[Diagnosis and management of tenosynovial giant cell tumor]. / Diagnostic et prise en charge de la tumeur à cellules géantes ténosynoviale.

Tenosynovial giant cell tumor is a benign condition that originates from synovial cells within joints, tendon sheaths, or bursae and may present either in localized (benign) or diffuse (locally aggressive) forms. Currently, the primary treatment approach is surgical, yielding satisfactory results with low recurrence rates in the localized forms, whereas the diffuse type displays high recurrence rates. In parallel, clinical trials are underway to explore pharmaceutical treatment options for the advanced diffuse type. This article aims at consolidating current knowledge about diagnosis and management of this rare tumor, additionally proposing a brief overview of novel therapeutic approaches.

La tumeur à cellules géantes ténosynoviale, bénigne, prend son origine dans les cellules synoviales des articulations, des gaines tendineuses ou des bourses et se présente dans une forme soit localisée (bénigne), soit diffuse (localement agressive). Le traitement principal est chirurgical, offrant des résultats satisfaisants à long terme, avec un faible risque de récidive dans la forme localisée, alors que le taux de récidives est élevé dans la forme diffuse. Parallèlement, des essais cliniques sont en cours pour explorer des options de traitement systémique pour les formes diffuses sévères. Cet article rappelle les connaissances actuelles pour le diagnostic et la prise en charge de cette tumeur rare. De plus, nous proposons un aperçu succinct des nouvelles approches thérapeutiques.

Induction of mitochondrial recycling reverts age-associated decline of the hematopoietic and immune systems.

Aging compromises hematopoietic and immune system functions, making older adults especially susceptible to hematopoietic failure, infections and tumor development, and thus representing an important medical target for a broad range of diseases. During aging, hematopoietic stem cells (HSCs) lose their blood reconstitution capability and commit preferentially toward the myeloid lineage (myeloid bias)1,2. These processes are accompanied by an aberrant accumulation of mitochondria in HSCs3. The administration of the mitochondrial modulator urolithin A corrects mitochondrial function in HSCs and completely restores the blood reconstitution capability of 'old' HSCs. Moreover, urolithin A-supplemented food restores lymphoid compartments, boosts HSC function and improves the immune response against viral infection in old mice. Altogether our results demonstrate that boosting mitochondrial recycling reverts the aging phenotype in the hematopoietic and immune systems.

Influence of smoking on the healing of conservatively treated displaced midshaft clavicle fractures.

Aims: Tobacco, in addition to being one of the greatest public health threats facing our world, is believed to have deleterious effects on bone metabolism and especially on bone healing. It has been described in the literature that patients who smoke are approximately twice as likely to develop a nonunion following a non-specific bone fracture. For clavicle fractures, this risk is unclear, as is the impact that such a complication might have on the initial management of these fractures. Methods: A systematic review and meta-analysis were performed for conservatively treated displaced midshaft clavicle fractures. Embase, PubMed, and Cochrane Central Register of Controlled Trials (via Cochrane Library) were searched from inception to 12 May 2022, with supplementary searches in Open Grey, ClinicalTrials.gov, ProQuest Dissertations & Theses, and Google Scholar. The searches were performed without limits for publication date or languages. Results: The meta-analysis included eight studies, 2,285 observations, and 304 events (nonunion). The random effects model predicted a pooled risk ratio (RR) of 3.68 (95% confidence interval 1.87 to 7.23), which can be considered significant (p = 0.003). It indicates that smoking more than triples the risk of nonunion when a fracture is treated conservatively. Conclusion: Smoking confers a RR of 3.68 for developing a nonunion in patients with a displaced middle third clavicle fracture treated conservatively. We know that most patients with pseudarthrosis will have pain and a poor functional outcome. Therefore, patients should be informed of the significantly higher risks of nonunion and offered smoking cessation efforts and counselling. Moreover, surgery should be considered for any patient who smokes with this type of fracture.

MarrowQuant 2.0: A Digital Pathology Workflow Assisting Bone Marrow Evaluation in Experimental and Clinical Hematology.

