Regionale neoadjuvant intendierte isolierte Extremitätenperfusion mit TNF-alpha und Melphalan zur Therapie primär nichtresektabler hochmaligner Weichgewebesarkome. Ziel ist die Größenreduktion und Devitalisierung des Tumors, um einen primär nichtresektablen Tumor in einen resektablen Zustand zu überführen. Primär nichtresektable (Indikation zur Amputation oder höhergradig mutilierenden Resektion), hochmaligne Weichgewebesarkome der Extremitäten. Gefäßverschlüsse, Thrombosen, akute Infektionen, insbesondere der betroffenen Extremität. Gefäßzugang zu Arterie und Vene proximal an der betroffenen Extremität. Arterielle und venöse Kanülierung der die Extremität und den Tumor versorgenden Gefäße. Anschluss an Herz-Lungen-Maschine. Anlage einer Blutsperremanschette oder eines elastischen Tourniquets proximal der Katheterspitzen. Nuklearmedizinische Leckratenmessung (Technetium 99m) zum Ausschluss eines systemischen Lecks. Perfusion der Extremität mit 1–2 mg rekombinantem TNF-alpha (Tasonermin/Beromun, Belpharma SA, Luxembourg) für 15 min, anschließend Hinzugeben von 11–13 mg Melphalan pro Liter Extremitätenvolumen und Fortsetzen der Perfusion für weitere 60 min. Auswaschen mit 2–5 l kristalloider Lösung, während die Extremität mit elastischen Esmarch-Bandagen mehrfach ausgewickelt wird. Entfernen der Blutsperre und der Katheter, Rekonstruktion der Gefäße, Wundverschluss. Hochlagern und Kühlen der Extremität (insbesondere Unterarm und Unterschenkel). Engmaschiges kardiozirkulatorisches und klinisches Monitoring bei bestehendem Risiko für ein TNF-alpha-induziertes SIRS und ein Kompartmentsyndrom (Auftreten innerhalb der ersten 24 h nach ILP). Vollbelastung der Extremität möglich. Fortsetzen der Lagerungstherapie abhängig vom Schwellungszustand. Krankenhausaufenthalt ca. 1 Woche. Gesamttherapieansprechen auf TM-ILP: 60–70

OBJECTIVE:Regional neoadjuvant isolated limb perfusion (ILP) with TNF-alpha and melphalan (TM-ILP) for the treatment of primarily unresectable highly malignant soft tissue sarcomas. The goal is to reduce the size and devitalize the tumor in order to convert a primarily unresectable tumor into a resectable state. INDICATIONS:Primarily nonresectable (indication for amputation or higher-grade mutilating resection), highly malignant soft tissue sarcomas of the extremities. CONTRAINDICATIONS:Vascular occlusions, thromboses, acute infections, especially of the affected extremity. SURGICAL TECHNIQUE:Vascular access to the artery and vein proximal to the affected limb. Arterial and venous cannulation of the vessels supplying the limb and tumor. Connection to a heart-lung machine. Application of a tourniquet or elastic bandage proximal to the catheter tips. Nuclear medicine leak rate measurement (technetium 99m) to rule out a systemic leak. Perfusion of the limb with 1-2 mg recombinant TNF-alpha (Tasonermin/Beromun, Belpharma SA, Luxembourg) for 15 min, followed by the addition of 11-13 mg melphalan per liter of limb volume and subsequent perfusion for an additional 60 min. Washing out with 2-5 l of crystalloid solution while wrapping the limb several times with elastic Esmarch bandages. Removal of the tourniquet and catheters, reconstruction of the vessels, wound closure. POSTOPERATIVE MANAGEMENT:Elevate and cool the limb (especially the forearm and lower leg). Close cardiovascular and clinical monitoring for existing risk of TNF-alpha-induced Septic Inflammatory Response Syndrome (SIRS) and compartment syndrome (occurring within the first 24 h after ILP). Full weight-bearing on the limb is possible. Continue elevated positioning therapy depending on the degree of swelling. The hospital stay is approximately 1 week. RESULTS:Overall treatment response to TM-ILP: 60-70%. Complete remissions observed in just under 20% of cases. Limb preservation is possible in over 80% of cases.

