Objective： We enrolled retrospective data to determine the efficacy of combined chemotherapy and surgery for local tumour control and survival in patients with high-risk soft-tissue sarcomas. Methods： We collected d...Objective： We enrolled retrospective data to determine the efficacy of combined chemotherapy and surgery for local tumour control and survival in patients with high-risk soft-tissue sarcomas. Methods： We collected data from 25 patients with high-risk soft-tissue sarcomas treated with four cycles of etoposide, ifosfamide and doxorubicin （EIA） followed by definitive surgery with or without postoperative radiotherapy and adjuvant chemotherapy. 21 patients received chemotherapy in a neoadjuvant/adjuvant clinical setting; eighteen of them completed adjuvant chemotherapy. Four patients received chemotherapy in an adjuvant setting only. Results： The objective response rate of neoadjuvant chemotherapy assessable in 21 patients was 43%. Including NED （n=7） and partial remissions （n=3）, the radiographic response rate was 47.6% with additional 42.9% stable diseases （n=9）. Surgery was performed in two patients before completing four neoadjuvant chemotherapy cycles because of disease progression. Median overall survival for all patients was 21.6＋ months. After completion of chemotherapy, in 62% of patients R0-resection could be performed. Conclusion： High proportion of R0-resections supports the idea of tumour down-staging after neoadjuvant treatment. Response to neoadjuvant chemotherapy is predictive for improved local tumour control resulting in long-term survival benefit.展开更多
Soft tissue sarcomas (STS) are rare neoplasm with frequencies around 1% of all neoplasms. Although it consists of a high heterogeneous group of tumors, surgery is the mean treatment. The STS surgery is still challengi...Soft tissue sarcomas (STS) are rare neoplasm with frequencies around 1% of all neoplasms. Although it consists of a high heterogeneous group of tumors, surgery is the mean treatment. The STS surgery is still challenging and complex procedure is usually required: this is because STS requires different types of resection and reconstruction due to various tissue-commitment</span><span style="font-family:"">s</span><span style="font-family:""> (nerve, arteries, skin and muscle). So, a multidisciplinary team must be prepared for STS approach to obtain the maximum local control and a limited extremity functional impairment. We, here, showing our experience, wish to introduce some technical contrivances in STS surgery, with special reference to tissue reconstruction. This may illustrate the necessity of a multidisciplinary team approach in this surgery.展开更多
The majority of sarcomas are under the influence of a tumor microenvironment that dampens immune activity,resulting in resistance to monoclonal antibodies targeting immune checkpoints and reduced clinical effectivenes...The majority of sarcomas are under the influence of a tumor microenvironment that dampens immune activity,resulting in resistance to monoclonal antibodies targeting immune checkpoints and reduced clinical effectiveness.Preclinical studies indicate that targeting abnormal neoangiogenesis by inhibiting vascular endothelial growth factor receptor(VEGFR)can alter the TME,thereby promoting T cell infiltration and increasing tumor immunogenicity.The REGOMUNE study,a phase II clinical trial,assessed the therapeutic combination of regorafenib,a multityrosine kinase inhibitor that targets VEGFR2 and the PD-L1 blocker avelumab,in individuals with advanced“cold”STS characterized by a lack of mature tertiary lymphoid structures(mTLS).Forty-nine mTLSnegative STS patients were enrolled,including leiomyosarcoma(45%),synovial sarcoma(18%),and other subtypes.The objective response rate was 11.0%(95%CI:4.0%-22.0%),with median progression-free survival and overall survival of 1.8 months(95%CI,1.7-3.5 months)and 15.1 months,respectively.Frequent adverse events included grade 1 or 2 palmar-plantar erythrodysesthesia,fatigue,and diarrhea.On-treatment multiplex immunofluorescence analysis revealed significant increases in CD8+T cell and B cell infiltration and PD1 expression on immune cells.Plasma analysis indicated significant upregulation of soluble PD-L1(sPD-L1)levels and tryptophan consumption.Overall,these results indicate that anti-angiogenic therapy modulates the tumor microenvironment in patients with cold STS and highlight the need for complementary strategies to enhance the functional activity of immune cells in this particular setting.展开更多
Immunoradiotherapy holds promise for improving outcomes in patients with advanced solid tumors,including in soft-tissue sarcoma(STS).However,the ideal combination of treatment modalities remains to be determined,and r...Immunoradiotherapy holds promise for improving outcomes in patients with advanced solid tumors,including in soft-tissue sarcoma(STS).However,the ideal combination of treatment modalities remains to be determined,and reliable biomarkers to predict which patients will benefit are lacking.Here,we report the results of the STS cohort of the SABR-PDL1 phase Il trial that evaluated the anti-PDL1 atezolizumab combined with stereotactic body radiation therapy(SBRT)delivered concurrently with the 2nd cycle to at least one tumor site,Eligible patients received atezolizumab until progression or unmanageable toxicity,with SBRT at 45 Gy in 3 fractions).The primary endpoint was one-year progression-free survival(PFS)rate with success defined as 13 patients achieving 1-year PFS.Sixty-one heavily pretreated patients with STS(median 5 prior lines;52%men;median age 54 years;28%leiomyosarcoma)were enrolled across two centers(France,Spain).SBRT was delivered to 55 patients(90%),with the lung being the most commonly irradiated site(50%).After a median follow-up of 45 months,the one-year PFS rate was 8.3%[95%Cl:3.6-18.1].Median PFS and overall survival were 2.5 and 8.6 months,respectively.Best responses included partial responses(5%)and stable disease(60%).Immune profiling revealed increased immunosuppressive tumor-associated macrophages(e.g,IL4l1,HES1)and monocyte-recruiting chemokines in non-responders.Higher monocyte/lymphocyte ratios(MonoLR)in tumor and blood correlated with progression.PD-L1 status,lymphoid infiltration,and tertiary-lymphoid structures were not predictive.Although the primary endpoint was not met,this study highlights MonoLR imbalance as a potential biomarker to identify STS patients likely to benefit from immunoradiotherapy.EudraCT No.2015-005464-42;Clinicaltrial.gov number:NCT02992912.展开更多
