成人肝脏未分化胚胎性肉瘤1例

患者女,46岁,以“右上腹部不适1月余”就诊.患者1个月前无明显诱因出现右上腹隐痛,伴有轻度腹胀,查体未见异常.B超:肝内6.3 cm×5.5 cm实性肿物,边界模糊,内呈不均质中低混合回声.CT:肝右叶有一不规则低密度肿块,约6.2 cm×5.4 cm,边界不清;增强扫描可见肿块呈囊状,边缘有包膜,内有多分隔,包膜及分隔强化,囊性部分无强化(图1).     

13例肝未分化(胚胎性)肉瘤临床病理学观察

目的 探讨肝未分化(胚胎性)肉瘤的临床病理、免疫组化特点和组织起源。方法 对13例肝未分化(胚胎性)肉瘤病例进行临床病理和免疫组化观察。结果13肝未分化(胚胎性)肉瘤男女之比为6：7，平均8．9岁。肿瘤位于肝右叶9例，两叶肝均有3例，肝左叶1例。肿瘤大小10～28cm，平均17．9cm。切面呈灰白、灰粉色胶冻样，均可见明显出血坏死，其中10例可见囊腔，囊腔直径1．5～10cm不等。光镜下见肿瘤黏液基质中散布未分化梭形或星形细胞，散在大的多形性或瘤巨细胞，细胞形态怪异；肿瘤边缘见包绕的肝细胞和扩张的胆管；9例可见胞质嗜酸性透明小体，PAS染色阳性；免疫组化瘤细胞表达a1．抗胰蛋白酶和波形蛋白。结论 肝未分化(胚胎性)肉瘤好发于儿童，具有独特的临床病理特点，可能起源于原始间质细胞，为预后较差之儿童肝肿瘤。     

老年人肝未分化胚胎性肉瘤临床病理观察

目的 分析老年人肝未分化胚胎性肉瘤的临床病理学特点,探讨其诊断和鉴别诊断.方法 对1例72岁女性患者肝未分化胚胎性肉瘤进行影像学、HE及免疫组化染色分析,并复习相关文献.结果 老年人肝未分化胚胎性肉瘤以女性多见,肿瘤多位于肝右叶,直径10 ～ 30 cm,切面灰白色、胶冻样,伴有出血、囊性变.镜检示黏液基质中散在分布未分化梭形或星形细胞,间有形态怪异、体积较大的多形细胞或多核巨细胞,肿瘤细胞胞质内可见PAS阳性嗜酸性透明小体.免疫组化:瘤细胞vimentin和al-抗胰蛋白酶弥漫(+).结论 发生于老年人的肝未分化胚胎性肉瘤极为罕见,确定诊断依靠组织形态学和免疫组化标记.治疗以手术切除为主,可辅以化疗,但预后极差.     

Neoadjuvant transcatheter arterial chemoembolization and systemic chemotherapy for the treatment of undifferentiated embryonal sarcoma of the liver in children

BACKGROUNDUndifferentiated embryonal sarcoma of the liver (UESL) is a rare and aggressive mesenchymal tumor in children. Herein, we describe our experience in neoadjuvant therapy (NAT) and subsequent surgery for the treatment of UESL in children.AIMTo evaluate the efficacy of NAT and explore a new choice for successful operation of UESL in children.METHODSWe retrospectively analyzed six patients newly diagnosed with unresectable UESL who received NAT and then surgery at our center between January 2004 and December 2019. The tumor was considered unresectable if it involved a large part of both lobes of the liver or had invaded the main hepatic vessels or inferior vena cava. The NAT included preoperative transcatheter arterial chemoembolization (TACE) and systemic chemotherapy. The patients were 4 boys and 2 girls with a mean age of 7 years. The longest tumor at presentation ranged from 8.6 to 14.8 cm (mean, 12 cm). Extrahepatic metastases were present in 2 cases. Preoperative systemic chemotherapy was administered 3 wk after TACE. Tumor resection was performed 3 wk after one or two cycles of NAT. The patients received systemic chemotherapy after surgery.RESULTSAll patients successfully underwent NAT and complete resection. The tumor volumes decreased by 18.2%–68.7%, with a mean decrease of 36% after 1 cycle of NAT (t = 3.524, P = 0.017). According to the Response Evaluation Criteria In Solid Tumors criteria, 4 patients had a partial response and underwent surgery, while 2 had stable disease and received another cycle of NAT before surgery. Massive tumor necrosis was seen on pathological examination of the surgical specimen: > 90% necrosis in two, > 50% necrosis in three, and 25% necrosis in 1, with an average of 71.8%. Post-NAT complications included fever, nausea and vomiting, and mild bone marrow suppression. Elevated alanine transaminase levels occurred in all patients, which returned to normal within 7–10 d after treatment. No cardiac or renal toxicity, severe hepatic dysfunction, bleeding and non-target embolization were observed in the patients. The median follow-up period was 8 years with an overall survival of 100%.CONCLUSIONNAT effectively reduced tumor volume, cleared the tumor margin, and caused massive tumor necrosis. This may be a promising choice for successful surgery of UESL in children.     

