肝脏未分化胚胎源性肉瘤1例

1 病例介绍 患者男,67岁,因"查体发现右上腹部包块3月余"入院.入院查体:右上腹可触及一质硬肿块,大小约10 cm×8 cm,活动度差.实验室检查:前白蛋白6.5 (17～40)mg/dL,白蛋白27 (40～55)g/L,红细胞3.74×1012/L,血清唾液酸101.10 mg/dL,其他血检包括甲胎蛋白、癌胚...     

肝脏未分化胚胎肉瘤一例

肝脏未分化肉瘤，又称胚胎肉瘤(undifferentiated embryonal sarcoma of liver，USL)，临床少见。随着影像诊断技术及肝脏外科的迅速发展，此病的发现可能会有增加。作者就经治的1例成人USL,并复习文献加以讨论，以提高对本病的诊治。     

肝脏未分化胚胎性肉瘤的研究进展

肝脏未分化胚胎性肉瘤（undifferentiated embryonal sarcoma of the liver,UESL）是发生于肝脏的高度恶性间叶源性肿瘤,常累及儿童及青少年,偶发于成人。UESL发病机制未知,发病隐匿,术前确诊率低且术后易复发,预后差。UESL特异性诊断标志物、分子生物学特征等尚待研究,主要依靠病理诊断进行确诊。手术、化疗和放疗的多模式治疗可显著改善UESL患者的预后。肝移植治疗也可以作为部分UESL患者的一种治疗手段。本综述拟从UESL的临床症状、组织病理学特点、发病机制、鉴别诊断、治疗及预后等方面进行介绍。     

肝脏未分化胚胎性肉瘤2例报道并文献复习

胚胎性肉瘤是肝脏罕见的恶性肿瘤,发病隐匿,术前确诊率低,术后易复发,预后较差,现将我院肝胆外科2005～2010年间2例此肿瘤进行回顾性报道并结合文献对其特点进行分析,以提高对此肿瘤的了解和认识.     

肝脏未分化胚胎性肉瘤一例

正病例患者,女,23岁,因"发现肝占位2个月余,右侧腹痛20天"入院。呈阵发性胀痛,变换体位及进食后加剧,无黄疸,查体腹膨隆,肝脏肿大,右肋下缘10 cm,质韧无压痛,肝区无叩痛,化验肿瘤标记物无异常。胸片示:右膈抬高,膈下病变;腹部B超提示肝右叶见一个大小12 cm×12 cm实质性低回声肿块,边界清楚、类圆形、周边见低回声晕、内部回声欠均匀;CDFI:肿块内血流丰富;诊断肝实质性占位病变,考虑     

儿童肝未分化胚胎性肉瘤临床病理特征分析

目的 探讨肝脏未分化胚胎性肉瘤（undifferentiated embryonal sarcoma of the liver,UESL）的 病理特征、诊断及鉴别诊断。方法 收集2013年至2018年浙江大学医学院附属儿童医院病理科5例UESL 病例,对其HE染色和免疫组织化学检测进行回顾分析,光镜下观察组织学特征及免疫表型。结果 5例 UESL患儿,年龄5～12岁,男女比例为4:1,其中肝右叶1例、肝门部1例、两叶均有3例,1例伴有肺转移。 肿物呈囊实性包块,囊内组织大多坏死出血,鱼肉样改变。镜下肿瘤黏液基质中散布未分化的不规则梭 形或星形细胞,散在大的多形性或瘤巨细胞,异型性明显。特征性病理表现为可见嗜酸性小体,PAS染色 阳性。免疫组织化学检测显示：Vimentin（+）、 Ki-67为50%～80%。结论 UESL嗜酸性小体为诊断UESL 的重要线索,临床上需要与肝母细胞瘤、肝胚胎性横纹肌肉瘤、肝脏间叶性错构瘤等疾病相鉴别。     

肝脏未分化性胚胎性肉瘤误诊分析

目的 加强对原发性肝脏未分化性胚胎性肉瘤的认识,提高其诊断率及生存率.方法 回顾分析我院2000年1月至2010年6月共13例肝脏未分化性胚胎性肉瘤临床资料,从临床及影像学角度,分析其误诊原因及随访结果.结果 13例患者中,男9例,女4例,最小年龄为8岁,最大年龄为63岁,中位年龄38岁.误诊为原发性肝癌6例,囊腺癌1...     

