BACKGROUND Primary intracranial alveolar soft-part sarcoma(PIASPS)is a rare malignancy.We aimed to investigate the clinical profiles and outcomes for PIASPS.CASE SUMMARY We firstly reported five consecutive cases from...BACKGROUND Primary intracranial alveolar soft-part sarcoma(PIASPS)is a rare malignancy.We aimed to investigate the clinical profiles and outcomes for PIASPS.CASE SUMMARY We firstly reported five consecutive cases from our institute.Then,the cases from previous studies were pooled and analyzed to delineate the characteristics of this disease.Our cohort included two males and three females.The median age was 21-years-old(range:8-54-years-old).All the patients received surgical treatment.Gross total resection(GTR),radiotherapy,and chemotherapy were administered in 3 patients,4 patients,and 1 patient,respectively.After a median follow-up of 36 mo,tumor progression was noticed in 4 patients;and 3 patients died of the disease.Pooled data(n=14)contained 5 males and 9 females with a median age of 19 years.The log-rank tests showed that GTR(P=0.011)could prolong progression-free survival,and radiotherapy(P<0.001)resulted in longer overall survival.CONCLUSION Patients with PIASPS suffer from poor outcomes.Surgical treatment is the first choice,and GTR should be achieved when the tumor is feasible.Patients with PIASPS benefit from radiotherapy,which should be considered as a part of treatment therapies.展开更多
BACKGROUND Alveolar soft part sarcoma(ASPS)is an extremely rare malignant sarcoma,accounting for less than 1%of all soft-tissue sarcomas.However,limited information is available on multimodal imaging[computed tomograp...BACKGROUND Alveolar soft part sarcoma(ASPS)is an extremely rare malignant sarcoma,accounting for less than 1%of all soft-tissue sarcomas.However,limited information is available on multimodal imaging[computed tomography(CT),magnetic resonance imaging(MRI),and positron emission computed tomography/computed tomography(PET/CT)]of ASPS.CASE SUMMARY This study reports a case of a 35-year-old female patient with ASPS of the left thigh with lung metastasis.The patient presented with a 1-year history of a palpable mass in the lower extremity,which exhibited rapid growth for 3 wk.CT,MRI,and F-deoxyglucose PET/CT examinations were performed.CT showed a slightly hypodense or isodense mass with patchy calcifications.On MRI examination,the mass manifested hyperintensity on T1-weighted,T2-weighted,and diffusion-weighted images with some signal voids.PET/CT images demonstrated an intensely hypermetabolic mass in the left thigh and hypermetabolic nodules in lungs.CONCLUSION ASPS should be considered as a possible diagnosis when a slow-growing mass is detected in the soft tissue of the extremities,with hyperintensity and numerous signal voids on T1-weighted,T2-weighted,and diffusion-weighted images and intense F-deoxyglucose uptake on PET/CT.ASPS can have calcifications on CT.展开更多
