AIM:To illustrate clinicopathological features of orbital non-rhabdomyosarcoma soft tissue sarcoma(NRSTS),and to compare the treatment outcome between postoperative radiotherapy(RT) and chemotherapy in a retrospective...AIM:To illustrate clinicopathological features of orbital non-rhabdomyosarcoma soft tissue sarcoma(NRSTS),and to compare the treatment outcome between postoperative radiotherapy(RT) and chemotherapy in a retrospective analysis nearly 20y.METHODS:A retrospective cohort study of 56 patients with orbital NRSTS were reviewed,34 of whom received postoperative RT,and 22 received postoperative chemotherapy.The clinicopathological features,local recurrence,metastases,and survival data were recorded.Survival analysis was performed using the Kaplan-Meier method.RESULTS:During follow-up(111.8mo,ranged 8-233mo) for 56 patients,19 patients of them developed local recurrence,and 7 patients developed distant metastases.Fifteen patients died during follow-up period.Overall survival rates considering the whole study group was 78.57% at 5y,and 72.16% at 10y after the initial diagnosis.Compared with chemotherapy,RT was associated with lower risk of local recurrence [hazard ratio for RT vs chemotherapy,0.263,95% confidence interval(CI),0.095-0.728,P=0.0015];with lower risk of distant metastasis(hazard ratio for RT vs chemotherapy,0.073,95%CI,0.015-0.364,P=0.0014);and with lower risk of death from disease(hazard ratio for RT vs chemotherapy,0.066,95%CI,0.022-0.200,P<0.0001).The 5-year survival rate in RT group was 97.06% compared to 50% in chemotherapy group.CONCLUSION:In patients with orbital NRSTS,postoperative RT provides better control of local recurrence,distant metastasis,and death from disease than chemotherapy.RT is the more preferrable adjuvant therapy compared to chemotherapy possibly.展开更多
Standard treatment for soft tissue sarcoma,based on complete surgical resection with or without adjuvant radiotherapy and chemotherapy,has not substantially changed during the last several decades.Nevertheless,recent ...Standard treatment for soft tissue sarcoma,based on complete surgical resection with or without adjuvant radiotherapy and chemotherapy,has not substantially changed during the last several decades.Nevertheless,recent advances have contributed to considerable improvement in the management of these patients;for example,new magnetic resonance imaging sequences such as diffusionweighted imaging and magnetic resonance imaging radiomics can better assess tumor extension and even estimate its grade.Detection of circulating genetic material(liquid biopsy)and next-generation sequencing are powerful techniques for genetic analysis,which will increase our understanding of the underlying molecular mechanisms and may reveal potential therapeutic targets.The role of chemotherapy in non-metastatic disease is still controversial,and there is a need to identify patients who really benefit from this treatment.Novel chemotherapeutic regimens have entered clinical praxis and can change the outcome of patients with metastatic disease.Advances in radiotherapy have helped decrease local adverse effects and sustain good local control of the disease.The following report provides an updated view of the diagnosis,treatment,and future perspectives on the management of patients with soft tissue sarcomas.