Alveolar soft part sarcoma （ASPS） is a rare tumor that was originally named by Christopherson in 1952.Until now, fewer than 200 cases have been described in the literature. The ASPS on the uterine cervix is a very r...Alveolar soft part sarcoma （ASPS） is a rare tumor that was originally named by Christopherson in 1952.Until now, fewer than 200 cases have been described in the literature. The ASPS on the uterine cervix is a very rare malignant tumor of the female reproductive organs. Including the first case described by Flint et al2 in 1985, there have been only a few cases reported to date, of which two were in China, and there is a lack of insightful analysis of treatment options. Therefore, to raise awareness of this disease, we performed a detailed literature review, which includes a case encountered by ourselves.展开更多
Background:In view of the ever-increasing representation of Staphylococcus spp.strains resistant to various antibiotics,the development of in vivo models for evaluation of novel antimicrobials is of utmost importance....Background:In view of the ever-increasing representation of Staphylococcus spp.strains resistant to various antibiotics,the development of in vivo models for evaluation of novel antimicrobials is of utmost importance.Methods:In this article,we describe the development of a fully immunocompetent porcine model of extensive skin and soft tissue damage suitable for testing topical anti-microbial agents that matches the real clinical situation.The model was developed in three consecutive stages with protocols for each stage amended based on the results of the previous one.Results:In the final model,10 excisions of the skin and underlying soft tissue were created in each pig under general anesthesia,with additional incisions to the fascia performed at the base of the defects and immediately inoculated with Staphylococcus aureus suspension.One pig was not inoculated and used as the negative control.Subsequently,the bandages were changed on Days 4,8,11,and 15.At these time points,a filter paper imprint technique(FPIT)was made from each wound for semi-quantitative microbiological evaluation.Tissue samples from the base of the wound together with the adjacent intact tissue of three randomly selected defects of each pig were taken for microbiological,histopathological,and molecular-biological examination.The infection with the inoculated S.aureus strains was sufficient during the whole experiment as confirmed by both FPIT and from tissue samples.The dynamics of the inflammatory markers and clinical signs of infection are also described.Conclusions:A successfully developed porcine model is suitable for in vivo testing of novel short-acting topical antimicrobial agents.展开更多
Dear Editor,Surgery remains the cornerstone of treatment for softtissue sarcomas(STS)[1].However,despite adequate locoregional treatment,30%-40%of the patients eventually develop metastases[2,3].Accurate risk assessme...Dear Editor,Surgery remains the cornerstone of treatment for softtissue sarcomas(STS)[1].However,despite adequate locoregional treatment,30%-40%of the patients eventually develop metastases[2,3].Accurate risk assessment is crucial to tailor therapeutic strategies and to identify high-risk patients who may benefit from perioperative chemotherapy.Hence,several efforts have been made to develop prognostic nomograms that enable individual prognosis prediction;representing an important decision-making aid for oncologists and surgeons involved in sarcoma care[4-6].展开更多
To the editor Soft-tissue sarcomas(STS)represent a very heterogeneous group of rare tumors including more than 100 different subtypes[1].Surgery and neo/adjuvant radiation therapy represent the cornerstone of treatmen...To the editor Soft-tissue sarcomas(STS)represent a very heterogeneous group of rare tumors including more than 100 different subtypes[1].Surgery and neo/adjuvant radiation therapy represent the cornerstone of treatment for STS.However,despite an optimal resection of the tumor,up to 40%of patients will develop metastatic relapse and will die from the disease[1].Doxorubicin represents the first-line standard of care for patients with advanced disease since the 1970s,despite several attempts to identify better regimens.The median overall survival(OS)of patients with metastatic disease is<18 months and has only modestly improved over the past 20 years[2].展开更多
Diagnosis of extremity soft-tissue tumors can be challenging.Characteristics of tumor margins can help precisely identify locally aggressive or non-aggressive behavior for surgical planning,but cannot differentiate be...Diagnosis of extremity soft-tissue tumors can be challenging.Characteristics of tumor margins can help precisely identify locally aggressive or non-aggressive behavior for surgical planning,but cannot differentiate benign from malignant lesions.Most malignant tumors can have inhomogeneous signals on T2-weighted images.Although a uniform signal on T2-weighted images can be a reliable indication of a benign lesion,a well-defined mass with homogeneous internal signal intensity does not definitively identify a benign lesion.Some common and distinctive soft-tissue lesions can have specific clinical and imaging features allowing a diagnosis without biopsy.These are known as determinate lesions.This illustrative report presents a diagnostic guide for extremity soft-tissue tumors based on tissue signal and morphological characteristics on magnetic resonance images.It is important for clinicians to be familiar with the imaging characteristics of common determinate lesions.展开更多
An experimental animal model of malignant soft-tissue tumor was established to investigate the applied value of multi-slice spiral CT perfusion imaging preliminarily. Ten New Zealand white rabbits which were implanted...An experimental animal model of malignant soft-tissue tumor was established to investigate the applied value of multi-slice spiral CT perfusion imaging preliminarily. Ten New Zealand white rabbits which were implanted with VX2 tumor in either proximal thigh were subjected to CT plain scan and perfusion scan two weeks later respectively, then the original perfusion images were transmitted to AW4.0 Workstation. The functional maps and perfusion parameters including blood flow （BF）, blood volume （BV）, mean transit time （MTT） and permeability surface （PS） were computed and analyzed. All the values of BF, BV and PS in VX2 soft-tissue tumors were obviously higher while the MTT-values were lower than those in the normal muscular tissues significantly. It was suggested that multi-slice spiral CT perfusion imaging is an accurate, convenient and relatively safe functional imaging technique, and can give a quantitative assessment to angiogenesis and blood perfusion of soft-tissue tumors.展开更多
