背景与目的:腺泡状软组织肉瘤罕见,其影像学表现至今尚未见系统研究,本文旨在探讨腺泡状软组织肉瘤(alveolar soft part sarcomas,ASPS)的临床特征和影像学表现,以提高诊断的准确率。方法:回顾性分析10例经病理证实的腺泡状软组织肉瘤...背景与目的:腺泡状软组织肉瘤罕见,其影像学表现至今尚未见系统研究,本文旨在探讨腺泡状软组织肉瘤(alveolar soft part sarcomas,ASPS)的临床特征和影像学表现,以提高诊断的准确率。方法:回顾性分析10例经病理证实的腺泡状软组织肉瘤的临床特征和影像学表现,所有患者(术前或活检前)均行X线、CT或MR检查。其中,9例行X线平片检查,9例行CT检查,6例行MRI检查。所有切除或活检组织均行HE染色,5例患者有比较完整的免疫组化结果。结果:ASPS发病年龄较轻,80%(8/10)为30岁以下。多表现为无痛性肿块。3例就诊时已有肺转移。70%(7/10)发生于下肢深部软组织以及臀部。另3例分别位于胸壁、颈部及眼眶内。ASPS的CT表现为软组织肿块影,增强后呈明显不均匀强化。MRI表现为T1WI等或略高信号,T2WI高信号,肿瘤内外可见血管流空信号,增强后肿瘤呈不均匀明显强化。镜下ASPS是由嗜伊红色的大多边形上皮样细胞组成,呈特征性的器官样或腺泡状排列,腺泡之间为衬覆单层扁平内皮细胞的裂隙状或血窦样毛细血管网。免疫组化显示3例神经元特异性烯醇化酶(NSE)阳性,两例抗淀粉酶消化染色(PAS)阳性,1例MyoD1横纹肌特异肌调节蛋白阳性(胞质染色),1例Desmin结蛋白阳性。结论:ASPS虽然是少见软组织肉瘤,但影像学很有特点,结合临床、影像和病理表现是诊断的关键。展开更多
Objective: Soft tissue sarcomas(STSs) are rare malignancies deriving from mesenchyme.In this study, we reported the epidemiology of STS in China using population-based cancer registry data.Methods: In 2017, qualified ...Objective: Soft tissue sarcomas(STSs) are rare malignancies deriving from mesenchyme.In this study, we reported the epidemiology of STS in China using population-based cancer registry data.Methods: In 2017, qualified data from 339 cancer registries were included in the national database.All STS cases were retrieved based on the morphological and topographical codes of International Classification of Diseases for Oncology, and were categorized into different histological subtypes and primary sites accordingly.Nationwide new STS cases were estimated using incidence rate of STS and the national population, and were reported for gastrointestinal stromal tumor(GIST) and STSs other than GIST separately by sex and region.Distribution of histological subtypes and primary sites of STS were calculated, as well as primary sites of GIST.Results: Approximately 39,900 new STS cases occurred nationwide in China in 2014, accounting for 1.05% of overall cancer incidence.The crude incidence rate was 2.91/100,000 and generally increased with age.An overall female predilection was found.GIST was the most common histological subtype, followed by nerve sheath tumor and malignant peripheral nerve sheath tumor,leiomyosarcoma, liposarcoma, and fibrosarcoma.About 67.5% of GIST occurred in stomach while 1.4% were recorded outside the gastrointestinal tract.Connective, subcutaneous and other soft tissues were the most common primary site, of which extremities were the major subsite.Conclusions: The burden of STS is not serious in China relatively.However, due to their histological and topographical complexity, STSs should not be unnoticed, and more basic and clinical studies should focus on STSs.展开更多
Background It is important to analyze and compare soft tissue sarcomas periodically so as to update the incidence, the clinical diagnosis, the treatment, and the ongoing research. The present study was conducted to de...Background It is important to analyze and compare soft tissue sarcomas periodically so as to update the incidence, the clinical diagnosis, the treatment, and the ongoing research. The present study was conducted to determine the relative frequency of each type of soft tissue sarcoma.Methods A total of 1118 cases of primary soft tissue sarcomas treated between January 1993 and December 2006 were evaluated in a retrospective analysis.Results According to the pathologic grouping, the diseases with the highest proportion were malignant fibrous histiocytomas (35.24%), synovial sarcomas (17.08%), liposarcomas (16.28%), and rhabdomyosarcomas (12.61%). Soft tissue sarcomas were detected in every age group and occurred in all parts of the body. The number of cases increased gradually over the years.Conclusions Malignant fibrous histiocytomas had the highest frequency among the soft tissue sarcomas. The number of cases increased gradually over the years.展开更多
