成人肝母细胞瘤1例

患者男，49岁。因低热、上腹隐痛40天余伴背部疼痛入院。体检：右肋压痛，无腹壁静脉曲张，肝脾未触及。实验室检查：HBSAg（＋），抗HBe（＋），抗HBC（＋），ALP73U／L，AKP487U／L，ASP112U／L，AFP＞400ug／L。B超未见肝内实性占位；CT示肝左叶占位；胸片示右下胸腔中等积液。胸水细胞学检查见大量间皮细胞增生，个别细胞有轻度异型。临床诊断为肝癌。患者术前行肝动脉介入化疗一周，化疗后AFPlug／ml。入院14天后行肝左叶切除术。术中见癌肿位于左叶，10×10×8cm3大小，与隔肌有粘连，癌肿边界清，肝门淋巴结肿大。术后…     

小儿肝母细胞瘤29例诊治分析

２９例小儿肝母细胞瘤依时间和治疗原则不同分为早期（１９６５～１９８０年行手术切除）、中期（１９８０～１９９１年行手术切除＋术后化疗）、后期（１９９１～１９９５年行术前化疗＋手术＋术后化疗）。早、中期总体手术切除率分别为３７．５％、３０％，总体１年无瘤生存率２５％和３０％。后期总体切除率６２．５％，１年无瘤生存率５０％。统计学分析表明，后期生存率提高是由于采用术前化疗，使进展期肿瘤切除病例增多。认为加强对Ⅲ、Ⅳ期肿瘤的术前化疗，是提高其切除率、改善本病总体预后的关键     

肝母细胞瘤肝切除术60例临床分析

目的 分析肝母细胞瘤(HB)肝切除术的方式及病理分型.方法 回顾性分析郑州大学附属儿童医院2017-04-2020-04间收治的60例HB患儿的临床资料.男40例,女20例;年龄(3.75±1.64)岁.11例瘤体直径≤5 cm,12例5～10 cm,37例＞10 cm.结果 右半肝切除15例(25.00％),右肝局部...     

以性早熟为临床表现的肝母细胞瘤1例

患儿,2岁,半年前出现阴茎逐渐肥大、增长、增粗,包皮上翻,食欲、大便正常,小便清,无发热。查体:腹膨隆,无明显压痛,未触及明显包块,肝脾触及不满意。化验:睾丸酮55.92μmol/d,尿17-羟1.73μmol24小时。B超示左上腹部实质性肿块。CT示腹块可能来自肝脏。手术见肿块来自肝左外叶,约7cm×10cm×10cm,占据大部分腹腔。切除肝左外叶和肿块,病理报告为肝母细胞瘤。术后睾丸酮0.44ng/dl。     

成人肾母细胞瘤（附12例报告）

自１９５８年１月～１９９１年１２月收治成人肾母细胞瘤１２例，均经病理证实，占同期收治的肾恶性肿瘤的２．５３％（１２／４７５）。患者男２例，女１０例。年龄１６～５４岁。临床分期，Ⅰ、Ⅱ期５例（４２％），Ⅲ、Ⅳ期７例（５８％）。症状主要为血尿，腹部肿块，腰腹部疼痛或发热。本组病人以手术加放化疗为主要治疗手段。随访其３、５年生存率均为３３％，２例术后无瘤生存达２０年以上。认为对成人肾母细胞瘤Ⅱ期病例应采取手术加放、化疗的综合治疗，以期提高治愈率     

成人肝母细胞瘤二例

成人肝母细胞瘤比较少见 ,我院近期收治两例 ,现报告如下。病例 1 :男 ,72岁 ,左上腹部疼痛不适 6个月 ,于 2 0 0 1年 2月 5日入院。ＡＦＰ >50 0ｍｇ/Ｌ ,ＣＴ :肝左外叶见一 8ｃｍ× 1 0ｃｍ密度均匀的低密度病灶 ,边界清晰。剖腹探查发现肝左叶有一 8ｃｍ× 1 0ｃｍ肿块 ,表面布满曲张血管(静脉 ) ,行肝左叶切除术。病理检查 :手术切除的肝左叶有一1 0ｃｍ× 8ｃｍ肿块 ,暗红色 ,表面平滑 ,可见较多曲张血管。切面呈红黄色。肿块周边区与肝组织分界清晰。残余肝组织内未见转移瘤结节或肝硬化现象。光镜观察 :瘤组织主要由胚胎性肝上皮样…     

