Hepatic perivascular epithelioid cell tumors:The importance of preoperative diagnosis

Accurate preoperative diagnosis is highly important for the treatment of perivascular epithelioid cell tumors(PEComas)because PEComas are mainly benign tumors and may not require surgical intervention.By analyzing the causes,properties and clinical manifestations of PEComas,we summarize the challenges and solutions in the diagnosis of PEComas.

Hepatic hemangiomas mimicking gastrointestinal stromal tumors: A case report

BACKGROUND Hepatic hemangiomas can be challenging to diagnose,particularly when they present with atypical features that mimic other conditions,such as gastrointestinal stromal tumors(GISTs).This case highlights the diagnostic difficulties encountered when imaging subepithelial lesions,especially when conventional methods such as computed tomography(CT)and endoscopic ultrasound(EUS)are used.CASE SUMMARY A 44-year-old woman presented with intermittent abdominal distension and heartburn for three months.Her medical history included iron deficiency anemia,menorrhagia,and previous cholecystectomy.One week prior to admission,an endoscopy suggested a bulging gastric fundus,which was likely a GIST,along with chronic nonatrophic gastritis and bile reflux.CT and EUS revealed nodules in the gastric fundus,which were initially considered benign tumors with a differential diagnosis of stromal tumor or leiomyoma.During surgery,unexpected lesions were found in the liver pressing against the gastric fundus,leading to laparoscopic liver resection.Postoperative pathology confirmed the diagnosis of hepatic cavernous hemangiomas.The patient recovered well and was discharged five days later,with normal follow-up results at three months.CONCLUSION This case underscores the challenges in the preoperative diagnosis of GISTs,particularly the limitations of the use of CT and EUS for the evaluation of subepithelial lesions.While CT is the primary tool for visualizing abdominal tumors,it is difficult to detect smaller lesions and assess the layers of the gastrointestinal wall on CT.EUS is recommended for the evaluation of nodules smaller than 2 cm and is useful for distinguishing GISTs from other lesions;however,its accuracy with regard to the differential diagnosis is relatively low.In this case,the gastric distension observed on imaging led to the compression of a liver tumor against the stomach,resulting in the misinterpretation of the tumor as a gastric wall lesion.

Primary hepatic neuroendocrine tumor with a suspicious pulmonary nodule:A case report and literature review

BACKGROUND Primary hepatic neuroendocrine tumors(PHNETs)are extremely rare tumors originating from neuroendocrine cells.Due to lack of neuroendocrine symptoms and specific radiographic characteristics,PHNETs are challenging to differentiate from other liver tumors.CASE SUMMARY This case involved a 67-year-old male who was admitted with a discovered hepatic mass and a suspicious lung lesion.Primary hepatic carcinoma was initially speculated based on the characteristic magnetic resonance imaging findings.The patient underwent a laparoscopic right partial hepatectomy,and subsequent immunohistochemical examination revealed a HNET.To exclude other potential origins,a positron emission tomography-computed tomography scan and gastrointestinal endoscopy were performed,leading to a final diagnosis of PHNETs.Then we conducted a literature review using the PubMed database,identifying 99 articles and 317 cases related to PHNETs.The characteristics,diagnostic methods,and treatment of PHNETs have been described.Finally,we elaborate on the presumed origins,pathological grades,clinical features,diagnosed methods,and treatments associated with PHNETs.CONCLUSION The diagnosis of PHNETs was primarily an exclusionary process.A definitive diagnosis of PHNETs relied mainly on immunohistochemical markers(chromogranin A,synaptophysin,and cluster of differentiation 56)and exclusion of primary foci in other organs.Radical surgery was the preferred treatment for early-stage tumors.

Efficient Computer Aided Diagnosis System for Hepatic Tumors Using Computed Tomography Scans

One of the leading causes of mortality worldwide is liver cancer.The earlier the detection of hepatic tumors,the lower the mortality rate.This paper introduces a computer-aided diagnosis system to extract hepatic tumors from computed tomography scans and classify them into malignant or benign tumors.Segmenting hepatic tumors from computed tomography scans is considered a challenging task due to the fuzziness in the liver pixel range,intensity values overlap between the liver and neighboring organs,high noise from computed tomography scanner,and large variance in tumors shapes.The proposed method consists of three main stages;liver segmentation using Fast Generalized Fuzzy C-Means,tumor segmentation using dynamic thresholding,and the tumor’s classification into malignant/benign using support vector machines classifier.The performance of the proposed system was evaluated using three liver benchmark datasets,which are MICCAI-Sliver07,LiTS17,and 3Dircadb.The proposed computer adided diagnosis system achieved an average accuracy of 96.75%,sensetivity of 96.38%,specificity of 95.20%and Dice similarity coefficient of 95.13%.