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:The aim of this study was to evaluate the efficacy,adverse reaction and survival of MAID protocol as first-line treatment on 137 metastatic soft tissue sarcomas patients.Methods:One hundredand thirty-seven p...Objective:The aim of this study was to evaluate the efficacy,adverse reaction and survival of MAID protocol as first-line treatment on 137 metastatic soft tissue sarcomas patients.Methods:One hundredand thirty-seven patients with metastatic soft tissue sarcoma were given MAID protocol chemotherapy which consists of ifosfamide at a total dose of 8-10 g/m2 in 4-5 days,adriamycin at a dose of 60 mg/m2 on first day and dacarbazine at a dose of 200 mg/m2/d in 4-5 days.Twenty one days were regarded as a cycle and 2-6 cycles were completed.The median cycle number was 4.Results:Four cases had complete remission(CR)(2.9%),thirteen cases achieved partial remission(PR)(9.5%),eighty-eight cases maintained a stable disease(SD)(64.2%),and thirty-two cases had progressive disease(PD)(23.4%).The overall response rate(RR) was 12.4%,disease control rate(DCR) was 76.6%.The progression-free survival(PFS) was 2-14 months and median PFS was 5.00 ± 1.12 months.The overall survival(OS) was 3-19 months and median OS was 8.00 ± 1.32 months.Three months PFS rate was 56.9%,six months PFS rate was 27.2% and one year PFS rate was 7.8%.Three months OS rate was 100.0%,six months OS rate was 41.5% and one year OS rate was 10.7%.The main adverse reactions were bone marrow supression,nausea/vomiting and alopecia.Other adverse reactions were seldom observed.Conclusion:The effect of MAID protocol as first-line treatment on metastatic soft tissue sarcomas patients was exact,the treatment can control disease progression effectively and the side effects can be tolerable.It was obviously very worthy in clinical use as one of the first-line chemotherapy protocols on advanced soft tissue sarcomas.展开更多
We report the case of a 17-year-old boy with a soft tissue aneurysmal bone cyst(STABC) located in the posterior aspect of the right mandible.Conventional radiography revealed no positive findings.On the computed tomog...We report the case of a 17-year-old boy with a soft tissue aneurysmal bone cyst(STABC) located in the posterior aspect of the right mandible.Conventional radiography revealed no positive findings.On the computed tomography scan,the lesion appeared to have a nonuniform intralesional density.Magnetic resonance imaging revealed an abnormal soft tissue masses with cystic component in the superficial part of right mandibular body and angle with intact cortex.Following histopathological examination,fibro-histiocytic proliferation,blood-filled spaces and multinucleated giant cells were seen and the lesion was diagnosed as a STABC.The mass together with underlying bone and periosteum on its periphery was surgically resected under general anesthesia.Thirty-six months after surgery the patient was assessed at outpatient clinic and found no sign of recurrence This may be only the first reported case of the mandible in the English literature of this extremely rare benign tumor occurring in soft tissue.展开更多
BACKGROUND Solitary fibrous tumor(SFT)is a remarkably uncommon mesenchymal tumor.STAT6 level and a combination of clinical,pathological,and molecular features are required to arrive at a proper diagnosis.CASE SUMMARY ...BACKGROUND Solitary fibrous tumor(SFT)is a remarkably uncommon mesenchymal tumor.STAT6 level and a combination of clinical,pathological,and molecular features are required to arrive at a proper diagnosis.CASE SUMMARY In this report,we present an intriguing case involving a 43-year-old woman who initially exhibited symptoms of a bleeding retroperitoneal tumor,initially resembling a gastrointestinal stromal tumor,but later confirmed as an SFT.However,a year later,what was initially believed to be a recurrence of her SFT was instead identified as a desmoid tumor.CONCLUSION Distinguishing SFT from other tumors was pivotal.Correcting misdiagnoses of tumor type initially and of recurrence later was necessary for appropriate treatment of the correct desmoid type.展开更多