Objective The aim of this study was to analyze the imaging features of alveolar soft part sarcoma （ASPS）. Methods The imaging features of 11 cases with ASPS were retrospectively analyzed. Results ASPS mainly exhibit...Objective The aim of this study was to analyze the imaging features of alveolar soft part sarcoma （ASPS）. Methods The imaging features of 11 cases with ASPS were retrospectively analyzed. Results ASPS mainly exhibited an isointense or slightly high signal intensity on Tl-weighted imaging （TlWl）, and a mixed high signal on T2-weighted imaging （T2Wl）. ASPS was partial, with rich tortuous flow voids, or ＂line-like＂ low signal septa. The essence of the mass was heterogeneous enhancement. The 1 H- MRS showed a slight choline peak at 3.2 ppm. Conclusion The well-circumscribed mass and blood voids, combined with ＂line-like＂ low signals play a significant role in diagnosis. The choline peak and the other signs may be auxiliary diagnoses.展开更多
Alveolar soft-part sarcoma (ASPS) is an uncommon, aggressive soft-tissue neoplasm, occurring in less than 1% of sarcomas. It is typically found in the head and neck tissues in children or in the deep soft tissues of t...Alveolar soft-part sarcoma (ASPS) is an uncommon, aggressive soft-tissue neoplasm, occurring in less than 1% of sarcomas. It is typically found in the head and neck tissues in children or in the deep soft tissues of the lower extremities in young adults. This tumor arises primarily within the skeletal muscles or musculofascial planes. Primary involvement of bone is extremely rare. ASPS has poor prognosis with a propensity for recurrence as well as distant metastasis. In this report, we present a 25-year-old male with primary ASPS in the left scapular bone and discuss the histopathological and immunohistochemical features of this sarcoma.展开更多
Soft alveolar sarcoma is a rare tumor (0.5% to 1% of soft-tissue sarcomas) of poor prognosis that often affects the young subject, with a moderate male predominance. The authors present the diagnostic and therapeutic ...Soft alveolar sarcoma is a rare tumor (0.5% to 1% of soft-tissue sarcomas) of poor prognosis that often affects the young subject, with a moderate male predominance. The authors present the diagnostic and therapeutic course of a 60-year-old patient from Niger with no particular previous history, consulted for a painless swelling of the right arm evolving for 6 months, in which the diagnosis of alveolar sarcoma of the soft parts of the arm was selected. A monobloc surgical excision decision was made, with the anatomy and function of the limb retained, with the filling of the area resected with cement and fixed by a locked nail. A conventional three-course chemotherapy regimen was instituted. Alveolar sarcoma of the soft tissues is a rare tumor whose imagery remains unclear, but the diagnosis is based on surgical excision with histopathological examination and immunohistochemical analysis.展开更多
Alveolar soft part sarcoma（ASPS）of the orbit is exceedingly rare and little is known regarding its radiologic features.Here,we reviewed the CT and MRI findings of one case of ASPS of the orbit with emphasis on its s...Alveolar soft part sarcoma（ASPS）of the orbit is exceedingly rare and little is known regarding its radiologic features.Here,we reviewed the CT and MRI findings of one case of ASPS of the orbit with emphasis on its salient imaging features.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