Avoiding misdiagnosis of multilocular thymic cysts as malignant tumors on computer tomography

This editorial provides insights from a case report by Sun et al published in the World Journal of Clinical Cases.The case report focuses on a case where a multilocular thymic cyst(MTC)was misdiagnosed as a thymic tumor,resulting in an unnecessary surgical procedure.Both MTCs and thymic tumors are rare conditions that heavily rely on radiological imaging for accurate diagnosis.However,the similarity in their imaging presentations can lead to misinterpretation,resulting in unnecessary surgical procedures.Due to the ongoing lack of comprehensive knowledge about MTCs and thymic tumors,we offer a summary of diagnostic techniques documented in recent literature and examine potential causes of misdiagnosis.When computer tomography(CT)values surpass 20 Hounsfield units and display comparable morphology,there is a risk of misdiagnosing MTCs as thymic tumors.Employing various differential diagnostic methods like biopsy,molecular biology,multi-slice CT,CT functional imaging,positron emission tomography/CT molecular functional imaging,magnetic resonance imaging and radiomics,proves advantageous in reducing clinical misdiagnosis.A deeper understanding of these conditions requires increased attention and exploration by healthcare providers.Moreover,the continued advancement and utilization of various diagnostic methods are expected to enhance precise diagnoses,provide appropriate treatment options,and improve the quality of life for patients with thymic tumors and MTCs in the future.continued advancement and utilization of various diagnostic methods are expected to enhance precise diagnoses,provide appropriate treatment options,and improve the quality of life for patients with thymic tumors and MTCs in the future.

Innovative applications and research progress of hepatic arterial infusion chemotherapy in the treatment of advanced hepatocellular carcinoma

This article provides an in-depth analysis of the study conducted by Wang et al,which explores hepatic arterial infusion chemotherapy and its synergistic strategies in managing advanced hepatocellular carcinoma(HCC).HCC ranks as the fourth most common cause of cancer-related mortality globally and is frequently associated with portal vein tumor thrombus(PVTT).The approach to managing HCC,particularly when PVTT is present,diverges markedly between Eastern and Western practices.These differences are rooted in variations in epidemiology,etiology,pathology,comorbidities,and prognosis.The paper delves into the diagnosis,classification,and treatment strategies for HCC with PVTT,as well as the evolving role and advancements of hepatic arterial infusion chemotherapy in the therapeutic landscape of HCC.

Utility of plasma D-dimer for diagnosis of venous thromboembolism after hepatectomy

BACKGROUND Venous thromboembolism(VTE)is a potentially fatal complication of hepatectomy.The use of postoperative prophylactic anticoagulation in patients who have undergone hepatectomy is controversial because of the risk of postoperative bleeding.Therefore,we hypothesized that monitoring plasma D-dimer could be useful in the early diagnosis of VTE after hepatectomy.AIM To evaluate the utility of monitoring plasma D-dimer levels in the early diagnosis of VTE after hepatectomy.METHODS The medical records of patients who underwent hepatectomy at our institution between January 2017 and December 2020 were retrospectively analyzed.Patients were divided into two groups according to whether or not they developed VTE after hepatectomy,as diagnosed by contrast-enhanced computed tomography and/or ultrasonography of the lower extremities.Clinicopathological factors,including demographic data and perioperative D-dimer values,were compared between the two groups.Receiver operating characteristic curve analysis was performed to determine the D-dimer cutoff value.Univariate and multivariate analyses were performed using logistic regression analysis to identify significant predictors.RESULTS In total,234 patients who underwent hepatectomy were,of whom(5.6%)were diagnosed with VTE following hepatectomy.A comparison between the two groups showed significant differences in operative time(529 vs 403 min,P=0.0274)and blood loss(530 vs 138 mL,P=0.0067).The D-dimer levels on postoperative days(POD)1,3,5,7 were significantly higher in the VTE group than in the non-VTE group.In the multivariate analysis,intraoperative blood loss of>275 mL[odds ratio(OR)=5.32,95%confidence interval(CI):1.05-27.0,P=0.044]and plasma D-dimer levels on POD 5≥21μg/mL(OR=10.1,95%CI:2.04-50.1,P=0.0046)were independent risk factors for VTE after hepatectomy.CONCLUSION Monitoring of plasma D-dimer levels after hepatectomy is useful for early diagnosis of VTE and may avoid routine prophylactic anticoagulation in the postoperative period.