The glenohumeral joint(GHJ)allows for a wide range of motion,but is also particularly vulnerable to episodes of instability.Anterior GHJ instability is especially frequent among young,athletic populations during conta...The glenohumeral joint(GHJ)allows for a wide range of motion,but is also particularly vulnerable to episodes of instability.Anterior GHJ instability is especially frequent among young,athletic populations during contact sporting events.Many first time dislocators can be managed non-operatively with a period of immobilization and rehabilitation,however certain patient populations are at higher risk for recurrent instability and may require surgical intervention for adequate stabilization.Determination of the optimal treatment strategy should be made on a case-by-case basis while weighing both patient specific factors and injury patterns(i.e.,bone loss).The purpose of this review is to describe the relevant anatomical stabilizers of the GHJ,risk factors for recurrent instability including bony lesions,indications for arthroscopic vs open surgical management,clinical history and physical examination techniques,imaging modalities,and pearls/pitfalls of arthroscopic soft-tissue stabilization for anterior glenohumeral instability.展开更多
Objective: To perform a contrast investigation of multi-slice spiral CT (MSCT) perfusion imaging and pathological findings in VX2 soft-tissue tumor of rabbits, and discuss the applicative value of multi-slice spiral C...Objective: To perform a contrast investigation of multi-slice spiral CT (MSCT) perfusion imaging and pathological findings in VX2 soft-tissue tumor of rabbits, and discuss the applicative value of multi-slice spiral CT perfusion imaging in soft-tissue tumors. Methods: 8 Newzealand white rabbits were implanted with 0.1 ml VX2 tumor tissue suspension in bilateral proximal thighs. 14 days and 21 days later, CT plain scan and perfusion scan were performed on these rabbits respectively, then the images were transmitted to AW4.0 workstation, the functional maps and perfusion parameters including blood flow (BF), blood volume (BV), mean transit time (MTT) and permeability surface (PS) were computed and analyzed. Subsequently, the rabbits were sacrificed, the tumors of which were taken out for pathological examination. The correlation between MSCT functional parametric images and pathological findings was analyzed. Results: All the values of BF, BV and PS of VX2 soft-tissue tumors were obviously higher while the MTT-values were lower than those of the normal muscular tissues significantly (P<0.001). Conclusion: Multi-slice spiral CT perfusion imaging is an accurate, convenient and relatively safe functional imaging technique, which can give a quantitative assessment to blood perfusion and angiogenesis of soft-tissue tumors.展开更多
BACKGROUND The classification of uterine sarcomas is based on distinctive morphological and immunophenotypic characteristics,increasingly supported by molecular genetic diagnostics.Data on neurotrophic tyrosine recept...BACKGROUND The classification of uterine sarcomas is based on distinctive morphological and immunophenotypic characteristics,increasingly supported by molecular genetic diagnostics.Data on neurotrophic tyrosine receptor kinase(NTRK)gene fusionpositive uterine sarcoma,potentially aggressive and morphologically similar to fibrosarcoma,are limited due to its recent recognition.Pan-TRK immunohistochemistry(IHC)analysis serves as an effective screening tool with high sensitivity and specificity for NTRK-fusion malignancies.CASE SUMMARY We report a case of a malignant mesenchymal tumor originating from the uterine cervix,which was pan-TRK IHC-positive but lacked NTRK gene fusions,accompanied by a brief literature review.A 55-year-old woman presented to the emergency department with abdominal pain and distension,exhibiting significant ascites and multiple solid pelvic masses.Pelvic examination revealed a tumor encompassing the uterine cervix,extending to the vagina and uterine corpus.A punch biopsy of the cervix indicated NTRK sarcoma with positive immunochemical pan-TRK stain.However,subsequent next generation sequencing revealed no NTRK gene fusion,leading to a diagnosis of poorly differentiated,advanced-stage sarcoma.CONCLUSION The clinical significance of NTRK gene fusion lies in potential treatment with TRK inhibitors for positive sarcomas.Identifying such rare tumors is crucial due to the potential applicability of tropomyosin receptor kinase inhibitor treatment.展开更多
Background: The low-grade fibromyxoid sarcoma (LGFMS) is an exceptionally uncommon sarcoma that primarily manifests in the extremities or trunk of young adults, presenting as painless lesions. The histological feature...Background: The low-grade fibromyxoid sarcoma (LGFMS) is an exceptionally uncommon sarcoma that primarily manifests in the extremities or trunk of young adults, presenting as painless lesions. The histological features of this tumor are benign, but it exhibits an exceptionally high rate of late recurrence and a significant potential for metastasis. Imaging examinations serve as a crucial method for detecting LGFMS, while the definitive diagnosis relies on histopathological assessment. Currently, the primary treatment modality for this neoplasm is surgical resection. Early aggressive surgery with negative margins is a critical factor in mitigating the risk of tumor recurrence and metastasis. The present study presented a case of LGFMS located in the right thigh. The patient underwent a mass resection procedure following an MRI examination. During the telephone follow-up one year post-surgery, despite the absence of an imaging review, the surgical site demonstrated satisfactory recovery with no reported abnormal symptoms. Case Presentation: The patient, a 31-year-old male, presented to our hospital for evaluation of an asymptomatic mass in his right thigh that was incidentally discovered 13 years ago. The MRI showed a well-defined mass measuring 8.2 cm × 6.8 cm × 9.6 cm in the right thigh. The tumor signals exhibit a mixed pattern, characterized by predominantly isointense and hypointense signals on T1-weighted imaging (T1WI), a central area of hyperintensity on T2-weighted imaging (T2WI), and peripheral circular enhancement observed on contrast-enhanced scans. The patient underwent surgical resection. Microscopically, the mass was composed of intricately interwoven fibrous matrix and a distinct mucoid region. The tumor cells exhibited a distinctive arrangement in a swirling or wheel-like pattern, with minimal variation in their karyotypic characteristics. The immunohistochemical examination revealed diffuse and intense MUC4 positivity in the tumor cells. The diagnosis of LGFMS was confirmed by post-operative histopathological examination. Conclusions: The LGFMS is an exceptionally uncommon mesenchymal tumor renowned for its benign histological manifestations and malignant behavior. It is crucial to provide a comprehensive summary of the research findings and thoroughly review the existing literature pertaining to this rare disease.展开更多