Recurrent undifferentiated embryonal sarcoma of the liver in adult patient treated by pembrolizumab: A case report

BACKGROUNDUndifferentiated embryonal sarcoma of the liver (UESL) is a neoplasm that rarely develops in adults. The main treatments for UESL are upfront gross total surgical resection and adjuvant multiagent chemotherapy. Here, we report a case of recurrent UESL in an adult treated with pembrolizumab and discuss a method to identify proper candidates for antibody of programmed cell death protein 1 (anti-PD-1) treatment.CASE SUMMARYA 69-year-old woman was admitted for abdominal pain that developed for 1 wk. Computed tomography showed a 16 cm mass in the right lobe of the liver. Right hemihepatectomy and lymphadenectomy were performed, and histological diagnosis was UESL. Six months later, the patient suffered from painless obstructive jaundice, and positron emission tomography-computed tomography revealed multiple metastases. Then, percutaneous transhepatic cholangial drainage was applied to reduce jaundice, and radiofrequency ablation was used to control the lesion near the hepatic hilum. However, the patient suffered from a serious fever caused by the tumor. The patient received treatment with pembrolizumab, and the prescribed dosage was 2 mg/kg every 3 wk. After the seventh dose, positron emission tomography-computed tomography revealed that the multiple metastases had nearly disappeared. Radiologic exam was used to evaluate the disease state, and no new lesions were found. Next-generation sequencing and immunohistology were applied to determine the reason why the patient had such a favorable response to pembrolizumab. Tumor mutation burden, microsatellite instability, and programmed death ligand 1 expression can be combined to predict the effect of PD-1 antibodies. When every one of these biomarkers are detected in a tumor patient, the patient may be a proper candidate for PD-1 antibodies.CONCLUSIONAnti-PD-1 treatment for tumors needs further research to identify indications and proper biomarkers.     

超声对儿童肝脏未分化胚胎性肉瘤与间叶错构瘤的鉴别诊断价值

目的 对比分析儿童肝脏未分化胚胎性肉瘤（UES）与间叶错构瘤（HMH）的声像图及临床特点,探讨超声对其的鉴别诊断价值。方法 收集我院经病理证实的19例UES患儿（UES组）与25例HMH患儿（HMH组）,均行超声检查,分析两组超声表现及临床资料的差异。经二元Logistic回归分析筛选,将具有统计学意义的指标纳入受试者工作特征（ROC）曲线并分析其诊断效能。结果 两组在发病部位、囊实比例及瘤体大小比较差异均无统计学意义,在回声均匀与否、厚壁囊腔、病变内出血及年龄比较差异均有统计学意义（均P＜0.05）。UES超声表现为实性区多为高低混杂不均回声,囊性区为多房分隔样,囊腔不规则,且无张力;HMH超声表现为实性区常为均匀偏高回声,囊腔较规则,囊壁有一定张力,亦可表现为一囊壁增厚的囊腔,即“厚壁囊腔”改变,此可看做HMH的特征性表现（这是属于HMH还是二者均有？）。二元Logistic回归分析显示,UES较HMH更易发生出血,且发病年龄明显高于HMH,差异均有统计学意义（均P＜0.05）;ROC曲线分析显示,病变内出血诊断UES的（什么的？）曲线下面积为0.861,敏感性84.2%,特异性88.0%;年龄鉴别二者的（什么的？）曲线下面积为0.931,敏感性89.5%,特异性88.0%,截断值为54个月。结论 超声在UES与HMH鉴别中具有重要的临床价值,结合患儿年龄及病变内是否出血,同时参考其回声特点,可对二者的鉴别做出提示。     