BACKGROUND:Primary malignant bone tumors are rare, biologically heterogeneous malignancies that require highly specialized, multidisciplinary treatment. Surgical excision of the primary tumor is considered a prerequisite for long-term survival in most affected patients. An association between hospital volume and outcome has been demonstrated for numerous oncological procedures but has been investigated less extensively in the context of bone sarcomas. MAIN FINDINGS:The surgical expertise concentrated at high-volume centers (HVC) appears to translate directly into superior oncologic outcomes and a better prognosis. Key contributing factors include significantly lower rates of positive surgical margins and significantly reduced perioperative mortality. Furthermore, specialized surgical expertise enables limb-sparing procedures even in most complex cases. UNRESOLVED QUESTIONS:The definition of HVC has been inconsistent so far. Furthermore, centralized treatment in HVC requires the establishment of clear referral pathways and optimal cooperation between specialized centers and the wider healthcare system. CONCLUSIONS:The surgical treatment of primary malignant bone tumors should be centralized in specialized, high-volume sarcoma centers. In this context, hospital volume should also be understood as a proxy for specialized multidisciplinary expertise, adherence to clinical practice guidelines, and systematic quality improvement, which are all essential factors for the optimal treatment of bone sarcomas. Further studies are necessary to better define the term HVC in different healthcare systems and settings.

Background: Soft tissue sarcomas (STS) in children, adolescents, and young adults are rare and biologically heterogeneous, and prognostic factors for local recurrence, metastasis, and survival remain incompletely defined. This study aimed to characterize oncological outcomes and to explore clinical, surgical, and treatment-related factors associated with these endpoints at a tertiary sarcoma center. Methods: This retrospective single-center cohort study included 42 consecutive patients aged ≤25 years with histologically confirmed STS who underwent definitive surgery between June 2018 and November 2025. Local recurrence-free (LRFS), metastasis-free (MFS), event-free (EFS), and overall survival (OS) were estimated by the Kaplan-Meier method with competing-risks sensitivity analyses. Given the limited number of events, pre-specified univariable comparisons (log-rank, Fisher exact, Mann-Whitney U) were performed and regarded as exploratory. Results: The median age was 16.7 years and 26 patients (61.9%) were female. A microscopically complete (R0) resection was achieved in 35 of 41 patients with assessable margins (85.4%), and limb preservation in 38 (90.5%). Over a median follow-up of 23.1 months, the 24- and 60-month estimates were LRFS 85.7% and 79.6%, MFS 93.3% and 84.8%, EFS 82.3% and 64.7%, and OS 96.0% and 81.5%, respectively. Metastatic disease at diagnosis was the only variable associated with metastatic status at last follow-up (p = 0.035). No factor was robustly associated with local recurrence; anatomical site (p = 0.68), resection-margin status (p = 0.23), and tumor volume (p = 0.97) were not significant, and an apparent association with a sub-millimeter margin rested on a single event (complete separation). Conclusions: Metastatic disease at diagnosis emerged as the most important prognostic factor for MFS in our cohort. Despite the complexity of treatment in this young patient population, high rates of complete resection and limb preservation were achieved, highlighting the value of multidisciplinary management in specialized sarcoma centers. While the limited cohort size did not allow the identification of further statistically significant prognostic factors for local recurrence, such associations may become detectable in larger multicenter studies.

Primäre maligne Knochentumoren sind seltene, biologisch heterogene Malignome, die eine hochspezialisierte, multidisziplinäre Behandlung erfordern. Die operative Behandlung des Primärtumors gilt als Voraussetzung für das Langzeitüberleben bei den meisten Betroffenen. Ein Zusammenhang zwischen Behandlungsvolumen und Ergebnis ist für zahlreiche onkologische Eingriffe belegt, für Knochensarkome jedoch weniger umfassend untersucht. Die Konzentration chirurgischer Expertise in High-Volume-Zentren (HVZ) korreliert direkt mit einer verbesserten onkologischen Ergebnisqualität und einer besseren Prognose. Wesentliche Aspekte, die dazu beitragen, sind die signifikant niedrigeren Raten positiver Resektionsränder und die signifikant niedrigere perioperative Mortalität. Darüber hinaus ermöglicht die spezialisierte chirurgische Expertise auch in komplexen Fällen meistens extremitätenerhaltende Operationen. High-Volume-Zentren sind bislang uneinheitlich definiert. Des Weiteren erfordert eine zentralisierte Behandlung in HVZ die Ausgestaltung von Zuweisungspfaden und die optimale Vernetzung zwischen Spezialzentren und der flächendeckenden Versorgung. Die operative Behandlung primärer maligner Knochentumoren sollte in spezialisierten HVZ für Sarkome konzentriert werden. Dabei ist das Klinikvolumen auch ein Surrogat für die spezialisierte multidisziplinäre Expertise, die Einhaltung klinischer Leitlinien sowie die systematische Qualitätsverbesserung, die für die optimale Behandlung von Knochensarkomen erforderlich sind. Weitere Studien sind notwendig, um den Begriff des HVZ in unterschiedlichen Gesundheitssystemen und Versorgungssettings besser zu definieren.

