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.     

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.     

Laparoscopic liver resections: A single center experience

Background Since the first report of laparoscopic liver resection, by Gagner et al. 1992, an increasing number of small prospective studies have been published. They have shown encouraging results for the feasibility and safety of the procedure. This paper prospectively evaluated the results of a single center’s experience with elective liver resections.Methods From January 1995 to January 2004 a prospective study of laparoscopic liver resections was undertaken in 31 patients with preoperative diagnosis of benign lesions (13 cases, 42.4%), hepatocellular carcinoma in absence of complicated cirrhosis (three cases, 9.1%), and liver metastases (15 cases, 45.5%). Mean tumor size was 34.9 mm (range 10–100 mm).Results The procedures included 11 (37.9%) major hepatectomies and 21 (62.1%) minor resections (one patient was submitted to repeat laparoscopic liver resection) . There were three conversions to open. Mean blood loss was 210 ml (range 0–700 ml). Mean operative time was 115 min (range 45–210 min). There were no deaths and no reoperations for complications. No port-site metastases occurred in patients with malignant lesions.Conclusions Laparoscopic liver resections, including major hepatectomies, are feasible and safe. Major and posterior resections are difficult, though, and conventional surgery remains an option.     

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.     

Undifferentiated embryonal sarcoma of the liver: case report and literature survey

Undifferentiated embryonal sarcoma (UES) of the liver was first identified as an independent clinicopathologic type of sarcoma in 1978. It is an uncommon hepatic tumor, of mesenchymal origin, usually observed in children, and cases in adults are rare: to our best knowledge, reports of only 51 cases have been published in the past 50 years. We present a case of UES of the liver in a previously healthy 22 year-old woman, admitted to our hospital due to a palpable mass in the right upper abdomen. On admission, laboratory studies showed mildly elevated aspartate aminotransferase, alkaline phosphatase, and gamma-GPT. Hepatitis and tumor markers were negative. Ultrasonography (US), computed tomography (CT), and magnetic resonance imaging (MRI) showed a large mass involving the right lobe and the medial segment of the liver, with compression of the bile duct. Right trisectionectomy with bile duct resection and reconstruction was performed. Microscopically, the tumor was composed of pleomorphic spindle cells in a myxoid stroma with focal staining of S-100 by immunohistochemistry. The histologic diagnosis was UES. Adjuvant therapy with vincristine, actinomycin-D, and cyclophosphamide was performed, and at 14 months of follow-up, the patient is alive without any evidence of recurrence. The clinical and histopathological features, as well as the therapeutic choices for adult UES, are described for this patient and in the literature of the past 50 years.     

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.     

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。     

肝脏移植10年120例经验回顾

目的：总结近年来我院肝移植的临床经验。方法：我院自1993年至今连续实施的120例人体原位肝移植病例，其中1993－1997年为第一阶段（2例），1999年为第二阶段（15例），2000年1月－2002年9月为第三阶段（103例），对手术适应证，外科技术，围手术期处理以及术后并发症防治等方面经验进行回顾性总结。结果：肝脏恶性病变在第一阶段两例受体均为恶性肿瘤，第二阶段为53％（8／15），第三阶段为34％（35／103），术后半年内良恶性病变受体的生存率无统计学差异，但恶性病变受体1年生存率明显低于良性病变者，肝细胞性肝癌患者则多死于癌复发与转移；第三阶段良性病变病人1年的生存率为83．1％，恶性疾病病人术后近期疗效较好，半年带癌生存率为81％，第三阶段无静脉转流技术的开展已显示其优越性；移植后血管并发症发生率逐渐下降；其发生率从29％下降到4．9％，而胆管并发症仍是长期存活病人的主要问题。结论：手术技术的完善与提高，术后并发症的及时合理诊治，受体的严格筛选以及原发疾病复发的防治是进一步提高我国肝脏移植疗效的努力方向。     

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.     

Laparoscopic liver resection: experience of 53 procedures at a single center

Background/Purpose The short-term outcome following laparoscopic liver resection at a single center is presented.Methods Fifty-three procedures were carried out in 47 patients, between August 1998 and April 2004 (6 patients were resected on two occasions). A previous laparotomy and/or hepatectomy had been done in 83% and 26% of the procedures, respectively. Colorectal metastasis was the main indication for treatment (42/53). A total laparoscopic approach was applied.Results Three of the 53 (6%) procedures were converted to laparotomy. In one additional procedure, radiofrequency ablation was done instead of resection. Sixty liver resections were done during the 49 procedures completed laparoscopically as planned (9 patients had concomitant resections performed). Nonanatomic (45/60) and anatomic (15/60; left lobectomies) resections were done. Tumor tissue was found in the resection margins of 6% of the specimens. The free margin was very short in 8% of the specimens. The morbidity was 16%. There was no mortality. Blood transfusions were given following 26% of the procedures. The median hospital stay was 3.5 days (range, 1–14 days) and the median number of days on which there was a need for opioids was 1 (range, 0–11 days).Conclusions Laparoscopic liver resection can be performed safely and seems to offer short-term benefits to the patients. Randomized studies are required to further evaluate the potential benefits of this treatment.     