背景与目的:腺泡状软组织肉瘤(alveolar soft part sarcoma,ASPS@是一种罕见的恶性软组织肿瘤,生长缓慢,无典型临床症状,常伴有远处转移(肺转移,脑转移及骨转移等@。本文旨在研究腺泡状软组织肉瘤的临床特点,评估其预后因素。方法:回顾...背景与目的:腺泡状软组织肉瘤(alveolar soft part sarcoma,ASPS@是一种罕见的恶性软组织肿瘤,生长缓慢,无典型临床症状,常伴有远处转移(肺转移,脑转移及骨转移等@。本文旨在研究腺泡状软组织肉瘤的临床特点,评估其预后因素。方法:回顾性研究了2003年1月-2008年12月就诊于北京大学人民医院骨与软组织肿瘤治疗中心的15例腺泡状软组织肉瘤患者的临床资料,并随访了他们的预后情况。15例患者中,女性9例,男性6例;小于或等于30岁者8例,大于30岁7例;肿瘤大于5cm者11例,小于5cm者4例;原发于软组织12例,原发于骨骼3例;未见其他部位转移6例,肺转移6例,骨转移3例,脑转移2例;术后局部复发2例;术后9例接受放疗,6例未接受放疗;术后接受化疗11例,未接受化疗4例,化疗方案MAID:多柔比星,氮唏米胺及异环磷酰胺。结果:患者预后情况较差,其中无瘤生存6例,带瘤生存4例,死亡5例,中位生存时间为42.0个月,5年生存率为41.7%。伴有远处转移(肺转移、脑转移及骨转移@的患者5年生存率远低于整体生存率。患者个人性别、年龄对预后无显著性差异。肿瘤大小、部位及有无远处转移是影响预后的重要因素。结论:腺泡状软组织肉瘤是高度恶性软组织肿瘤。尽早评估病情,制定合适的治疗方案,原发病灶采取广泛切除,以降低复发及转移风险。但由于本组数据较少,放化疗是否有效还需进一步研究。展开更多
目的:探讨颅骨腺泡状软组织肉瘤(Alveolar soft part sarcoma,ASPS)的影像学特征。资料与方法:回顾性分析8例经手术病理证实的颅骨ASPS的CT(n=8)、MRI(n=8)及MR静脉造影(MRV)(n=2)表现。结果:8例ASPS均以推压头皮软组织向外生长为主,向...目的:探讨颅骨腺泡状软组织肉瘤(Alveolar soft part sarcoma,ASPS)的影像学特征。资料与方法:回顾性分析8例经手术病理证实的颅骨ASPS的CT(n=8)、MRI(n=8)及MR静脉造影(MRV)(n=2)表现。结果:8例ASPS均以推压头皮软组织向外生长为主,向内则与硬脑膜分界不清。CT表现:6例原发性APSP呈溶骨性骨质破坏,见不均匀密度肿块,含斑片状残留骨质,边缘示不完整骨性薄壳;2例继发性ASPS呈溶骨性骨质破坏,肿块密度较均匀。MRI表现:6例原发性ASPS外观呈"蘑菇征",呈以等T_1、长T_2、高DWI信号为主的混杂信号,内部可见片状T_2WI高信号(n=6)、点条状低信号(n=4)或簇状血管影(n=2),MR增强示明显不均匀强化,并于肿瘤两侧出现"硬膜尾征";2例继发性ASPS呈卵圆形,呈均匀等T_1、等T_2信号,明显均匀强化;6例原发性ASPS的ADC值平均为2.19×10^(-4)mm^2/s,2例继发性ASPS的ADC值平均为1.76×10^(-4) mm^2/s。MRV表现:上矢状窦受压,肿块内见大量引流静脉。结论:颅骨ASPS具有特征性的影像表现,结合其临床、病理特征,有助于本病的诊断。展开更多
目的探讨腺泡状软组织肉瘤（alveolar soft part sarcoma,ASPS）的临床病理、免疫表型以及分子遗传学特征。方法对16例ASPS进行临床病理学、细胞化学和免疫表型观察,其中2例行FISH检测。结果 16例ASPS中男性6例,女性10例,发病年龄8~58...目的探讨腺泡状软组织肉瘤（alveolar soft part sarcoma,ASPS）的临床病理、免疫表型以及分子遗传学特征。方法对16例ASPS进行临床病理学、细胞化学和免疫表型观察,其中2例行FISH检测。结果 16例ASPS中男性6例,女性10例,发病年龄8~58岁（中位年龄31.7岁）,临床表现为局部缓慢生长的肿块。肿瘤发生部位包括四肢、肩背部、舌、声带、肺、子宫颈、输尿管。镜下见典型的器官样或腺泡状结构,形成窦隙状血管及纤维间隔,肿瘤胞质内含丰富嗜酸性颗粒。PAS染色显示肿瘤细胞内可有结晶体形成,免疫组化标记肿瘤细胞TFE3阳性,FISH检测肿瘤细胞存在ASPL-TFE3基因融合。结论 ASPS好发于青少年,肿瘤好发部位为四肢,少见部位（舌、声带、子宫颈、输尿管等）发生的ASPS易被误诊为上皮性恶性肿瘤。病理诊断时需与原发或转移性腺癌、副神经节瘤鉴别。肿瘤细胞形成典型的腺泡状结构并且免疫组化标记TFE3和组织蛋白酶K对诊断有意义,诊断时需结合ASPL-TFE3基因融合检测技术。展开更多
文摘BACKGROUND Primary intracranial alveolar soft-part sarcoma(PIASPS)is a rare malignancy.We aimed to investigate the clinical profiles and outcomes for PIASPS.CASE SUMMARY We firstly reported five consecutive cases from our institute.Then,the cases from previous studies were pooled and analyzed to delineate the characteristics of this disease.Our cohort included two males and three females.The median age was 21-years-old(range:8-54-years-old).All the patients received surgical treatment.Gross total resection(GTR),radiotherapy,and chemotherapy were administered in 3 patients,4 patients,and 1 patient,respectively.After a median follow-up of 36 mo,tumor progression was noticed in 4 patients;and 3 patients died of the disease.Pooled data(n=14)contained 5 males and 9 females with a median age of 19 years.The log-rank tests showed that GTR(P=0.011)could prolong progression-free survival,and radiotherapy(P<0.001)resulted in longer overall survival.CONCLUSION Patients with PIASPS suffer from poor outcomes.Surgical treatment is the first choice,and GTR should be achieved when the tumor is feasible.Patients with PIASPS benefit from radiotherapy,which should be considered as a part of treatment therapies.