Clinical value of oral contrast-enhanced ultrasonography in diagnosis of gastric tumors

BACKGROUND The incidence of gastric cancer remains high,and it is the sixth most common cancer and the fourth leading cause of cancer deaths worldwide.Oral contrastenhanced ultrasonography is a simple,non-invasive,and painless method for the diagnosis of gastric tumors.AIM To explore the diagnostic value of oral contrast-enhanced ultrasonography for the detection of gastric tumors.METHODS The screening results based on oral contrast-enhanced ultrasonography and electronic gastroscopy were compared with those of the postoperative pathological examination.RESULTS Among 42 patients with gastric tumors enrolled in the study,the diagnostic accordance rate was 95.2%for oral contrast-enhanced ultrasonography(n=40)and 90.5%for electronic gastroscopy(n=38)compared with postoperative pathological examination.The Kappa value of consistency test with pathological findings was 0.812 for oral contrast-enhanced ultrasonography and 0.718 for electronic gastroscopy,and there was no significant difference between them(P=0.397).For the TNM staging of gastric tumors,the accuracy rate of oral contrast enhanced ultrasonography was 81.9%for the overall T staging and 50%,77.8%,100%,and 100%for T1,T2,T3,and T4 staging,respectively.The sensitivity and specificity were both 100%for stages T3 and T4.The diagnostic accuracy rate of oral contrast-enhanced ultrasonography was 93.8%,80%,100%,and 100%for stages N0,N1-N3,M0,and M1,respectively.CONCLUSION The accordance rate of qualitative diagnosis by oral contrast-enhanced ultrasonography is comparable to that of gastroscopy,and it could be used as the preferred method for the early screening of gastric tumors.

Massive simultaneous hepatic and renal perivascular epithelioid cell tumor benefitted from surgery and everolimus treatment:A case report

BACKGROUND Perivascular epithelioid cell tumor(PEComa)is a rare mesenchymal neoplasm that predominantly affects the kidney and uterus.The occurrence of this tumor in the liver,particularly with simultaneous involvement of the liver and kidney,is exceedingly uncommon.Pathological diagnosis is the gold standard.PEComas usually show positive immunohistochemical staining for melanocytic(HMB-45,Melan-A)and myoid(SMA,muscle-specific actin)markers.CASE SUMMARY We presented a noteworthy case of malignant PEComa affecting both the liver and kidney in a 53-year-old man with tuberous sclerosis complex(TSC).FAT2 and TP73 mutations in the kidney were identified and positive expression of diagnostic markers including HMB-45,Melan A,and TFE3 were detected.In addition,we demonstrated that hepatic artery perfusion chemotherapy was ineffective for hepatic PEComa,while surgery remained the most effective approach.Everolimus showed an excellent efficacy in the postoperative treatment of the tumor.CONCLUSION Surgical treatment is preferred for malignant PEComa affecting liver and kidney,especially with TSC;everolimus is effective postoperatively.

Cost analysis of radical resection of malignant breast tumors under the China Healthcare Security Diagnosis Related Groups payment system

BACKGROUND Breast cancer is one of the most common malignant tumors in women worldwide and poses a severe threat to their health.Therefore,this study examined patients who underwent breast cancer surgery,analyzed hospitalization costs and structure,and explored the impact of China Healthcare Security Diagnosis Related Groups(CHS-DRG)management on patient costs.It aimed to provide medical institutions with ways to reduce costs,optimize cost structures,reduce patient burden,and improve service efficiency.AIM To study the CHS-DRG payment system’s impact on breast cancer surgery costs.METHODS Using the CHS-DRG(version 1.1)grouping criteria,4073 patients,who underwent the radical resection of breast malignant tumors from January to December 2023,were included in the JA29 group;1028 patients were part of the CHS-DRG payment system,unlike the rest.Through an independent sample t-test,the length of hospital stay as well as total hospitalization,medicine and consumables,medical,nursing,medical technology,and management expenses were compared.Pearson’s correlation coefficient was used to test the cost correlation.RESULTS In terms of hospitalization expenses,patients in the CHS-DRG payment group had lower medical,nursing,and management expenses than those in the diagnosis-related group(DRG)non-payment group.For patients in the DRG payment group,the factors affecting the total hospitalization cost,in descending order of relevance,were medicine and consumable costs,consumable costs,medicine costs,medical costs,medical technology costs,management costs,nursing costs,and length of hospital stay.For patients in the DRG nonpayment group,the factors affecting the total hospitalization expenses in descending order of relevance were medicines and consumable expenses,consumable expenses,medical technology expenses,the cost of medicines,medical expenses,nursing expenses,length of hospital stay,and management expenses.CONCLUSION The CHS-DRG system can help control and reduce unnecessary medical expenses by controlling medicine costs,medical consumable costs,and the length of hospital stay while ensuring medical safety.