目的探讨腱鞘纤维瘤(fibroma of tendon sheath,FTS)的临床病理学特征。方法对39例FTS的临床表现、影像学及病理学特征进行观察。结果男性24例(61.54%),女性15例(38.46%),男女比为1.6∶1,年龄5~69岁,平均34.56岁。74.36%的病例累及上肢...目的探讨腱鞘纤维瘤(fibroma of tendon sheath,FTS)的临床病理学特征。方法对39例FTS的临床表现、影像学及病理学特征进行观察。结果男性24例(61.54%),女性15例(38.46%),男女比为1.6∶1,年龄5~69岁,平均34.56岁。74.36%的病例累及上肢,尤以掌指部为主。临床上,66.67%病例表现为局部无痛性缓慢生长的肿块,33.33%病例伴局部压痛、肿痛症状。10.26%病例曾有局部外伤史,患者术后复发率为14.29%。以MRI为代表的影像学成像显示病变与肌腱、腱鞘或关节囊紧密相连。大体上,病变包膜纤薄或无包膜。镜下病变呈分叶状,每个小叶由稀疏散在的纤维母细胞、裂隙状血管腔隙和大量玻璃样变胶原间质所组成。部分病变形态多样,局部与促结缔组织增生性纤维母细胞瘤、纤维组织细胞瘤或纤维瘤病、结节性筋膜炎及微静脉型血管瘤病变相似。免疫组化示所有成分均表达vimentin,部分梭形及星形细胞表达α-SMA、calponin及CD163,少数病变局灶弱阳性表达β-catenin(胞质)、CD68、MSA及h-Caldesmon,裂隙状腔隙内衬扁平细胞表达CD34、CD31及FⅧRAg,提示为血管性腔隙。结论 FTS是一种相对少见的发生于肌腱、腱鞘或关节滑囊的良性纤维母/肌纤维母细胞增生性病变。典型的FTS多发生于青年人及成年人,以男性居多,好发于上肢,掌指部占多数,少数病变切除后可复发。展开更多
基金Supported by the National Natural Science Foundation of China (No.82171099,No.82000940,No.81970835,No.81800867)the Natural Science Foundation of Shanghai (No.20ZR1409500)。
文摘AIM:To illustrate clinicopathological features of orbital non-rhabdomyosarcoma soft tissue sarcoma(NRSTS),and to compare the treatment outcome between postoperative radiotherapy(RT) and chemotherapy in a retrospective analysis nearly 20y.METHODS:A retrospective cohort study of 56 patients with orbital NRSTS were reviewed,34 of whom received postoperative RT,and 22 received postoperative chemotherapy.The clinicopathological features,local recurrence,metastases,and survival data were recorded.Survival analysis was performed using the Kaplan-Meier method.RESULTS:During follow-up(111.8mo,ranged 8-233mo) for 56 patients,19 patients of them developed local recurrence,and 7 patients developed distant metastases.Fifteen patients died during follow-up period.Overall survival rates considering the whole study group was 78.57% at 5y,and 72.16% at 10y after the initial diagnosis.Compared with chemotherapy,RT was associated with lower risk of local recurrence [hazard ratio for RT vs chemotherapy,0.263,95% confidence interval(CI),0.095-0.728,P=0.0015];with lower risk of distant metastasis(hazard ratio for RT vs chemotherapy,0.073,95%CI,0.015-0.364,P=0.0014);and with lower risk of death from disease(hazard ratio for RT vs chemotherapy,0.066,95%CI,0.022-0.200,P<0.0001).The 5-year survival rate in RT group was 97.06% compared to 50% in chemotherapy group.CONCLUSION:In patients with orbital NRSTS,postoperative RT provides better control of local recurrence,distant metastasis,and death from disease than chemotherapy.RT is the more preferrable adjuvant therapy compared to chemotherapy possibly.
文摘Standard treatment for soft tissue sarcoma,based on complete surgical resection with or without adjuvant radiotherapy and chemotherapy,has not substantially changed during the last several decades.Nevertheless,recent advances have contributed to considerable improvement in the management of these patients;for example,new magnetic resonance imaging sequences such as diffusionweighted imaging and magnetic resonance imaging radiomics can better assess tumor extension and even estimate its grade.Detection of circulating genetic material(liquid biopsy)and next-generation sequencing are powerful techniques for genetic analysis,which will increase our understanding of the underlying molecular mechanisms and may reveal potential therapeutic targets.The role of chemotherapy in non-metastatic disease is still controversial,and there is a need to identify patients who really benefit from this treatment.Novel chemotherapeutic regimens have entered clinical praxis and can change the outcome of patients with metastatic disease.Advances in radiotherapy have helped decrease local adverse effects and sustain good local control of the disease.The following report provides an updated view of the diagnosis,treatment,and future perspectives on the management of patients with soft tissue sarcomas.
