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
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
Objective:To examine the efficacy and safety of anlotinib as first-line therapy to treat locally advanced or metastatic soft-tissue sarcoma.Methods:This is a single-arm trial.Treatment-naïve patients(≥14 years)w...Objective:To examine the efficacy and safety of anlotinib as first-line therapy to treat locally advanced or metastatic soft-tissue sarcoma.Methods:This is a single-arm trial.Treatment-naïve patients(≥14 years)with locally advanced or metastatic soft tissue sarcoma were eligible.Each treatment cycle lasted for 3 weeks,and included liposomal doxorubicin(40-50 mg/m^(2))on day 1 and anlotinib(12 mg)on days 8-21.Starting from the 9th cycle,treatment consisted of only anlotinib.Treatment continued until disease progression or intolerable toxicities.The primary efficacy end point was progression-free survival(PFS).Results:Eight patients were enrolled between July 25,2019 and January 8,2020.The median number of treatment cycles was 5.5.Within 5.9 months median follow-up,PFS events occurred in 4(4/8,50%)patients.The median PFS was 11.3 months and the 6-month PFS rate was 56%.No patients attained complete response and 2 patients(fibrosarcoma,1 patient and undifferentiated pleomorphic sarcoma,1 patient)achieved partial response.Three patients(fibrosarcoma,2 patients and synovial sarcoma,1 patient)had stable disease.The objective response rate was 25%(2/8)for the study population,and the disease control rate was 75%(6/8).No new safety concerns emerged.Conclusions:Anlotinib plus liposomal doxorubicin demonstrated antitumor activities in previously untreated locally advanced or metastatic soft tissue sarcomas.Due to the small sample size,further investigations with a larger population should be undertaken to confirm the study findings.展开更多
Low-grade myofibroblastic sarcoma is a recently characterized tumor showing features of myofibroblastic differen-tiation that is part of the spectrum of malignant mesenchymal tumors. This extremely rare type of tumor ...Low-grade myofibroblastic sarcoma is a recently characterized tumor showing features of myofibroblastic differen-tiation that is part of the spectrum of malignant mesenchymal tumors. This extremely rare type of tumor occurs most commonly in superficial locations. The case we describe herein is that of a 60- year-old man with two large hepatic masses. The patient＇s tumor was removed radically through an incision due to the inconclusive imaging findings. Follow-up computed tomography showed no recurrence and metastasis after 37 months, suggesting that enucleation was adequate for tumor eradication. To our knowl-edge, this is the first reported case of low-grade myofibroblastic sarcoma in the liver in the English language literature.展开更多
Importance:As the most common subtype of pediatric rhabdomyosarcoma(RMS),the prognosis of embryonal RMS has rarely been investigated solely.Objective:To perform a population-based study to characterize the prognosis o...Importance:As the most common subtype of pediatric rhabdomyosarcoma(RMS),the prognosis of embryonal RMS has rarely been investigated solely.Objective:To perform a population-based study to characterize the prognosis of embryonal RMS in children and adolescents.Methods:Demographic and clinical features were retrospectively evaluated in selected patients with embryonal RMS registered in the Surveillance,Epidemiology,and End Results(SEER)program from 1988 to 2016.Survival curves were compared using the log-rank test.A multivariate Cox proportional hazards model was developed to assess the impact of each factor on the overall survival.A nomogram was constructed based on the results of Cox regression model.Results:A total of 464 patients were included in the analysis,among which 64.6%were male and 70.2%were white patients.About 38.6%and 26.3%of the patients were at 1-4 years and 5-9 years,respectively.Cox analysis showed that patients at age group 5-9 years had the lowest risk of mortality(hazard ratio[HR],0.277;95%confidential interval[CI],0.123-0.620),compared with patients diagnosed at less than 1-year-old,and age group 1-4 years had the second-best prognosis.Patients having distant tumors had significantly higher mortality risk(HR,4.842;95%CI,2.804-8.362)than the patients with localized tumor.Compared with receiving no surgery or radiotherapy,receiving any combination of surgery and radiotherapy would lower the risk of mortality significantly(for surgery without radiotherapy:HR,0.418;for radiotherapy without surgery:HR,0.405;and for surgery plus radiotherapy:HR,0.410).Interpretation:Age,stage at diagnosis,and treatment received were found to be the most important predictors of the overall survival of pediatric embryonal RMS.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.
