Liver transplantation for polycystic liver with massive hepatomegaly： A case report

A previous study has shown that liver or combined liver-kidney transplantation can be a valuable surgical technique for the treatment of polycystic liver disease.Herein, we present the case of a 35-year-old woman with polycystic liver disease, who underwent orthotopic liver transplantation (OLT) on November 11, 2008.The whole-size graft was taken from a deceased donor (a 51-year-old man who died of a heart attack).Resection in a patient with massive hepatomegaly is very difficult. Thus, after intercepting the portal hepatic vein, left hepatectomy was performed, then the vena cava was intercepted, the second and third porta hepatic isolated, and finally, right hepatectomy was performed. OLT was performed successfully.The recipient did well after transplantation. This case suggested that OLT is an effective therapeutic option for polycystic liver disease and left hepatectomy can be performed first during OLT if the liver is over enlarged.     

Liver transplantation for recurrent hepatic adenoma

Hepatic adenoma (HA) is a rare indication for liver transplantation (LTx). So far 20 cases of LTx for HA are reported in PubMed. In rare cases HA presents as multiple hepatic adenomas or recurrent adenoma after initial liver resection and in such cases LTx is the only potential cure and prevents the risk of bleeding or cancer transformation into hepatocellular carcinoma. We report the case of a 56 years old lady who underwent a left hepatectomy for giant adenoma in 2005 and resection of segment Ⅴ-Ⅵ for recurrence of liver adenoma in 2007. She developed a second recurrence of HA with 3 new lesions in the right liver in 2008. The patient underwent LTx. After 3 years the patient is alive with no evidence of disease. LTx is indicated in patients with HA in which resection is not technically feasible.     

肝移植术后丙型肝炎复发受者肝组织病理学特征分析

目的：探讨肝移植术后丙型肝炎复发时受者肝组织病理学特征。方法收集28例明确诊断为肝移植术后丙型肝炎复发患者的54份肝组织病理学资料,回顾性分析丙型肝炎复发时肝组织病理学特点。结果肝移植术后丙型肝炎复发的主要病理学特点是：肝细胞变性和坏死,汇管区以淋巴细胞为主的炎性细胞聚集,以及较早出现的肝纤维化,同时部分病例合并有排斥反应和药物性肝损伤的组织学特点;移植术后远期（大于12 m）丙型肝炎复发者肝纤维化程度较近期（小于12 m）复发者严重[肝纤维化计分为（1.82±1.12）对（1.13±1.08）,P＜0.05;不同丙型肝炎病毒基因型导致的丙型肝炎在病理学表现上的差异无统计学意义;合并排斥反应及药物性肝损害患者肝炎活动度评分分别为（2.32±0.64）和（2.33±0.88）,显著高于无合并组患者（1.64±0.59）,（P＜0.05）。结论肝移植术后丙型肝炎复发时有特征性的肝脏病理学改变,肝组织病理学检查对移植术后丙型肝炎复发的病情判断具有重要的价值。     

Liver transplantation for metastatic neuroendocrine tumor： A case report and review of the literature

Neuroendocrine tumors are divided into gastrointestinal carcinoids and pancreatic neuroendocrine tumors. The WHO has updated the classification of these lesions and has abandoned the term "carcinoid". Both types of tumors are divided into functional and non-functional tumors. They are characterized by slow growth and frequent metastasis to the liver and may be limited to the liver for long periods. The therapeutic approach to hepatic metastases should consider the number and distribution of the liver metastases as well as the severity of symptoms related to hormone production and tumor bulk. Surgery is generally considered as the first line therapy. In patients with unresectable liver metastases, alternative treatments are dependent on the type and the growth rate. Initial treatments consist of long acting somatostatin analogs and/or interferon. Streptozocin-based chemotherapy is usually reserved for symptomatic patients with rapidly advancing disease, but generally the therapy is poorly tolerated and its effects are short-lived. Locoregional therapy directed such as hepatic-artery embolization and chemoembolization, radiofrequency thermal ablation and cryosurgery, is often used instead of systemic therapy, if the disease is limited to the liver. However, liver transplantation should be considered in patients with neuroendocrine metastases to the liver that are not accessible to curative or cytoreductive surgery and if medical or locoregional treatment has failed and if there are life threatening hormonal symptoms. We report a case of liver transplantation for metastatic neuroendocrine tumor of unknown primary source and provide a detailed review of the world literature on this controversial topic.     