BACKGROUND Enhanced recovery after surgery(ERAS)programs provide recommendations for an optimized management of patients undergoing surgery.An ERAS program tailored on surgery for retroperitoneal sarcomas(RPS)may impr...BACKGROUND Enhanced recovery after surgery(ERAS)programs provide recommendations for an optimized management of patients undergoing surgery.An ERAS program tailored on surgery for retroperitoneal sarcomas(RPS)may improve patient outcomes and it has still not been established.AIM To determine how an ERAS program tailored to RPS surgery can be agreed upon,structured,and implemented.METHODS Twenty-five candidate items from existing ERAS programs,potentially relevant for RPS surgery,were identified via literature review and expert input.These were included in a questionnaire refined through cognitive interviews and pilot testing.Expert sarcoma surgeons rated each item’s relevance and feasibility on a 6-point scale.The survey was recirculated after one year.Intra-observer reproducibility,inter-observer concordance,and agreement with the modal value of the most experienced participants were analyzed.RESULTS Thirteen sarcoma surgeons from 6 centers participated in the survey.Although surgeons agreed on several items,their overall concordance was low.After recirculating the survey,the intraobserver reproducibility was low.Interestingly,the median concordance with the reference increased for relevance and decreased for feasibility.CONCLUSION Despite interest in ERAS for RPS,surgeon concordance on item relevance and feasibility remains low,underscoring the need for collaborative efforts toward a standardized,consensus-based protocol.展开更多
Splenic histiocytic sarcoma(SHS)is a rare,aggressive hematological malignancy with unclear progression and management.Our case illustrates the progression and pathophysiological processes of SHS and provides key data ...Splenic histiocytic sarcoma(SHS)is a rare,aggressive hematological malignancy with unclear progression and management.Our case illustrates the progression and pathophysiological processes of SHS and provides key data for the diagnosis,treatment and management of SHS.A 60-year-old female with incidentally detected splenic mass(6.0 cm×5.7 cm)underwent splenectomy,confirmed as SHS in 2020.Post-op imatinib therapy was given.In 2022,hepatic metastases(2.4 cm×2.9 cm)with pancytopenia led to supportive care.Lesions enlarged to 4.3 cm×2.7 cm,leading to multi-organ failure and death at 33 months.The case was categorized into three distinct stages based on the pathophysiology of SHS:Early-stage splenic tumor growth,mid-stage liver metastasis with hematological abnormalities,and late-stage tumor infiltration leading to multiorgan failure.For SHS,this case highlights the pivotal role of early intervention and the value of personalized treatment strategies.展开更多
Myofibroblastic sarcoma(MS)is a rare malignant soft tissue tumor characterized by myofibroblasts.It most commonly arises in the head and neck region,especially the tongue,with rare occurrences in the limbs.MS exhibits...Myofibroblastic sarcoma(MS)is a rare malignant soft tissue tumor characterized by myofibroblasts.It most commonly arises in the head and neck region,especially the tongue,with rare occurrences in the limbs.MS exhibits varying histopathology,ranging from low-to high-grade,with diverse subtypes showing different clinical behaviors and prognoses.This article reports the first case of high-grade MS in the hand,adding to the limited documentation of this rare condition.Here,we present the case of a 30-year-old healthy female with a year-long history of progressive shortening,mobility loss,and weakness in the first finger of the left hand.Left-hand imaging revealed a lytic,cottony tumor involving the entire first metacarpal.Following surgical resection,which included metatarsal grafting and joint reconstruction,a diagnosis of high-grade MS was confirmed based on histological manifestations and immunohistochemical staining,which was further classified as grade 2 according to the French Federation of Cancer Centers Sarcoma Group system.Postoperative radiotherapy was administered and the patient experienced a successful recovery without graft osteonecrosis.The patient regained 90%mobility and strength,without shortening,after surgical resection and radiotherapy.Six months post-surgery,the patient reported full hand functionality.MS is a rare tumor that infrequently affects bones and is often misdiagnosed owing to its controversial characteristics.The initial treatment should focus on complete resection with negative margins,followed by reconstructive surgery to preserve function.Further case studies are needed to establish standardized surgical treatment protocols.展开更多
Synovial sarcoma is a high-grade soft tissue malignancy characterized by a unique fusion gene known as SS18-SSX.The SS18-SSX fusion protein acts as an oncogenic driver of synovial sarcoma,and it has thus been commonly...Synovial sarcoma is a high-grade soft tissue malignancy characterized by a unique fusion gene known as SS18-SSX.The SS18-SSX fusion protein acts as an oncogenic driver of synovial sarcoma,and it has thus been commonly accepted that disruption of SS18-SSX function represents a therapeutic means of treating synovial sarcoma,but emerging evidence suggests that upon depletion of SS18-SSX,an anti-apoptotic signal surprisingly arises to protect synovial sarcoma cell survival.In this article,we discuss the controversial roles of SS18-SSX’s transcriptional activity in synovial sarcoma biology and outline a synergistic strategy for overcoming the resistance of synovial sarcoma cells to SS18-SSX targeted therapeutics.展开更多