AIM:To study computed tomography(CT)features of abdominal malignant fibrous histiocytoma(MFH)in various rare locations.METHODS:We retroprospectively identified cases of MFH involving the abdominal cavity.Particular a... AIM:To study computed tomography(CT)features of abdominal malignant fibrous histiocytoma(MFH)in various rare locations.METHODS:We retroprospectively identified cases of MFH involving the abdominal cavity.Particular attention was paid to details regarding imaging features and histological types.RESULTS:The study population consisted of seven men and one woman,with a mean age of 52.5 years.Seven patients had some physical symptoms,while one was incidentally detected.The sites of origin were liver(n=3),greater omentum(n=1),superior mesentery(n=1),ileum(n=1),right psoas muscle(n=1)and right kidney(n=1).With the exception of the ileum lesion,all were of huge size.The contour of the lesions was more or less clear.Foci of necrosis were present in six lesions(n=6).On plain CT scan,all lesions were hypo to iso dense.The lesion in the greater omentum was cystic.One lesion(n=1)showed significant enhancement and the cystic lesion showed mild peripheral enhancement.An abundance of blood vessels surrounding the mass was seen in two lesions(n=2)and both were of the inflammatory variety.Pathological examination revealed storiform-pleomorphic variety(n=4),inflammatory variety(n=3)and myxoid variety(n=1).Two of the patients with inflammatory MFH had a clinical presentation of fever and one was afebrile,however,blood investigations in all three showed leukocytosis.CONCLUSION:Primary MFHs of the abdominal viscera and gastrointestinal tract are generally huge soft tissue masses containing areas of low attenuation and mild to moderate contrast enhancement.展开更多
Pediatric soft tissue sarcomas are a group of malignant neoplasms arising within embryonic mesenchymal tissues during the process of differentiation into muscle, fascia and fat. The tumors have a biphasic peak for age...Pediatric soft tissue sarcomas are a group of malignant neoplasms arising within embryonic mesenchymal tissues during the process of differentiation into muscle, fascia and fat. The tumors have a biphasic peak for age of incidence. Rhabdomyosarcoma(RMS) is diagnosed more frequently in younger children, whereas adult-type non-RMS soft tissue sarcoma is predominately observed in adolescents. The latter group comprises a variety of rare tumors for which diagnosis can be difficult and typically requires special studies, including immunohistochemistry and molecular genetic analysis. Current management for the majority of pediatric sarcomas is based on the data from large multi-institutional trials, which has led to great improvements in outcomes over recent decades. Although surgery remains the mainstay of treatment, the curative aim cannot be achieved without adjuvant treatment. Pre-treatment staging and risk classification are of prime importance in selecting an effective treatment protocol. Tumor resectability, the response to induction chemotherapy, and radiation generally determine the risk-group, and these factors are functions of tumor site, size and biology. Surgery provides the best choice of local control of small resectable tumors in a favorable site. Radiation therapy is added when surgery leaves residual disease or there is evidence of regional spread. Chemotherapy aims to reduce the risk of relapse and improve overall survival. In addition, upfront chemotherapy reduces the aggressiveness of the required surgery and helps preserve organ function in a number of cases. Long-term survival in low-risk sarcomas is feasible, and the intensity of treatment can be reduced. In high-risk sarcoma, current research is allowing more effective disease control.展开更多
Objective: To elucidate the role and prognostic significance of lymphocyte activation-gene-3(LAG-3) in soft tissue sarcoma(STS).Methods: The expression of LAG-3 in patient and matched normal blood samples was analyzed...Objective: To elucidate the role and prognostic significance of lymphocyte activation-gene-3(LAG-3) in soft tissue sarcoma(STS).Methods: The expression of LAG-3 in patient and matched normal blood samples was analyzed by flow cytometry. The localization and prognostic values of LAG-3^+ cells in 163 STS patients were analyzed by immunohistochemistry. In addition, the expression of tumor-infiltrating CD3^+ T, CD4^+ T, and CD8^+ T cells and their role in the prognosis of STS were evaluated by immunohistochemistry. The effect of LAG-3 