文摘BACKGROUND Alveolar soft part sarcoma(ASPS)is an extremely rare malignant sarcoma,accounting for less than 1%of all soft-tissue sarcomas.However,limited information is available on multimodal imaging[computed tomography(CT),magnetic resonance imaging(MRI),and positron emission computed tomography/computed tomography(PET/CT)]of ASPS.CASE SUMMARY This study reports a case of a 35-year-old female patient with ASPS of the left thigh with lung metastasis.The patient presented with a 1-year history of a palpable mass in the lower extremity,which exhibited rapid growth for 3 wk.CT,MRI,and F-deoxyglucose PET/CT examinations were performed.CT showed a slightly hypodense or isodense mass with patchy calcifications.On MRI examination,the mass manifested hyperintensity on T1-weighted,T2-weighted,and diffusion-weighted images with some signal voids.PET/CT images demonstrated an intensely hypermetabolic mass in the left thigh and hypermetabolic nodules in lungs.CONCLUSION ASPS should be considered as a possible diagnosis when a slow-growing mass is detected in the soft tissue of the extremities,with hyperintensity and numerous signal voids on T1-weighted,T2-weighted,and diffusion-weighted images and intense F-deoxyglucose uptake on PET/CT.ASPS can have calcifications on CT.
基金Supported by a grant from the National Scientific foundation of China(No.81320108013,31170899)
文摘Objective The aim of this study was to analyze the imaging features of alveolar soft part sarcoma （ASPS）. Methods The imaging features of 11 cases with ASPS were retrospectively analyzed. Results ASPS mainly exhibited an isointense or slightly high signal intensity on Tl-weighted imaging （TlWl）, and a mixed high signal on T2-weighted imaging （T2Wl）. ASPS was partial, with rich tortuous flow voids, or ＂line-like＂ low signal septa. The essence of the mass was heterogeneous enhancement. The 1 H- MRS showed a slight choline peak at 3.2 ppm. Conclusion The well-circumscribed mass and blood voids, combined with ＂line-like＂ low signals play a significant role in diagnosis. The choline peak and the other signs may be auxiliary diagnoses.
文摘Alveolar soft-part sarcoma (ASPS) is an uncommon, aggressive soft-tissue neoplasm, occurring in less than 1% of sarcomas. It is typically found in the head and neck tissues in children or in the deep soft tissues of the lower extremities in young adults. This tumor arises primarily within the skeletal muscles or musculofascial planes. Primary involvement of bone is extremely rare. ASPS has poor prognosis with a propensity for recurrence as well as distant metastasis. In this report, we present a 25-year-old male with primary ASPS in the left scapular bone and discuss the histopathological and immunohistochemical features of this sarcoma.