A 63-year-old man was admitted to the hospital with a>1-year history of repeated acid reflux and belching and a 1-month history of an abdominal mass.On admission,the patient was in good condition,and his vital sign...A 63-year-old man was admitted to the hospital with a>1-year history of repeated acid reflux and belching and a 1-month history of an abdominal mass.On admission,the patient was in good condition,and his vital signs were stable.Laboratory examinations revealed no significant abnormalities.展开更多
BACKGROUND Ulcerative colitis(UC)is a chronic inflammatory bowel disease treated with immunosuppressants to control inflammation.Drugs like azathioprine(AZA)and anti-tumor necrosis factor agents increase the risk of e...BACKGROUND Ulcerative colitis(UC)is a chronic inflammatory bowel disease treated with immunosuppressants to control inflammation.Drugs like azathioprine(AZA)and anti-tumor necrosis factor agents increase the risk of extraintestinal malignancies.However,no association has been established between these therapies and endometrial stromal sarcoma.This report presents a rare case of endometrial stromal sarcoma in a patient with UC undergoing immunosuppressive treatment and includes a literature review to explore any possible correlation between the disease,the therapies used,and the development of this rare tumor.CASE SUMMARY Female,49 years old,with UC pancolitis extension since 2017.She used aminosalicylates and AZA with non-response.She started infliximab and AZA combination therapy in 2020,with optimization in 2021 due to endoscopic activity.In the same year,the patient presented to the emergency room with ascitis and underwent diagnostic paracentesis,which showed serum-ascites albumin gradient<1.1 g/dL,absence of neoplastic cells,and abdominal and pelvic tomography reported a hypoechoic nodular lesion in the posterior wall of the uterus and elevated carbohydrate antigen 125.Given the suspicion of neoplasia,the suspension of immunosuppressive therapy was indicated.The patient underwent a total abdominal hysterectomy and bilateral salpingo-oophorectomy,and the pathological result reported low-grade endometrial sarcoma.It was decided to introduce vedolizumab for the management of UC;however,even after induction therapy,intense clinical and endoscopic disease activity was maintained,with total proctocolectomy being indicated due to clinical refractoriness and a history of neoplasia.CONCLUSION Patients with inflammatory bowel disease have a higher risk of cancer due to inflammation or treatment.Proper screening with multidisciplinary care can improve outcomes.展开更多
文摘Objective： We enrolled retrospective data to determine the efficacy of combined chemotherapy and surgery for local tumour control and survival in patients with high-risk soft-tissue sarcomas. Methods： We collected data from 25 patients with high-risk soft-tissue sarcomas treated with four cycles of etoposide, ifosfamide and doxorubicin （EIA） followed by definitive surgery with or without postoperative radiotherapy and adjuvant chemotherapy. 21 patients received chemotherapy in a neoadjuvant/adjuvant clinical setting; eighteen of them completed adjuvant chemotherapy. Four patients received chemotherapy in an adjuvant setting only. Results： The objective response rate of neoadjuvant chemotherapy assessable in 21 patients was 43%. Including NED （n=7） and partial remissions （n=3）, the radiographic response rate was 47.6% with additional 42.9% stable diseases （n=9）. Surgery was performed in two patients before completing four neoadjuvant chemotherapy cycles because of disease progression. Median overall survival for all patients was 21.6＋ months. After completion of chemotherapy, in 62% of patients R0-resection could be performed. Conclusion： High proportion of R0-resections supports the idea of tumour down-staging after neoadjuvant treatment. Response to neoadjuvant chemotherapy is predictive for improved local tumour control resulting in long-term survival benefit.
文摘Soft tissue sarcomas (STS) are rare neoplasm with frequencies around 1% of all neoplasms. Although it consists of a high heterogeneous group of tumors, surgery is the mean treatment. The STS surgery is still challenging and complex procedure is usually required: this is because STS requires different types of resection and reconstruction due to various tissue-commitment</span><span style="font-family:"">s</span><span style="font-family:""> (nerve, arteries, skin and muscle). So, a multidisciplinary team must be prepared for STS approach to obtain the maximum local control and a limited extremity functional impairment. We, here, showing our experience, wish to introduce some technical contrivances in STS surgery, with special reference to tissue reconstruction. This may illustrate the necessity of a multidisciplinary team approach in this surgery.
基金Institut National du Cancer,from the Association pour la Recherche contre le Cancer and by the Agence Nationale de la Recherche(ANR 21 RHUS 0010).
文摘The majority of sarcomas are under the influence of a tumor microenvironment that dampens immune activity,resulting in resistance to monoclonal antibodies targeting immune checkpoints and reduced clinical effectiveness.Preclinical studies indicate that targeting abnormal neoangiogenesis by inhibiting vascular endothelial growth factor receptor(VEGFR)can alter the TME,thereby promoting T cell infiltration and increasing tumor immunogenicity.The REGOMUNE study,a phase II clinical trial,assessed the therapeutic combination of regorafenib,a multityrosine kinase inhibitor that targets VEGFR2 and the PD-L1 blocker avelumab,in individuals with advanced“cold”STS characterized by a lack of mature tertiary lymphoid structures(mTLS).Forty-nine mTLSnegative STS patients were enrolled,including leiomyosarcoma(45%),synovial sarcoma(18%),and other subtypes.The objective response rate was 11.0%(95%CI:4.0%-22.0%),with median progression-free survival and overall survival of 1.8 months(95%CI,1.7-3.5 months)and 15.1 months,respectively.Frequent adverse events included grade 1 or 2 palmar-plantar erythrodysesthesia,fatigue,and diarrhea.On-treatment multiplex immunofluorescence analysis revealed significant increases in CD8+T cell and B cell infiltration and PD1 expression on immune cells.Plasma analysis indicated significant upregulation of soluble PD-L1(sPD-L1)levels and tryptophan consumption.Overall,these results indicate that anti-angiogenic therapy modulates the tumor microenvironment in patients with cold STS and highlight the need for complementary strategies to enhance the functional activity of immune cells in this particular setting.