Combination of serum tumor markers dickkopf-1,DCP and AFP for the diagnosis of primary hepatocellular carcinoma

Objective:To evaluate the detection accuracy of the biomarkers dickkopf-1,DCP and AFP as a serum biomarker panel by comparing the sensitivity of the panel with those of the individual biomarkers.Methods:The study was composed of three groups,one with HCC patients,one with non-HCC liver diseases and one with healthy controls.Serum AFP was measured using a chemiluminescence assay and serum dickkopf-1 and DCP were measured with ELISA.The sensitivity and specificity of the biomarkers were analyzed as single parameters and as a serum panel.Results:The HCC group showed higher levels of dickkopf-1,DCP and AFP than the other two groups(P<0.05).Dickkopf-1 showed better sensitivity(73.26%vx.58.13%.P<0.05) and better specificity(44.00%vs.29.00%,P>0.05) than AFP.DCP also had better sensitivity(74.42%vs.58.13%.P<0.05) than AFP,but their specificity was similar(30.00%vs.29.00%.P>0.05).The combination of the biomarkers as a scrum panel produced much better sensitivity(93.02%) and specificity(78.00%) than each of the markers individually(P<0.05).Conclusion:The combination of AFP.DCP and dickkopf-1 as a biomarker panel can significantly improve the detection power with much higher sensitivity and specificity for HCC than any of the biomarkers alone.The tests are convenient and inexpensive,and may serve as a valuable addition to current options for the diagnosis of HCC.

Diagnosis of bile duct hepatocellular carcinoma thrombus without obvious intrahepatic mass

AIM:To study the diagnosis of hepatocellular carcinoma(HCC)presenting as bile duct tumor thrombus with no detectable intrahepatic mass.METHODS:Six patients with pathologically proven bile duct HCC thrombi but no intrahepatic mass demonstrated on the preoperative imaging or palpated intrahepatic mass during operative exploration,were collected.Their clinical and imaging data were retrospectively analyzed.The major findings or signs on comprehensive imaging were correlated with the surgical and pathologic findings.RESULTS:Jaundice was the major clinical symptom of the patients.The elevated serum total bilirubin,direct bilirubin and alanine aminotransferase levels were in concordance with obstructive jaundice and the underlying liver disease.Of the 6 patients showing evidence of viral hepatitis,5 were positive for serum alpha fetoprotein and carbohydrate antigen 19-9,and 1 was positive for serum carcinoembryonic antigen.No patient was correctly diagnosed by ultrasound.The main features of patients on comprehensive imaging were filling defects with cup-shaped ends of the bile duct,with large filling defects presenting as casting moulds in the expanded bile duct,hypervascular intraluminal nodules,debris or blood clots in the bile duct.No obvious circular thickening of the bile duct walls was observed.CONCLUSION:Even with no detectable intrahepatic tumor,bile duct HCC thrombus should be considered in patients predisposed to HCC,and some imaging signs are indicative of its diagnosis.