Treatment of ruptured undifferentiated sarcoma of the liver in children: a report of two cases and review of the literature

Undifferentiated (embryonal) sarcoma of the liver (USL) is a highly malignant tumor of early life. Treatment choices for USL, especially with intraperitoneal rupture, are uncertain. Outcomes have been almost uniformly poor until recently. We describe two 7-year-old girls treated for ruptured USL. In the more recent patient, operative biopsy was followed by three cycles of cisplatin (CDDP), adriamycin (ADR), and cyclophosphamide (CPM). A fluid-filled cavity in the tumor showed enlargement and was drained. Two cycles of CDDP, ADR, vincristine (VCR), and ifosfamide were accompanied by reduction in tumor size, and trisegmentectomy was performed. She has no evidence of disease 3.5 years after surgery. In the other patient, left lobectomy was followed by a less intensive regimen, including CPM, VCR, and fluorouracil. This patient died of dissemination within 5 months. In 170 reported pediatric patients with USL, the 2-year disease-free survival was 17%. For the 96 such patients reported since 1980, 2-year disease-free survival had improved to 27%. More aggressive chemotherapy has been associated with this change. Of 8 patients with tumor rupture whose details have been reported (including the 2 present patients) after resection of the tumor, 4 died, 1 was alive with disease, and 3 were free of disease at 8, 49, and 58 months, respectively, after diagnosis. Ruptured USL should be treated with combination chemotherapy including CDDP and ADR, as well as with curative resection. Received: June 19, 2000 / Accepted: October 12, 2000     

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.     

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.     

Undifferentiated embryonal sarcoma of the liver: results of clinical management in one center

PURPOSE: This study was undertaken to review the authors' clinical experience with undifferentiated embryonal sarcoma of the liver (UES) in children, focusing on the clinical presentation and results of treatment. METHODS: A retrospective analysis of all children who have undergone treatment for UES during the 15-year period from 1984 through 1998 was performed. RESULTS: Seven patients (4 boys and 3 girls) ranging in age from 20 months to 12 years at the time of diagnosis were identified. All presented with large abdominal masses and normal liver function test results. All underwent complete tumor resection; trisegmentectomy was required in 4 of these cases. All patients received postoperative chemotherapy. Two patients suffered tumor recurrence at 12 and 29 months; both of these patients died of their disease. Another patient died of complications related to chemotherapy. The other 4 patients are alive with no evidence of disease after 19 to 150 months' follow-up. CONCLUSIONS: Undifferentiated embryonal sarcoma of the liver presents as a large hepatic tumor. Operative resection is difficult, but combined with adjuvant chemotherapy offers the best hope for cure.     

22例小儿肝脏肿物的治疗体会

【摘要】〓目的〓探讨小儿肿物的临床特征、治疗方法及效果。方法〓回顾性分析我科在2011年1月~2013年6月收治22例小儿肝脏肿物的临床资料,17例患儿行手术治疗,8例肝母细胞瘤患儿术前进行了介入栓塞治疗,10例患儿手术过程中未阻断肝门,7例患儿手术应用了CUSA和(或)Ligasure能量平台。结果〓病理证实肝母细胞瘤14例,未分化胚胎性肉瘤1例,局灶性结节性增生3例,婴儿型血管内皮瘤2例,纤维性错构瘤1例,单纯性囊肿1例。肝切除手术应用CUSA和Ligasure的有8例,平均失血量为121 mL;钳夹法肝切除手术9例,平均失血量为248 mL;两组失血量间的差异有统计学意义(P<0.05)。术中阻断肝门的有7例,平均失血量为170 mL;未阻断肝门的有10例,平均失血量为198.64 mL;两组失血量间的差异无统计学意义。手术7例良性肿瘤术后均无病生存。1例未分化肝胚胎性肉瘤手术后6个月死亡。14例肝母细胞瘤中,9例行手术切除患儿至今仍存活;5例未手术患儿4例死亡,1例存活。结论〓利用精准肝切除的方法治疗小儿肝脏占位性病变,不论良恶性,都是安全有效的,但对于侵犯肝门或超过3个肝叶以上的肿瘤还需进一步的提高治疗手段。     

Undifferentiated (embryonal) sarcoma of the liver: Report of three cases

Undifferentiated (embryonal) sarcoma is a rare malignant tumor of the liver. It typically presents in late childhood and its prognosis is poor. We experienced three cases of such a tumor during the period of 1976–1989; two of these patients are still alive without disease. Each case was independently treated with a combination of surgery and preand/or postoperative chemotherapy, which was found to be effective. In one surviving patient, cyclophosphamide and vincristine were found to be effective, while in the other a combination of cisplatin, Adriamycin (ADM), vincristine and cyclophosphamide was observed to induce a rapid reduction in the size of the recurrent tumor. Thus, an adequate combination of surgery and chemotherapy may improve the prognosis of this tumor.