基金supported by the French National Research Agency under the FRANCE2030 investment plan(grant application No.ANR-21-RHUS-0005)by the imCORE research network(No.SG40863)+1 种基金The authors would like to thank the collaborators from the histopathology department of Gustave Roussythe Experimental and Translational Pathology(PETRA,AMMICA,INSERM US23/CNRS)。
文摘Immunoradiotherapy holds promise for improving outcomes in patients with advanced solid tumors,including in soft-tissue sarcoma(STS).However,the ideal combination of treatment modalities remains to be determined,and reliable biomarkers to predict which patients will benefit are lacking.Here,we report the results of the STS cohort of the SABR-PDL1 phase Il trial that evaluated the anti-PDL1 atezolizumab combined with stereotactic body radiation therapy(SBRT)delivered concurrently with the 2nd cycle to at least one tumor site,Eligible patients received atezolizumab until progression or unmanageable toxicity,with SBRT at 45 Gy in 3 fractions).The primary endpoint was one-year progression-free survival(PFS)rate with success defined as 13 patients achieving 1-year PFS.Sixty-one heavily pretreated patients with STS(median 5 prior lines;52%men;median age 54 years;28%leiomyosarcoma)were enrolled across two centers(France,Spain).SBRT was delivered to 55 patients(90%),with the lung being the most commonly irradiated site(50%).After a median follow-up of 45 months,the one-year PFS rate was 8.3%[95%Cl:3.6-18.1].Median PFS and overall survival were 2.5 and 8.6 months,respectively.Best responses included partial responses(5%)and stable disease(60%).Immune profiling revealed increased immunosuppressive tumor-associated macrophages(e.g,IL4l1,HES1)and monocyte-recruiting chemokines in non-responders.Higher monocyte/lymphocyte ratios(MonoLR)in tumor and blood correlated with progression.PD-L1 status,lymphoid infiltration,and tertiary-lymphoid structures were not predictive.Although the primary endpoint was not met,this study highlights MonoLR imbalance as a potential biomarker to identify STS patients likely to benefit from immunoradiotherapy.EudraCT No.2015-005464-42;Clinicaltrial.gov number:NCT02992912.
文摘Alveolar soft part sarcoma （ASPS） is a rare tumor that was originally named by Christopherson in 1952.Until now, fewer than 200 cases have been described in the literature. The ASPS on the uterine cervix is a very rare malignant tumor of the female reproductive organs. Including the first case described by Flint et al2 in 1985, there have been only a few cases reported to date, of which two were in China, and there is a lack of insightful analysis of treatment options. Therefore, to raise awareness of this disease, we performed a detailed literature review, which includes a case encountered by ourselves.
基金Supported by the Ministry of Health of the Czech Republic,Grant/Award Number:NU22-05-00475 and NV19-05-00214。
文摘Background:In view of the ever-increasing representation of Staphylococcus spp.strains resistant to various antibiotics,the development of in vivo models for evaluation of novel antimicrobials is of utmost importance.Methods:In this article,we describe the development of a fully immunocompetent porcine model of extensive skin and soft tissue damage suitable for testing topical anti-microbial agents that matches the real clinical situation.The model was developed in three consecutive stages with protocols for each stage amended based on the results of the previous one.Results:In the final model,10 excisions of the skin and underlying soft tissue were created in each pig under general anesthesia,with additional incisions to the fascia performed at the base of the defects and immediately inoculated with Staphylococcus aureus suspension.One pig was not inoculated and used as the negative control.Subsequently,the bandages were changed on Days 4,8,11,and 15.At these time points,a filter paper imprint technique(FPIT)was made from each wound for semi-quantitative microbiological evaluation.Tissue samples from the base of the wound together with the adjacent intact tissue of three randomly selected defects of each pig were taken for microbiological,histopathological,and molecular-biological examination.The infection with the inoculated S.aureus strains was sufficient during the whole experiment as confirmed by both FPIT and from tissue samples.The dynamics of the inflammatory markers and clinical signs of infection are also described.Conclusions:A successfully developed porcine model is suitable for in vivo testing of novel short-acting topical antimicrobial agents.
文摘Dear Editor,Surgery remains the cornerstone of treatment for softtissue sarcomas(STS)[1].However,despite adequate locoregional treatment,30%-40%of the patients eventually develop metastases[2,3].Accurate risk assessment is crucial to tailor therapeutic strategies and to identify high-risk patients who may benefit from perioperative chemotherapy.Hence,several efforts have been made to develop prognostic nomograms that enable individual prognosis prediction;representing an important decision-making aid for oncologists and surgeons involved in sarcoma care[4-6].
基金MSD(Merck Sharp and Dohme)AVENIR.Grant Number:HEART。
文摘To the editor Soft-tissue sarcomas(STS)represent a very heterogeneous group of rare tumors including more than 100 different subtypes[1].Surgery and neo/adjuvant radiation therapy represent the cornerstone of treatment for STS.However,despite an optimal resection of the tumor,up to 40%of patients will develop metastatic relapse and will die from the disease[1].Doxorubicin represents the first-line standard of care for patients with advanced disease since the 1970s,despite several attempts to identify better regimens.The median overall survival(OS)of patients with metastatic disease is<18 months and has only modestly improved over the past 20 years[2].
文摘Diagnosis of extremity soft-tissue tumors can be challenging.Characteristics of tumor margins can help precisely identify locally aggressive or non-aggressive behavior for surgical planning,but cannot differentiate benign from malignant lesions.Most malignant tumors can have inhomogeneous signals on T2-weighted images.Although a uniform signal on T2-weighted images can be a reliable indication of a benign lesion,a well-defined mass with homogeneous internal signal intensity does not definitively identify a benign lesion.Some common and distinctive soft-tissue lesions can have specific clinical and imaging features allowing a diagnosis without biopsy.These are known as determinate lesions.This illustrative report presents a diagnostic guide for extremity soft-tissue tumors based on tissue signal and morphological characteristics on magnetic resonance images.It is important for clinicians to be familiar with the imaging characteristics of common determinate lesions.
文摘An experimental animal model of malignant soft-tissue tumor was established to investigate the applied value of multi-slice spiral CT perfusion imaging preliminarily. Ten New Zealand white rabbits which were implanted with VX2 tumor in either proximal thigh were subjected to CT plain scan and perfusion scan two weeks later respectively, then the original perfusion images were transmitted to AW4.0 Workstation. The functional maps and perfusion parameters including blood flow （BF）, blood volume （BV）, mean transit time （MTT） and permeability surface （PS） were computed and analyzed. All the values of BF, BV and PS in VX2 soft-tissue tumors were obviously higher while the MTT-values were lower than those in the normal muscular tissues significantly. It was suggested that multi-slice spiral CT perfusion imaging is an accurate, convenient and relatively safe functional imaging technique, and can give a quantitative assessment to angiogenesis and blood perfusion of soft-tissue tumors.
