目的:分析儿童肝脏未分化胚胎性肉瘤(undifferentiated embryonal sarcoma of the liver,UESL)的超声、CT及MRI影像学表现,以提高对该病的认识水平。方法:回顾性收集2009年12月至2021年12月首都儿科研究所附属儿童医院经手术及病理证实...目的:分析儿童肝脏未分化胚胎性肉瘤(undifferentiated embryonal sarcoma of the liver,UESL)的超声、CT及MRI影像学表现,以提高对该病的认识水平。方法:回顾性收集2009年12月至2021年12月首都儿科研究所附属儿童医院经手术及病理证实的11例儿童UESL,分析所有患儿的超声、CT及MRI影像表现,总结其影像学特点。结果:病灶均为单发,肿块最大径11.5~19.8 cm。CT平扫显示所有病灶边缘较清楚、其内呈不均匀低密度,内可见厚薄不均的分隔;囊性密度区边缘或分隔周围可见少许片絮样软组织密度影。6例超声图像均提示实性占位,表现为混合回声的实性包块内可见大小不等的无回声区。3例MRI表现为边界清楚肿块影,T1WI呈不均匀混杂低信号,病灶内可见高信号区;T2WI呈混合高信号区和条状低信号区。9例病灶增强扫描动脉期均以边缘强化为主,8例可见迂曲增粗血管影;延迟期病灶呈持续不均匀强化,边缘持续强化,且从外围向中心充盈,强化范围增大,8例延迟期可见假包膜。结论:儿童UESL影像学表现有一定特征性,了解其影像特点并结合临床特征,有助于早期诊断。展开更多
AIM: To evaluate the undifferentiated embryonal sarcoma of liver (UESL) in adults in order to improve its diagnosis and treatment. METHODS: Four primary and one recurrent cases of UESL were clinicopathologically evalu...AIM: To evaluate the undifferentiated embryonal sarcoma of liver (UESL) in adults in order to improve its diagnosis and treatment. METHODS: Four primary and one recurrent cases of UESL were clinicopathologically evaluated and immunohistochemically investigated with a panel of antibodies using the EnVision+ system. Relevant literature about UESL in adults was reviewed. RESULTS: Three males and one female were enrolled in this study. Their chief complaints were abdominal pain, weight loss, or fever. Laboratory tests, imaging and pathological features of UESL in adults were similar to those in children. Immunohistochemistry showed evidence of widely divergent differentiation into mesenchymal and epithelial phenotypes. The survival time of patients who underwent complete tumor resection followed by adjuvant transcatheter arterial chemoembolization (TACE) was significantly longer than that of those who underwent surgical treatment alone.CONCLUSION: UESL in adults may undergo pluripotential differentiation and its diagnosis should be made based on its morphological and immunohistochemical features. Complete tumor resection after adjuvant TACE may improve the survival time of such patients.展开更多
Undifferentiated embryonal sarcoma of the liver （UESL） was first described by Stocker and Ishak in 1978. It is a rare tumor that most often presents in childhood between 6 to 10 years of age.2 The adult patients di...Undifferentiated embryonal sarcoma of the liver （UESL） was first described by Stocker and Ishak in 1978. It is a rare tumor that most often presents in childhood between 6 to 10 years of age.2 The adult patients diagnosed with UESL often show nonspecific symptoms with normal laboratory results. Ultrasonography, CT, or MRI often reveals a cystic mass. Complete surgical resection plus pre- and/or post-operative systemic chemotherapy offers the possibility of a cure. We report a case of primary UESL in an adult with no specific symptoms, tumor markers, or image results. The diagnosis of the case was based on the resulting pathology from surgery.展开更多
目的:探讨肝脏未分化性胚胎性肉瘤(undifferentiated embryonal sarcoma of the liver,UESL)的临床特点,加深对该病的认识,提高术前诊断准确性。方法:回顾性分析16例经手术病理证实、术前误诊UESL患者的临床、影像及病理资料,并对比分...目的:探讨肝脏未分化性胚胎性肉瘤(undifferentiated embryonal sarcoma of the liver,UESL)的临床特点,加深对该病的认识,提高术前诊断准确性。方法:回顾性分析16例经手术病理证实、术前误诊UESL患者的临床、影像及病理资料,并对比分析。结果:16例患者中诊断为原发性肝癌9例,肝母细胞瘤3例,肝内恶性占位4例。12例以腹痛不适就诊;超声显示实性为主肿瘤,而CT及MRI呈囊性为主的肿瘤,瘤内成分混杂;增强后病灶内囊壁下软组织、分隔及边缘可见强化。术后病理见肿瘤切面呈囊实性,内见不同程度出血坏死、凝胶样区域和肿瘤组织,癌细胞间可见特征性嗜酸性小体。结论:未分化胚胎性肉瘤临床罕见,临床资料无明显特异性,超声与CT、MRI表现不一致性为重要鉴别要点,有助于提高术前诊断率。展开更多
近年来小儿实体肿瘤的发病率一直在缓慢增长,其中肝脏恶性肿瘤的发病率占1%~4%,是较常见的小儿实体肿瘤[1]。小儿肝脏恶性肿瘤中最为常见的是肝母细胞瘤和肝细胞癌,其次是肝脏未分化胚胎性肉瘤(undifferentiated embryonal sarcoma of t...近年来小儿实体肿瘤的发病率一直在缓慢增长,其中肝脏恶性肿瘤的发病率占1%~4%,是较常见的小儿实体肿瘤[1]。小儿肝脏恶性肿瘤中最为常见的是肝母细胞瘤和肝细胞癌,其次是肝脏未分化胚胎性肉瘤(undifferentiated embryonal sarcoma of the liver,UESL)[2]。UESL于1978年由Stocker和Ishak[3]首次报道,多发于小儿,成人较少见。UESL始发于肝内间叶组织,又被称为恶性间叶瘤或未分化间叶肉瘤,常见的临床症状是腹部包块及腹痛;恶性程度较高,进展迅速,且临床症状、体征与其他肝脏肿瘤相比无明显特异性,早期诊断困难[4]。展开更多