Diagnosis and surgical treatment of primary hepatic lymphoma

AIM: To assess the benefi ts and limits of surgery for primary hepatic lymphoma (PHL), and probability of survival after postoperative chemotherapy. METHODS: A retrospective analysis was undertaken to determine the results of surgical treatment of PHL over the past 8 years. Only nine patients underwent such treatment. The detailed data of diagnosis, treatment, and prognosis were carefully studied. RESULTS: All patients were mistaken as having α-fetoprotein-negative hepatic cancer before pathological diagnosis. The mean delay time between initial symptoms and final diagnosis was 26.8 d (range:14-47 d). Hepatitis B virus infection was noted in 33.3% of these patients. Most of the lesions were found to be restricted to a solitary hepatic mass. The surgical procedure performed was left hepatectomy in five cases, including left lateral segmentectomy in three. Right hepatectomy was performed in three cases and combined procedures in one. One patient died on the eighth day after surgery,secondary to hepatic insufficiency. The cumulative 6-mo, 1-year, and 2-year survival rates after hepatic surgery were, respectively, 85.7%,71.4%, and 47.6%. One patient survived for>5 years after surgery without any signs of recurrence until latest follow-up, who received routine postoperative chemotherapy every month for 2 years and then regular follow-up. By univariate analysis, postoperative chemotherapy was a significant prognostic factor that influenced survival (P=0.006). CONCLUSION: PHL is a rare entity that is often misdiagnosed, and has a potential association with chronic hepatitis B infection. The prognosis is variable, with good response to early surgery combined with postoperative chemotherapy in strictly selected patients.

Hepatic epithelioid hemangioendothelioma: Dilemma and challenges in the preoperative diagnosis

Hepatic epithelioid hemangioendothelioma(HEHE) is a rare category of vascular tumor with uncertain malignant potential. It commonly presents nonspecific and variable clinical manifestations, ranging from asymptomatic to hepatic failure. In addition, laboratory measurements and imaging features also lack specificity in the diagnosis of HEHE. The aim of the present study is to highlight the dilemma and challenges in the preoperative diagnosis of HEHE, and to enhance awareness of the range of hepatobiliary surgery available in patients with multiple hepatic nodular lesions on imaging. In these patients, HEHE should at least be considered in the differential diagnosis.

Role of diffusion-weighted magnetic resonance imaging in the differential diagnosis of focal hepatic lesions

AIM： To evaluate the utility of diffusion-weighted imaging （DWl） in screening and differential diagnosis of benign and malignant focal hepatic lesions. METHODS： Magnetic resonance imaging （MRI） examinations were performed using the Signa Excite Xl Twin Speed 1.5T system （GE Healthcare, Milwaukee, Wl, USA）. Seventy patients who had undergone MRI of the liver [29 hepatocellular carcinomas （HCC）, four cholangiocarcinomas, 34 metastatic liver cancers, 10 hemangiomas, and eight cysts] between April 2004 and August 2008 were retrospectively evaluated. Visualization of lesions, relative contrast ratio （RCR）, and apparent diffusion coefficient （ADC） were compared between benign and malignant lesions on DWl. Su- perparamagnetic iron oxide （SPIO） was administered to 59 patients, and RCR was compared pre- and postadministration.RESULTS： DWI showed higher contrast between malignant lesions （especially in multiple small metastatic cancers） and surrounding liver parenchyma than did contrast-enhanced computed tomography. ADCs （mean±SD × 10^-3 mm2/s） were significantly lower （P 〈 0.05） in malignant lesions （HCC： 1.31 ± 0.28 and liver metastasis： 1.11 ± 0.22） and were significantly higher in benign lesions （hemangioma： 1.84 ± 0.37 and cyst： 2.61 ± 0.45） than in the surrounding hepatic tissues. RCR between malignant lesions and surrounding he- patic tissues significantly improved after SPIO administration, but RCRs in benign lesions were not improved.CONCLUSION： DWI is a simple and sensitive method for screening focal hepatic lesions and is useful for differential diagnosis.

History of the infantile hepatic hemangioma:From imaging to generating a differential diagnosis

We aim to provide an up-to-date summary of infantile hepatic hemangioma(IHH) and its misnomers and to dialectically present the differential diagnosis of these rare entities of the liver.Eligible peer-reviewed articles on hepatic infantile hemangiomas,published between 2000 and 2015,were reviewed for this study.IHH is the most common hepatic vascular tumor in children.Once a liver mass is identified in an infant,the differential diagnosis ranges from vascular malformations to benign and malignant tumors including mesenchymal hamartoma,hepatoblastoma,metastatic neuroblastoma,so careful physical examination,imaging studies,and,if indicated,tumor markers and biopsy,are of pivotal importance to ascertain the correct diagnosis.Despite the benign nature of IHHs,some of these lesions may demand medical and/or surgical intervention,especially for multiple and diffuse IHH.Complications can include hepatomegaly,hypothyroidism and cardiac failure.Therefore,a close follow-up is required until complete involution of the lesions.We propose an algorithm to guide the physicians towards the proper management of hepatic lesions.

