of osseointegration and targeted muscle reinnervation surgery. Rehabilitation included graded weight training, range of motion exercises, biofeedback, table-top prosthesis training, and controlling the actual device. The impairment in daily life, health-related quality of life, and pain before and after the intervention was evaluated in these patients. Their shoulder range of motion, prosthesis embodiment, and function were documented at a 2- to 5-year follow-up. Results: All 3 patients attended rehabilitation and used their myoelectric prosthesis on a daily basis. Two patients had full shoulder range of motion with the prosthesis, while the other patient had 55° of abduction and 45° of anteversion. They became more independent in their daily life activities after the intervention and incorporated their prosthesis into their body scheme to a high extent. Conclusion: These results indicate that patients can benefit from the combined procedure. However, the patients’ perspective, risks of the surgical procedures, and the relatively long rehabilitation procedure need to be incorporated in the decision-making.

Summary: Background: A variety of treatment options for unicameral bone cysts (UBC) exist. The controversy of open management of UBC is discussed. The aim of this study was to analyze a single institution’s experience in the open surgical treatment of UBC. Patients and methods: By retrospective analysis of the Vienna Bone and Soft Tissue Tumor Registry, 119 patients with open surgery and histologically verified UBC with a mean follow up of 4.8 years (range 1–30 years) were included. Lesion treatment failure was defined as surgically addressed UBC undergoing revision surgery due to persistence or recurrence. Results: Local revision-free survival for lesion treatment failure was 93% after 1 year, 80% after 2 years, 60% after 5 years and 57% after 10 years. Of the patients 34 (29%) had at least 1 revision surgery due to lesion treatment failure. We found that patients with lesion treatment failure were younger (p = 0.03), had UBC with less minimal distance to the growth plate (p = 0.02) and more septation chambers in radiologic imaging (p = 0.02). Patients with open revision surgery were less likely to require a second revision due to lesion treatment failure than patients with percutaneous revision surgery (p = 0.03). Conclusion: Open surgery for UBC can only be recommended as reserve treatment in younger children with actively growing lesions. Open UBC surgery carries a relatively high risk of almost 30% of lesion treatment failure and therefore the indications should be limited to extensive osteolysis with high risk of pathological fractures, lesions with displaced pathological fractures, and lesions with an ambiguous radiological presentation that require tissue collection.

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.

Purpose: Little is known about the effect of sports activity levels on health-related quality of life (HRQOL) in long-term survivors of lower-extremity sarcoma. Methods: Eighty-three long-term survivors of bone and soft tissue sarcoma of the lower extremities with a median follow-up of 14 (range: 5–35) years completed the University of California and Los Angeles (UCLA) activity scores before tumor resection, 1 year after surgery and at the latest follow-up, as well as a Short Form 36 (SF-36) health survey at the latest follow-up. Simple linear regression models as well as stepwise variable selection with Akaike information criterion (AIC) were undertaken. Results: The preoperative UCLA activity level (median: 9, range: 2–10) dropped to a median of 4 (range: 1–10) 1 year after surgery before increasing to a score of 6 (range: 2–10) 5 years after surgery. The long-term SF-36 physical health component summary score (PCS) was 49 (SD: 9), and the mental health component summary score (MCS) was 54 (SD: 7). A linear model with stepwise variable selection identified a negative correlation of PCS with age at surgery (estimate: –0.2; p = 0.02), UCLA score at the last follow-up (estimate: 1.4; p = 0.02) and UCLA score 1 year after surgery (estimate: 1.0; p = 0.02). Conclusion: As not only the final activity levels but also the status immediately after surgery affect the PCS, higher early activity levels should be a goal of modern rehabilitation after sarcoma treatment. Further studies are needed to weigh the potential postoperative risks of higher sport activity levels against the benefits described in this study. Level of evidence: Level 4.

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.