Bone marrow (BM) cellularity assessment is a crucial step in the evaluation of BM trephine biopsies for hematologic and nonhematologic disorders. Clinical assessment is based on a semiquantitative visual estimation of the hematopoietic and adipocytic components by hematopathologists, which does not provide quantitative information on other stromal compartments. In this study, we developed and validated MarrowQuant 2.0, an efficient, user-friendly digital hematopathology workflow integrated within QuPath software, which serves as BM quantifier for 5 mutually exclusive compartments (bone, hematopoietic, adipocytic, and interstitial/microvasculature areas and other) and derives the cellularity of human BM trephine biopsies. Instance segmentation of individual adipocytes is realized through the adaptation of the machine-learning-based algorithm StarDist. We calculated BM compartments and adipocyte size distributions of hematoxylin and eosin images obtained from 250 bone specimens, from control subjects and patients with acute myeloid leukemia or myelodysplastic syndrome, at diagnosis and follow-up, and measured the agreement of cellularity estimates by MarrowQuant 2.0 against visual scores from 4 hematopathologists. The algorithm was capable of robust BM compartment segmentation with an average mask accuracy of 86%, maximal for bone (99%), hematopoietic (92%), and adipocyte (98%) areas. MarrowQuant 2.0 cellularity score and hematopathologist estimations were highly correlated (R2 = 0.92-0.98, intraclass correlation coefficient [ICC] = 0.98; interobserver ICC = 0.96). BM compartment segmentation quantitatively confirmed the reciprocity of the hematopoietic and adipocytic compartments. MarrowQuant 2.0 performance was additionally tested for cellularity assessment of specimens prospectively collected from clinical routine diagnosis. After special consideration for the choice of the cellularity equation in specimens with expanded stroma, performance was similar in this setting (R2 = 0.86, n = 42). Thus, we conclude that these validation experiments establish MarrowQuant 2.0 as a reliable tool for BM cellularity assessment. We expect this workflow will serve as a clinical research tool to explore novel biomarkers related to BM stromal components and may contribute to further validation of future digitalized diagnostic hematopathology workstreams.

Prosthetic fitting associated with better survival at 5 years after above-knee amputation due to vascular insufficiency.

BACKGROUND: Individuals requiring non-traumatic Gritti-Stokes amputation or mid-thigh amputation usually have multiple comorbidities that place them at high risk of mortality. OBJECTIVE: To determine survival rate 5 years after Gritti-Stokes and mid-thigh amputation in individuals with vascular insufficiency and to identify the predictors of survival. METHODS: We conducted a retrospective observational study including all individuals with vascular insufficiency who underwent amputation from September 2007 to December 2015 in our University Hospital. The indication for amputation was limb necrosis in 86% of cases, infection in 10%, and complications with the stump (discomfort, neuroma or scar dehiscence) in 4%. Medical records were analysed to determine factors and comorbidities. The date of death was retrieved from the national death registry at a minimum of 5 years after amputation. Cox proportional-hazard regression was used to estimate associations between factors and post-amputation survival with hazard ratios (HR) and 95% confidence intervals (CIs). RESULTS: We included 126 people with vascular insufficiency (83 men), mean age was 70 years [20; 97]; eighty-nine participants (71%) died during the study period. Survival rate was 68% at 1 year, 48% at 3 years and 37% at 5 years. Survival was associated with prosthetic fitting (HR 0.306 [95% CI 0.180; 0.521], p<0.001) and length of stay (HR 0.992 [95% CI 0.987; 0.997], p = 0.003). Conversely, limb necrosis was associated with a lower survival rate (HR 3.801 [95% CI 1.615; 8.949], p = 0.002). In a secondary multivariable analysis, Gritti-Stokes amputation was the only factor positively associated with prosthetic fitting (odds ratio 7.407 [95% CI 2.439; 22.489], p<0.001). CONCLUSIONS: The survival rate at 5 years after Gritti-Stokes and mid-thigh amputation in people with vascular insufficiency was 37%. Prosthetic fitting was independently associated with better survival, and Gritti-Stokes amputation was the only factor positively related to prosthetic fitting.

Lymphatic flow through (LyFT) ALT flap: an original solution to reconstruct soft tissue loss with lymphatic leakage or lower limb lymphedema.