OBJECTIVE:Long-term stable fixation of the leg to the remaining ilium or sacrum following internal hemipelvectomy including resection of the acetabulum. INDICATIONS:Bone sarcomas, soft tissue sarcomas infiltrating the bone, solitary late metastases, acetabular reconstruction failure. CONTRAINDICATIONS:Palliative treatment goal, mutilating resection (especially when the tumor extends beyond the midline of the sacrum). SURGICAL TECHNIQUE:Facilitation of stable fixation of the femoral head or proximal femur replacement to the remaining bone (ilium or sacrum,) following periacetabular pelvic resection. Insertion of 2-3 bone anchors in the ilium or sacrum, depending on the extent of tumor resection, alternatively transosseous sutures and attachment of a partially resorbable mesh, closed at the proximal end. Depending on the muscular coverage, repositioning of the hip or bipolar cup proximally. Attachment of the mesh to the remaining hip capsule or the megaprosthesis and the muscles. The reconstruction length must not compromise sufficient muscle coverage. POSTOPERATIVE MANAGEMENT:Elastic hip spica. Bed rest for 1-2 weeks, depending on the weight of the leg. Mobilization with a walker or 2 crutches with 20 kg weight bearing for the following 4-6 weeks. Lymphatic drainage/venous foot pump as required. Adjuvant chemo- or radiotherapy as per multidisciplinary tumor board recommendation. RESULTS:The goal is the development of a stable scar around the neo-joint with minimal dead space. Young patients can often walk for several kilometers, typically using a walking stick on the contralateral side. Sole lift, lengthening of the femur at a later point if desired. There is a risk of wound-healing disorders or deep infections postoperatively in approximately 30% of cases. In case of infection, removal of the mesh and possibly of the proximal femoral replacement, as well as vacuum-assisted closure therapy may be necessary.