Multi-slice computed tomography manifestations of hepatic epithelioid angiomyolipoma

AIM:To explore the characteristics of multi-slice computed tomography(CT)manifestations of hepatic epithelioid angiomyolipoma(HEA),improve the rate of accurate diagnosis,and reduce the misdiagnostic rate.METHODS:The multi-slice CT manifestations in five patients who were diagnosed with HEA definitely by postoperative pathological examination were analysed retrospectively.Three female patients and two male patients were included.Before operation,four patients received plain CT scanning and dynamic enhancement scanning,and the other patient only received enhancement scanning,with immunohistochemical analysis conducted after postoperative pathological examination.Four patients were misdiagnosed by CT,including three patients misdiagnosed with hepatic cell carcinoma and one patient with focal nodular hyperplasia.RESULTS:Upper abdominal multi-slice spiral CT scanning and three-stage enhancement scanning were conducted in five patients with HEA before operation.HEA had certain characteristic CT manifestations:low density masses,a few relatively high-density masses or fatdensity masses diffusely shown in foci,clear boundary,round or oval and large focus,and tumour size ranging from 3.1 cm×2.5 cm to 7.0 cm×5.2 cm.During enhancement scanning,the foci were significantly enhanced uniformly or non-uniformly during the arterial phase,while during the venous and equilibrium phases,the foci were enhanced continuously or showed obvious low-density masses.Obviously enhanced and widened vessels could be found adjacent to foci or in the central area of foci during the arterial phase.CONCLUSION:CT manifestations of HEA have certain characteristics.Primary diagnosis can be obtained by combining CT findings with clinical data,but pathological examination is still needed for a definite diagnosis.     

Liver transplantation for hepatic tumors:a systematic review

Improvements in the medical and pharmacological management of liver transplantation(LT)recipients have led to a better long-term outcome and extension of the indications for this procedure.Liver tumors are relevant to LT;however,the use of LT to treat malignancies remains a debated issue because the high risk of recurrence.In this review we considered LT for hepatocellular carcinoma(HCC),cholangiocarcinoma(CCA),liver metastases(LM)and other rare tumors.We reviewed the literature,focusing on the past 10 years.The highly selected Milan criteria of LT for HCC(single nodule<5 cm or up to 3 nodules<3cm)have been recently extended by a group from the University of S.Francisco(1 lesion<6.5 cm or up to3 lesions<4.5 cm)with satisfying results in terms of recurrence-free survival and the"up-to-seven criteria".Moreover,using these criteria,other transplant groups have recently developed downstaging protocols,including surgical or loco-regional treatments of HCC,which have increased the post-operative survival of recipients.CCA may be treated by LT in patients who cannot undergo liver resection because of underlying liver disease or for anatomical technical challenges.A well-defined protocol of chemoirradiation and staging laparotomy before LT has been developed by the Mayo Clinic,which has resulted in long term diseasefree survival comparable to other indications.LT for LM has also been investigated by multicenter studies.It offers a real benefit for metastases from neuroendocrine tumors that are well differentiated and when a major extrahepatic resection is not required.If LT is an option in these selected cases,liver metastases from colorectal cancer is still a borderline indication because data concerning the disease-free survival are still lacking.Hepatoblastoma and hemangioendothelioma represent rare primary tumors for which LT is often the only possible and effective cure because of the frequent multifocal,intrahepatic nature of the disease.LT is a very promising procedure for both primary and secondary liver malignancies;however,it needs an accurate evaluation of the costs and benefits for each indication to balance the chances of cure with actual organ availability.     

Living-donor or deceased-donor liver transplantation for hepatic carcinoma:A case-matched comparison