INTRODUCTION: The lympho-venous shunt using the distal vein of ALT flap pedicle allowed at the same time the coverage of the inguinal defects and to perform lymphovenous shunt into a run-in vein of the descending branch of the lateral circumflex femoral pedicle, draining the lymph through the flap pedicle. Surgical technique, complications and final outcomes (both clinical and lymphoscintigraphic) are reported. METHODS: Five patients (45.8 y.o.[22-70]) with groin soft tissue loss with lymphatic leakage or lower limb lymphedema, benefited of the described technique. The ALT flap was used to cover the defect and, at the same time, we could perform a lymphovenous shunt between afferent lymphatics to the thigh and the descending branch of the lateral circumflex femoral pedicle, distal to the perforator nourishing the flap. Clinical and lymphoscintigraphic assessment of the limbs, cease of lymphorrhea or cellulitis/lymphangitis episodes, eventual downstaging of physiologic/physical therapy were recorded. LYMphatic Quality Of Life in leg (LYMQoLLeg) and patient satisfaction were evaluated. RESULTS: Average flap size was 88.8cm2 (range 84-126). The mean number of multi-lymphovenous anastomosis (MLVA) performed was 1.8 (range 1-3) per patient with 1-3 lymphatics shunted into each vein. Only one hemato-seroma requiring surgical revision. Mean improvement of perometer values was 48.2% (range 27.7-67.7) with an average follow-up of 13.6 months (range 12-17). Lymphoscintigraphy showed disappearing of the lymphatic leak and lymphedema with a high satisfaction of LYMQoL score. DISCUSSION: The combination of pedicle flap with lympho-venous bypass as lymphatic derivation concept, improving the chronic morbidity scenarios of lymphatic complications.

The Performances of Conventional Titanium and Silver-Coated Megaprostheses in Non-oncological and Post-oncological Patients: An Analysis of Infection Failures in 142 Patients.

Background: Megaprostheses are one of the preferred choices of reconstruction after tumor resection. Periprosthetic joint infections are one of the most serious complications of joint prostheses surgeries. In this study, our aim was to analyze the efficacy of silver-coated megaprostheses in reducing the risk of prosthesis-related infection. Methods: One hundred forty-two patients who had undergone implantation of a mega-endoprosthesis for non-neoplastic or post-neoplastic conditions were included in this retrospective study. The end-point of the survival analysis was the prosthesis failure due to infection. Results: Thirty-eight patients had undergone implantation of a silver-coated megaprosthesis and 104 patients a megaprosthesis without silver coating. The survival analysis showed an overall infection-free survival rate of 82.3% at five years and 61.9% at 10 years. Silver-coated prostheses had an HR of 0.72 (95% CI: 0.26-2.05; P=0.54). Conclusion: Implantation of a silver-coated mega-prosthesis in non-oncological patients did not significantly reduce the risk of prosthesis-related infection.

Case Report: Tyrosine Kinase Inhibitors Induced Lymphadenopathy in Desmoid Tumor Patients.

Tyrosine kinase inhibitors (TKIs) are nowadays a valuable treatment of desmoid tumors, a rare mesenchymal neoplasm. Although many side effects of imatinib and pazopanib, commonly or rarely occurring, have been described, reactional lymphadenopathy has not yet been reported. In this publication, we report two cases of patients with desmoid tumors, treated with pazopanib and imatinib, who developed reactional lymphadenopathy. As this side effect is presented as a newly formed mass, it can result in new diagnostic questions and added imaging tests and can even lead to discontinuation of the treatment. This report may help the clinicians facing similar problems adopt a "watch and wait" approach.

Life or limb: an international qualitative study on decision making in sarcoma surgery during the COVID-19 pandemic.

OBJECTIVES: The COVID-19 pandemic is unprecedented as a global crisis over the last century. How do specialist surgeons make decisions about patient care in these unprecedent times? DESIGN: Between April and May 2020, we conducted an international qualitative study. Sarcoma surgeons from diverse global settings participated in 60 min interviews exploring surgical decision making during COVID-19. Interview data were analysed using an inductive thematic analysis approach. SETTING: Participants represented public and private hospitals in 14 countries, in different phases of the first wave of the pandemic: Australia, Argentina, Canada, India, Italy, Japan, Nigeria, Singapore, Spain, South Africa, Switzerland, Turkey, UK and USA. PARTICIPANTS: From 22 invited sarcoma surgeons, 18 surgeons participated. Participants had an average of 19 years experience as a sarcoma surgeon. RESULTS: 17/18 participants described a decision they had made about patient care since the start of the pandemic that was unique to them, that is, without precedence. Common to 'unique' decisions about patient care was uncertainty about what was going on and what would happen in the future (theme 1: the context of uncertainty), the impact of the pandemic on resources or threat of the pandemic to overwhelm resources (theme 2: limited resources), perceived increased risk to self (theme 3: duty of care) and least-worst decision making, in which none of the options were perceived as ideal and participants settled on the least-worst option at that point in time (theme 4: least-worst decision making). CONCLUSIONS: In the context of rapidly changing standards of justice and beneficence in patient care, traditional decision-making frameworks may no longer apply. Based on the experiences of surgeons in this study, we describe a framework of least-worst decision making. This framework gives rise to actionable strategies that can support decision making in sarcoma and other specialised fields of surgery, both during the current crisis and beyond.