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Objective:To examine the efficacy and safety of anlotinib as first-line therapy to treat locally advanced or metastatic soft-tissue sarcoma.Methods:This is a single-arm trial.Treatment-naïve patients(≥14 years)with locally advanced or metastatic soft tissue sarcoma were eligible.Each treatment cycle lasted for 3 weeks,and included liposomal doxorubicin(40-50 mg/m^(2))on day 1 and anlotinib(12 mg)on days 8-21.Starting from the 9th cycle,treatment consisted of only anlotinib.Treatment continued until disease progression or intolerable toxicities.The primary efficacy end point was progression-free survival(PFS).Results:Eight patients were enrolled between July 25,2019 and January 8,2020.The median number of treatment cycles was 5.5.Within 5.9 months median follow-up,PFS events occurred in 4(4/8,50%)patients.The median PFS was 11.3 months and the 6-month PFS rate was 56%.No patients attained complete response and 2 patients(fibrosarcoma,1 patient and undifferentiated pleomorphic sarcoma,1 patient)achieved partial response.Three patients(fibrosarcoma,2 patients and synovial sarcoma,1 patient)had stable disease.The objective response rate was 25%(2/8)for the study population,and the disease control rate was 75%(6/8).No new safety concerns emerged.Conclusions:Anlotinib plus liposomal doxorubicin demonstrated antitumor activities in previously untreated locally advanced or metastatic soft tissue sarcomas.Due to the small sample size,further investigations with a larger population should be undertaken to confirm the study findings.
文摘Low-grade myofibroblastic sarcoma is a recently characterized tumor showing features of myofibroblastic differen-tiation that is part of the spectrum of malignant mesenchymal tumors. This extremely rare type of tumor occurs most commonly in superficial locations. The case we describe herein is that of a 60- year-old man with two large hepatic masses. The patient＇s tumor was removed radically through an incision due to the inconclusive imaging findings. Follow-up computed tomography showed no recurrence and metastasis after 37 months, suggesting that enucleation was adequate for tumor eradication. To our knowl-edge, this is the first reported case of low-grade myofibroblastic sarcoma in the liver in the English language literature.
基金Beihang University&Capital Medical University Advanced Innovation Center for Big Data-Based Precision Medicine Plan(BHME-201801)。
文摘Importance:As the most common subtype of pediatric rhabdomyosarcoma(RMS),the prognosis of embryonal RMS has rarely been investigated solely.Objective:To perform a population-based study to characterize the prognosis of embryonal RMS in children and adolescents.Methods:Demographic and clinical features were retrospectively evaluated in selected patients with embryonal RMS registered in the Surveillance,Epidemiology,and End Results(SEER)program from 1988 to 2016.Survival curves were compared using the log-rank test.A multivariate Cox proportional hazards model was developed to assess the impact of each factor on the overall survival.A nomogram was constructed based on the results of Cox regression model.Results:A total of 464 patients were included in the analysis,among which 64.6%were male and 70.2%were white patients.About 38.6%and 26.3%of the patients were at 1-4 years and 5-9 years,respectively.Cox analysis showed that patients at age group 5-9 years had the lowest risk of mortality(hazard ratio[HR],0.277;95%confidential interval[CI],0.123-0.620),compared with patients diagnosed at less than 1-year-old,and age group 1-4 years had the second-best prognosis.Patients having distant tumors had significantly higher mortality risk(HR,4.842;95%CI,2.804-8.362)than the patients with localized tumor.Compared with receiving no surgery or radiotherapy,receiving any combination of surgery and radiotherapy would lower the risk of mortality significantly(for surgery without radiotherapy:HR,0.418;for radiotherapy without surgery:HR,0.405;and for surgery plus radiotherapy:HR,0.410).Interpretation:Age,stage at diagnosis,and treatment received were found to be the most important predictors of the overall survival of pediatric embryonal RMS.