AIM:To compare the surgical outcomes between living-donor and deceased-donor liver transplantation in patients with hepatic carcinoma.METHODS:From January 2007 to December 2010,257 patients with pathologically confirmed hepatic carcinoma met the eligibility criteria of the study.Forty patients who underwent living-donor liver transplantation(LDLT)constituted the LDLT group,and deceaseddonor liver transplantation(DDLT)was performed in217 patients.Patients in the LDLT group were randomly matched(1:2)to patients who underwent DDLT using a multivariate case-matched method,so 40 patients in the LDLT group and 80 patients in the DDLT group were enrolled into the study.We compared the two groups in terms of clinicopathological characteristics,postoperative complications,long-term cumulative survival and relapse-free survival outcomes.The modified Clavien-Dindo classification system of surgical complications was used to evaluate the severity of perioperative complications.Furthermore,we determined the difference in the overall biliary complication rates in the perioperative and follow-up periods between the LDLT and DDLT groups.RESULTS:The clinicopathological characteristics of the enrolled patients were comparable between the two groups.The duration of operation was significantly longer(553 min vs 445 min,P<0.001)in the LDLT group than in the DDLT group.Estimated blood loss(1188 mL vs 1035 mL,P=0.055)and the proportion of patients with intraoperative transfusion(60.0%vs 43.8%,P=0.093)were slightly but not significantly greater in the LDLT group.In contrast to DDLT,LDLT was associated with a lower rate of perioperative gradeⅡcomplications(45.0%vs 65.0%,P=0.036)but a higher risk of overall biliary complications(27.5%vs 7.5%,P=0.003).Nonetheless,21 patients(52.5%)in the LDLT group and 46 patients(57.5%)in the DDLT group experienced perioperative complications,and overall perioperative complication rates were similar between the two groups(P=0.603).No significant difference was observed in 5-year overall survival(74.1%vs 66.6%,P=0.372)or relapse-free survival(72.9%vs 70.9%,P=0.749)between the LDLT and DDLT groups.CONCLUSION:Although biliary complications were more common in the LDLT group,this group did not show any inferiority in long-term overall survival or relapse-free survival compared with DDLT.     

原发性肝癌肝移植135例

目的:探讨肝癌肝移植适应证、禁忌证、手术时机及术后肿瘤复发的防治.方法:对2005-04/2010-04连续实施的135例原发性肝癌患者进行随访和回顾性分析.结果:Milan标准组患者术后1、2年总体生存率分别为97.0%、89.5%,无瘤患者生存率分别为91.0%、71.6%;UCSF标准组患者术后1、2年总体生存率分别为95.1%、78.6%,无瘤患者生存率分别为90.2%、65.6%;超过UCSF标准组患者术后1年总体生存率为71.4%,无瘤患者1年总体生存率为57.1%.Milan、UCSF标准组生存率和无瘤生存率与超过UCSF标准组比较,差异有统计学意义(P<0.01).结论:肝移植是治疗肝癌的一个有效手段,术后应重视免疫抑制剂的应用调整,这对于防止肝癌术后复发有一定的意义.     

肝血管平滑肌脂肪瘤的MRI及临床病理特征

目的探讨肝血管平滑肌脂肪瘤(hepatic angiomyolipoma,HAML)的MRI表现及临床病理特征。方法回顾性分析经病理证实的9例HAML的MRI特征及临床表现。结果 9例均为单发肿块,中位直径4.0 cm(1.4~15.3 cm)。7例无不适症状,2例上腹部不适,均行手术切除,中位随访10个月(2~24个月),无复发。组织学上分为混合型4例和肌瘤型5例。免疫组化均HMB-45(+)、SMA(+)、CD34(+)。5例MRI回波序列见大量脂质成分。增强MRI分两种类型:Ⅰ型,动脉期明显强化,门脉期和延迟期仍强化(混合型和肌瘤型各1例),其对应病理为肿瘤组织含较少血管;Ⅱ型,动脉期强化,门脉期和延迟期消退,其病理为肿瘤富含血管(混合型3例及肌瘤型4例)。结论 HAML临床表现无特异性,HAML的增强MRI特征与肿瘤组织富含血管多少有关。确诊需病理及免疫组化检测HMB-45、SMA、CD34阳性。     

特发性门脉高压肝移植术后随访第3年再发1例报告及文献复习

目的 复习1例特发性门脉高压(IPH)患者接受肝移植(LT)后第3年出现病情“再发”,并进行了相关文献复习,以提高对该病的认识。方法 报道1例我们诊治的IPH患者的病例资料,并检索MEDLINE、EMBASE、万方等数据库经LT治疗的IPH患者的研究报道,分析其治疗和转归。结果 本文报道的病例为57岁女性,因消化道出血、腹水行LT术,组织病理学检查诊断为IPH;术后随访第3年病情复发,行经皮肝穿刺活检术,病理学检查提示结节性再生性增生(NRH)、轻度汇管区炎症及纤维化,提示IPH再发;文献检索到81例LT治疗的IPH患者,其中42例在LT前诊断为肝硬化;LT后最长随访时间为248个月,8例死亡,其中5例分别在首次LT后3.5月～14年进行肝活检,组织病理学检查提示NRH,3例分别于LT后第7月、第3年和第14年出现具有门脉高压表现的NRH。结论 具有严重的门脉高压或肝功能衰竭的IPH患者需要LT治疗。少数患者在LT后可能出现IPH“再发”。     

Radiofrequency ablation of recurrent cholangiocarcinoma after orthotopic liver transplantation—a case report

AIM: To report the use of radiofrequency ablation in the treatment of recurrenct cholangiocarcinoma in the transplanted liver. METHODS: A lady who underwent orthotopic liver transplantation (OLT) for intrahepatic cholangiocarcinoma recurrence of tumour 13 mo after tralsplantation inspite of adjuvant chemotherapy. Her recurrent tumour was treated with radiofrequency ablation. RESULTS: She survived for 18 mo following the recurrence of her tumour. CONCLUSION: Radiofrequency ablation can be used safely in the transplanted liver to treat recurrent tumour.     