A live single-cell reporter assay links intratumor heterogeneity to metastatic proclivity in Ewing sarcoma.

Targeting of the most aggressive tumor cell subpopulations is key for effective management of most solid malignancies. However, the metastable nature of tumor heterogeneity, which allows cells to transition between strong and weak tumorigenic phenotypes, and the lack of reliable markers of tumor-promoting properties hamper identification of the most relevant cells. To overcome these obstacles, we designed a functional microRNA (miR)-based live-cell reporter assay to identify highly tumorigenic cells in xenotransplants of primary Ewing sarcoma (EwS) 3D cultures. Leveraging the inverse relationship between cell pluripotency and miR-145 expression, we successfully separated highly tumorigenic, metastasis-prone (miR-145low) cells from poorly tumorigenic, nonmetastatic (miR-145high) counterparts. Gene expression and functional studies of the two cell populations identified the EPHB2 receptor as a prognostic biomarker in patients with EwS and a major promoter of metastasis. Our study provides a simple and powerful means to identify and isolate tumor cells that display aggressive behavior.

Pelvic Chondrosarcoma Treated by En Bloc Resection with Patient-Specific Osteotomy Guides and Reimplantation of the Extracorporeally Irradiated Bone as an Osseocartilaginous Structural Orthotopic Autograft: A Report of Two Cases with Description of the Surgical Technique.

Primary tumors of the pelvis are considered difficult to treat due to the complex anatomy and the proximity of important neurovascular structures. The surgical armamentarium for the treatment of these tumors has evolved with the help of cutting-edge technology from debilitating hemipelvectomies to solutions such as precise resections guided by patient-specific instruments or computer navigation and reconstruction by modular prostheses, 3D-printed custom-made implants, or orthotopic autograft reimplantation after extracorporeal irradiation. Different combinations of these techniques have been described in the literature with various rates of success. We present two cases of pelvic chondrosarcomas successfully treated by a combination of periacetabular resection with patient-specific osteotomy guides and orthotopic reimplantation of the extracorporeally irradiated autograft resulting in retention of the native hip.

Unrecognized Chondrosarcoma as a Cause of Total Hip Arthroplasty Failure.

BACKGROUND: Total hip arthroplasty (THA) is one of the most successful procedures in orthopedic surgery. The most frequent THA indications are osteoarthritis and avascular necrosis, whereas symptomatic aseptic loosening is the most common indication to revision surgery. Chondrosarcoma (CS) is the most frequent bone sarcoma in adults, and proximal femur is the most prevalent location. Wide resection is the treatment of choice.We report 3 cases of unrecognized high-grade CS in the setting of primary or revision THA and reviewed the literature on this rare clinical presentation. METHODS: A systematic literature review on CS in the setting of THA, published between 1980 and 2020, was performed on PubMed, Embase, Medline, Ovid SP, and Web of Science, using the guidelines set in the Preferred Reporting Items for Systematic Reviews and Mata-analyses (PRISMA). RESULTS: Case series: Three patients were referred to our sarcoma center after failure of THA due to unrecognized high-grade CS. All 3 had rapid fatal outcome. Literature review: Fifty-nine articles were identified, of which 8 were included in the study. They confirmed that primary or revision THA failure due to unrecognized CS is extremely rare, with only few cases reported in the literature. CONCLUSIONS: Before proceeding to primary or revision arthroplasty, diagnosis must be ascertained. Atypical presentation of a common pathology, such as osteoarthritis, avascular necrosis, or aseptic loosening of an endoprosthesis, should raise suspicion for another cause to symptoms, and additional workup be performed. As our cases demonstrated, unrecognized or inadequately managed bone sarcoma may lead to poor or even fatal outcome.

Opposing immune and genetic mechanisms shape oncogenic programs in synovial sarcoma.