Hepatic epithelioid hemangioendothelioma:Update on diagnosis and therapy

With an estimated incidence of only 1-2 cases in every 1 million people,hepatic epithelioid hemangioendothelioma(HEHE)is a rare vascular endothelial cell tumor occurring in the liver and consisting of epithelioid and histiocyte-like vascular endothelial cells in mucus or a fibrotic matrix.HEHE is characterized as a low-to-moderate grade malignant tumor and is classified into three types:solitary,multiple,and diffuse.Both the etiology and characteristic clinical manifestations of HEHE are unclear.However,HEHE has a characteristic appearance on imaging including ultrasound,magnetic resonance imaging,and positron emission tomography/computerized tomography.Still,its diagnosis depends mainly on pathological findings,with immunohistochemical detection of endothelial markers cluster of differentiation 31(CD31),CD34,CD10,vimentin,and factor VIII antigen as the basis of diagnosis.Hepatectomy and/or liver transplantation are the first choice for treatment,but various chemotherapeutic drugs are reportedly effective,providing a promising treatment option.In this review,we summarize the literature related to the diagnosis and treatment of HEHE,which provides future perspectives for the clinical management of HEHE.

Tools for the diagnosis of hepatitis C virus infection and hepatic fibrosis staging

Hepatitis C virus(HCV)infection represents a major public health issue.Hepatitis C can be cured bytherapy,but many infected individuals are unaware of their status.Effective HCV screening,fast diagnosis and characterization,and hepatic fibrosis staging are highly relevant for controlling transmission,treating infected patients and,consequently,avoiding end-stage liver disease.Exposure to HCV can be determined with high sensitivity and specificity with currently available third generation serology assays.Additionally,the use of point-of-care tests can increase HCV screening opportunities.However,active HCV infection must be confirmed by direct diagnosis methods.Additionally,HCV genotyping is required prior to starting any treatment.Increasingly,high-volume clinical laboratories use different types of automated platforms,which have simplified sample processing,reduced hands-on-time,minimized contamination risks and human error and ensured full traceability of results.Significant advances have also been made in the field of fibrosis stage assessment with the development of non-invasive methods,such as imaging techniques and serum-based tests.However,no single test is currently available that is able to completely replace liver biopsy.This review focuses on approved commercial tools used to diagnose HCV infection and the recommended hepatic fibrosis staging tests.

Diagnosis and surgical treatment of hepatic hilar cholangiocarcinoma

BACKGROUND: Hepatic hilar cholangiocarcinoma can be diagnosed early with the progress in diagnostic imaging, and thus the rate of resection of the tumor has increased markedly. To assess the effectiveness of resection, we reviewed 185 cases of hepatic hilar cholangiocarcinoma diagnosed and treated at our hospital. METHODS: The clinical data of 185 patients with hepatic hilar cholangiocarcinoma who had been treated surgically from 1972 to 2006 were retrospectively analyzed. RESULTS: The records of the 185 patients were divided into first stage (1972-1986) or second stage (1987-2006) according to the incidence of the tumor and its resection rate. Primary symptoms included upper abdominal discomfort or pain, anorexia, tiredness, weight loss and progressive jaundice. Ultrasonography, computed tomography (CT), magnetic resonance imaging (MRI), and magnetic resonance cholangiopancreatography (MRCP) were first line methods for atraumatic diagnosis. If the patients displayed intrahepatic bile duct dilatation or were diagnosed as suffering from extrahepatic obstructive jaundice, percutaneous transhepatic cholangiography (PTC), MRCP or endoscopic retrograde cholangiopancreatography (ERCP) should be used. In this series, 87 patients underwent resection of the tumor (47.0%). Of the 87 patients, 43 received radical resection and 44 palliative resection. Fifteen patients underwent resection in the first stage and 72 in the second stage. A total of 74 patients were followed up after the resection. The median survival time of the radical resection group was 37 months and that of the palliative resection group was 17 months (P<0.001). The other 62 patients receiving no resection died within 1.5 years. CONCLUSIONS: Once patients are diagnosed with hepatic hilar cholangiocarcinoma, they should undergo exploratory laparotomy. Resection is the most effective method for the treatment of hepatic hilar cholangiocarcinoma.

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

BACKGROUND Hepatic 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 largescale retrospective studies are required to understand better this disease.AIM To determine the characteristics of HEHE and identify its optimal treatments and prognostic factors.METHODS The 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.RESULTS HEHE 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).CONCLUSION The 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.