| BACKGROUND&AIMHepatic malignant fibrous histiocytoma(HMFH)is a rare malignant tumor of liver originating from mesenchyme.It was first reported by Alberti-Flor in 1985 that there was no reliable clinical study ever since.So far,HMFH has not become clinically specific and does not show any specific tumor markers.This article primarily analyzes the clinical features of hepatic malignant fibrous tissue Case histories of oncocytoma(HMFH)raise awareness of the disease.METHODSKeyword searches were performed in databases such as PubMed,web of science,and Springer until the end of January,1988,January 1985.Key words:"malignant fibrous histiocytoma","histiocytoma,malignant fibrous histiocytoma","sarcoma","liver neoplasmas" "liver OR hepatic OR hepato" as the keyword search,a total of 174 articles were found.Except for the topics of non-MFH 25 articles,11 experimental papers,8 literature reviews,8 non-English literature,and MFH primary extrahepatic tissues and organs of other 81 articles,reports of clinical cases of HMFH literature only 41 Articles,covering a total of 72 patients,of which 50 cases of relatively complete data from 34 medical units,analysis of clinical data.Retrieved from 1985 in our hospital biopsy or surgical pathological diagnosis and treatment of HMFH clinical and follow-up data,the final enrolled 5 patients,5 patients were performed preoperative abdominal enhanced CT examination,of which 1 case to consider Diagnosis of hepatic sarcoma,but no HMFH diagnosis.Surgical treatment of radical excision in 3 cases,palliative resection in 1 case,symptomatic surgery in 1 case.No simple HMFH,all with comorbidity,HMFH with hepatitis B in 4 cases,HMFH with cirrhosis in 3 cases,HMFH with hepatobiliary stones in 1 case.Postoperative tissue specimens routine histopathological examination and immunohistochemistry.Regular follow-up,2 years after every 3 months 1,2 years after every 6 months.Retrospective analysis of 5 patients with gender,age,current medical history,past history,laboratory tests,imaging studies,surgical procedures,intraoperative findings,surgical specimens of the microscopic examination,immunohistochemistry,postoperative follow-up and treatment results.And the case of the literature and our hospital compared with each other cases.RESULTSThe total number of cases included in this study was 50,including 29 males and 21 females,and the male patients accounted for 58.0%of the total cases.The average age of patients was 56.6 years and ranged from 34 to 87 years.Patients under 40 years of age accounted for 12%of the total number of cases,44 to 70 years of age patients accounted for 50%of the total number of cases.Five patients in our hospital were 46 years old,54 years old,31 years old,42 years old and 37 years old.The average age was 42 years old.The standard deviation was 8.75.The minimum age was 31 years old and the maximum age was 54 years old.Four cases of male patients and one female patient were enrolled in our hospital.Male patients accounted for 80%of the total enrollment.The general condition of the patientAmong the 36 patients with symptoms described,58.3%of patients with abdominal pain,36.1%of patients with discomfort,13.9%of patients with fever,33.3%of patients with anorexia,nausea and loss of appetite,and 44.4%of patients with weight loss;with urine yellow,skin,sclera Only one case of yellow dye patients.Among the 50 patients,7 were hepatitis B virus,1 was hepatitis C virus,and no other hepatitis was reported.One of the hepatitis patients reported cirrhosis.Described in 2 cases of positive ascites signs,including 1 case of bloody ascites;10 cases described positive signs of physical examination,of which 7 cases can reach the abdominal mass,and upper abdominal tenderness.There were 3 cases of previous history of tumor and tumor surgery,1 case of surgical resection for malignant fibrous histiocytoma of spleen 2 years ago.One patient reported a history of smoking.The symptoms of 5 patients in our hospital were as follows:3 cases(60%)of abdominal pain,2 cases of discomfort(40%),2 cases of fever(40%),1 case of weight loss(80%)had a history of hepatitis B or serological diagnosis of hepatitis B,4(80%)had a history of smoking,and 2(20%)had a history of alcohol abuse,Abdominal tenderness signs were positive in 3 cases.Primary liver tumor siteIn this study,47 cases described the location of the tumor,including 14 cases of tumor located in the left lobe of the liver,accounting for 29.8%of the total number of cases;29 cases located in the right lobe of the liver,accounting for 61.7%of the total cases;,Accounting for 8.5%of the total number of cases.Only two cases of multiple intrahepatic tumors and one case of multiple tumor nodules were reported.Can be seen from the above,mostly single tumor tumors,most of the tumor in the right lobe of the liver.Among the 5 patients enrolled in this study,4 were single tumor and 1 was multiple tumor.Among them,3 were primary in the right lobe and 2 were in the left lobe of the liver.The upper right shows that the liver malignant fibrous histiocytoma mostly single tumor,a small number of multiple tumors,mostly in the right lobe of the liver.Tumor related markers29 cases described serological indicators,including 2 cases of AFP-positive,CEA and CA199 tumor markers common in two liver tumors and no positive report of a