肝脏未分化胚胎性肉瘤患者围术期护理

对收治的13例肝脏未分化胚胎肉瘤患者采取手术切除肿瘤,术后7例行肝动脉化疗栓塞术治疗,其中1例术后行静脉化学治疗(5-FU+VP-16+顺铂)、伽马刀治疗及3次B超引导下瘤内注射无水乙醇及超液化碘油,6例未行其他治疗。结果患者症状缓解出院。全部病例随访时间4～20个月,均出现肝内肿瘤复发,中位生存期为7个月。提示术前给予发热、疼痛、营养支持护理,术后做好患者及家属的心理护理、化疗及引流管护理,提供支持可提高患者治疗信心,改善患者对疾病的态度,配合治疗,提高生命质量。     

肝未分化(胚胎性)肉瘤的CT表现和病理特点

目的 分析肝未分化(胚胎性)肉瘤的CT影像和病理特点,以提高对这种罕见肿瘤的认识.方法 2000-2006年间,10例病理证实的肝未分化(胚胎性)肉瘤病人(5～56岁),对其临床、CT影像及病理特点进行回顾性分析.结果 肿瘤直径平均为11.2 cm(范围9～25 cm);CT影像表现为多房、囊实性,囊性为主(略高于水密度),增强后囊壁下软组织及分隔可见强化.肿瘤切面囊实性,主要包括出血坏死、凝胶样内容物和肿瘤组织,肿瘤组织由多种间叶性肉瘤成分组成,异型明显,另可见较多多形性细胞和瘤巨细胞,在细胞内和间质中有特征性小圆形嗜酸性小体.免疫组化肿瘤呈多种间叶性成分分化.结论 肝未分化(胚胎性)肉瘤恶性程度高、预后差.在青少年或儿童病人中发现肝脏囊实性占位,应考虑肝UES可能,尽早手术及综合治疗能改善预后.     

小儿肝脏胚胎性肉瘤一例

1临床资料患儿女,6岁。因间断腹痛2个月于2012年12月12日入院。2012年1O月患儿无明显诱因出现腹痛,以脐周为著,呈间歇性钝痛,与进食无关,无恶心、呕吐,无发热等不适,在当地医院诊断为肝囊肿并进行局部麻醉B超引导下肝囊肿穿刺术,引流出酱油色液体约450mL,并注入平阳霉素5mg,患儿腹痛症状缓解后出院。为求进一步诊断与治疗来我科就诊。入院后体格检查：右季肋区肋缘下可触及直径约5cm质地峰硬包块,压痛阳性。既往无肝炎等特殊疾病史。     

成人肝脏未分化胚胎性肉瘤的CT及病理表现

肝脏未分化胚胎性肉瘤(undifferentiated embryonal sarcoma,UES)好发于儿阐童和青少年,成人罕见.     

肝脏未分化胚胎性肉瘤九例的临床病理特点及其治疗

目的 总结肝脏未分化胚胎性肉瘤(UESL)的临床病理学特点.方法 对11年来第二军医大学东方肝胆外科医院手术切除的9例UESL病人的临床资料进行回顾性分析.结果 该组9例年龄5～63岁,其中6例年龄5～10岁;肿瘤长径平均15.1 cm.术前6例误诊为肝囊腺瘤(癌),2例误诊为原发性肝癌,1例误诊为肝良性囊性占位,病理学检查均证实为未分化胚胎性肉瘤.结论 UESL为少见高度恶性肿瘤;CT与B超表现的不一致性为本病的重要特点;治疗上目前仍以手术切除肿瘤为首选,手术前后联合化疗及放疗等综合治疗可提高疗效.     

Nursing care of patients with undifferentiated embryonal sarcoma of the liver

对收治的13例肝脏未分化胚胎肉瘤患者采取手术切除肿瘤,术后7例行肝动脉化疗栓塞术治疗,其中1例术后行静脉化学治疗(5-FU+ VP-16+顺铂)、伽马刀治疗及3次B超引导下瘤内注射无水乙醇及超液化碘油,6例未行其他治疗.结果患者症状缓解出院.全部病例随访时间4～20个月,均出现肝内肿瘤复发,中位生存期为7个月.提示术前给予发热、疼痛、营养支持护理,术后做好患者及家属的心理护理、化疗及引流管护理,提供支持可提高患者治疗信心,改善患者对疾病的态度,配合治疗,提高生命质量.     

Childhood undifferentiated embryonal liver sarcoma: clinical features and immunohistochemistry analysis

Purpose

The aim of the study was to report on 3 cases of childhood undifferentiated embryonal liver sarcoma (UELS) and to highlight the clinical features, laboratory findings, diagnosis, and management of this rare disease.

Methods

The patients' age, sex, clinical features, laboratory findings, pathologic results, and therapy were reviewed. Immunohistochemistry analysis was performed on the resected mass sections.