blockade was evaluated in an immunocompetent MCA205 fibrosarcoma mouse model.Results: Peripheral CD8^+ and CD4^+ T cells from STS patients expressed higher levels of LAG-3 than those from healthy donors.LAG-3 expression in STS was significantly associated with a poor clinical outcome(P = 0.038) and was correlated with high pathological grade(P < 0.001), advanced tumor stage(P = 0.016). Additionally, LAG-3 expression was highly correlated with CD8^+ T-cell infiltration(r = 0.7034, P < 0.001). LAG-3 was expressed in murine tumor-infiltrating lymphocytes, and its blockade decreased tumor growth and enhanced secretion of interferon-gamma by CD8^+ and CD4^+ T cells.Conclusions: LAG-3 blockade may be a promising strategy to improve the effects of targeted therapy in STS.展开更多
文摘背景与目的:腺泡状软组织肉瘤罕见,其影像学表现至今尚未见系统研究,本文旨在探讨腺泡状软组织肉瘤(alveolar soft part sarcomas,ASPS)的临床特征和影像学表现,以提高诊断的准确率。方法:回顾性分析10例经病理证实的腺泡状软组织肉瘤的临床特征和影像学表现,所有患者(术前或活检前)均行X线、CT或MR检查。其中,9例行X线平片检查,9例行CT检查,6例行MRI检查。所有切除或活检组织均行HE染色,5例患者有比较完整的免疫组化结果。结果:ASPS发病年龄较轻,80%(8/10)为30岁以下。多表现为无痛性肿块。3例就诊时已有肺转移。70%(7/10)发生于下肢深部软组织以及臀部。另3例分别位于胸壁、颈部及眼眶内。ASPS的CT表现为软组织肿块影,增强后呈明显不均匀强化。MRI表现为T1WI等或略高信号,T2WI高信号,肿瘤内外可见血管流空信号,增强后肿瘤呈不均匀明显强化。镜下ASPS是由嗜伊红色的大多边形上皮样细胞组成,呈特征性的器官样或腺泡状排列,腺泡之间为衬覆单层扁平内皮细胞的裂隙状或血窦样毛细血管网。免疫组化显示3例神经元特异性烯醇化酶(NSE)阳性,两例抗淀粉酶消化染色(PAS)阳性,1例MyoD1横纹肌特异肌调节蛋白阳性(胞质染色),1例Desmin结蛋白阳性。结论:ASPS虽然是少见软组织肉瘤,但影像学很有特点,结合临床、影像和病理表现是诊断的关键。
基金supported by CAMS Innovation Fund for Medical Sciences (CIFMS) (Grant No.2016-12M-2-004)Ministry of Science and Technology (Grant No.2014FY121100)
文摘Objective: Soft tissue sarcomas(STSs) are rare malignancies deriving from mesenchyme.In this study, we reported the epidemiology of STS in China using population-based cancer registry data.Methods: In 2017, qualified data from 339 cancer registries were included in the national database.All STS cases were retrieved based on the morphological and topographical codes of International Classification of Diseases for Oncology, and were categorized into different histological subtypes and primary sites accordingly.Nationwide new STS cases were estimated using incidence rate of STS and the national population, and were reported for gastrointestinal stromal tumor(GIST) and STSs other than GIST separately by sex and region.Distribution of histological subtypes and primary sites of STS were calculated, as well as primary sites of GIST.Results: Approximately 39,900 new STS cases occurred nationwide in China in 2014, accounting for 1.05% of overall cancer incidence.The crude incidence rate was 2.91/100,000 and generally increased with age.An overall female predilection was found.GIST was the most common histological subtype, followed by nerve sheath tumor and malignant peripheral nerve sheath tumor,leiomyosarcoma, liposarcoma, and fibrosarcoma.About 67.5% of GIST occurred in stomach while 1.4% were recorded outside the gastrointestinal tract.Connective, subcutaneous and other soft tissues were the most common primary site, of which extremities were the major subsite.Conclusions: The burden of STS is not serious in China relatively.However, due to their histological and topographical complexity, STSs should not be unnoticed, and more basic and clinical studies should focus on STSs.
文摘Background It is important to analyze and compare soft tissue sarcomas periodically so as to update the incidence, the clinical diagnosis, the treatment, and the ongoing research. The present study was conducted to determine the relative frequency of each type of soft tissue sarcoma.Methods A total of 1118 cases of primary soft tissue sarcomas treated between January 1993 and December 2006 were evaluated in a retrospective analysis.Results According to the pathologic grouping, the diseases with the highest proportion were malignant fibrous histiocytomas (35.24%), synovial sarcomas (17.08%), liposarcomas (16.28%), and rhabdomyosarcomas (12.61%). Soft tissue sarcomas were detected in every age group and occurred in all parts of the body. The number of cases increased gradually over the years.Conclusions Malignant fibrous histiocytomas had the highest frequency among the soft tissue sarcomas. The number of cases increased gradually over the years.