Synovial sarcoma (SyS) is an aggressive neoplasm driven by the SS18-SSX fusion, and is characterized by low T cell infiltration. Here, we studied the cancer-immune interplay in SyS using an integrative approach that combines single-cell RNA sequencing (scRNA-seq), spatial profiling and genetic and pharmacological perturbations. scRNA-seq of 16,872 cells from 12 human SyS tumors uncovered a malignant subpopulation that marks immune-deprived niches in situ and is predictive of poor clinical outcomes in two independent cohorts. Functional analyses revealed that this malignant cell state is controlled by the SS18-SSX fusion, is repressed by cytokines secreted by macrophages and T cells, and can be synergistically targeted with a combination of HDAC and CDK4/CDK6 inhibitors. This drug combination enhanced malignant-cell immunogenicity in SyS models, leading to induced T cell reactivity and T cell-mediated killing. Our study provides a blueprint for investigating heterogeneity in fusion-driven malignancies and demonstrates an interplay between immune evasion and oncogenic processes that can be co-targeted in SyS and potentially in other malignancies.

Image-guided percutaneous cryoablation of unresectable sacrococcygeal chordoma: Feasibility and outcome in a selected group of patients with long term follow-up.

BACKGROUND: Chordoma is a rare malignant tumor of the axial skeleton. Percutaneous cryoablation (PCA) is a minimally invasive technique that allows freezing of tumors under imaging control. The purpose of our retrospective study was to investigate the outcome of PCA in a selected cohort of patients with sacrococcygeal chordoma, with a minimum of 5 years follow-up. MATERIALS AND METHODS: Four patients were treated in 10 sessions. The mean follow-up was 57.3 months. We evaluated the feasibility, the procedure-related complications, the impact on pain control and oncological outcomes. RESULTS: Freezing of 100% of the tumor volume was possible in 60%. Pain control was not reliably evaluable. Local recurrence occurred in 90% of the treated lesions; the mean time to progression was 8.1 months (range 1.5-16). At last follow-up, one patient had died of the disease, one of another cause and one was receiving the best supportive care. The only patient alive without the disease had received additional carbon-ion radiotherapy. The 5-year survival rate after index PCA was 50%. CONCLUSION: Complete freezing of the tumor was technically challenging, mainly due to the complex local anatomy. Recurrence occurred in 90% of the lesions treated. PCA should be considered with caution in the curative management of sacrococcygeal chordoma.

The chromatin landscape of primary synovial sarcoma organoids is linked to specific epigenetic mechanisms and dependencies.

Synovial sarcoma (SyS) is an aggressive mesenchymal malignancy invariably associated with the chromosomal translocation t(X:18; p11:q11), which results in the in-frame fusion of the BAF complex gene SS18 to one of three SSX genes. Fusion of SS18 to SSX generates an aberrant transcriptional regulator, which, in permissive cells, drives tumor development by initiating major chromatin remodeling events that disrupt the balance between BAF-mediated gene activation and polycomb-dependent repression. Here, we developed SyS organoids and performed genome-wide epigenomic profiling of these models and mesenchymal precursors to define SyS-specific chromatin remodeling mechanisms and dependencies. We show that SS18-SSX induces broad BAF domains at its binding sites, which oppose polycomb repressor complex (PRC) 2 activity, while facilitating recruitment of a non-canonical (nc)PRC1 variant. Along with the uncoupling of polycomb complexes, we observed H3K27me3 eviction, H2AK119ub deposition and the establishment of de novo active regulatory elements that drive SyS identity. These alterations are completely reversible upon SS18-SSX depletion and are associated with vulnerability to USP7 loss, a core member of ncPRC1.1. Using the power of primary tumor organoids, our work helps define the mechanisms of epigenetic dysregulation on which SyS cells are dependent.

Cryoablation of Extra-Abdominal Desmoid Tumors: A Single-Center Experience with Literature Review.

Cryoablation (CA) has gained popularity in the treatment of benign and malignant musculoskeletal tumors. While extra-abdominal desmoid (EAD) tumors are not malignant, they remain challenging to treat because of their high local recurrence rate. We reviewed all EAD tumors treated with CA at our institution between November 2012 and March 2020. Fourteen procedures were performed on nine females and one male (mean age, 33 ± 18 years) as either first-line (n = 4) or salvage therapy (n = 6) with curative intent (n = 8) or tumor debulking (n = 2). Mean tumor size was 63.6 cm3 (range, 3.4-169 cm3). Contrast-enhanced MRI was performed before treatment and at 3-, 6-, and 12-month follow-up. Treatment outcome was based on the change in enhanced tumor volume (ET-V). For curatively treated patients, the mean ET-V change was -97 ± 7%, -44 ± 143%, and +103 ± 312% at 3, 6, and 12 months, respectively. For debulking patients, the mean ET-V change was -98 ± 4%, +149 ± 364%, and +192 ± 353% at 3, 6, and 12 months, respectively. During a mean follow-up of 53.7 months (range, 12-83 months), one grade III and one grade IV complication were noted. We found CA to be safe and well tolerated in patients with EAD.