文摘The glenohumeral joint(GHJ)allows for a wide range of motion,but is also particularly vulnerable to episodes of instability.Anterior GHJ instability is especially frequent among young,athletic populations during contact sporting events.Many first time dislocators can be managed non-operatively with a period of immobilization and rehabilitation,however certain patient populations are at higher risk for recurrent instability and may require surgical intervention for adequate stabilization.Determination of the optimal treatment strategy should be made on a case-by-case basis while weighing both patient specific factors and injury patterns(i.e.,bone loss).The purpose of this review is to describe the relevant anatomical stabilizers of the GHJ,risk factors for recurrent instability including bony lesions,indications for arthroscopic vs open surgical management,clinical history and physical examination techniques,imaging modalities,and pearls/pitfalls of arthroscopic soft-tissue stabilization for anterior glenohumeral instability.
文摘Objective: To perform a contrast investigation of multi-slice spiral CT (MSCT) perfusion imaging and pathological findings in VX2 soft-tissue tumor of rabbits, and discuss the applicative value of multi-slice spiral CT perfusion imaging in soft-tissue tumors. Methods: 8 Newzealand white rabbits were implanted with 0.1 ml VX2 tumor tissue suspension in bilateral proximal thighs. 14 days and 21 days later, CT plain scan and perfusion scan were performed on these rabbits respectively, then the images were transmitted to AW4.0 workstation, the functional maps and perfusion parameters including blood flow (BF), blood volume (BV), mean transit time (MTT) and permeability surface (PS) were computed and analyzed. Subsequently, the rabbits were sacrificed, the tumors of which were taken out for pathological examination. The correlation between MSCT functional parametric images and pathological findings was analyzed. Results: All the values of BF, BV and PS of VX2 soft-tissue tumors were obviously higher while the MTT-values were lower than those of the normal muscular tissues significantly (P<0.001). Conclusion: Multi-slice spiral CT perfusion imaging is an accurate, convenient and relatively safe functional imaging technique, which can give a quantitative assessment to blood perfusion and angiogenesis of soft-tissue tumors.
基金Supported by Grant of the Korea Health Technology R&D Project through the Korea Health Industry Development Institute,funded by the Ministry of Health&Welfare,Republic of Korea,No.RS-2022-KH129889.
文摘BACKGROUND The classification of uterine sarcomas is based on distinctive morphological and immunophenotypic characteristics,increasingly supported by molecular genetic diagnostics.Data on neurotrophic tyrosine receptor kinase(NTRK)gene fusionpositive uterine sarcoma,potentially aggressive and morphologically similar to fibrosarcoma,are limited due to its recent recognition.Pan-TRK immunohistochemistry(IHC)analysis serves as an effective screening tool with high sensitivity and specificity for NTRK-fusion malignancies.CASE SUMMARY We report a case of a malignant mesenchymal tumor originating from the uterine cervix,which was pan-TRK IHC-positive but lacked NTRK gene fusions,accompanied by a brief literature review.A 55-year-old woman presented to the emergency department with abdominal pain and distension,exhibiting significant ascites and multiple solid pelvic masses.Pelvic examination revealed a tumor encompassing the uterine cervix,extending to the vagina and uterine corpus.A punch biopsy of the cervix indicated NTRK sarcoma with positive immunochemical pan-TRK stain.However,subsequent next generation sequencing revealed no NTRK gene fusion,leading to a diagnosis of poorly differentiated,advanced-stage sarcoma.CONCLUSION The clinical significance of NTRK gene fusion lies in potential treatment with TRK inhibitors for positive sarcomas.Identifying such rare tumors is crucial due to the potential applicability of tropomyosin receptor kinase inhibitor treatment.
文摘Background: The low-grade fibromyxoid sarcoma (LGFMS) is an exceptionally uncommon sarcoma that primarily manifests in the extremities or trunk of young adults, presenting as painless lesions. The histological features of this tumor are benign, but it exhibits an exceptionally high rate of late recurrence and a significant potential for metastasis. Imaging examinations serve as a crucial method for detecting LGFMS, while the definitive diagnosis relies on histopathological assessment. Currently, the primary treatment modality for this neoplasm is surgical resection. Early aggressive surgery with negative margins is a critical factor in mitigating the risk of tumor recurrence and metastasis. The present study presented a case of LGFMS located in the right thigh. The patient underwent a mass resection procedure following an MRI examination. During the telephone follow-up one year post-surgery, despite the absence of an imaging review, the surgical site demonstrated satisfactory recovery with no reported abnormal symptoms. Case Presentation: The patient, a 31-year-old male, presented to our hospital for evaluation of an asymptomatic mass in his right thigh that was incidentally discovered 13 years ago. The MRI showed a well-defined mass measuring 8.2 cm × 6.8 cm × 9.6 cm in the right thigh. The tumor signals exhibit a mixed pattern, characterized by predominantly isointense and hypointense signals on T1-weighted imaging (T1WI), a central area of hyperintensity on T2-weighted imaging (T2WI), and peripheral circular enhancement observed on contrast-enhanced scans. The patient underwent surgical resection. Microscopically, the mass was composed of intricately interwoven fibrous matrix and a distinct mucoid region. The tumor cells exhibited a distinctive arrangement in a swirling or wheel-like pattern, with minimal variation in their karyotypic characteristics. The immunohistochemical examination revealed diffuse and intense MUC4 positivity in the tumor cells. The diagnosis of LGFMS was confirmed by post-operative histopathological examination. Conclusions: The LGFMS is an exceptionally uncommon mesenchymal tumor renowned for its benign histological manifestations and malignant behavior. It is crucial to provide a comprehensive summary of the research findings and thoroughly review the existing literature pertaining to this rare disease.