457 Background: Fibroblast activation protein (FAP) is highly expressed on tumor-associated fibroblasts. FAP-directed radioligand positron emission tomography ( 68 Ga-FAPI-46 PET) is a novel tool for cancer imaging. However, its diagnostic performance, especially in genitourinary (GU) cancers, is still unclear. Here, we aim to compare diagnostic performance of 68 Ga-FAPI-46 and 18 F-FDG PET, validated by histopathology. Methods: Patients (pts) at initial staging/restaging with (a) proven or suspected malignancy, (b) any tumor diameter > 1 cm, (c) planned or performed biopsy/surgery within 8 weeks prior to or after 68 Ga-FAPI-46 PET, and (d) no prior external beam radiation or systemic tumor therapy within 1 month underwent 68 Ga-FAPI-46 and clinical 18 F-FDG PET on subsequent days. For comparison, patient-based and region-based (local, nodal, distant organ or soft tissue, bone) image analysis was performed by three blinded nuclear medicine physicians, separate for each PET modality. Positive predictive value (PPV) and sensitivity (SE) were evaluated based on histopathologic results, and compared in the subgroups of GU cancers, sarcoma, and others. Inter-reader reproducibility was assessed using Fleiss‘ kappa. Results: A total of n=158 pts (median age: 62 years, male/female: n=101 (63.9%)/57 (36.1%)) were enrolled. 155 (98.1%)/152 (96.2%) pts underwent 68 Ga-FAPI-46/ 18 F-FDG PET/CT. Histopathologic validation was available in n=145 (91.8%) pts. N=54 (34.2%)/28 (17.7%)/76 (48.1%) pts were diagnosed with GU cancers/sarcoma/others. Patient-based PPV/SE for 68 Ga-FAPI-46 ( 18 F-FDG) PET were 94/72% (97/71%) for GU cancers, 100/96% (100/96%) for sarcoma, and 100/91% (100/88%) for others. Region-based PPV/SE for 68 Ga-FAPI-46 ( 18 F-FDG) PET were 93/71% (97/70%) for GU cancers, 100/94% (97/90%) for sarcoma, and 99/90% (93/85%) for others. Patient-based inter-reader reproducibility for 68 Ga-FAPI-46 ( 18 F-FDG) PET was 0.71 (0.43) for GU cancers, 1.0 (0.21) for sarcoma, and 0.6 (0.53) for others. Conclusions: 68 Ga-FAPI-46 and 18 F-FDG PET demonstrate similar accuracy for tumor detection and localization of GU cancers. However, inter-reader reproducibility was superior for 68 Ga-FAPI-46 (substantial agreement) compared to 18 F-FDG PET (moderate agreement). Clinical trial information: NCT05160051 . Patient characteristics. All patients n=158 All regions n=186 Diagnosis n (pts) % (pts) n (regions) % (regions) Genitourinary CancersRenal cell carcinomaUrothelial carcinomaProstate cancerSeminomaOthers 54 3314322 34.2 20.98.91.91.31.3 63 3816414 33.9 20.48.62.20.52.2 Sarcoma 28 17.7 32 17.2 Others* 76 48.1 91 48.9 *Including breast cancer, cholangiocarcinoma, colorectal cancer, endometrial cancer, esophageal cancer, head and neck cancer, lymphoma, multiple myeloma, neuroendocrine, NSCLC, pancreatic cancer, thyroid cancer, unknown primary, others.

Introduction Bone resection followed by endoprosthetic reconstruction (EPR) in the treatment of soft tissue sarcoma (STS) is rare and associated with unique challenges. This study aimed to analyze the indications, results and factors affecting the results of these cases. Materials and methods Twelve patients (7 men and 5 women, median age 49 years) who underwent resection and endoprosthetic reconstruction due to soft tissue sarcoma of the extremity between 2010 and 2021 were analyzed retrospectively. The most common localization was the thigh (66%), and the most common diagnosis myxofibrosarcoma (33%). The most frequent Indications for the endoprosthetic reconstruction after soft tissue tumor resections were the close relationship of the tumor to the bone ( n = 6), and suspicious bone infiltration on magnetic resonance imaging (MRI) ( n = 5). Results Eight patients (66%) had no evidence of disease at the last follow-up examination (median 62 months), while 4 patients died after an average of 14 months. In 4 of 5 cases in which suspicious bone infiltration was detected on magnetic resonance imaging, the bone lesion was confirmed histopathologically. A Whoops procedure history was significantly negative prognostic in terms of limb survival ( p < 0.045). Conclusions In bone-infiltrating or highly bone surrounding soft tissue sarcomas, wide resection including resection of the affected bone followed by endoprosthetic reconstruction seem to be a recommendable limb-salvage option with good oncological results and acceptable complication rate. The presence of bone infiltration at time of surgery does not increase the risk of local recurrence. A Whoops procedure history significantly reduce the limb survival.

Langfristig stabile Verankerung des Beines nach interner Hemipelvektomie inklusive des Acetabulum am restlichen Os ilium/Os sacrum. Knochensarkome, Weichteilsarkome mit Knochenbeteiligung, solitäre Spätmetastasen, Versagen einer azetabulären Rekonstruktion. Palliative Therapiesituation, mutilierende Resektion (insbesondere bei Tumorausdehnung über die Mittellinie des Os sacrum). Nach periazetabulärer Becken(teil)resektion Ermöglichung einer stabilen Fixation des Hüftkopfes/proximalen Femurersatzes am verbliebenen Knochen. Einbringung von 2 bis 3 Knochenankern in das Os ilium/Os sacrum je nach Resektionsausmaß, alternativ transossäre Nähte und Fixation des proximal blind verschlossenen, partiell resorbierbaren Netzes. Je nach muskulärer Deckung Proximalisierung des Hüft- oder Duokopfes. Fixation des Netzes an den Resten der Hüftkapsel bzw. der Tumorprothese und der Muskulatur. Die Länge der Rekonstruktion darf die ausreichende muskuläre Deckung nicht beeinträchtigen. Elastische Hüftwickel. Bettruhe für 1 bis 2 Wochen je nach Gewicht des Beines. Mobilisation mit Gehwagen oder 2 Unterarmgehstützen mit 20 kg Teilbelastung für weitere 4 bis 6 Wochen. Gegebenenfalls Lymphdrainage/Venenpumpe. Je nach Tumorboardbeschluss adjuvante Chemo- oder Strahlentherapie. Ziel ist die Ausbildung einer stabilen Narbenplatte um das Neogelenk mit wenig Totraum. Bei jungen Patienten ist häufig eine Gehstrecke von mehreren Kilometern unter Verwendung eines Gehstocks auf der Gegenseite möglich. Schuhausgleich, ggf. später Verlängerung des Femurs. Postoperativ Risiko einer tiefen Wundheilungsstörung oder Infektion in ca. 30