LIN28B Underlies the Pathogenesis of a Subclass of Ewing Sarcoma LIN28B Control of EWS-FLI1 Stability.

Ewing sarcoma (EwS) is associated with poor prognosis despite current multimodal therapy. Targeting of EWS-FLI1, the fusion protein responsible for its pathogenesis, and its principal downstream targets has not yet produced satisfactory therapeutic options, fueling the search for alternative approaches. Here, we show that the oncofetal RNA-binding protein LIN28B regulates the stability of EWS-FLI1 mRNA in ~10% of EwSs. LIN28B depletion in these tumors leads to a decrease in the expression of EWS-FLI1 and its direct transcriptional network, abrogating EwS cell self-renewal and tumorigenicity. Moreover, pharmacological inhibition of LIN28B mimics the effect of LIN28B depletion, suggesting that LIN28B sustains the emergence of a subset of EwS in which it also serves as an effective therapeutic target.

Lymphatic Leaks of the Thigh and Inguinal Region: Combined Plastic Surgery Approaches for an Effective Treatment Algorithm.

BACKGROUND: Surgical procedures interfering with the draining nodes in the inguinal region carry the intrinsic risk of lymphatic complications. Lesions of the inguinal lymphatic network can lead to lymphocele or lymphocutaneous fistulas and can eventually be associated to limb lymphedema with consequent high morbidity. OBJECTIVES: This article describes a new surgical algorithm based on wound properties to properly address lymphatic complications of the inguinal area. Based on our experience, surgical solutions ranged from selective lymphatic vessel ligation to microsurgical lymphatic fistula treatment and free tissue transfer. METHODS: Fourteen consecutive patients underwent surgery in our department following failed attempts to address persistent lymphatic leaks. Patient characteristics such as smoking, previous surgeries, comorbidities, and wound properties were considered. Identification of the leak was performed using blue patent dye and indocyanine green fluorescence. Surgical reconstruction occurred, according to our algorithm. RESULTS: Lymphatic leaks were visualized in 11 of 14 patients. Direct closure of the wound after leak ligation could be performed in 4 of 14 patients. Multilymphatic into vein anastomosis was performed in 3 of 14 patients, and the remaining patients benefited from flap surgery based on the wound defects. All 14 patients had successful outcomes (100%) with early drain removal (average, 6 [SD, 6] days) and definitive wound healing 2 weeks postoperatively. After a mean follow-up of 12 (SD, 2.9) months, no clinical infection, lymphatic complication, or wound breakdown occurred. One patient had a partial recurrence that did not require surgical intervention. CONCLUSIONS: A stepwise approach, combining lymphatic surgery principles and plastic surgery flap techniques, can lead to an effective treatment algorithm where surgical options are wound tailored to guarantee the best functional outcomes.

Oncological outcome, functional results and costs after unplanned excision of musculoskeletal soft tissue sarcoma.

BACKGROUND: Treatment of soft tissue sarcomas (STS) should only be initiated once the diagnosis is fully established. Resection of tumors of unknown nature should be avoided. Nevertheless, specialized centers continue to face numbers of unplanned excisions (UPE) in STS. AIM: To compare oncologic and functional outcomes, number of surgeries, length of hospital stay and treatment costs of UPE versus planned excision (PE) in STS. METHOD: A retrospective single tertiary center study was performed on 201 patients. Survival, local and distant recurrence rates were compared between PE (n = 137) and UPE (n = 64). In a subgroup analysis of 60 patients, functional outcome (MSTS and TESS scores), and socio-economic impact (number of surgeries, length of hospital stay and treatment costs) in "functional planned excision" (fPE) group (n = 30) and "functional unplanned excision" (fUPE) group (n = 29) were compared. RESULTS: There was no significant difference in oncological outcome between PE and UPE. In the subgroup analysis, we found a non-significant difference in functional outcome. Patients in the fUPE had significantly more surgeries (3.5 vs. 1.4; p < 0.00001) and costs of their management was 64% higher than fPE (p = 0.048). Hospital stay was longer after fUPE but not statistically significant (18.3 days vs. 11.8 days; p = 0.13). CONCLUSION: Even though oncological and functional outcomes are comparable after PE and UPE of STS, the number of surgeries, length of hospital stay and treatment costs were higher in patients with UPE. Our data underscore the importance of specialized STS treatment centers including multidisciplinary management.