老年人肝母细胞瘤一例

病人,女,68岁.因右侧腰背部隐痛7 d入院.无肝炎病史.体检肝右肋下3 cm,质中等,表面尚光滑,无明显压痛.腹壁静脉无曲张.脾不大.乙肝5项(-) .肝功能正常.AFP <20 μg/L,CEA 10.97 μg/L,β-HCG >3.1 μg/L(正常<3.1 μg/L).B超:肝右叶下缘10.8 cm×7.9 cm不均质低回声,可见部分包膜,血流丰富,考虑为肝癌.CT:肝右叶下段10.8 cm×8.5 cm卵圆形低密度影,边清,增强后动脉期病灶边缘呈结节状强化.延迟5 min扫描,病灶呈均匀等密度强化.考虑肝血管瘤.手术探查:肝无硬化表现.肝Ⅴ、Ⅵ段悬垂性肿瘤,10 cm×10 cm×8 cm大小,质软,血管瘤样,边不清.行肝肿瘤切除及胆囊切除.术后顺利恢复出院.病检结果:成人型肝母细胞瘤.免疫组化:AFP(+),HCG(-),AAT(-),CK(±),EMA(±).     

小儿肝母细胞瘤的临床特点与诊治

小儿肝母细胞瘤又称幼儿型肝细胞瘤，也属肝脏原发性恶性肿瘤，在临床上较为罕见。我院自1985年以来共收治了6例。鉴于本瘤在临床病理特点、诊治等方面与小儿肝细胞癌有不同之处，现予报告并分析如下。1临床资料6例中男4例，女2例。年龄6个月至4岁，其中＜3岁者5例。均以发现右上腹肿块而就诊。其它伴随症状与体征：出现不同程度的发热3例，短期内体重下降伴贫血貌3例，纳差、腹胀2例，腹壁静脉曲张2例，黄疸1例。辅助检查：2例血清AFP对流法（＋），4例放免法除1例＜20ng／ml；余3例均有不同程度的升高。B超或CT检查提示肝脏弥漫性肿大…     

小儿肝母细胞瘤的诊治回顾分析

小儿肝母细胞瘤早期无特殊症状，发现时肿瘤已较大，加上既往医生对肝脏解剖不熟悉，对肝部分切除缺乏信心，故肝部分切除率很低，仅作探查肿物活检或劝家属放弃治疗，故疗效差。１９８５年以后，随着肝脏外科飞速发展，同时Ｂ超、ＣＴ、ＭＲＩ的广泛应用，肝部分切除率明显提高，疗效大为改观，联合化疗，２年成活率达７２％。本文回顾分析了１９５５～１９９９年收治的６７例，旨在提高疗效。１　临床资料本组６７例，男４３例，女２４例；年龄最小１月，最大９岁，３岁以内６２例，占９２．５％。临床表现：腹胀６７例，腹部包块５４例，…     

Hepatoblastoma of the adult with pericardial metastasis: A case report

BackgroundHepatoblastoma is the most frequent liver tumor in children, but very rare in the adult and associated with an unfavorable prognosis. The diagnosis is always postoperative or post mortem and biopsy is not useful. Surgery is the only accepted treatment.Case presentationOur patient underwent surgery in the suspect of liver metastasis from a previous gastric cancer. Surgery consisted in left lobectomy with partial diaphragm resection and partial pericardiectomy for a pericardial lesion, found after the opening of the thorax. The diaphragm defect was corrected with a biological mesh.ResultsThe histopathological examination indicated hepatoblastoma of the adult with pericardial metastases. The patient was asymptomatic and without recurrence after 21 months of follow up.ConclusionThe hepatoblastoma of the adult is related to a poor prognosis with median survival time less than 5 months. Surgery is the only curative treatment, but in many cases tumor resection requires complex operations. Vascular and thoracic expertise could be useful in the management of hepatoblastoma.     