目的:探讨肝脏未分化胚胎性肉瘤(undifferen-tiated embryonal sarcoma of the liver,UESL)的诊断与治疗.方法:回顾性总结分析本院2005-2011年住院手术治疗并经术后病理组织学检查确认的4例UESL患者的临床资料.结果:男1例,女3例,年龄分...目的:探讨肝脏未分化胚胎性肉瘤(undifferen-tiated embryonal sarcoma of the liver,UESL)的诊断与治疗.方法:回顾性总结分析本院2005-2011年住院手术治疗并经术后病理组织学检查确认的4例UESL患者的临床资料.结果:男1例,女3例,年龄分别为6岁、14岁、14岁和46岁.既往无肝病背景,其临床表现主要为腹部包块、腹痛和发热等.术前常规化验检查显示肝功能基本正常,肝炎病毒标志物和甲胎蛋白等肿瘤标志物均为阴性.术前影像学检查确诊较困难,术后病理组织学检查均确诊为UESL.所有患者均行根治性手术切除,其中2例因出现发热和腹膜炎体征而疑有肿瘤破裂出血,转行急诊手术治疗,并于术后联合行化学药物辅助治疗.结论:UESL是一种少见的高度恶性肿瘤,无特异性临床表现,术前难以确诊;治疗上仍以手术完整切除肿瘤为首选,联合化疗等综合辅助治疗可进一步提高疗效.展开更多
During the first year of life, most of the liver neoplasms are benign in origin, but some of these histologically benign lesions may be challenging in their management. Although most hepatic hemangiomas can be safely ...During the first year of life, most of the liver neoplasms are benign in origin, but some of these histologically benign lesions may be challenging in their management. Although most hepatic hemangiomas can be safely observed until involution is documented, some patients will need treatment due to progressive hepatomegaly, hypothyroidism and/or cardiac failure. Large mesenchymal hamartomas may require extensive hepatic resection and an appropriate surgical plan is critical to obtain good results. For malignant neoplasms such as hepatoblastoma, complete surgical resection is the mainstay of curative therapy. The decision about whether to perform an upfront or delayed resection of a primary liver malignant tumor is based on many considerations, including the ease of resection, surgical expertise, tumor histology and stage, and the likely chemosensitivity of the tumor. This article reviews the initial management of the more common hepatic tumors of infancy, focusing on the differential diagnosis and treatment options.展开更多
文摘目的:分析儿童肝脏未分化胚胎性肉瘤(undifferentiated embryonal sarcoma of the liver,UESL)的超声、CT及MRI影像学表现,以提高对该病的认识水平。方法:回顾性收集2009年12月至2021年12月首都儿科研究所附属儿童医院经手术及病理证实的11例儿童UESL,分析所有患儿的超声、CT及MRI影像表现,总结其影像学特点。结果:病灶均为单发,肿块最大径11.5~19.8 cm。CT平扫显示所有病灶边缘较清楚、其内呈不均匀低密度,内可见厚薄不均的分隔;囊性密度区边缘或分隔周围可见少许片絮样软组织密度影。6例超声图像均提示实性占位,表现为混合回声的实性包块内可见大小不等的无回声区。3例MRI表现为边界清楚肿块影,T1WI呈不均匀混杂低信号,病灶内可见高信号区;T2WI呈混合高信号区和条状低信号区。9例病灶增强扫描动脉期均以边缘强化为主,8例可见迂曲增粗血管影;延迟期病灶呈持续不均匀强化,边缘持续强化,且从外围向中心充盈,强化范围增大,8例延迟期可见假包膜。结论:儿童UESL影像学表现有一定特征性,了解其影像特点并结合临床特征,有助于早期诊断。
文摘AIM: To evaluate the undifferentiated embryonal sarcoma of liver (UESL) in adults in order to improve its diagnosis and treatment. METHODS: Four primary and one recurrent cases of UESL were clinicopathologically evaluated and immunohistochemically investigated with a panel of antibodies using the EnVision+ system. Relevant literature about UESL in adults was reviewed. RESULTS: Three males and one female were enrolled in this study. Their chief complaints were abdominal pain, weight loss, or fever. Laboratory tests, imaging and pathological features of UESL in adults were similar to those in children. Immunohistochemistry showed evidence of widely divergent differentiation into mesenchymal and epithelial phenotypes. The survival time of patients who underwent complete tumor resection followed by adjuvant transcatheter arterial chemoembolization (TACE) was significantly longer than that of those who underwent surgical treatment alone.CONCLUSION: UESL in adults may undergo pluripotential differentiation and its diagnosis should be made based on its morphological and immunohistochemical features. Complete tumor resection after adjuvant TACE may improve the survival time of such patients.