肝脏未分化胚胎性肉瘤的临床诊治

目的:探讨肝脏未分化胚胎性肉瘤的临床特征、治疗方案及预后。方法:回顾性分析17例肝脏未分化胚胎性肉瘤患者的临床资料,总结临床诊治经验。结果:17例患者中,男性10例、女性7例,年龄11～66岁(中位年龄47岁)。临床主要表现为腹部包块,可伴有腹痛、腹胀;诊断时肿瘤直径中位数为13 cm(6.5～40 cm)。15例患者接受外科手术治疗,其中8例接受单纯外科手术治疗,1例接受外科手术+经导管肝动脉化疗栓塞术(TACE),2例接受外科手术+化疗,4例接受外科手术+TACE+化疗;另外2例仅接受TACE。17例患者中位随访时间为11个月(1～60个月),2例失访,1例复发。完成随访的患者中,1年生存率为42.9%(6/14),2年生存率为28.6%(4/14)。6例术后联合化疗的患者中,1例至随访7个月时未复发,3例获得疾病缓解,2例出现疾病进展;6例患者均出现4级骨髓抑制。结论:肝脏未分化胚胎性肉瘤较罕见,病灶大,术后复发转移率高,治疗以外科手术为主,联合介入、化疗可提高患者生存率,但预后仍较差。     

Hepatic epithelioid hemangioendothelioma: Clinical characteristics,diagnosis, treatment,and prognosis

BACKGROUNDHepatic epithelioid hemangioendothelioma (HEHE) is a rare hepatic vascular tumor with unpredictable malignant potential. The etiology, characteristics, diagnosis, treatment, and prognosis of HEHE are not well-understood, and large-scale retrospective studies are required to understand better this disease.AIMTo determine the characteristics of HEHE and identify its optimal treatments and prognostic factors.METHODSThe clinical data of two patients diagnosed with HEHE at the Fourth Hospital of Hebei Medical University and 258 previously reported cases retrieved from the China National Knowledge Infrastructure and PubMed databases between 1996 and 2021 were combined and summarized. All cases were pathologically identified as HEHE. Information such as clinical features, laboratory examination findings, imaging findings, pathological characteristics, treatment, and survival periods was reviewed. Kaplan-Meir curves were used for survival analysis. Prognostic factors were identified by Cox regression analysis.RESULTSHEHE primarily affected middle-aged women. The typical manifestations included epigastric pain, hepatosplenomegaly, inappetence, distension, weight loss, and fatigue. Tumor markers were expressed normally. The incidence of extrahepatic metastasis was 34.5% at the time of diagnosis. The most common sites of extrahepatic involvement were the lungs (22.3%), lymph nodes (5.6%), peritoneum (3.6%), bones (6.6%), and spleen (5.1%). Furthermore, “capsular retraction”, “target sign”, and “lollipop sign” were the characteristic features of HEHE on imaging. The immunohistochemical profile for HEHE (expression of vascular markers, such as factor VIII-related antigen, CD31, and CD34; expression levels of D2-40) can facilitate and ensure an accurate diagnosis. The management options for patients with HEHE include liver resection (29.7%), liver transplantation (16.1%), palliative treatments (12.7%), transhepatic arterial chemotherapy and embolization (TACE, 10.2%), chemotherapy (11.0%), antiangiogenic therapy (15.3%), and other treatments (5.1%); the mean survival time was 158.6, 147.3, 4.2, 90.8, 71.4, 83.1, and 55.0 mo, respectively. The survival time of patients who underwent surgical treatment was longer than that of patients who did not. TACE and antiangiogenic therapy tended to prolong survival compared with other nonsurgical treatments. The 1-, 5-, and 10-year survival rates were 82%, 71%, and 64%, respectively. Multivariate analysis showed that liver function (P = 0.045), intrahepatic metastasis (P = 0.029), and treatment (P = 0.045) were independent prognostic factors. The presence of extrahepatic metastases was not an independent risk factor for poor prognosis (P = 0.558).CONCLUSIONThe clinical course of HEHE is rare and variable, and patients with intrahepatic metastases and liver dysfunction may have a poorer prognosis than those without. Surgical intervention, whether liver resection or transplantation, might be warranted regardless of extrahepatic metastasis. For patients without the option for surgery, clinicians should consider the use of TACE with antiangiogenic drugs in the treatment of HEHE.     

Undifferentiated embryonal sarcoma of the liver treated with chemotherapy: CT imaging in four patients

Background: The purpose of this study was to describe the computed tomography (CT) findings of undifferentiated embryonal sarcoma after chemotherapy and to correlate the CT imaging findings with pathologic findings. Methods: Ten CT images obtained before and after chemotherapy in four patients with hepatic undifferentiated embryonal sarcoma were retrospectively reviewed and correlated with pathologic findings. Results: After chemotherapy, tumor volume decreased by 50–90% and initially nonresectable tumor or gross residual tumor was successfully excised in three patients. In all patients, enhancing peripheral solid portions and septations changed to low-attenuation areas, and in three patients increased or de novo calcifications were found at the periphery of the tumor. Resected pathologic specimen after chemotherapy showed well-encapsulated masses with central necrosis, fibrosis, and dystrophic calcifications. Conclusions: These CT findings will be useful in monitoring the treatment response of hepatic undifferentiated embryonal sarcoma during chemotherapy.     