成人婴儿型肝脏血管内皮细胞瘤的诊治：附7例报告

目的：总结成人婴儿型肝脏血管内皮细胞瘤（IHHE）的临床病理特征,提高对该病的诊治水平。 方法：回顾性分析2003年1月—2013年12月东方肝胆外科医院手术治疗并病理证实的7例成年IHHE患者（32~63岁）临床病理资料。 结果：7例患者中,5例患者术前无明显临床症状,另2例分别表现为右上腹间隙性疼痛和食欲减退;血常规、肝功能指标均无明显异常,肿瘤指标、肝炎病毒指标均为阴性,CD34免疫组化均为阳性;病灶均为单个,且术前影像学检查术前均未确诊。所有患者术后均定期随访,至2014年8月,未出现肿瘤复发。 结论：IHHE在成人肝脏肿瘤中极为罕见,临床上患者无肝炎背景,肿瘤指标均为阴性,结合CT及MRI表现,排除海绵状血管瘤,可以考虑该病的可能。     

Adult hepatoblastoma: A case report and literature review

INTRODUCTIONAdult hepatoblastoma is a rare malignant liver neoplasm. Surgery is the only cure, but recurrence is common even after complete resection. No therapeutic strategy has been established.PRESENTATION OF CASEA 22-year-old man presented with a rapidly expanding right hypochondrial mass. Pain preceded the appearance of the mass. No definitive diagnosis was established in the referring hospital. In addition, two attempts of embolization failed to reach the tumor due to its large size and vascular displacement. Clinical examination revealed a 26 cm × 23 cm mass occupying the right hypochondrium and epigastrium as far as the right iliac fossa, compressing the stomach, spleen, kidneys and liver. The preoperative diagnosis was gastrointestinal stromal tumor because it appeared to originate from the stomach. During surgery, we found a mass arising from the liver, adhering to the omentum, stomach, and left hemidiaphragm, and infiltrating the pericardium. The tumor was completely resected off the inferior vena cava and pericardium. The histopathological diagnosis was a 30 cm × 30 cm hepatoblastoma weighing 4 kg. The postoperative treatment course went smoothly until day 10, when the patient developed complications like bilateral atrial thrombi and left ventricular hypokinesia and expired on day 16.DISCUSSIONDue to the rarity of hepatoblastoma in adults and non-specific initial symptoms, hepatoblastoma is often overlooked as a diagnosis. Early detection may lead to improved prognosis and survival.CONCLUSIONWe report here the first case of adult hepatoblastoma in the Middle East and the largest such tumor ever reported in literature.     

Hepatoblastoma in Children

Hepatoblastoma is the most common primary liver tumour in children. Complete surgical removal is the treatment of choice for cure ; however, in most cases the tumour is unresectable because of its extensive hepatic involvement.

Nineteen pediatric cases (11 boys, 8 girls) with ages ranging from three months to 17 years were referred for management to our clinic from 1982 until 2000. All but three suffered from abdominal distention. The other frequent complaints were abdominal mass, anorexia, fatigue, abdominal pain and fever. Physical examination revealed enlarged liver in all patients. In addition to laboratory studies, they were pre-operatively examined by ultrasonography and, in recent cases, computed tomography was also used. Serum alpha-fetoprotein levels were found to be elevated in all patients. In thirteen cases, hepatic resections (10 lobectomies, 2 trisegmentectomies, 1 segmentectomy) were performed. In six children only liver biopsies could be done because of the huge tumour size. However, in three of them the tumours were excised at the second laparotomy, but only one patient survived. All of the patients-except two who were lost in the early postoperative period-received chemotherapy whether the tumour was excised or biopsied. In this series the mortality rate was found to be very high (91%) in the 1980s, and more reasonable (50%) in the 1990s, with an overall mortality rate of 73 per cent.

This result might be explained with late referral and advanced stage at diagnosis. In addition, we speculate that a combination of improved chemotherapy and technical advances in anesthesia and hepatic resection caused the obvious differences in the survival rates between the two periods.     

成人活体肝移植71例报道

目的 探讨开展成人活体肝移植初始阶段如何确保供、受者安全.方法 回顾性分析我院2007年4月至11月71例成人活体肝移植供、受者临床资料,分析评估方案、手术策略和并发症.结果 切取供者右半肝68例,其中带肝中静脉4例;切取带肝中静脉左半肝3例.术后出现并发症2例,1例胆漏,1例腹腔内出血,无供者死亡.受者外科并发症18例,其中胆道并发症12例,血管并发症3例,小肝综合征3例;病死率为10%(7/71).结论 在开展成人活体肝移植的初始阶段,采用严格的供、受者评估、选择合理的手术方式和术后处理可以最大程度地保证供、受者安全.     