文摘Undifferentiated embryonal sarcoma of the liver （UESL） was first described by Stocker and Ishak in 1978. It is a rare tumor that most often presents in childhood between 6 to 10 years of age.2 The adult patients diagnosed with UESL often show nonspecific symptoms with normal laboratory results. Ultrasonography, CT, or MRI often reveals a cystic mass. Complete surgical resection plus pre- and/or post-operative systemic chemotherapy offers the possibility of a cure. We report a case of primary UESL in an adult with no specific symptoms, tumor markers, or image results. The diagnosis of the case was based on the resulting pathology from surgery.
文摘目的:探讨肝脏未分化性胚胎性肉瘤(undifferentiated embryonal sarcoma of the liver,UESL)的临床特点,加深对该病的认识,提高术前诊断准确性。方法:回顾性分析16例经手术病理证实、术前误诊UESL患者的临床、影像及病理资料,并对比分析。结果:16例患者中诊断为原发性肝癌9例,肝母细胞瘤3例,肝内恶性占位4例。12例以腹痛不适就诊;超声显示实性为主肿瘤,而CT及MRI呈囊性为主的肿瘤,瘤内成分混杂;增强后病灶内囊壁下软组织、分隔及边缘可见强化。术后病理见肿瘤切面呈囊实性,内见不同程度出血坏死、凝胶样区域和肿瘤组织,癌细胞间可见特征性嗜酸性小体。结论:未分化胚胎性肉瘤临床罕见,临床资料无明显特异性,超声与CT、MRI表现不一致性为重要鉴别要点,有助于提高术前诊断率。
文摘近年来小儿实体肿瘤的发病率一直在缓慢增长,其中肝脏恶性肿瘤的发病率占1%~4%,是较常见的小儿实体肿瘤[1]。小儿肝脏恶性肿瘤中最为常见的是肝母细胞瘤和肝细胞癌,其次是肝脏未分化胚胎性肉瘤(undifferentiated embryonal sarcoma of the liver,UESL)[2]。UESL于1978年由Stocker和Ishak[3]首次报道,多发于小儿,成人较少见。UESL始发于肝内间叶组织,又被称为恶性间叶瘤或未分化间叶肉瘤,常见的临床症状是腹部包块及腹痛;恶性程度较高,进展迅速,且临床症状、体征与其他肝脏肿瘤相比无明显特异性,早期诊断困难[4]。
文摘目的:探讨肝脏未分化胚胎性肉瘤(undifferen-tiated embryonal sarcoma of the liver,UESL)的诊断与治疗.方法:回顾性总结分析本院2005-2011年住院手术治疗并经术后病理组织学检查确认的4例UESL患者的临床资料.结果:男1例,女3例,年龄分别为6岁、14岁、14岁和46岁.既往无肝病背景,其临床表现主要为腹部包块、腹痛和发热等.术前常规化验检查显示肝功能基本正常,肝炎病毒标志物和甲胎蛋白等肿瘤标志物均为阴性.术前影像学检查确诊较困难,术后病理组织学检查均确诊为UESL.所有患者均行根治性手术切除,其中2例因出现发热和腹膜炎体征而疑有肿瘤破裂出血,转行急诊手术治疗,并于术后联合行化学药物辅助治疗.结论:UESL是一种少见的高度恶性肿瘤,无特异性临床表现,术前难以确诊;治疗上仍以手术完整切除肿瘤为首选,联合化疗等综合辅助治疗可进一步提高疗效.
文摘During the first year of life, most of the liver neoplasms are benign in origin, but some of these histologically benign lesions may be challenging in their management. Although most hepatic hemangiomas can be safely observed until involution is documented, some patients will need treatment due to progressive hepatomegaly, hypothyroidism and/or cardiac failure. Large mesenchymal hamartomas may require extensive hepatic resection and an appropriate surgical plan is critical to obtain good results. For malignant neoplasms such as hepatoblastoma, complete surgical resection is the mainstay of curative therapy. The decision about whether to perform an upfront or delayed resection of a primary liver malignant tumor is based on many considerations, including the ease of resection, surgical expertise, tumor histology and stage, and the likely chemosensitivity of the tumor. This article reviews the initial management of the more common hepatic tumors of infancy, focusing on the differential diagnosis and treatment options.