文摘Soft alveolar sarcoma is a rare tumor (0.5% to 1% of soft-tissue sarcomas) of poor prognosis that often affects the young subject, with a moderate male predominance. The authors present the diagnostic and therapeutic course of a 60-year-old patient from Niger with no particular previous history, consulted for a painless swelling of the right arm evolving for 6 months, in which the diagnosis of alveolar sarcoma of the soft parts of the arm was selected. A monobloc surgical excision decision was made, with the anatomy and function of the limb retained, with the filling of the area resected with cement and fixed by a locked nail. A conventional three-course chemotherapy regimen was instituted. Alveolar sarcoma of the soft tissues is a rare tumor whose imagery remains unclear, but the diagnosis is based on surgical excision with histopathological examination and immunohistochemical analysis.
文摘Alveolar soft part sarcoma（ASPS）of the orbit is exceedingly rare and little is known regarding its radiologic features.Here,we reviewed the CT and MRI findings of one case of ASPS of the orbit with emphasis on its salient imaging features.
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.
文摘背景与目的:腺泡状软组织肉瘤(alveolar soft part sarcoma,ASPS@是一种罕见的恶性软组织肿瘤,生长缓慢,无典型临床症状,常伴有远处转移(肺转移,脑转移及骨转移等@。本文旨在研究腺泡状软组织肉瘤的临床特点,评估其预后因素。方法:回顾性研究了2003年1月-2008年12月就诊于北京大学人民医院骨与软组织肿瘤治疗中心的15例腺泡状软组织肉瘤患者的临床资料,并随访了他们的预后情况。15例患者中,女性9例,男性6例;小于或等于30岁者8例,大于30岁7例;肿瘤大于5cm者11例,小于5cm者4例;原发于软组织12例,原发于骨骼3例;未见其他部位转移6例,肺转移6例,骨转移3例,脑转移2例;术后局部复发2例;术后9例接受放疗,6例未接受放疗;术后接受化疗11例,未接受化疗4例,化疗方案MAID:多柔比星,氮唏米胺及异环磷酰胺。结果:患者预后情况较差,其中无瘤生存6例,带瘤生存4例,死亡5例,中位生存时间为42.0个月,5年生存率为41.7%。伴有远处转移(肺转移、脑转移及骨转移@的患者5年生存率远低于整体生存率。患者个人性别、年龄对预后无显著性差异。肿瘤大小、部位及有无远处转移是影响预后的重要因素。结论:腺泡状软组织肉瘤是高度恶性软组织肿瘤。尽早评估病情,制定合适的治疗方案,原发病灶采取广泛切除,以降低复发及转移风险。但由于本组数据较少,放化疗是否有效还需进一步研究。
文摘目的:探讨颅骨腺泡状软组织肉瘤(Alveolar soft part sarcoma,ASPS)的影像学特征。资料与方法:回顾性分析8例经手术病理证实的颅骨ASPS的CT(n=8)、MRI(n=8)及MR静脉造影(MRV)(n=2)表现。结果:8例ASPS均以推压头皮软组织向外生长为主,向内则与硬脑膜分界不清。CT表现:6例原发性APSP呈溶骨性骨质破坏,见不均匀密度肿块,含斑片状残留骨质,边缘示不完整骨性薄壳;2例继发性ASPS呈溶骨性骨质破坏,肿块密度较均匀。MRI表现:6例原发性ASPS外观呈"蘑菇征",呈以等T_1、长T_2、高DWI信号为主的混杂信号,内部可见片状T_2WI高信号(n=6)、点条状低信号(n=4)或簇状血管影(n=2),MR增强示明显不均匀强化,并于肿瘤两侧出现"硬膜尾征";2例继发性ASPS呈卵圆形,呈均匀等T_1、等T_2信号,明显均匀强化;6例原发性ASPS的ADC值平均为2.19×10^(-4)mm^2/s,2例继发性ASPS的ADC值平均为1.76×10^(-4) mm^2/s。MRV表现:上矢状窦受压,肿块内见大量引流静脉。结论:颅骨ASPS具有特征性的影像表现,结合其临床、病理特征,有助于本病的诊断。