文摘 AIM:To study computed tomography(CT)features of abdominal malignant fibrous histiocytoma(MFH)in various rare locations.METHODS:We retroprospectively identified cases of MFH involving the abdominal cavity.Particular attention was paid to details regarding imaging features and histological types.RESULTS:The study population consisted of seven men and one woman,with a mean age of 52.5 years.Seven patients had some physical symptoms,while one was incidentally detected.The sites of origin were liver(n=3),greater omentum(n=1),superior mesentery(n=1),ileum(n=1),right psoas muscle(n=1)and right kidney(n=1).With the exception of the ileum lesion,all were of huge size.The contour of the lesions was more or less clear.Foci of necrosis were present in six lesions(n=6).On plain CT scan,all lesions were hypo to iso dense.The lesion in the greater omentum was cystic.One lesion(n=1)showed significant enhancement and the cystic lesion showed mild peripheral enhancement.An abundance of blood vessels surrounding the mass was seen in two lesions(n=2)and both were of the inflammatory variety.Pathological examination revealed storiform-pleomorphic variety(n=4),inflammatory variety(n=3)and myxoid variety(n=1).Two of the patients with inflammatory MFH had a clinical presentation of fever and one was afebrile,however,blood investigations in all three showed leukocytosis.CONCLUSION:Primary MFHs of the abdominal viscera and gastrointestinal tract are generally huge soft tissue masses containing areas of low attenuation and mild to moderate contrast enhancement.
文摘Pediatric soft tissue sarcomas are a group of malignant neoplasms arising within embryonic mesenchymal tissues during the process of differentiation into muscle, fascia and fat. The tumors have a biphasic peak for age of incidence. Rhabdomyosarcoma(RMS) is diagnosed more frequently in younger children, whereas adult-type non-RMS soft tissue sarcoma is predominately observed in adolescents. The latter group comprises a variety of rare tumors for which diagnosis can be difficult and typically requires special studies, including immunohistochemistry and molecular genetic analysis. Current management for the majority of pediatric sarcomas is based on the data from large multi-institutional trials, which has led to great improvements in outcomes over recent decades. Although surgery remains the mainstay of treatment, the curative aim cannot be achieved without adjuvant treatment. Pre-treatment staging and risk classification are of prime importance in selecting an effective treatment protocol. Tumor resectability, the response to induction chemotherapy, and radiation generally determine the risk-group, and these factors are functions of tumor site, size and biology. Surgery provides the best choice of local control of small resectable tumors in a favorable site. Radiation therapy is added when surgery leaves residual disease or there is evidence of regional spread. Chemotherapy aims to reduce the risk of relapse and improve overall survival. In addition, upfront chemotherapy reduces the aggressiveness of the required surgery and helps preserve organ function in a number of cases. Long-term survival in low-risk sarcomas is feasible, and the intensity of treatment can be reduced. In high-risk sarcoma, current research is allowing more effective disease control.
基金supported by grants from the National Key R & D Program of China (Grant No. 2017YFA0505600-04)National Natural Science Foundation of China (Grant No. 81372887, 81572403, and 81772863)
文摘Objective: To elucidate the role and prognostic significance of lymphocyte activation-gene-3(LAG-3) in soft tissue sarcoma(STS).Methods: The expression of LAG-3 in patient and matched normal blood samples was analyzed by flow cytometry. The localization and prognostic values of LAG-3^+ cells in 163 STS patients were analyzed by immunohistochemistry. In addition, the expression of tumor-infiltrating CD3^+ T, CD4^+ T, and CD8^+ T cells and their role in the prognosis of STS were evaluated by immunohistochemistry. The effect of LAG-3 blockade was evaluated in an immunocompetent MCA205 fibrosarcoma mouse model.Results: Peripheral CD8^+ and CD4^+ T cells from STS patients expressed higher levels of LAG-3 than those from healthy donors.LAG-3 expression in STS was significantly associated with a poor clinical outcome(P = 0.038) and was correlated with high pathological grade(P < 0.001), advanced tumor stage(P = 0.016). Additionally, LAG-3 expression was highly correlated with CD8^+ T-cell infiltration(r = 0.7034, P < 0.001). LAG-3 was expressed in murine tumor-infiltrating lymphocytes, and its blockade decreased tumor growth and enhanced secretion of interferon-gamma by CD8^+ and CD4^+ T cells.Conclusions: LAG-3 blockade may be a promising strategy to improve the effects of targeted therapy in STS.