While the usual etiology of slipped capital femoral epiphysis (SCFE) is idiopathic, there are many other factors that increase the predisposition to slippage. Chemotherapy can be one of them. In this article, we report a rare case of acute SCFE after tumor prosthesis implantation in a patient who received chemotherapy. A 10-year-old girl with osteosarcoma of the right distal femur underwent (neo-) adjuvant chemotherapy, wide tumor resection, and reconstruction using a growing tumor prosthesis and a short non-cemented femoral stem. Half a year after implantation, she developed aseptic loosening. Revision surgery was performed using a hydroxyapatite (HA)-coated cementless femoral stem. Postoperative plain radiographs revealed SCFE that was treated by closed reduction and screw fixation. The patient recovered without complications, and unaffected hip showed no radiographic signs of slippage on follow-up. The forces of implanting a tumor prosthesis, particularly with a non-cemented stem, can increase the risk of an acute SCFE. The controversy over prophylactic pinning of the uninvolved hip in chemotherapy-associated SCFE is unresolved. Pinning can be considered only in the presence of abnormal prodromal radiological findings.

Megaendoprosthetic reconstruction of bone defects in skeletally immature patients has led to the development of unique complications and secondary deformities not observed in adult patient cohorts. With an increasing number of megaendoprosthetic replacements performed, orthopedic oncologists still gain experience in the incidence and type of secondary deformities caused. In this study, we report the incidence, probable cause and management outcome of two secondary deformities after megaendoprosthetic reconstruction of the proximal femur: hip dysplasia and genu valgum. Retrospective analysis of 14 patients who underwent primary and/or repeat reconstruction/surgery with a megaendoprosthetic proximal femur replacement between 2018 and 2022. Mean patient age was 9.1 years (range 4–17 years). Stress shielding was observed in 71.4

Clear cell sarcoma (CCS) of tendons and aponeuroses and CCS-like malignant gastrointestinal neuroectodermal tumor/sarcoma (GINET) are characterized by frequent local and distant relapses, alongside with low efficacy of all systemic treatments. We aimed to collect a comprehensive dataset to identify prognostic factors and treatment outcomes. We performed a retrospective single center analysis for diagnosed CCS and GINET on demographic, tumor, treatment and survival data. We identified 43 patients (w:25, m:18) with a median follow-up of 35mo and a 5y-OS-rate of 42

BackgroundIsolated limb perfusion (ILP) is a well-established surgical procedure for the administration of high dose chemotherapy to a limb for the treatment of advanced extremity malignancy. Although the technique of ILP was first described over 60 years ago, ILP is utilised in relatively few specialist centres, co-located with tertiary or quaternary cancer centres. The combination of high dose cytotoxic chemotherapy and the cytokine tumour necrosis factor alpha (TNFα), mandates leakage monitoring to prevent potentially serious systemic toxicity. Since the procedure is performed at relatively few specialist centres, an ILP working group was formed with the aim of producing technical consensus guidelines for the procedure to streamline practice and to provide guidance for new centres commencing the technique.MethodsBetween October 2021 and October 2023 a series of face to face online and hybrid meetings were held in which a modified Delphi process was used to develop a unified consensus document. After each meeting the document was modified and recirculated and then rediscussed at subsequent meeting until a greater than 90% consensus was achieved in all recommendations.ResultsThe completed consensus document comprised 23 topics in which greater than 90 % consensus was achieved, with 83% of recommendations having 100% consensus across all members of the working group. The consensus recommendations covered all areas of the surgical procedure including pre-operative assessment, drug dosing and administration, perfusion parameters, hyperthermia, leakage monitoring and theatre logistics, practical surgical strategies and also post-operative care, response evaluation and staff training.ConclusionWe present the first joint expert-based consensus statement with respect to the technical aspects of ILP that can serve as a reference point for both existing and new centres in providing ILP.