文摘BACKGROUND Enhanced recovery after surgery(ERAS)programs provide recommendations for an optimized management of patients undergoing surgery.An ERAS program tailored on surgery for retroperitoneal sarcomas(RPS)may improve patient outcomes and it has still not been established.AIM To determine how an ERAS program tailored to RPS surgery can be agreed upon,structured,and implemented.METHODS Twenty-five candidate items from existing ERAS programs,potentially relevant for RPS surgery,were identified via literature review and expert input.These were included in a questionnaire refined through cognitive interviews and pilot testing.Expert sarcoma surgeons rated each item’s relevance and feasibility on a 6-point scale.The survey was recirculated after one year.Intra-observer reproducibility,inter-observer concordance,and agreement with the modal value of the most experienced participants were analyzed.RESULTS Thirteen sarcoma surgeons from 6 centers participated in the survey.Although surgeons agreed on several items,their overall concordance was low.After recirculating the survey,the intraobserver reproducibility was low.Interestingly,the median concordance with the reference increased for relevance and decreased for feasibility.CONCLUSION Despite interest in ERAS for RPS,surgeon concordance on item relevance and feasibility remains low,underscoring the need for collaborative efforts toward a standardized,consensus-based protocol.
基金Supported by the Program of General Hospital of Western Theater,No.2021-XZYG-C33.
文摘Splenic histiocytic sarcoma(SHS)is a rare,aggressive hematological malignancy with unclear progression and management.Our case illustrates the progression and pathophysiological processes of SHS and provides key data for the diagnosis,treatment and management of SHS.A 60-year-old female with incidentally detected splenic mass(6.0 cm×5.7 cm)underwent splenectomy,confirmed as SHS in 2020.Post-op imatinib therapy was given.In 2022,hepatic metastases(2.4 cm×2.9 cm)with pancytopenia led to supportive care.Lesions enlarged to 4.3 cm×2.7 cm,leading to multi-organ failure and death at 33 months.The case was categorized into three distinct stages based on the pathophysiology of SHS:Early-stage splenic tumor growth,mid-stage liver metastasis with hematological abnormalities,and late-stage tumor infiltration leading to multiorgan failure.For SHS,this case highlights the pivotal role of early intervention and the value of personalized treatment strategies.
文摘Myofibroblastic sarcoma(MS)is a rare malignant soft tissue tumor characterized by myofibroblasts.It most commonly arises in the head and neck region,especially the tongue,with rare occurrences in the limbs.MS exhibits varying histopathology,ranging from low-to high-grade,with diverse subtypes showing different clinical behaviors and prognoses.This article reports the first case of high-grade MS in the hand,adding to the limited documentation of this rare condition.Here,we present the case of a 30-year-old healthy female with a year-long history of progressive shortening,mobility loss,and weakness in the first finger of the left hand.Left-hand imaging revealed a lytic,cottony tumor involving the entire first metacarpal.Following surgical resection,which included metatarsal grafting and joint reconstruction,a diagnosis of high-grade MS was confirmed based on histological manifestations and immunohistochemical staining,which was further classified as grade 2 according to the French Federation of Cancer Centers Sarcoma Group system.Postoperative radiotherapy was administered and the patient experienced a successful recovery without graft osteonecrosis.The patient regained 90%mobility and strength,without shortening,after surgical resection and radiotherapy.Six months post-surgery,the patient reported full hand functionality.MS is a rare tumor that infrequently affects bones and is often misdiagnosed owing to its controversial characteristics.The initial treatment should focus on complete resection with negative margins,followed by reconstructive surgery to preserve function.Further case studies are needed to establish standardized surgical treatment protocols.
文摘Synovial sarcoma is a high-grade soft tissue malignancy characterized by a unique fusion gene known as SS18-SSX.The SS18-SSX fusion protein acts as an oncogenic driver of synovial sarcoma,and it has thus been commonly accepted that disruption of SS18-SSX function represents a therapeutic means of treating synovial sarcoma,but emerging evidence suggests that upon depletion of SS18-SSX,an anti-apoptotic signal surprisingly arises to protect synovial sarcoma cell survival.In this article,we discuss the controversial roles of SS18-SSX’s transcriptional activity in synovial sarcoma biology and outline a synergistic strategy for overcoming the resistance of synovial sarcoma cells to SS18-SSX targeted therapeutics.
基金supported by the National Natural Science Foundation of China(Project No.82160348)the Yunnan Province Major Special Plan(No.202302AA310018-D-8)+1 种基金the Youth Talent Project of Yunnan Province's“Xingdian Talent Support Program”(No.XDYC-QNRC-2022-0608)the 2024 Senior Health Technology and Medical Discipline Leader of Yunnan Provincial Health Commission(No.D-2024056).
文摘A 63-year-old man was admitted to the hospital with a>1-year history of repeated acid reflux and belching and a 1-month history of an abdominal mass.On admission,the patient was in good condition,and his vital signs were stable.Laboratory examinations revealed no significant abnormalities.
文摘BACKGROUND Ulcerative colitis(UC)is a chronic inflammatory bowel disease treated with immunosuppressants to control inflammation.Drugs like azathioprine(AZA)and anti-tumor necrosis factor agents increase the risk of extraintestinal malignancies.However,no association has been established between these therapies and endometrial stromal sarcoma.This report presents a rare case of endometrial stromal sarcoma in a patient with UC undergoing immunosuppressive treatment and includes a literature review to explore any possible correlation between the disease,the therapies used,and the development of this rare tumor.CASE SUMMARY Female,49 years old,with UC pancolitis extension since 2017.She used aminosalicylates and AZA with non-response.She started infliximab and AZA combination therapy in 2020,with optimization in 2021 due to endoscopic activity.In the same year,the patient presented to the emergency room with ascitis and underwent diagnostic paracentesis,which showed serum-ascites albumin gradient<1.1 g/dL,absence of neoplastic cells,and abdominal and pelvic tomography reported a hypoechoic nodular lesion in the posterior wall of the uterus and elevated carbohydrate antigen 125.Given the suspicion of neoplasia,the suspension of immunosuppressive therapy was indicated.The patient underwent a total abdominal hysterectomy and bilateral salpingo-oophorectomy,and the pathological result reported low-grade endometrial sarcoma.It was decided to introduce vedolizumab for the management of UC;however,even after induction therapy,intense clinical and endoscopic disease activity was maintained,with total proctocolectomy being indicated due to clinical refractoriness and a history of neoplasia.CONCLUSION Patients with inflammatory bowel disease have a higher risk of cancer due to inflammation or treatment.Proper screening with multidisciplinary care can improve outcomes.