Clinical characteristics and outcomes of patients with hepatic angiomyolipoma: A literature review

First reported in 1976, hepatic angiomyolipoma (HAML) is a rare mesenchymal liver tumor occurring mostly in middle-aged women. Diagnosis of the liver mass is often incidental on abdominal imaging due to the frequent absence of specific symptoms. Nearly 10% of HAMLs are associated with tuberous sclerosis complex. HAML contains variable proportions of blood vessels, smooth muscle cells and adipose tissue, which renders radiological diagnosis hazardous. Cells express positivity for HMB-45 and actin, thus these tumors are integrated into the group of perivascular epithelioid cell tumors. Typically, a HAML appears on magnetic resonance imaging (or computed tomography scan) as a hypervascular solid tumor with fatty areas and with washout, and can easily be misdiagnosed as other liver tumors, particularly hepatocellular carcinoma. The therapeutic strategy is not clearly defined, but surgical resection is indicated for symptomatic patients, for tumors showing an aggressive pattern (i.e., changes in size on imaging or high proliferation activity and atypical epithelioid pattern on liver biopsy), for large (> 5 cm) biopsy-proven HAML, and if doubts remain on imaging or histology. Conservative management may be justified in other conditions, since most cases follow a benign clinical course. In summary, the correct diagnosis of HAML is challenging on imaging and relies mainly on pathological findings.     

原发性胆汁性肝硬化肝移植术后复发的研究进展

原发性胆汁胜肝硬化（primary biliary cirrhosis，PBC）是一种缓慢的进行性自身免疫性肝脏疾病，其组织病理学主要表现为门脉区炎症和免疫介导的肝内胆管破坏，进而引起胆汁分泌减少和代谢过程中的有毒物质在肝脏蓄积，最终导致肝脏损伤、肝纤维化、肝硬化，直至肝功能衰竭。目前治疗PBC最有效的药物是熊去氧胆酸（ursodeoxycholic acid，UDCA），它能有效改善患者肝功能，早期患者及早并长期应用UDCA可能延缓肝脏组织学进展，但不能改善乏力和瘙痒等临床症状，也无足够证据支持UDCA能延长患者的生存期。     

肝恶性淋巴瘤伴高钙血症1例

1.病例资料:男性患者,69岁,农民.因发热伴呕吐1周,外院抗炎治疗后,症状缓解,但近2日精神逐渐变差,于2008年3月24日转入我院急诊内科进一步诊治.入院时体检结果:体温37.5℃,呼吸22次/min,脉搏84次/min,血压170/90 mm Hg(1 mm Hg=0.133 kPa),平侧卧位,神清,精神萎靡,口唇不绀,双肺呼吸音稍低,右下肺可闻及少许湿音,心率84次/min,心律齐,未闻及杂音,腹平软,脐周轻压痛,无反跳痛,肝脾肋下未及.     

Two-stage liver transplant for ruptured hepatic adenoma:A case report

BACKGROUND Only one case of liver transplantation for hepatic adenoma has previously been reported for patients with rupture and uncontrolled hemorrhage.We present the case of a massive ruptured hepatic adenoma with persistent hemorrhagic shock and toxic liver syndrome which resulted in a two-stage liver transplantation.This is the first case of a two-stage liver transplantation performed for a ruptured hepatic adenoma.CASE SUMMARY A 23 years old African American female with a history of pre-diabetes and oral contraceptive presented to an outside facility complaining of right-sided chest pain and emesis for one day.She was found to be in hemorrhagic shock due to a massive ruptured hepatic hepatic adenoma.She underwent repeated embolizations with interventional radiology with ongoing hemorrhage and the development of renal failure,hepatic failure,and hemodynamic instability,known as toxic liver syndrome.In the setting of uncontrolled hemorrhage and toxic liver syndrome,a hepatectomy with porto-caval anastomosis was performed with liver transplantation 15 h later.She tolerated the anhepatic stage well,and has done well over one year later.CONCLUSION When toxic liver syndrome is recognized,liver transplantation with or withouthepatectomy should be considered before the patient becomes unstable.     