This study aimed to retrospectively analyze the follow-up results of cases in which the adjacent joint was preserved using a custom-made uncemented short-stem design (hollow stem) with optional external flanches in tumor endoprosthetic replacement due to bone sarcomas in 13 patients (with an average age of 9.6 years) between 2017 and 2023. Reconstructions were proximal femur (n = 6), intercalary femur (n = 4), intercalary tibia (n = 2), and proximal humerus (n = 1) tumor prostheses. The hollow body was used distally in 10 of the megaprotheses, proximally in 1, and both proximally and distally in 2 of them. The average distance from the joints was 6 cm in stems with flanches and 11.8 cm in stems without flanches. No aseptic loosening or deep infection was observed during an average follow-up of 34 months. Except for one case with a tibial intercalary prosthesis that needed a revision, all cases were well osteointegrated and all lower extremity cases could bear full weight without pain. In cases where the remaining bone stock after bone resection is insufficient for a standard stem implantation, reconstruction with a patient-specific short hollow-stem design appears to be a good alternative to protect healthy joints with high prosthesis survival and low revision rates in the short-term follow-up.

Weichgewebesarkome sind selten. Die Symptome sind unspezifisch, die Tumoren treten an unterschiedlichsten Lokalisationen auf. Wegen des hohen Verteilungsgrads dieser Tumoren auf unterschiedliche Akteure des Gesundheitswesens ist das klinische Management von Weichgewebesarkomen problembehaftet. Die kontrastmittelverstärkte Magnetresonanztomographie (MRT) ist unverzichtbar zur Diagnostik. Die Weichenstellung der Therapie erfolgt durch eine korrekt durchgeführte und im Referenzzentren analysierte Biopsie. Grundpfeiler der lokalen Tumorbehandlung ist die Abwägung im prätherapeutischen Tumorboard. Grundzüge von Epidemiologie, Diagnostik, Verlaufskontrolle und v. a. der Entscheidungsfindung, basierend auf der Biopsie, werden dargestellt. Der multimodale Behandlungsansatz wird unter Hinweis auf die einschlägige und aktuelle Literatur skizziert. Die fachübergreifende S3-Leitlinie Weichgewebesarkome ist dabei ein wichtiges Instrument zur Steigerung von Behandlungsqualität und Patientensicherheit.

Introduction: As survival rates associated with the multimodal treatment of malignant bone cancer (osteosarcoma and Ewing's sarcoma) are satisfactory, health-related quality of life and the return to activity and sports by the affected patients have attracted increasing attention in recent years. Nowadays, limbs can be salvaged for most patients using modular endoprostheses. Such patients are typically adolescents and young adults who have high activity levels and thereby high demands for multimodal cancer treatment. This study aimed to evaluate the activity levels and sporting proficiencies that can be attained after modular endoprosthetic treatment of bone sarcomas as well as the extent to which physiotherapeutic and sports interventions influence functional outcome and activity levels. Methods: This non-systematic review of the literature focused on the return to activity and sports after modular endoprosthetic treatment of lower extremities bone sarcomas in adolescents and young adults. The electronic database PubMed was screened for relevant publications on this issue. A treatment algorithm for return to activity and sports in tumor orthopaedics is proposed. Results and Discussion: The objective activity level (gait cycles per day and gait intensities) in patients treated for bone sarcomas is reduced in short- and long-term follow-ups compared with healthy controls and patients with other cancers (leukaemia). Although a negative impact is observed in terms of motor performance, it shows improvement over time. Functional assessment at 12 postoperative months is sensible as neoadjuvant chemotherapy is completed by then. In long-term followup, patients with bone sarcomas can achieve high sports activity levels, i. e., type of sport, frequency/week and UCLA score, after modular endoprosthetic reconstruction. The maximum level is attained at 5 years postoperatively. Nevertheless, there is a shift from high- and intermediate- to low-impact sports. Only 20% of the patients participate in school sports regularly without limitations. The localisation of bone sarcoma, but not the rate of postoperative complications, influences the postoperative activity level. Individualised sports-related interventions during and after multimodal treatment can improve the short-term activity levels; moreover, "serious games" can improve motor performance and postural control. There is no evidence that intense activity levels leads to early loosening of the endoprosthesis. There is insufficient valid data on activity and sports after modular endoprosthetic treatment of bone sarcomas of the upper extremities. Conclusion: High preoperative activity levels of young patients with bone sarcomas must be considered in tumour orthopaedics. Limitations on sports activities have a significant negative impact on the quality of life and mental health of such patients. Therefore, tumour orthopaedic treatment has to focus on preserving an improvement in these factors. The overall existing evidence concerning this issue is weak. Additional studies to evaluate the ability to return to specific sports activities are desirable, as well as prospective interventional studies.