Results

In this study, 2 cases were female (aged 8 and 12 years) and 1 was male (aged 9 years). The causes of hospitalization were mainly abdominal pain, mass, or fever. An elevated erythrocyte sedimentation rate was noted in 2 available cases, and α fetoprotein (AFP) was within the normal range. Imaging findings indicated a well-defined heterogeneous large mass in the right lobe. Histopathologic evaluation of the mass confirmed the diagnosis of UELS. Immunohistochemical staining showed that vimentin and CD68 antigen were positive in all samples, whereas desmin was positive in one sample. Surgery with chemotherapy was performed in 2 cases.

Conclusion

The diagnosis of UELS depends mainly on the pathologic findings. Undifferentiated embryonal liver sarcoma should be included in the differential diagnosis of mass in the liver, especially with well-defined heterogeneous imaging findings and normal AFP. Diagnosis and management should be made early as UELS is a potentially treatable tumor.     

Liver transplant for relapsed undifferentiated embryonal sarcoma in a young child

Undifferentiated embryonal sarcoma of the liver is a rare hepatic malignancy of childhood with a historically poor prognosis. Recent improvements in outcomes have been reported in small numbers of cases with the use of combination therapy involving aggressive surgical resection and chemotherapy. Complete surgical resection is frequently difficult to achieve when the location of the tumor is along the margins of the major hepatic vessels (portal vein, hepatic vein, and hepatic artery). Here we report a case of undifferentiated embryonal sarcoma of the liver that recurred along surgical hepatic vein margins in a 9-year-old boy who subsequently underwent orthotopic liver transplantation from a cadaveric donor. The patient has been in continuous clinical remission for the last 5 years.     

Treatment of undifferentiated embryonal sarcoma of the liver in children—single center experience

Background

Undifferentiated embryonal sarcoma of the liver (UESL) represents less than 5% of all malignant hepatic tumors in childhood. It is considered an aggressive neoplasm with an unfavorable prognosis. The aim of this paper is to present a single center experience in the treatment of children with UESL.

Materials and methods

Ten children with UESL were treated between 1981 and 2012. Age at diagnosis ranged from 4 months to 17 years (median age, 6 years and 9 months). Surgery after neoadjuvant chemotherapy (CHT) was performed in 7 patients, and in 3 patients primary surgery was done. Adjuvant chemotherapy was administered in all 10 patients (CYVADIC, CAV, CAV/ETIF/IF + ADM, CDDP/PLADO). Right hemihepatectomy was performed in 1 patient, extended right hemihepatectomy in 6, and partial resection of the right lobe (segments V-VI, segment V) in 2 patients. One patient with unresectable tumor affecting both lobes was listed for liver transplantation (LTx).

Results

Follow-up from diagnosis ranged from 50 to 222 months (mean 138 months). Among 9 patients treated with partial liver resection, distant metastases/local recurrence was not observed in any, and disease-free survival in this group is 100% (9 patients alive). The patient that underwent liver transplantation died of multiorgan failure 4 months postoperatively.However, this patient was misdiagnosed as having hepatoblastoma (HBL) and received PLADO chemotherapy. The overall survival rate is 90%.

Conclusion

Excellent results with long-term survival can be achieved in children with UESL with conventional therapy, including a combination of neoadjuvant and adjuvant chemotherapy and surgery, even in large extensively growing tumors.     

手术切除加行射频消融术在肝脏原发性未分化胚胎性肉瘤外科治疗中的意义

目的 研究根治性切除+射频消融术治疗原发性肝脏未分化胚胎性肉瘤的根治效果.方法 回顾性分析2005年1月-2015年2月中国人民解放军总医院肝胆外科17例肝脏原发性未分化胚胎性肉瘤患者的诊断、治疗和预后.所有17例患者均行外科手术切除,其中8例患者加行切缘组织的射频消肖融治疗(S+ RFA组),另外9例患者仅行手术切除病灶(S组).结果 所有病例均经术后病理证实为原发性肝脏未分化胚胎性肉瘤.与S组患者相比,S+ RFA组手术时间较长(P =0.0449);但术后肝功能(转氨酶、胆红素水平)和术中出血量的差异无统计学意义.回访时间4 ～38个月,其中9例患者死亡;S+RFA组患者病死率33.3％(3/9),S组患者病死率75.0％(6/8).Kaplan-Meier生存曲线法分析,两组患者总生存期差异无统计学意义(P＞0.05).S组和S+ RFA组患者24个月无病生存率分别为0和56.8％,中位生存期分别为15个月和29个月(P =0.0476).结论 手术切除+肝脏射频消融术能进一步保证原发性肝脏未分化胚胎性肉瘤切缘的阴性率,进而降低肿瘤的复发和转移,结合全身化疗等综合治疗,能够明显延长原发性肝脏未分化胚胎性肉瘤患者的生存期、降低肿瘤复发转移率.