positive result.Only 1 of the 5 patients enrolled in our hospital was positive for AFP,and the other A serological AFP indicators were negative,and all patients serological CEA,CA199 two indicators were negative results.From the above we can see that in the malignant fibrous histiocytoma AFP may be a positive result,but no diagnostic significance,so far,CEA,CA199 has not found the significance of the tumor.Serum liver function levelStatistics in this group of patients,in almost all cases,except for serum liver function as the representative of laboratory tests,such as renal function and coagulation were reported no abnormalities,only 1 case of hepatitis B-related antibodies;white blood cells in the non-normal range of 5 cases;serology Liver function abnormalities in 5 cases.In our hospital,5 cases of patients with liver function:According to serological liver dysfunction in 4 patients,including only ALT,AST,BIL index was abnormal in 1 case,only showed hypoproteinemia in 2 cases,ALT,AST,BIL,ALB indicators were significantly abnormal in 1 case.Preoperative based on Child-pugh liver function grading,liver function in 5 patients were A grade.Histological classificationOne case of inflammatory malignant fibrous histiocytoma and one case of mucinous malignant fibrous histiocytoma were reported in the literature,and the rest were myxoid-pleomorphic cases.At the same time,the ultrastructure of tumor cells under the electron microscope was diagnosed by one of the tumor cells in the tumor area.The others were diagnosed by electron microscopy and immunohistochemistry.According to the results of immunohistochemistry,the positive rate of Vim was 100%(13/13),the positive rate of CD68 was 81.2%(13/16),and the positive rate of?-AAT in tumor pathology,including surgical pathology,biopsy and autopsy pathology.Rate of 100%(8/8),?-1-ACT positive rate of 100%(11/11).My courtyard 5 patients,according to the diagnosis of tumor pathology,in histology belong to the striated pleomorphic in 2 cases,cytomegalovirus in 2 cases,inflammation in 1 case;microscopy mostly round or round-like tumor,Only 1 case was irregular,the section showed gray,gray,brown,hard texture,may have envelope,internal hemorrhage and necrosis visible;but the final diagnosis of liver MFH relies mainly on histology and immunohistochemistry.Histological light microscope shows more obvious cell growth,arrangement,atypia and other characteristics Immunohistochemistry showed that Vim was positive in 5 cases,positive in CD68,positive in 2 cases by Mac387,positive in a-AAT and negative in ?-1-ACT.From the above we can see,liver malignant fibrous histiocytoma,mostly striated-pleomorphic cases,while the giant cell type,inflammatory and mucinous are rare,hemangioma is rarely reported.Surgical situationStatistics in this group of patients in detail the treatment of a total of 41 cases,of which 2 patients admitted to hospital after the sharp deterioration in the condition,no chance of surgery,1 case of liver rupture and rupture of the second day after admission lost the chance of surgery,another case Almost all liver tumors,lung pneumonia died 6 days after admission;diagnosis of loss of operation after surgery in 1 case,liver resection surgery in 32 cases,the surgical approach is:left hepatectomy,expansion of the left hepatectomy,right lobe of the liver Resection,expansion of the right lobe resection,local tumor resection of the liver,etc.,accompanied by vascular thrombosis,violations of the diaphragm,the invasion of the omentum and violations of the pleura,lung lobes and other tumors,then the inferior vena cava thrombectomy,partial resection of the diaphragm Surgery,omentum and right lower lobe resection and so on.Two patients died of liver failure within 3 months after operation,and one patient died of bilirubin and renal failure after 14 days.Adjuvant treatmentEffective statistics were included in the treatment of 41 cases in this group of patients,in which patients give up treatment in 1 case,line radiotherapy in 1 case,line chemotherapy in 2 cases,line TACE chemotherapy in 2 cases.Among the 5 patients enrolled in our hospital,1 patient underwent TACE treatment.Tumor general situationIn the description of the general situation of the tumor in 28 cases,mainly CT,ultrasound,MRI report and intraoperative findings,the tumor diameter diameter of the three reports of the longest diameter,the smallest diameter of its tumor was 1.50cm,the largest 27.00 cm,an average of 11.34 cm,of which the diameter of 5cm to 15cm cases temporarily describe the number of 75%.In our hospital,5 patients were enrolled.The size of the tumor was evaluated by imaging analysis and surgical pathology.The smallest diameter of the tumor in 5 patients was 6cm,the largest diameter was 15.3cm,the average diameter was 10.66cm,and the tumor size of 5cm or more was 100%.From the above we can see that the primary liver malignant fibrous histiocytoma,tumor diameter larger,most of the tumor diameter greater than 5cm.Can be located in the left lobe of the liver,right lobe of the liver,but also invading the bile duct around the two leaves,but also multiple.Tumor growthIncluded in this study,can be a total of 35 cases of tumor statistics,including 1 case of tumor in the inferior vena cava thrombosis,tumor infiltration to the liver plate,gallbladder bed in 1 case;Among the 5 