子宫内膜间质肉瘤13例临床病理分析

目的提高子宫内膜间质肉瘤的早期诊断率,以确定手术范围,避免二次手术。方法对13例子宫内膜间质肉瘤临床病理资料进行回顾性分析。结果确诊主要依靠病理结果,术前诊断性刮宫结合宫腔镜,宫颈赘生物活检确诊率为60%(3/5);临床分期为I期11例,Ⅱ期1例,Ⅳ期1例;行全子宫及双侧附件(包括次广泛)切除6例;行全子宫切除或全子宫及单侧附件切除5例,残端复发灶切除1例,行宫颈赘生物摘除1例;术后11例行全身化疗。结论对此类患者,术前诊断性刮宫是必要的。疑有恶性应送冰冻病理检查;以采用全子宫加双侧附件切除术辅以化疗者疗效最好。     

肝未分化胚胎性肉瘤的CT及MRI表现

目的 探讨肝未分化胚胎性肉瘤(UESL)的螺旋CT及高场强MR的影像学表现,提高UESL诊断准确率.方法 回顾性分析14例经手术病理证实的UESL患者.螺旋CT检查5例,MR检查9例.结果 5例患者CT扫描均表现为平扫边界清晰的囊状低密度影,其内含有不规则的软组织影,增强后动脉期软组织强化,门脉期及延迟期继续强化.MRI 9例患者中6例T1WI表现为囊状高低信号混杂影,T2WI病灶以高信号为主.增强后动脉期病灶边缘强化,门脉期及延迟期继续强化;3例患者T1WI表现为囊状低信号影,T2WI呈高信号.增强后动脉期病灶边缘强化,门脉期及延迟期继续强化.结论 平扫和动态增强螺旋CT及高场强MRI能反应UESL的影像学特点,提高了UESL诊断准确率.     

急性药物性肝损伤64例临床分析

目的探讨64例急性药物性肝损伤（drug-induced liver injury,DILI）患者的临床特征。方法采用回顾性分析法对64例急性DILI住院患者的用药史、临床表现及治疗转归进行分析。结果多种药物均可引起急性DILI,以抗结核药物为主,占23.4%,其次为中药23.4%,抗菌药11.0%;临床类型为肝细胞型多见,占84.4%,胆汁淤积型占11.0%,混合型占4.7%;主要临床表现有纳差、乏力、黄疸、恶心、呕吐等;绝大多数患者治疗后预后较好,但重症患者仍可危及生命。结论多种药物均可引起急性DILI,以抗结核药和中药为主;急性DILI以肝细胞损伤型最为常见;大多患者预后良好,但重症患者仍可危及生命。     

老年急腹症的临床特点及诊治要点

目的探讨老年急腹症的临床特点及诊治要点。方法回顾性分析老年急腹症216例（老年组）和中青年156例（中青年组）的临床资料,对其病情、症状、伴随疾病、实验室及辅助检查、诊断与治疗、住院时间等进行比较分析。结果老年组以腹胀、肛门停止排气和排便、腹腔积液居多（P〈0．01）,中青年组则以体温升高、腹部压痛、反跳痛及肌紧张多见（P〈0．01）;老年组伴随疾病发生率为92．1％,中青年组为29．5％,两组比较差异有统计学意义（P〈0．01）;老年组手术治疗161例,非手术治疗55例,治愈或好转209例,死亡7例;中青年组手术治疗149例,非手术治疗7例,均治愈出院。老年组平均住院时间（25．5±2．5）d,中青年组（16．5±1．5）d,两组比较差异有统计学意义（P〈0．01）。结论老年组急腹症临床表现不典型、伴随疾病多、病情变化快,应重视各种辅助检查,及时早期诊断,合理选择个体化治疗方案,是提高老年急腹症治愈率的关键。     

胃原发胚胎性横纹肌肉瘤临床诊断学特征及文献复习

目的探讨胃原发性胚胎性横纹肌肉瘤的临床诊断学特征。 方法回顾性分析1例临床表现为成人原发于胃的胚胎性横纹肌肉瘤患者的临床资料,并进行相关文献复习。 结果患者表现为腹痛、腹部肿物等,胃镜显示胃贲门、胃底、胃体占位,贫血胃,胃窦息肉。胃镜病理结果提示胚胎性横纹肌肉瘤,镜下肿瘤细胞弥漫分布,排列呈片状、巢状,瘤细胞较小,呈圆形,核深染,居中或偏位,胞质少,核分裂易见。免疫组化Myogenin＋,Desmin＋。 结论成人原发于胃的胚胎性横纹肌肉瘤是一罕见的高度恶性软组织肿瘤,需要结合临床影像学、病理学、免疫学等多种诊断方法,可作出正确诊断。     