腹腔镜肝切除术的体会(附6例临床报告)

目的 探讨腹腔镜肝切除术的适应证和可行性.方法 回顾分析5例病灶位于肝脏Ⅱ～Ⅵ段表面及边缘,1例病灶位于Ⅷ段表面的患者的临床资料.其中肝脏海绵状血管瘤4例,肝脏局灶性结节样增生2例,肝脏占位直径5～9.6 cm,平均(6.64±2.60)cm,6例肝功能Child评分均为A级.结果 6例均成功完成腹腔镜肝切除术,无中转...     

Surgical Management and Outcomes of Patients with Multifocal Hepatoblastoma

ObjectiveTo compare the outcomes of patients with multifocal hepatoblastoma (HB) treated at our institution with either orthotopic liver transplant (OLTx) or hepatic resection to determine outcomes and risk factors for recurrence.BackgroundMultifocality in HB has been shown to be a significant prognostic factor for recurrence and worse outcome. The surgical management of this type of disease is complex and primarily involves OLTx to avoid leaving behind microscopic foci of disease in the remnant liver.MethodsWe performed a retrospective chart review on all patients <18 years of age with multifocal HB treated at our institution between 2000 and 2021. Patient demographics, operative procedure, post-operative course, pathological data, laboratory values, short- and long-term outcomes were analyzed.ResultsA total of 41 patients were identified as having complete radiologic and pathologic inclusion criteria. Twenty-three (56.1%) underwent OLTx and 18 (43.9%) underwent partial hepatectomy. Median length of follow-up across all patients was 3.1 years (IQR 1.1–6.6 years). Cohorts were similar in rates of PRETEXT designation status identified on standardized imaging re-review (p = .22). Three-year overall survival (OS) estimate was 76.8% (95% CI: 60.0%–87.3%). There was no difference in rates of recurrence or overall survival in patients who underwent either resection or OLTx (p = .54 and p = .92 respectively). Older patients (>72 months), patients with a positive porta hepatis margin, and patients with associated tumor thrombus experienced worse recurrence rates and survival. Histopathology demonstrating pleomorphic features independently associated with worse rates of recurrence.ConclusionsThrough proper patient selection, multifocal HB was adequately treated with either partial hepatectomy or OLTx with comparable outcome results. HB with pleomorphic features, increased patient age at diagnosis, involved porta hepatis margin on pathology, and the presence of associated tumor thrombus may be associated with worse outcomes regardless of the local control surgery offered.Level of EvidenceIII.     

肝、肾联合移植12例报告

目的 对肝、肾联合移植的临床情况进行总结。方法 为12例肝、肾功能异常患者施行肝、肾联合移植，采用多器官联合切取术整块切取供者器官。8例行经典式肝移植，4例行背驮式肝移植，均未行体外静脉转流；肾移植为常规术式。术前进行抗CD25单克隆抗体和抗胸腺细胞球蛋白诱导治疗，术后应用他克莫司（FK506）、霉酚酸酯及泼尼松预防排斥反应。结果 12例手术均获成功，移植肝及肾功能恢复良好。术后的并发症有移植肝急性排斥反应、FK506中毒、消化道出血、腹腔出血、肺部感染、腹腔感染（各1例次），所有患者均未发生移植肾急性排斥反应。结论 肝、肾联合移植是治疗终末期肝病合并肾功能衰竭的理想选择。     

成人活体肝移植单中心56例临床报告

目的探讨成人间右半肝移植手术中保证供、受者安全的方法。方法2002年1月至2006年9月四川大学华西医院对56例受者施行了成人右半肝移植,其中52例不含肝中静脉,4例双供肝肝移植。受者原发病为乙型肝炎肝硬化35例（62．5％,含急性肝功能衰竭12例）,肝细胞肝癌17例（30．4％）,其他4例;MELD评分〉25分者10例。供者常规行三维CT计算全肝体积及右半肝体积,并进行了移植手术技术改进。结果58例供者共摘取55例右半供肝及3例左半供肝。右半供肝均不含肝中静脉,重量为400—860g（中位数550g）,右半供肝与受者标准肝重比为31．7％-71．7％（中位数45．4％）,供者残肝体积均大于全肝体积的35％。58例供者发生并发症7例（12．5％）,无死亡。术后住院时间7—30d（中位数11d）。术后对56例受者随访2—52个月（中位数11个月）,发生并发症15例（26．8％）,死亡4例（7．2％）。1年实际生存率92．8％。结论采用不包含肝中静脉的右半供肝,术前CT测量残肝体积〉35％,同时右半供肝与受者标准肝重比〉40％者进行右半肝移植可保证供受者安全,反之则应考虑采用双供肝肝移植。