patients enrolled in our hospital,1 patient had bile duct invasion and 1 patient had multiple intrahepatic tumors.Far away from the situationAmong the patients enrolled in this study,1 patient had lung metastasis,1 patient had pleural metastasis,1 patient had thrombus in the atrium,and 1 patient had invaded heart and lung.5 patients admitted to our hospital,no distant metastasis was found.Preoperative diagnosisThere were no patients diagnosed with malignant fibrous histiocytoma before the pathological findings.Among them,12 cases were diagnosed as preoperative diagnosis,including 2 cases of liver tumor,2 cases of stromal tumor,1 case of intrahepatic cystadenocarcinoma and 1 case of liver abscess,1 case of hepatocellular carcinoma,1 case of hepatic granuloma,1 case of invasive hepatoma,1 case of primary hepatocellular carcinoma and 1 case of undifferentiated mesenchymal malignancy(biopsy).Our hospital into the group of 5 patients,no preoperative tumor biopsy,are frozen in the surgical pathology or surgical pathology,it is based on clinical manifestations,past history,serological test,impact study,consider the diagnosis of primary 3 cases of liver cancer,consider the diagnosis of hepatic sarcoma in 1 case,consider the diagnosis of cholangiocellular carcinoma,intrahepatic bile duct stones complicated by obstructive jaundice in 1 case.In summary,no matter in the literature,or our hospital into the group of patients,none of the cases can be diagnosed as malignant fibrous histiocytoma preoperatively,for this type of preoperative diagnosis of the urgent need to improve.PrognosisAccording to the Kaplan-Meier survival function,the mean survival time was 20.54 months and the median survival time was 15.00 months.According to the literature report Two of these patients survived 60 and 63 months after radical resection and were all surviving in the report.Other treatment mean survival was 3.9 months,the median survival was 4.00 months.However,in the radical resection of the patients,1 case of local recurrence within 1 month and invasion of liver and stomach space,loss of surgery almost died after 3 months;1 case died of tumor invasion of the peritoneum and 12 The intestine,eventually died of duodenal hemorrhage and necrosis;another one case of progressive hyperbilirubinemia and renal failure within 2 weeks after surgery,and finally died after 2 weeks;2 patients after 1 year After recurrence and death,1 patient recurred and died 4 years after the operation.Among the patients who did not undergo radical resection,one patient underwent laparotomy and found that the tumor had invaded the main hepatic veins and diaphragm,no resection of the tumor was performed,the postoperative condition deteriorated and died of liver failure after two months.One patient suffered from liver failure Died within 1 month after the autopsy showed that died of pulmonary embolism;1 patient admitted to hospital the day after admission,rupture of the liver under the capsule tumor hemorrhage,leading to death,autopsy.Five patients in our hospital 5-year survival rate higher than those reported in other credible cases.Case 3 is currently the longest surviving patients,the current recurrence-free metastasis-free survival of 102 months,did not die;Case 4 is also a radical surgery patients,no recurrence without metastasis in 84 months,and did not die;Case 2 surgery Palliative treatment,died in February after surgery,died of liver failure;case 5 multiple tumors,postoperative arterial embolization treatment 1,died of liver rupture bleeding,survival 104 days,the prognosis is still poor.From the above we can see,malignant fibrous histiocytoma,radical resection of the liver tumor increased the survival time of patients,some patients can be more than 5 years of survival,but within 2 months after surgery,the possibility of serious complications may be higher,may be Lead to the deterioration of the disease,accelerate tumor recurrence or metastasis.CONCLUSIONSThe onset of HMFH is obscure and the cause of the disease is unknown.The main clinical manifestations are abdominal pain,discomfort,nausea,loss of appetite and weight loss,which are not specific to other liver tumors and difficult to be diagnosed preoperatively.AFP,CEA,CA 19-9 Tumor markers such as detection of no help in the diagnosis,although the imaging examination can show tumor mass,can show the size of the tumor,the number and internal structure of the tumor and the surrounding situation,but these manifestations are not clearly characteristic,not with other Liver tumor phase identification.The diagnosis of HMFH relies on histopathology and immunohistochemical examination.Histologically,the classification of HMFH has been controversial in recent years.However,the classification of HMFH is as follows:myxoid-pleomorphic,myxoid,giant cell type,Inflammatory,hemangioma-like.Immunohistochemically,HMFH is characterized by positive immunoglobulin(Vim),positive for pan-macrophage marker(CD68),positive for macrophage marker(Mac3 87),alpha-antitrypsin Positive and anti-chymase(?-1-ACT)positive.Surgical resection of the tumor is the preferred method of treatment of HMFH,the prognosis of patients with the thoroughness of tumor resection,radical resection may be obtained after 5 years of long-term survival results. |
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