血脂正常的脂肪肝病人的临床特征--附137例分析

目的:分析血脂正常者发生脂肪肝的危险因素及其]临床特征.方法:回顾性分析137例血脂正常的脂肪肝(血脂正常脂肪肝组)和137例血脂正常的非脂肪肝(对照组)病人的临床资料,对肥胖等相关因素进行Logistic回归分析,并比较血脂正常脂肪肝组和高脂血症脂肪肝组病人的临床特征、肝功能改变差异.结果:血脂正常患者发生脂肪肝的独立相关危险因素为嗜酒、肥胖、2型糖尿病、空腹血糖升高、高尿酸血症,其比数比(odds rate,0R)分别为27.49、26.95、3.14、1.60和1.00.血脂正常脂肪肝组患者的γ-谷氨酰转移酶(27.7%)和球蛋白(5.8%)升高及A/G异常(13.1%)的比例均显著低于高脂血症脂肪肝组,后者相应为38.8%、12.3%和23.9%,均为P＜0.05.结论:嗜酒、肥胖、2型糖尿病、空腹血糖升高和高尿酸血症是血脂正常者发生脂肪肝的独立相关危险因素.血脂正常脂肪肝组患者的肝功能损害较高脂血症脂肪肝组患者为轻.     

15例肺梭形细胞癌患者的临床特征及预后

目的探讨肺梭形细胞癌的临床病理特征及预后。 方法收集2012年1月至2014年12月郑州大学第一附属医院确诊的15例肺梭形细胞癌患者的临床资料,应用Kaplan-Meier法计算生存率并绘制生存曲线。 结果6058例肺癌患者中15例确诊为肺梭形细胞癌,其中男性12例,女性3例,中位年龄58岁,9例有大量吸烟史。CT显示肿瘤平均直径（53.6±20.3）mm,其中2例侵犯相邻结构,12例为外周型。7例患者行PET-CT,肿块平均标准摄取值为12.8±4.3,高于其他同分期的非小细胞型肺癌（NSCLC）（P=0.003）。中位生存期为12.1个月,接受外科治疗的11例患者中复发和转移较常见,手术治疗患者较非手术者生存时间更长（P=0.01）,术后化疗患者较未行化疗手术患者在生存时间未能明显获益（P=0.03）。 结论肺梭形细胞癌极为罕见,侵袭性强,易复发、转移,对化疗反应差,目前早期手术为主要治疗办法。     

残胃癌的临床特征及外科治疗

目的探讨残胃癌的临床特征及治疗方法.方法回顾分析2000年1月至2010年12月上海新华医院普外科收治的32例残胃癌患者的临床资料.结果 32例患者确诊残胃癌距首次手术时间平均为21年;初次手术行Billrotn II式胃大部切除术24例;残胃癌发生在吻合口18例;残胃手术切除率为73.3%.结论 残胃癌大多发生于初次胃部分切除术后10年以上,多发生于Billroth II术式,癌病变主要位于吻合口附近.早期诊断、早期治疗,根治性手术是残胃癌的有效治疗方法.     

Dieulafoy溃疡15例临床特征

目的 探讨Dieulafoy溃疡的临床特点与治疗方法,以提高临床早期诊治水平.方法 对北京大学第三医院急诊科15例Dieulafoy溃疡的临床资料进行回顾性分析.结果 男性多于女性,男女比例为6.5∶1,平均年龄48.3岁,大部分患者无明显诱因,少数患者存在饮酒(1/15)、服用NSAID药物(2/15),进食刺激性食物(2/15)等诱因,所有患者既往均无消化性溃疡及肝硬化病史,部分患者有糖尿病(3/15)、高血压(5/15)病史,患者均以突发呕血、便血发病伴失血性休克,中重度贫血,病灶部位以胃后壁(9/15)、近小弯侧多见(10/15).急诊内镜检查为首选的诊断方法,14例内镜下药物注射、钳夹止血成功(14/15),1例手术治疗.结论 Dieulafoy溃疡以突发致命性大出血为特点,中老年男性多见,好发于胃后壁、近胃小弯侧区域,内镜检查是诊断Dieulafoy病最简单有效的手段,急诊内镜下钳夹止血治疗Dieulafoy溃疡的疗效可靠.