BACKGROUND:The classic type of epithelioid sarcoma (ES) is a rare, aggressive soft tissue neoplasm that most commonly affects the distal upper extremities of young patients. This study aimed to assess clinical features and provide a long-term report of the oncological outcome.METHODS:We retrospectively analyzed our clinical database for patients with ES of the distal upper extremities.RESULTS:Twenty-three patients with ES of the distal upper extremity were treated surgically between January 1990 and August 2018. ES affected most commonly the palmar side of young patients. The most common site affected by a sarcoma was the wrist in 47.8% of cases, followed by metacarpals and fingers with 34.8% and 17.4%, respectively. Most of the patients were treated according to the protocols of interdisciplinary tumor boards with multimodal therapy. A local recurrence was observed in 7 patients (30.4%). The 5 - and 10-year recurrence-free survival was 80.4% (95% confidence interval [CI]: 68.6-76.8) and 60.9% (95% CI: 53.5-68.3), respectively. The 5- and 10-years disease-specific survival was 89.9% (95% CI: 87-92.8) and 61.9% (95% CI: 56.5-67.3), respectively. Five patients (21.7%) had metastasis in regional lymph nodes.CONCLUSION:The classic type of ES represents a group of high-grade sarcomas, which affect the dominantly distal upper extremity. Specific clinical, diagnostic, and oncological characteristics make it difficult to diagnose and therapy. Wide tumor resection as a part of multimodal therapy remains a more viable and common treatment option for patients with ES on distal extremities. High rates of lymph node metastasis are typical for ES.

Objective: The aim of this study was to investigate the response rates of different extremity soft-tissue sarcoma subtypes (eSTS) after isolated limb perfusion (ILP), based on an international multi-centre study. Materials and methods: The retrospective cohort comprised eSTS patients from 17 specialised ILP centres that underwent melphalan-based ILP, with or without recombinant human tumour necrosis factor (rhTNF & alpha;) (TM-ILP and M-ILP, respectively). Response was measured on imaging (magnetic resonance imaging) and/or clinical response, for which M-ILPs were excluded. Results: A total of 1109 eSTS patients were included. The three most common histological subtypes were undifferentiated pleomorphic sarcoma (17%, n = 184), synovial sarcoma (16%, n = 175) and myxofibrosarcoma (8%, n = 87). rhTNF & alpha; was used in 93% (TM-ILP) and resulted in a significantly better overall response rate (ORR, p = 0.031) and complete responses (CR, p < 0.001) in comparison to M-ILP, without significant differences among histological subgroups. The ORR of TM-ILP was 68%, including 17% CR. Also, 80% showed progressive disease. Significantly higher response rates were shown for Kaposi sarcoma (KS) with 42% CR and 96% ORR (both p < 0.001), and significantly higher CR rates for angiosarcoma (AS, 45%, p < 0.001) and clear cell sarcoma (CCS, 31%, p = 0.049). ILP was followed by resection & LE; 6 months in 80% of the patients. The overall limb salvage rate was 88%, without significant differences among histological subgroups, but was significantly higher for ILP responders compared to non-responders (93% versus 76%, p < 0.001). Conclusion: ILP resulted in high response and LRS among all eSTS subtypes, however, with significant differences between subtypes with most promising results for KS, AS and CCS.& COPY; 2023 Elsevier Ltd. All rights reserved.