BACKGROUND Undifferentiated embryonal sarcoma of the liver(UESL)is a rare liver malignancy originating from primary mesenchymal tissue.The clinical manifestations,laboratory tests,and imaging examinations of the disea...BACKGROUND Undifferentiated embryonal sarcoma of the liver(UESL)is a rare liver malignancy originating from primary mesenchymal tissue.The clinical manifestations,laboratory tests,and imaging examinations of the disease lack specificity and the preoperative misdiagnosis rate is high.The overall prognosis is poor and survival rate is low.AIM To investigate the diagnosis,treatment,and prognosis of UESL.METHODS We performed a retrospective,single-center cohort study in Shengjing Hospital of China Medical University,which is a central hospital in northeast China.From 2005 to 2017,we recruited 14 patients with pathologically confirmed UESL.We analyzed the clinical manifestations,laboratory tests,imaging examinations,pathological examinations,therapy,and prognosis of these patients.RESULTS There were nine males and five females aged 2-60 years old included in the study.The major initial symptoms were abdominal pain(71.43%)and fever(57.14%).Preoperative laboratory tests revealed that seven patients had increased leukocyte levels,four showed a decrease in hemoglobin levels,seven patients had increased glutamyl transpeptidase levels,nine had increased lactate dehydrogenase levels,and three showed an increase in carbohydrate antigen 199.There was no difference in the rate of misdiagnosis in preoperative imaging examinations of UESL between adults and children(6/6 vs 5/8,P=0.091).The survival rate after complete resection was 6/10,while that after incomplete resection was 0/4(P=0.040),suggesting that complete resection is important to improve survival rate.In total,five out of the eight children achieved survival.During the follow-up,the maximum survival time was shown to be 11 years and minimum survival time was 6 mo.Six adult patients relapsed late after surgery and all of them died.CONCLUSION Preoperative imaging examination for UESL has a high misdiagnosis rate.Multidisciplinary collaboration can improve the diagnostic accuracy of UESL.Complete surgical resection is the first choice for treatment of UESL.展开更多
背景与目的:宫颈微偏腺癌(minimal deviation adenocarcinoma of cervix,MDA)是一种罕见疾病,误诊率较高,尚无规范的治疗方法。本研究旨在分析总结MDA的临床病理特点,以提高对该病的认识。方法:回顾性分析中山大学附属第二医院1991年1月...背景与目的:宫颈微偏腺癌(minimal deviation adenocarcinoma of cervix,MDA)是一种罕见疾病,误诊率较高,尚无规范的治疗方法。本研究旨在分析总结MDA的临床病理特点,以提高对该病的认识。方法:回顾性分析中山大学附属第二医院1991年1月至2006年12月诊治的5例MDA患者的临床病理资料。结果:5例患者的中位年龄47岁(38～59岁)。主要临床表现为大量稀薄粘液性白带、阴道不规则出血等。MDA在组织学上表现为腺体分化好,镜下难与宫颈内膜的正常腺体相区别,但浸润深度超过正常宫颈腺体的深度。免疫组化示2例患者癌胚抗原(CEA)和P53阳性。4例患者接受手术治疗,其中手术+放疗1例,手术+化疗+放疗3例;1例不能手术的患者接受了姑息性放疗。随访6～88个月,无失访病例,2例无瘤生存的患者中有1例无瘤生存时间超过5年,3例死于肿瘤复发转移。结论:MDA的诊断主要依靠临床表现和腺体浸润深度超过正常宫颈腺体深度的病理特点,以手术为主的综合治疗可能是治疗MDA的较好方法。展开更多
基金The National Science and Technology Major Project,No.2017ZX10201201 and No.2017ZX10202202Liaoning Provincial Natural Science Foundation,No.20180550096。
文摘BACKGROUND Undifferentiated embryonal sarcoma of the liver(UESL)is a rare liver malignancy originating from primary mesenchymal tissue.The clinical manifestations,laboratory tests,and imaging examinations of the disease lack specificity and the preoperative misdiagnosis rate is high.The overall prognosis is poor and survival rate is low.AIM To investigate the diagnosis,treatment,and prognosis of UESL.METHODS We performed a retrospective,single-center cohort study in Shengjing Hospital of China Medical University,which is a central hospital in northeast China.From 2005 to 2017,we recruited 14 patients with pathologically confirmed UESL.We analyzed the clinical manifestations,laboratory tests,imaging examinations,pathological examinations,therapy,and prognosis of these patients.RESULTS There were nine males and five females aged 2-60 years old included in the study.The major initial symptoms were abdominal pain(71.43%)and fever(57.14%).Preoperative laboratory tests revealed that seven patients had increased leukocyte levels,four showed a decrease in hemoglobin levels,seven patients had increased glutamyl transpeptidase levels,nine had increased lactate dehydrogenase levels,and three showed an increase in carbohydrate antigen 199.There was no difference in the rate of misdiagnosis in preoperative imaging examinations of UESL between adults and children(6/6 vs 5/8,P=0.091).The survival rate after complete resection was 6/10,while that after incomplete resection was 0/4(P=0.040),suggesting that complete resection is important to improve survival rate.In total,five out of the eight children achieved survival.During the follow-up,the maximum survival time was shown to be 11 years and minimum survival time was 6 mo.Six adult patients relapsed late after surgery and all of them died.CONCLUSION Preoperative imaging examination for UESL has a high misdiagnosis rate.Multidisciplinary collaboration can improve the diagnostic accuracy of UESL.Complete surgical resection is the first choice for treatment of UESL.
文摘背景与目的:宫颈微偏腺癌(minimal deviation adenocarcinoma of cervix,MDA)是一种罕见疾病,误诊率较高,尚无规范的治疗方法。本研究旨在分析总结MDA的临床病理特点,以提高对该病的认识。方法:回顾性分析中山大学附属第二医院1991年1月至2006年12月诊治的5例MDA患者的临床病理资料。结果:5例患者的中位年龄47岁(38～59岁)。主要临床表现为大量稀薄粘液性白带、阴道不规则出血等。MDA在组织学上表现为腺体分化好,镜下难与宫颈内膜的正常腺体相区别,但浸润深度超过正常宫颈腺体的深度。免疫组化示2例患者癌胚抗原(CEA)和P53阳性。4例患者接受手术治疗,其中手术+放疗1例,手术+化疗+放疗3例;1例不能手术的患者接受了姑息性放疗。随访6～88个月,无失访病例,2例无瘤生存的患者中有1例无瘤生存时间超过5年,3例死于肿瘤复发转移。结论:MDA的诊断主要依靠临床表现和腺体浸润深度超过正常宫颈腺体深度的病理特点,以手术为主的综合治疗可能是治疗MDA的较好方法。