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:The aim of this study was to evaluate the efficacy,adverse reaction and survival of MAID protocol as first-line treatment on 137 metastatic soft tissue sarcomas patients.Methods:One hundredand thirty-seven patients with metastatic soft tissue sarcoma were given MAID protocol chemotherapy which consists of ifosfamide at a total dose of 8-10 g/m2 in 4-5 days,adriamycin at a dose of 60 mg/m2 on first day and dacarbazine at a dose of 200 mg/m2/d in 4-5 days.Twenty one days were regarded as a cycle and 2-6 cycles were completed.The median cycle number was 4.Results:Four cases had complete remission(CR)(2.9%),thirteen cases achieved partial remission(PR)(9.5%),eighty-eight cases maintained a stable disease(SD)(64.2%),and thirty-two cases had progressive disease(PD)(23.4%).The overall response rate(RR) was 12.4%,disease control rate(DCR) was 76.6%.The progression-free survival(PFS) was 2-14 months and median PFS was 5.00 ± 1.12 months.The overall survival(OS) was 3-19 months and median OS was 8.00 ± 1.32 months.Three months PFS rate was 56.9%,six months PFS rate was 27.2% and one year PFS rate was 7.8%.Three months OS rate was 100.0%,six months OS rate was 41.5% and one year OS rate was 10.7%.The main adverse reactions were bone marrow supression,nausea/vomiting and alopecia.Other adverse reactions were seldom observed.Conclusion:The effect of MAID protocol as first-line treatment on metastatic soft tissue sarcomas patients was exact,the treatment can control disease progression effectively and the side effects can be tolerable.It was obviously very worthy in clinical use as one of the first-line chemotherapy protocols on advanced soft tissue sarcomas.
文摘We report the case of a 17-year-old boy with a soft tissue aneurysmal bone cyst(STABC) located in the posterior aspect of the right mandible.Conventional radiography revealed no positive findings.On the computed tomography scan,the lesion appeared to have a nonuniform intralesional density.Magnetic resonance imaging revealed an abnormal soft tissue masses with cystic component in the superficial part of right mandibular body and angle with intact cortex.Following histopathological examination,fibro-histiocytic proliferation,blood-filled spaces and multinucleated giant cells were seen and the lesion was diagnosed as a STABC.The mass together with underlying bone and periosteum on its periphery was surgically resected under general anesthesia.Thirty-six months after surgery the patient was assessed at outpatient clinic and found no sign of recurrence This may be only the first reported case of the mandible in the English literature of this extremely rare benign tumor occurring in soft tissue.
文摘BACKGROUND Solitary fibrous tumor(SFT)is a remarkably uncommon mesenchymal tumor.STAT6 level and a combination of clinical,pathological,and molecular features are required to arrive at a proper diagnosis.CASE SUMMARY In this report,we present an intriguing case involving a 43-year-old woman who initially exhibited symptoms of a bleeding retroperitoneal tumor,initially resembling a gastrointestinal stromal tumor,but later confirmed as an SFT.However,a year later,what was initially believed to be a recurrence of her SFT was instead identified as a desmoid tumor.CONCLUSION Distinguishing SFT from other tumors was pivotal.Correcting misdiagnoses of tumor type initially and of recurrence later was necessary for appropriate treatment of the correct desmoid type.
文摘目的探讨腱鞘纤维瘤(fibroma of tendon sheath,FTS)的临床病理学特征。方法对39例FTS的临床表现、影像学及病理学特征进行观察。结果男性24例(61.54%),女性15例(38.46%),男女比为1.6∶1,年龄5~69岁,平均34.56岁。74.36%的病例累及上肢,尤以掌指部为主。临床上,66.67%病例表现为局部无痛性缓慢生长的肿块,33.33%病例伴局部压痛、肿痛症状。10.26%病例曾有局部外伤史,患者术后复发率为14.29%。以MRI为代表的影像学成像显示病变与肌腱、腱鞘或关节囊紧密相连。大体上,病变包膜纤薄或无包膜。镜下病变呈分叶状,每个小叶由稀疏散在的纤维母细胞、裂隙状血管腔隙和大量玻璃样变胶原间质所组成。部分病变形态多样,局部与促结缔组织增生性纤维母细胞瘤、纤维组织细胞瘤或纤维瘤病、结节性筋膜炎及微静脉型血管瘤病变相似。免疫组化示所有成分均表达vimentin,部分梭形及星形细胞表达α-SMA、calponin及CD163,少数病变局灶弱阳性表达β-catenin(胞质)、CD68、MSA及h-Caldesmon,裂隙状腔隙内衬扁平细胞表达CD34、CD31及FⅧRAg,提示为血管性腔隙。结论 FTS是一种相对少见的发生于肌腱、腱鞘或关节滑囊的良性纤维母/肌纤维母细胞增生性病变。典型的FTS多发生于青年人及成年人,以男性居多,好发于上肢,掌指部占多数,少数病变切除后可复发。