Current status and prospect of treatments for recurrent hepatocellular carcinoma

Owing to its heterogeneous and highly aggressive nature, hepatocellular carcinoma (HCC) has a high recurrence rate, which is a non-negligible problem despite the increasing number of available treatment options. Recent clinical trials have attempted to reduce the recurrence and develop innovative treatment options for patients with recurrent HCC. In the event of liver remnant recurrence, the currently available treatment options include repeat hepatectomy, salvage liver transplantation, tumor ablation, transcatheter arterial chemoembolization, stereotactic body radiotherapy, systemic therapies, and combination therapy. In this review, we summarize the strategies to reduce the recurrence of high-risk tumors and aggressive therapies for recurrent HCC. Additionally, we discuss methods to prevent HCC recurrence and prognostic models constructed based on predictors of recurrence to develop an appropriate surveillance program.     

Granulomatous reaction in hepatic inflammatory angiomyolipoma after chemoembolization and spontaneous rupture

A 77-year-old Japanese woman was transported to a nearby hospital due to sudden abdominal pain and transient loss of consciousness. Abdominal computed tomography (CT) suggested hemoperitoneum and hepatic nodule. She was conservatively treated. Contrast-enhanced CT two months later revealed an increased mass size, and the enhancement pattern suggested the possibility of hepatocellular carcinoma (HCC). Under a clinical diagnosis of HCC, transcatheter arterial chemoembolization (TACE) was performed. A subsequent imaging study revealed that most of the lipiodol used for the embolization was washed out. Therefore, surgical resection was performed. Histologically, the nodule contained numerous inflammatory cells including small lymphocytes, plasma cells and macrophages. Notably, epithelioid granulomatous features with multinucleated giant cells were observed in both the nodule and background liver. Some of the multinucleated giant cells contained oil lipid. Among the infiltrating inflammatory cells, spindle-shaped, histiocytoid or myoid tumor cells with eosinophilic cytoplasm were found. The tumor cells were positive for Melan A and HMB45. The nodule contained many IgG4-positive plasma cells; these were counted and found to number 72.6 cells/HPF (range: 61-80). The calculated IgG4:IgG ratio was 33.2%. The nodule was finally diagnosed as previously ruptured inflammatory angiomyolipoma modified by granulomatous reaction after TACE.     

肝移植术后急性排斥反应1例

一、病例资料患者,男,58岁,于2008年2月15日入院.确诊为酒精性肝硬化6年,经内科治疗后病情稳定,于2006年、2007年10月分别出现消化道出血,经脾切除术等治疗后好转.     

Role of surgery and transplantation in the treatment of hepatic metastases from neuroendocrine tumor

Neuroendocrine tumors (NET) are a heterogeneous group of cancers, with indolent behavior. The most common primary origin is the gastro-intestinal tract but can also appear in the lungs, kidneys, adrenals, ovaries and other organs. In general, NET is usually discovered in the metastatic phase (40%-80%). The liver is the most common organ involved when metastases occur (40%-93%), followed by bone (12%-20%) and lung (8%-10%).A number of different therapeutic options are available for the treatment of hepatic metastases including surgical resection, transplantation, ablation, trans-arterial chemoembolization, chemotherapy and somatostatin analogues. Recently, molecular targeted therapies have been used, usually in combination with other treatment options, to improve outcomes in patients with metastases. This article emphasizes on the role of surgery in the treatment of liver metastases from NET.     

Primary hepatic neuroendocrine tumour requiring live donor liver transplantation: case report and concise review

Primary hepatic neuroendocrine tumours are rare tumours effecting relatively young patients. As metasta- tic neuroendocrine tumours to the liver are much more common, extensive investigations are crucial to exclude a primary tumour elsewhere. We report a case of a 27 year old woman who presented with fati- gue, increased abdominal girth and feeling of early satiety and bloating. Extensive work up failed to show tumour at another primary site. Hepatic artery embolization showed no effect, so the patient underwent total hepatectomy and live-donor liver transplant. Grossly the tumour measured 27 cm. Microscopic exami- nation showed bland, monomorphic cells growing in tubuloglandular and trabecular growth patterns. Cells were positive for neuroendocrine (synaptophysin, chromogranin, CD56) and epithelial markers (MOC31, CK7, CK19). Cytoplasmic dense neurosecretory vesicles were seen on ultrastructural examination. Based on the Ki-67 rate, mitotic count, lack of marked nuclear atypia and absence of necrosis, a diagnosis of primary neuroendocrine grade 2 was conferred.